DDrare: Database of Drug Development for Rare Diseases

299. 嚢胞性線維症　［臨床試験数：856，薬物数：981（DrugBank：220），標的遺伝子数：84，標的パスウェイ数：158］

Searched query = "Cystic fibrosis"
The queries were searched in Public_title, Scientific_title, and Condition of the data. Export date: 11/21/2019, 11/20/2019. Trials are sorted by Date_enrolment from most recent to oldest in the table.

Drug = 0.12% saline; 0.9% isotonic saline (is); 0.9% isotonic saline (is), 4ml; 0.9% w/v sodium chloride solution; 033841; 1,5 (butylimino)-1,5-dideoxy-d-glucitol; 1-cyclopropyl-6-fluoro-1,4-dihydro-4-oxo-7-[1-piperazinyl]-3-quinoline carboxylic acid; 100 mg vx-445/50 mg tez /75 mg iva fdc; 100 mg/m2 dose; 2% chlorhexidine solution wipes; 200 mg/m2 dose; 25-hydroxyvitamin d3; 291844; 4% chlorhexidine gluconate liquid skin cleanser; 4-phenylbutyrate; 4.2% nacl/inhalation solution; 5-methyltetrahydrofolate and vitamin b12; 5000 iu of cholecalciferol; 552-02; 6% hypertonic saline (hs), 4ml; 7% hypertonic saline (hs); 7% nacl; 7% saline; Aac hexal; Abbv-2222; Abbv-3067; Abelcet; Acc600tabs; Acebilustat; Acetate; Acetylcysteine; Acetylcysteine sodium; Active treatment group 7% hypertonic saline; Actrapid 100 iu/ml, solution for injection in a vial; Actrapid penfill 100ui/ml; Acute (1 hour) sildenafil; Acute antioxidant; Adempas; Adempas 0.125 mg; Adempas 0.5 mg; Adempas 1.0 mg; Adempas 2.0 mg; Adeno-associated virus-cftr vector; Advagraf prolonged-release hard capsules; Aer002; Aerosolized, human, plasma-derived alpha-1 antitrypsin; Aerosoltherapy; Aerovanc; Air dnase; Air dnase™; Ajulemic acid; Alanine; Albuterol; Albuterol inhaler.; Alendronate; Alendronate sodium; Alginate; Alginate oligosaccharide; Allo-hmscs; Alpha-1 hc 100 mg; Alpha-1 hc 200 mg; Alpha1-proteinase inhibitor; Alpha1-proteinase inhibitor (human); Altu-135; Alx-009; Amikacin; Amikacin nebulization; Amikacine; Amikacine nebulization; Amikin, amiklin, amicacin, bb-k8, biklin, fabianol, kaminax, mikavir, novamin, pierami; Amikin®, amiklin, amicacin, bb-k8, biklin®, fabianol®, kaminax, mikavir, novamin®, pierami; Amiklin; Amiloride; Amiloride hcl; Amiloride solution for inhalation; Amitriptylin; Amitriptylin-ct; Amitriptylin-ct tabletten; Amitriptyline; Amphotericin b; Amylase; Anakinra; Andecaliximab; Anti-pseudomonas igy gargle; Anti-reflux pharmacotherapy; Antibiotic; Antioxidant cocktail; Aquadek; Aquadeks-2; Ard-3150; Arginine; Arikace; Arikace™; Arikayce™; Aspartate; Ataluren; Ataluren (ptc124); Ataluren (ptc124®); Atorvastatin; Aurexis® (tefibazumab); Avian polyclonal igy antibody against pa; Avibactam; Azd1236; Azd5634; Azd5634 for infusion; Azd5634 for inhalation; Azd9668; Azithromycin; Azithromycin 250 mg tablets; Azithromycin 500 mg film-coated tablet; Azithromycin dihydrate; Azli; Azli 75 mg three times a day (tid); Azli 75 mg three times daily (tid); Azli 75 mg two times a day (bid)/ three times a day (tid); Azli 75 mg two times a day (bid)/three times a day (tid); Aztreonam; Aztreonam for inhalation (ai); Aztreonam for inhalation solution (azli); Aztreonam lysine; Aztreonam lysine for inhalation; Balance; Bay 85-8501; Bay q 3939; Bay63-2521; Bayq3939; Bb-k8; Beta-adrenergic cocktail; Bethkis; Bh4 20mg; Bh4 5mg; Bi 1265162; Bi 443651; Biaxin ( clarithromycin); Biil 283 bs (amelubent); Biil 284 bs, high dose; Biil 284 bs, high dose, adult patients; Biil 284 bs, high dose, pediatric patients; Biil 284 bs, low dose; Biil 284 bs, low dose, adult patients; Biil 284 bs, low dose, pediatric patients; Biil 284 bs, medium dose, adult patients; Biil 284 bs, medium dose, pediatric patients; Bio-25 probiotic; Bisphosphonate treatment; Blood sample; Blood test; Boost vhc; Boots high strength garlic odour controlled; Bramitob; Bramitob 300mg/4ml nebuliser solution; Bramitob*nebul 28f 300mg/4ml; Bramitob*nebul 56f 300mg/4ml; Bramitob® administered by pari eflow® rapid electronic nebulizer; Bramitob® administered by pari lc® plus nebulizer; Broccoli; Broccoli sprouts; Bronchitol; Bucelipase alfa; Budesonide; Budesonide-formoterol single inhaler; Burlulipase; Calcium; Calcium carbonate; Calfactant; Carbonate; Cavosonstat; Cayston; Cayston 75 mg pulver und lösungsmittel zur herstellung einer lösung für einen vernebler; Cefepime; Ceftaroline; Ceftazidim; Ceftazidim fresenius kabi; Ceftazidim fresenius kabi 2000 mg poeder voor oplossing voor injectie of infusie; Ceftazidime; Ceftazidime and tobramycin; Ceftazidime pentahydrate; Ceftazidime/avibactam; Ceftolozane; Ceftolozane/tazobactam; Cf patients with a g551d mutation and treated with ivacaftor; Chf 6333; Chf6333 (part 1 - sad); Chf6333 (part 2 - mad); Chlorhexidine; Chlorhexidine gluconate; Chlorhexidine gluconate oral rinse; Chloride; Cholecalciferol; Cholecalciferol (vitamin d3); Choline; Choline chloride; Choline supplementation; Chronic antioxidant; Cipro (ciprofloxacin, bayq3939); Cipro inhale; Ciprofloxacin; Ciprofloxacin (cipro inhale, bayq3939); Ciprofloxacin (cipro, bayq3939); Ciprofloxacin (ciproxin); Ciprofloxacin dispersion for inhalation; Ciprofloxacin dpi; Ciprofloxacin for inhalation; Ciprofloxacin hydrochloride; Ciprofloxacin hydrochloride monohydrate; Ciproxin; Ciproxin suspension; Ciproxin*6cpr riv 500mg; Clarithromycin; Claritromycin; Claritromycin 500mg film coated tablets; Clavulanate; Cloruro sodico 7%; Cobamamide; Colimycine inhalation; Colimycine injectable; Colistimethat-natrium; Colistimethate; Colistimethate 75 mg inhaled two times daily; Colistimethate sodium; Colistimethate sodium (a component); Colistimethate sodium (ban, usan), colistimethatum natricum, colistin sodium methanesulfonate; Colistimethate sodium (promixin); Colistin; Colistin cf; Colistin forest - trockenstechampullen mit lösungsmittel; Colistin mesilate sodium; Colistineb; Colistineb 2mui; Colobreathe; Colomycin; Compound 1a; Continuous infusion cefepime; Continuous infusion ceftazidime; Continuous infusion meropenem; Continuous infusion piperacillin tazobactam; Continuous infusion ticarcillin-clavulanate; Control group 0.9% isotonic saline; Control multivitamin; Cr002; Cr002 liquid api; Crc3357, zk-355322, leukoton; Creon; Creon 10000; Creon 25000; Creon ir; Creon n 25000; Creon® 25,000; Crestor; Ctp-656, 100 mg (qd); Ctp-656, 150 mg (qd); Ctp-656, 20 mg (qd); Ctx-4430; Curcuma longa; Curcuma longa extract; Curcuma longa extract cpl formaat 00 23,2 x 8,5 mm 723 mg; Curcumin; Cyc202; Cystagon; Cystagon (cysteamine bitartrate) immediate-release capsules; Cysteamine; Cysteamine bitartrate; Cysteamine bitartrate (cystagon); Cystic fibrosis ready to use supplemental food; D-a-tocopheryl peg 1000 succinate; D-glucitol; D6-25-hydroxyvitamin d3; Dci 1020; Decolonization; Denosumab; Denufosol; Denufosol tetrasodium (ins37217); Denufosol tetrasodium (ins37217) inhalation solution; Denufosol tetrasodium inhalation solution; Depelestat; Desoxyribonuclease; Detemir; Detoxifying agents for antineoplastic treatment; Deuterated ivacaftor; Dexamethasone; Dexamethasone sodium phosphate 250 mg/10 ml solution; Diet; Digitoxin; Docosahexaenoic acid; Docosahexaenoic acid (dha); Docosahexaenoic acid administration: 50 mg/kg/day during 12 months; Docosahexanoic acid supplement; Doribax; Doripenem; Doripenem hydrate; Dornase alfa; Dornase alfa (pulmozyme); Dornase alfa [pulmozyme®]; Dornase alpha; Doxycycline; Dp mannitol; Dry powder tobramycin; Dtpa; Duramycin; Duramycin, 2622u90; Ebselen; Ecgc; Egcg; Egcg + tocotrienol; Elexacaftor; Elexacaftor-tezacaftor-ivacaftor exposure; Elx-02; Elx/tez/iva; Emea/h/c/000125; Engineerd protein inhibitor of human neutrophil elastase; Engineered protein inhibitor of human neutrophil elastase; Ensure high protein; Ensure plus; Epi-hne4 dx-890; Ergocalciferol; Ergocalciferol (vitamin d2); Esomeprazole; Essential amino acid intake + leucine vs total aa supplement; Estradiol; Estradiol 2 mg; Ethambutol; Ethinyl estradiol; Eu orphan designation number eu/3/02/120; Eur-1008; Eur-1008 (apt-1008); Ez-2053; Ez-2053 5mg/kg; Fdl169; Fdl176; Fdl176 & fdl169 coadministration; Fenretinide; Ferric carboxymaltose; Ferrous sulfate 325mg; Finely ground flaxseed powder; Fish oil; Flucloxacillin; Flucloxacillin 250mg/5ml sugar-free powder for oral solution; Flucloxacillin sodium; Flucloxicillin; Flumist®; Fluticasone; Formoterol; Fortum; Fti, azli; G1117337; G957389; Gallium nitrate; Garlic; Genistein; Genistein (unconjugated isoflavones 100); Genisteine plus 150 mg cpl formaat 1 18,7 x 6,4 mm 438 mg; Gentamicin; Gernebcin 80 mg; Ghrelin; Giving dha-rich supplement; Glargine; Glargine insulin; Glp-1; Glpg1837; Glpg2222; Glpg2222 150 mg q.d.; Glpg2222 300 mg q.d.; Glpg2222 dose 1 for cohort a; Glpg2222 dose 1 for cohort b; Glpg2222 dose 2 for cohort a; Glpg2222 dose 2 for cohort b; Glpg2451 dose regimen a; Glpg2451 dose regimen b; Glpg2737; Glpg2737 single dose; Glpg3067 single dose; Glucose; Glutamine; Glutathione; Glutathione sodium; Glycine; Glycine buffer; Growth hormone; Gs-5737; Gs-9411; Gsh (glutathione); Helium; Helium-3; Heparin; Heparin 25mg inhalation powder, hard capsule; Heparin sodium; High dose of budesonide; High-dose vitamin d3; Hmscs; Hp 129xe; Hp 3he; Human recombinant growth hormone; Hyaluronan; Hydrocortisone; Hydroxychloroquine; Hyperglycemic clamp; Hyperglycemic clamp with concurrent gip infusion; Hyperglycemic clamp with concurrent glp-1 infusion; Hyperpolarized helium-3; Hyperpolarized helium-3 mri of the chest; Hyperpolarized xenon; Hyperpolarized xenon gas; Hypertonic bicarbonate; Hypertonic saline; Hypertonic saline (7 %) and isotonic saline (0.9%); Hypertonic saline (7%); Hypertonic saline 6% -; Hypertonic saline 7%; Hypertonic saline solution; Ibuprofen; Ics+laba; Idpm; Idpm: inhaled dry powder mannitol; Ige025; Impact; Impact-nutridrink; Increlex*sc 1fl 4ml 10mg/ml; Indium; Indium-dtpa; Indusil*os gtt fl 30mg+fl 15ml; Inhaled 7% hypertonic saline (hs); Inhaled ar-501; Inhaled dry powder mannitol; Inhaled hypertonic saline (7%); Inhaled mannitol; Inhaled reduced glutathione; Inhaled snsp113; Inhaled sodium pyruvate; Inhaled tobramycin; Inhaled vancomycin; Inomax 400ppm mol/mol inhalation gas; Ins1007 10 mg oral tablet; Ins1007 25 mg oral tablet; Insulin; Insulin asparte; Insulin detemir; Insulin detemir [rdna origin] injection; Insulin glargine; Insuline humaine recombinante; Interferon gamma-1b; Intermittent, short infusion cefepime; Intermittent, short infusion ceftazidime; Intermittent, short infusion meropenem; Intermittent, short infusion piperacillin tazobactam; Intermittent, short infusion ticarcillin-clavulanate; Inulin; Ionis-enacrx; Isotonic saline; Itraconazole; Itraconazole/voriconazole; Iv amikacin; Iv ceftazidime; Iv meropenem; Iv piperacillin-tazobactam; Iv ticarcillin-clavulanate; Iv tobramycin; Iv vancomycin hydrochloride; Ivacaftor; Ivacaftor 125mg tablet; Ivacaftor 150 mg or 250 mg; Ivacaftor 25 mg/75 mg; Ivacaftor 75 mg/150 mg; Ivacaftor exposed; Ivacaftor+lumacaftor; Ivacaftor/kalydeco; J01gb01; Jbt-101; Jbt-101 (lenabasum); Kalydeco; Kalydeco 150 mg film-coated tablets; Kalydeco 150mg; Kalydeco, 150 mg tablet (bid; Kb001; Kb001-a; Kineret; Kreon 25 000; Kreon 25000; L-alanine; L-arginine; L-lysine; L-lysine-n-acetylcysteinate; Lactic acid; Lactic acid producing organisms; Lactizole nebulization; Lactobacillus reuteri; Lactobacillus rhamnosus; Lactobacillus rhamnosus gg; Lansoprazole; Lantus 100 units/ml solution for injection in a cartridge; Lantus*sc 5car3ml100ui/ml; Lantus® 100 units/ml solution for injection in a vial. lantus® 100 units/ml solution for injection in a cartridge. lantus® 100 units/ml solution for injection in a pre-filled pen; Lau-7b; Lenabasum; Lenabasum 20 mg; Lenabasum 5 mg; Leucine; Levemir 100 u/ml solution for injection in a cartridge, levemir 100 u/ml solution for injection in a pre-filled pen; Levemir flexpen; Levemir insulin; Levemir penfill; Levofloxacin; Linezolid; Lipase clec; Liposomal amikacin (arikace™); Liposomal amikacin for inhalation; Liposomal amikacin for inhalation (arikayce™) using the pari investigational eflow® nebulizer.; Liposomal amphotericin b (ambisome®); Liprotamase; Liprotamase powder for oral solution; Loestrin (norethindrone acetate and ethinyl estradiol); Losartan; Lu 70274; Lubiprostone; Lucinactant; Lucinactant first; Lum/iva; Lum/iva fixed-dose combination; Lumacaftor; Lumacaftor / ivacaftor; Lumacaftor plus ivacaftor; Lumacaftor plus ivacaftor combination; Lumacaftor-ivacaftor; Lumacaftor-ivacaftor treatment; Lumacaftor/ ivacaftor; Lumacaftor/ivacaftor; Lumacaftor/ivacaftor 100mg/125mg tablets; Lumacaftor/ivacaftor 200mg/125mg tablets; Lumacaftor/ivacaftor 200mg/83mg tablet; Lym-x-sorb powder; Lysine; Lysomucil 10 %; Mag-dha; Mannitol; Mannitol + pulmozyme; Mannitol inhalation powder; Matched isoglycemic clamp; Mecasermin; Medical grade oxygen; Menadiol diphosphate; Menadiol sodium diphosphate; Meropenem; Meropenem fresenius kabi; Meropenem fresenius kabi 1 g poeder voor oplossing voor injectie of infusie; Meropenem infusion; Meropenem trihydrate; Mesenchymal stem cells; Metformine; Metformine hcl 500 pch; Miglustat; Minocycline; Minocycline hydrochloride; Mirnas isolation from blood samples of patients and control; Modified lipid formulation; Modified oral glucose tolerance test (50g 4-hours); Molgramostim; Molgramostim nebulizer solution; Moli1901; Moli1901 (2622u90, duramycin); Moxifloxacin; Moxifloxacin hydrochloride; Mp-376; Mp-376 (levofloxacin solution for inhalation); Mri with pfp; Mrt5005; Ms1819; Ms1819-sd; Msi-1995; Mucoid exopolysaccharide p. aeruginosa immune globulin iv; Mucolysin; Mucomyst; Mupirocin; Mupirocin intranasal creme; N-(5-hydroxy-2,4-bis(trimethylsilyl)phenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide; N-(trans-3-(5-((r)-1-hydroxyethyl)-1,3,4-oxadiazol-2-yl)cyclobutyl)-3-phenylisoxazole-5-carboxamide; N-acetylcystein; N-acetylcysteine; N-acetylcysteine (nac); N6022; N91115; Nacystelyn®; Nasal aztreonam; Neb/01-07; Nebcin; Nebcinal; Nebcinal tobi; Nebcine; Nebulization of amikacin during niv (rr: 15 cycles/minute); Nebulization of amikacin during niv (rr: 25 cycles/minute); Nexium; Nexium 20; Nitrate; Nitric oxide; Nitric oxide 160 ppm; Nitric oxide for inhalation; Nitrite; Nitrogen; None; None assigned; Norethindrone; Norgestimate; Normal saline; Normal saline iv, salt tablets; Normal saline solution; Normal saline:; Norvir; Norvir 100 mg film-coated tablets; Novo rapid; Novo rapid insulin (novonordisk); Novolog insulin; Novonorm 0.5 mg; Novonorm 1 mg; Novonorm 2 mg; Novorapid / humalog short acting insulin; Novorapid 100 u/ml, solution for injection in a vial. novorapid penfill 100 u/ml, solution for injection in a cartridge. novorapid flexpen 100 u/ml, solution for injection in a pre-filled pen.; Novorapid aspartate; Noxafil®; Nutridrink-impact; Nutropin aq; Nutropin aq [somatropin (dna origin) injection]; Ogt 918; Oligog; Oligog (60 mg/ml); Oligog cf-5/20; Oligog dpi; Oligog dry powder for inhalation; Oligog-cf5/20, alginate oligosaccharide (g-block) fragment; Oligomer of sodium alginate; Omalizumab; Omega 3; Omega 3 premium; Omega-3; Omega-3-acid triglycerides; Omeprazole; Omeprazole 40mg capsule; Once-daily insulin detemir; Oral aztreonam; Oral ciprofloxacin; Oral ciprofloxacin plus inhaled colistin; Oral glucose tolerance test (75g 2-hour); Oral glutathione; Oral reduced l-glutathione; Oral roflumilast; Orkambi; Orkambi 100 mg/125 mg film-coated tablets; Orkambi 200 mg/125 mg film-coated tablets; Orkambi®; Ortho-cyclen, ethinyl estradiol/norgestimate, 0.035 mg/0.250 mg; Oscn-; Oseltamivir; Other non-sponsor pancreatic enzyme replacement therapy; Oxygen; P-1037; Pancreas powder; Pancrease mt; Pancrease mt 10.5, or mt 21; Pancreaslipase; Pancreatin; Pancreatin (pancreas powder); Pancreaze; Pancrecarb® (pancrelipase); Pancrelipase; Pancrelipase amylase; Pancrelipase delayed release; Pancrelipase delayed release capsule; Pancrelipase microtablets; Pancrelipase with bicarbonate; Pancrelipase/pancreas powder; Panzytrat; Panzytrat 25000; Panzytrat® ok; Pbi4050; Pc945; Perfluorinated gas/oxygen mixture; Pertzye; Pgm169/gl67a; Phenylbutyrate; Phosphate; Physiologic serum nebulized nasally; Physiological solution; Pilocarpine; Pilocarpine nitrate 5%; Pioglitazone; Piperacillin; Piperacillin sodium; Piperacillin with tazobactam (tazocin); Piperacillin-tazobactam combination product; Piperacilline/tazobactam fresenius kabi 2 g/0,25 g poeder voor oplossing voor infusie; Piperacilline/tazobactam fresenius kabi 4 g/0,5 g poeder voor oplossing voor infusie; Pl 0010/0211; Plerixafor; Plerixafor mobilization of autologous cd117 stem cells; Po azithromycin; Po ciprofloxacin; Pol6014; Pol7080; Porcine (pig) pert; Porcine pert; Posaconazole; Ppi treatment; Prednisolone; Prednisone; Prefolic*30cpr gastror 15mg; Prograft; Prograft capsules; Prolastin (drug); Promixin 1 million international units (iu) powder for nebuliser solution; Promixin*nebul 30monod 1mui; Promixin, 1 million international units (iu) powder for nebuliser solution; Promixin/tadim; Protease; Psaer igy; Ptc-0161480; Ptc-124; Ptc-c124; Ptc124; Pti-428; Pti-801; Pti-808; Pulmaquin®; Pulmozyme; Pulmozyme (dornase alfa); Pulmozyme (dornase alpha); Pulmozyme single use ampule; Pur118; Pyruvate; Qau145; Qbw251; Qr-010; Quercetin; R03ac02; R03cc02; R05c b01; Radiolabelled oligog cf-5/20 6% solution; Radiolabelled oligog cf-5/20 6% solution for nebulisation; Radiolabelled oligog cf-5/20 dpi; Radiolabelled oligog cf-5/20 dry powder for inhalation (dpi); Rapeglinide; Ravicti high dose; Ravicti low dose; Recombiant human desoxyribonuclease ( rhdnase); Recombinant human dnase (pulmozyme); Recombinant human igf-1; Recombinant truncated spint2 protease inhibitor; Reduced glutathione; Repaglinide; Repaglinide and insulin; Reparixin; Resunab, ajulemic acid, anabasum; Rhbssl; Rhdnase; Rifampicin; Rifampin; Riociguat; Roflumilast; Roscovitine; Rosuvastatin; Rosuvastatin calcium; Roxithromycin; Rpl554; Rps 2505; Répaglinide; S-1226; Salbutamol; Saline; Saline 0.9% inhalation solution; Saline solution; Salmeterol; Saxagliptin; Sb-656933 tablets; Sb-656933-aaa; Sb656933; Se10599; Seliciclib; Short-acting insulin (actrapid); Sildenafil; Sildenafil 40mg oral capsule; Simvastatin; Simvastatin treatment for 28 days; Sitagliptin; Slit amikacin; Slit(tm) amikacin; Snack beverage; Sodium (r)-8-methyl-2-(3-methylbenzofuran-2-yl)-5-(1-(tetrahydro-2h-pyran-4-yl)ethoxy)quinoline-4-ca; Sodium 4-phenylbutyrate; Sodium 4-phenylbutyrate (4pba); Sodium bicarbonate; Sodium bicarbonate bp; Sodium chloride; Sodium chloride (7%); Sodium chloride 0,9%; Sodium chloride 6%; Sodium nitrite; Sodium pyruvate in 0.9% sodium chloride solution; Soluble fiber supplementation; Somalgen; Somatropin; Spi-1005 ebselen 200mg capsule x1; Spi-1005 ebselen 200mg capsule x2; Spi-1005 ebselen 200mg capsule x3; Spiriva respimat 2.5 mcg; Spiriva respimat 2.5 microgram, solution for inhalation; Sputum; Spx-101; Ss-adrenergic cocktail; Standard care; Standard formula; Standard formula (enfamil); Standard of care iv antibiotic(s); Standardized turmeric root extract; Study medication, azithromycin; Sub-chronic (4 weeks) sildenafil; Succinate; Sulfamethoxazole; Sulfate; Sunflower oil; Supplementation with vitamin d2/d3; Symbiotic formula with dha and antioxidants; Symdeko; T100; T100 pari; Tacrolimus; Tacrolimus and mmf; Tacrolimus capsules; Tacrolimus prolonged-release hard capsules; Tad 600; Talniflumate; Tauroursodeoxycholic acid; Tazobactam; Tazobactam sodium; Tbm100; Tbm100c; Technetium sulfur colloid; Tedizolid; Tedizolid iv/po; Tedizolid po/iv; Telavancin; Telavancin injection; Tez/iva; Tezacaftor; Tezacaftor (tez); Tezacaftor/ivacaftor; Tezacaftor/ivacaftor + ivacaftor; Tezacaftor/ivacaftor 100mg/150mg; Tham; The gut mycobiota and microbiota profile; The lung mycobiota and microbiota profile; Theophylline; Ticarcillin; Tiotropium; Tiotropium bromide high; Tiotropium bromide low; Tiotropium bromide medium; Tiotropium bromide monohydrate; Tiotropium respimat; Tiotropium respimat 1.25 microgram solution for inhalation; Tip (tobramycin inhalation powder); Tis (tobramycin inhalation solution); Tis or tip; Tnse; Tobi; Tobi (tobramycin); Tobi 300 mg / 5 ml nebuliser solution; Tobi 300 mg/5 ml nebuliser solution; Tobi 300mg/5ml nebuliser solution.; Tobi inhalant product; Tobi nebcinal; Tobi podhaler; Tobi podhaler 28 mg inhalációs por, kemény kapszula; Tobi*nebul 56f 1d 300mg/5ml; Tobi®; Tobi™ podhaler™ 112 mg inhaled twice daily; Tobramycin; Tobramycin (gernebcin®); Tobramycin / bramitob; Tobramycin / tobi; Tobramycin 100 pari; Tobramycin dry powder; Tobramycin for inhalation; Tobramycin inhalation powder; Tobramycin inhalation powder (tip); Tobramycin inhalation solution; Tobramycin inhalation solution (tis); Tobramycin inhalation solution using a pari lc® plus nebulizer.; Tobramycin nebulized nasally; Tobramycin powder; Tobramycin solution for inhalation; Tobramycin solution for inhalation (tobi); Tobramycin solution for inhalation 300 mg; Tobramycin steri-neb 300 mg/5 ml, inhalation solution; Tobramycin sulfate; Tobramycine; Tobramycinum; Tobrineb/bramitob; Tocotrienol; Tr02; Trimethoprim; Trimethoprim sulfamethoxazole (tmp/smx); Trimethoprim-sulfamethoxazole; Trimethoprim/sulfamethoxazole; Trimethoprim/sulfamethoxazole (tmp/smx); Triple combination therapy; Turmeric; Ultrase® mt12; Ultrase® mt20; Ultresa; Unconjugated isoflavones 100 (pti g-4660, 87% genistein); Uridine; Urinary levels of piiinp as a marker of changes in renal function; Ursodiol; Vaccin grippal pandémique h1n1; Vaccination with vaccine against hepatitis a and b; Valganciclovir; Valganciclovir [valcyte]; Vancomycin; Vancomycin inhalation powder; Vantobra (tobramycin); Vardenafil; Vedrop; Vfend; Vitamin b12; Vitamin d; Vitamin d oil; Vitamin d powder; Vitamin d2; Vitamin d3; Vitamin e; Vitamin e - tgps; Vitamin: choline bitartrate (2-hydroxyethyl) trimethylammonium salt 1:1; Voriconazole; Vrt-813077; Vrt-826809; Vrt-893661; Vrt-893661 vrt-0893661; Vx-121; Vx-152; Vx-371; Vx-371 in hypertonic saline; Vx-371 in saline; Vx-440; Vx-445; Vx-445/tez/iva; Vx-561; Vx-561 (ctp-656); Vx-659; Vx-659/tez/iva; Vx-659/tezacaftor/ivacaftor; Vx-661; Vx-661, vrt-893661; Vx-661/ ivacaftor; Vx-661/ivacaftor; Vx-661/ivacaftor (vx-770) 100mg/150 mg; Vx-661/ivacaftor 100 mg/ 150 mg; Vx-661/ivacaftor 100mg/150mg; Vx-661/ivacaftor 50 mg/ 75 mg; Vx-661/vx-770; Vx-770; Vx-770, vrt-8130077; Vx-770, vrt-813077; Vx-809; Vx-809 & vx-770; Vx-809 / vx-770; Vx-809, vrt-826809; Vx-809/vx-770; Water; Water for injections; Water for injections, ep; Xenon; Xenon-129; Xolair; Xolair 150 mg pulver und injektionslösung zur herstellung einer injektionslösung; Xylitol; Zavesca; Zenpep; Zinc; Zinc acetate; Zinc acetate (20mg/d); Zithomax 40 mg/ml enfants; Zithromax; Zithromax 250 mg; Zitromax; Zoledronic acid

No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
1	NCT03931252	February 2020	11 November 2019	Thermic Effect of Feeding in Cystic Fibrosis	Thermic Effect of Feeding in Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Boost VHC;Dietary Supplement: Ensure High Protein	Virginia Commonwealth University		Recruiting	18 Years	N/A	All	10	N/A	United States
2	NCT03698448	December 1, 2019	22 October 2019	A Dose-finding Study of Inhaled OligoG vs Placebo in Patients With Cystic Fibrosis	A Randomized, Double Blind, Dose-finding Study of Inhaled Oligosaccharide (OligoG) vs Placebo in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: OligoG Dry powder for inhalation	AlgiPharma AS	Imperial College London;Cystic Fibrosis Foundation;European Cystic Fibrosis Society;University Hospital of Cologne	Not recruiting	12 Years	N/A	All	120	Phase 2/Phase 3	Germany
3	NCT02547116	December 2019	9 September 2019	Epidemiology and Treatment of Small-colony Variant Staphylococcus Aureus in Cystic Fibrosis	Epidemiology and Treatment of Small-colony Variant Staphylococcus Aureus in Cystic Fibrosis	Cystic Fibrosis;MRSA	Drug: Rifampin	Johns Hopkins University		Not recruiting	12 Years	N/A	All	16	Phase 4	United States
4	NCT03058068	December 2019	15 July 2019	Human MesenchymAl Stem Cells Infusion in Patients With Cystic Fibrosis	A Phase I, Randomized and Placebo-controlled Trial to Evaluate the Safety, Tolerability, and Potential Efficacy of Allogeneic Human MesenchymAl Stem Cells Infusion in Patients With Cystic Fibrosis - HAPI	Cystic Fibrosis	Biological: Allo-hMSCs;Biological: Placebo	Joshua M Hare		Not recruiting	20 Years	45 Years	All	18	Phase 1	United States
5	NCT03921060	December 2019	7 October 2019	Markers of Osteoporosis in Cystic Fibrosis	Markers of Osteoporosis in Cystic Fibrosis	Cystic Fibrosis	Drug: Denosumab	University of Texas Southwestern Medical Center		Not recruiting	12 Years	64 Years	All	100	Phase 4	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
6	NCT04058366	November 2019	30 September 2019	Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy	A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Gating or Residual Function Mutation (F/G and F/RF Genotypes)	Cystic Fibrosis	Drug: ELX/TEZ/IVA;Drug: IVA	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	250	Phase 3
7	NCT04135495	October 16, 2019	4 November 2019	A Phase 2 Study to Evaluate the Safety, Tolerability, PK and PD of ELX-02 in Cystic Fibrosis Patients With G542X Allele	A Phase 2 Open Label Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Multiple Dose Levels of Subcutaneously Administered ELX-02 in Patients With Cystic Fibrosis With at Least One G542X Allele	Cystic Fibrosis	Drug: ELX-02	Eloxx Pharmaceuticals, Inc.		Recruiting	18 Years	N/A	All	8	Phase 2	United States
8	NCT03435939	October 15, 2019	7 October 2019	Effect of Losartan in Cystic Fibrosis (CF)-NIH Grant #133240	Anti-Inflammatory Therapy to Augment CFTR Rescue in CF Patients	Cystic Fibrosis	Drug: Losartan	University of Kansas Medical Center		Not recruiting	18 Years	N/A	All	16	Early Phase 1	United States
9	NCT04105972	October 3, 2019	11 November 2019	A Study Evaluating the Efficacy and Safety of VX-445/Tezacaftor/Ivacaftor in Cystic Fibrosis Subjects, Homozygous for F508del	A Phase 3b, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445/Tezacaftor/Ivacaftor in Cystic Fibrosis Subjects, Homozygous for F508del	Cystic Fibrosis	Drug: ELX/TEZ/IVA;Drug: TEZ/IVA;Drug: IVA	Vertex Pharmaceuticals Incorporated		Recruiting	12 Years	N/A	All	158	Phase 3	Australia;Belgium;Germany;United Kingdom
10	NCT04126473	October 3, 2019	4 November 2019	A Phase 2 Study to Evaluate the Safety, Tolerability, PK and PD in Cystic Fibrosis Patients With at Least 1 G542X Allele	A Phase 2 Open Label Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Multiple Dose Levels of Subcutaneously Administered ELX-02 in Patients With Cystic Fibrosis With at Least One G542X Allele	Genetic Disease;Nonsense Mutation;Cystic Fibrosis With Meconium Ileus	Drug: ELX-02	Eloxx Pharmaceuticals, Inc.		Recruiting	16 Years	N/A	All	16	Phase 2	Israel
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
11	NCT03924947	October 2, 2019	4 November 2019	A Study to Compare US Marketed Pancrelipase Drug Product With Drug Product Manufactured With a Modernized Process at an Alternate Manufacturing Site, in Participants With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis.	A Phase 4 Study to Compare US Marketed Creon Drug Product With Drug Product Manufactured With a Modernized Process at an Alternate Manufacturing Site, in Subjects With Exocrine Pancreatic Insufficiency (EPI) Due to Cystic Fibrosis	Cystic Fibrosis	Drug: Pancrelipase	AbbVie		Recruiting	12 Years	N/A	All	28	Phase 4	United States;Spain
12	NCT03925194	October 1, 2019	6 May 2019	A Study to Evaluate Safety and Efficacy of Subcutaneous Administration of Anakinra in Patients With CF	A Phase IIa, Randomized, Placebo-controlled, Double-blind, Cross-over Study to Evaluate Safety and Efficacy of Subcutaneous Administration of Anakinra in Patients With Cystic Fibrosis	Cystic Fibrosis, 10011762	Drug: Anakinra	Heidelberg University		Not recruiting	12 Years	N/A	All	52	Phase 2	Germany
13	NCT04090294	October 1, 2019	14 October 2019	Assessment of Early Outcomes of Roflumilast in Patients With Non Cystic Fibrosis Bronchiectasis	Assessment of Early Outcomes of Roflumilast in Patients With Non Cystic Fibrosis Bronchiectasis	Bronchiectasis	Drug: Roflumilast	Assiut University		Not recruiting	18 Years	75 Years	All	35	Early Phase 1
14	NCT04118010	October 2019	22 October 2019	Vitamin D and Prebiotics for Intestinal Health in Cystic Fibrosis	Vitamin D and Prebiotics for Intestinal Health in Cystic Fibrosis	Cystic Fibrosis;Dysbiosis	Drug: Vitamin D3;Drug: Placebo vitamin D3;Drug: Inulin;Drug: Placebo Inulin	Emory University		Not recruiting	18 Years	N/A	All	40	Phase 4	United States
15	NCT04059094	September 16, 2019	11 November 2019	A 4-week Study to Test Different Doses of BI 1265162 in Adolescents and Adults With Cystic Fibrosis Using the Respimat® Inhaler	A Randomised, Double-blind, Placebo-controlled and Parallel Group Trial to Evaluate Efficacy and Safety of Twice Daily Inhaled Doses of BI 1265162 Delivered by Respimat® Inhaler as add-on Therapy to Standard of Care Over 4 Weeks in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: BI 1265162;Drug: Placebo	Boehringer Ingelheim		Recruiting	12 Years	N/A	All	98	Phase 2	United States;Belgium;France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
16	NCT04038710	September 5, 2019	4 November 2019	Clinical Outcomes of Triple Combination Therapy in Severe Cystic Fibrosis Disease.	An Observational Study of the Effects on Clinical Outcomes of Expanded Access Program of Vertex Triple Combination Therapy.	Cystic Fibrosis	Drug: Triple combination therapy	National Jewish Health		Recruiting	12 Years	N/A	All	7	Phase 2	United States
17	NCT04039087	September 5, 2019	4 November 2019	Sildenafil Exercise: Role of PDE5 Inhibition	Mechanisms of Exercise Intolerance in Cystic Fibrosis: Role of PDE5 Inhibition	Cystic Fibrosis	Drug: Sildenafil 40mg oral capsule;Drug: Placebo Oral capsule	National Jewish Health	Augusta University;Cystic Fibrosis Foundation	Recruiting	9 Years	N/A	All	40	Phase 2/Phase 3	United States
18	NCT04056702	September 5, 2019	4 November 2019	Impact of Triple Combination CFTR Therapy on Sinus Disease.	Impact of Elexacaftor-tezacaftor-ivacaftor Triple Combination CFTR Therapy on Sinus Disease: Quantitative Sinus Computed Tomography, Patient Reported Outcomes and Cellular and Molecular Changes	Cystic Fibrosis	Drug: Elexacaftor-tezacaftor-ivacaftor exposure	Jennifer Taylor-Cousar	Cystic Fibrosis Foundation	Recruiting	18 Years	89 Years	All	70	Phase 4	United States
19	NCT03506061	September 4, 2019	30 September 2019	Symdeko in Cystic Fibrosis Patients	iPS Cell Response to CFTR Modulators: Study of Symdeko in CF Patients Carrying Partial Function Mutations	Cystic Fibrosis	Drug: Symdeko	Emory University	National Heart, Lung, and Blood Institute (NHLBI)	Recruiting	12 Years	N/A	All	22	Phase 2	United States
20	NCT04006873	September 3, 2019	4 November 2019	Gut Imaging for Function & Transit in Cystic Fibrosis Study 2	A Randomised Crossover Pilot Study of the Effects of Tezacaftor/Ivacaftor and Ivacaftor on Gastrointestinal Function Using Magnetic Resonance Imaging Parameters in People With Cystic Fibrosis	Cystic Fibrosis	Drug: Tezacaftor/Ivacaftor + Ivacaftor;Drug: Placebo oral tablet	Nottingham University Hospitals NHS Trust	Vertex Pharmaceuticals Incorporated;Cystic Fibrosis Foundation;Cystic Fibrosis Trust;University of Nottingham;Nottingham University Hospitals Charity	Recruiting	12 Years	40 Years	All	12	Phase 2	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
21	NCT04058353	August 28, 2019	4 November 2019	A Phase 3 Study of VX-445 Combination Therapy in Cystic Fibrosis (CF) Subjects Heterozygous for F508del and a Gating or Residual Function Mutation (F/G and F/RF Genotypes)	A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Gating or Residual Function Mutation (F/G and F/RF Genotypes)	Cystic Fibrosis	Drug: ELX/TEZ/IVA;Drug: IVA;Drug: TEZ/IVA	Vertex Pharmaceuticals Incorporated		Recruiting	12 Years	N/A	All	250	Phase 3	United States
22	NCT03969888	August 27, 2019	11 June 2019	A Phase 2 Study of ABBV-3067 Alone and in Combination With ABBV-2222	A Phase 2 Study of ABBV-3067 Alone and in Combination With ABBV-2222 in Cystic Fibrosis Subjects Who Are Homozygous for the F508del Mutation	Cystic Fibrosis	Drug: ABBV-3067;Drug: Placebo ABBV-3067;Drug: ABBV-2222;Drug: Placebo ABBV-2222	AbbVie		Not recruiting	18 Years	N/A	All	189	Phase 2	United States
23	NCT04043806	August 9, 2019	11 November 2019	A Study Evaluating the Long-term Safety of VX-445 Combination Therapy	A Phase 3, Open-label Study Evaluating the Long-term Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: ELX/TEZ/IVA;Drug: IVA	Vertex Pharmaceuticals Incorporated		Recruiting	12 Years	N/A	All	480	Phase 3	United States
24	NCT03587961	August 1, 2019	14 October 2019	Personalized Theratyping Trial	Personalized Theratyping Trial	Cystic Fibrosis	Drug: Symdeko	University of Alabama at Birmingham		Recruiting	6 Years	N/A	All	20	Early Phase 1	United States
25	NCT04058340	July 30, 2019	2 September 2019	Taste Receptors Regulation in CF Patients	The Effects of Taste Receptors Regulation in Upper Airway Innate Immunity of CF Patients	Cystic Fibrosis	Dietary Supplement: lactizole nebulization;Other: Placebo	Medical Universtity of Lodz		Recruiting	6 Years	N/A	All	30	N/A	Poland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
26	NCT03988816	July 2019	1 July 2019	Effect of Roflumilast on Quality of Life, Lung Function and Mucus Properties in Patients With Non-cystic Fibrosis Bronchiectasis.	Effect of Roflumilast on Quality of Life, Lung Function and Mucus Properties in Patients With Non-cystic Fibrosis Bronchiectasis: a Cross-over, Unicentric, Double-blind and Placebo-controlled Study	Bronchiectasis Adult	Drug: Roflumilast;Drug: Placebo oral tablet	University of Sao Paulo General Hospital		Recruiting	18 Years	N/A	All	30	Phase 2	Brazil
27	NCT03597347	June 20, 2019	30 September 2019	Trial of Inhaled Molgramostim in CF Subjects With NTM Infection	An Open-label, Non-controlled, Multicenter, Pilot Trial, Using Inhaled Molgramostim in Cystic Fibrosis Subjects With Nontuberculous Mycobacterial (NTM) Infection	Mycobacterium Infections, Nontuberculous;Cystic Fibrosis (CF)	Drug: Molgramostim nebulizer solution;Device: PARI eFlow nebulizer system	Savara Inc.		Recruiting	18 Years	N/A	All	30	Phase 2	United States
28	NCT03734744	June 17, 2019	26 August 2019	PK/PD of Vitamin D3 in Adults With CF	A Pilot Study Evaluating Single, High-dose Pharmacokinetics/Pharmacodynamics of Vitamin D3 in CF	Vitamin D Deficiency;Cystic Fibrosis	Dietary Supplement: Vitamin D3	University of Southern California		Recruiting	18 Years	N/A	All	12	N/A	United States
29	NCT03485456	May 29, 2019	26 August 2019	DPI-Tobra-Kind Cyclops® in Children With Cystic Fibrosis	Pharmacokinetic Evaluation and Tolerability of Dry Powder Tobramycin Via the Cyclops® in Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Tobramycin	University Medical Center Groningen		Recruiting	6 Years	18 Years	All	10	Phase 1/Phase 2	Netherlands
30	NCT04010799	May 27, 2019	15 July 2019	A Clinical Study to Investigate Safety, Tolerability and Distribution of CHF 6333 After One or After Repeated Inhalation in Patients With Cystic Fibrosis (CF) and in Patients With Non Cystic Fibrosis (NCFB) Bronchiectasis	A Phase Ib, Randomised, Double-blind, Placebo-controlled Study to Investigate the Safety, Tolerability and Pharmacokinetics of Inhaled CHF 6333 After Single and Repeated Ascending Doses in Patients Affected by Cystic Fibrosis and Non Cystic Fibrosis Bronchiectasis	Cystic Fibrosis;Non-Cystic Fibrosis Bronchiectasis	Drug: CHF 6333;Drug: Placebo	Chiesi Farmaceutici S.p.A.		Recruiting	18 Years	N/A	All	48	Phase 1	Germany
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
31	NCT03965832	May 24, 2019	15 July 2019	HFNT During Exercise in CF	A Pilot Study to Evaluate the Feasibility of Using High-flow Nasal Therapy During Exercise in Patients With Cystic Fibrosis and Severe Lung Disease	Cystic Fibrosis	Device: HFNT during exercise;Device: Standard oxygen therapy/RA	The Leeds Teaching Hospitals NHS Trust		Recruiting	18 Years	N/A	All	20	N/A	United Kingdom
32	NCT03822455	May 15, 2019	29 July 2019	A Phase 2b Randomised, Placebo Controlled Study of OligoG in Patients With Cystic Fibrosis	A Phase 2b Randomised, Double-blind, Parallel-group Study of Alginate Oligosaccharide (OligoG) Dry Powder Inhalation in Addition to Standard of Care Compared to Placebo in Addition to Standard of Care in Patients With Cystic Fibrosis (CF)	Cystic Fibrosis	Drug: OligoG DPI	AlgiPharma AS	AlgiPharma Australia Pty. Ltd.	Recruiting	18 Years	N/A	All	33	Phase 2/Phase 3	Australia
33	NCT02748798	May 2019	23 April 2019	Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders	Developing Optimal Parameters for Hyperpolarized Noble Gas (3He and 129Xe) and Inert Fluorinated Gas Magnetic Resonance Imaging of Lung Disorders	Lung Transplant;Lung Resection;Lung Cancer;Asthma;Cystic Fibrosis;Chronic Obstructive Pulmonary Disease;Emphysema;Mesothelioma;Asbestosis;Pulmonary Embolism;Interstitial Lung Disease;Pulmonary Fibrosis;Bronchiectasis;Seasonal Allergies;Cold Virus;Lung Infection;Pulmonary Hypertension;Pulmonary Dysplasia;Obstructive Sleep Apnea	Drug: HP 3He;Drug: HP 129Xe;Drug: PFP;Drug: SF6;Device: 129Xe Small and Large Human Lung Coil;Device: 3He Human Lung Coil;Device: PFP and SF6 Human Lung Coil	Thunder Bay Regional Research Institute	Thunder Bay Regional Health Sciences Centre;St. Joseph's Care Group;Lakehead University	Not recruiting	18 Years	N/A	All	160	Early Phase 1	Canada
34	NCT03903913	May 2019	23 April 2019	Safety, Tolerability and Efficacy of S-1226 in Cystic Fibrosis	A Phase IIa Open Label Study to Evaluate the Safety, Tolerability and Efficacy of S-1226 Administered by Nebulization in Subjects With Mild to Moderate Cystic Fibrosis	Cystic Fibrosis	Drug: S-1226	SolAeroMed Inc.		Recruiting	14 Years	50 Years	All	12	Phase 2	Canada
35	NCT03912233	April 30, 2019	22 October 2019	A Study to Evaluate the Safety and Efficacy of VX-121 Combination Therapy in Subjects With Cystic Fibrosis	A Phase 2, Randomized, Double-blind, Controlled Study to Evaluate the Safety and Efficacy of VX-121 Combination Therapy in Subjects Aged 18 Years and Older With Cystic Fibrosis	Cystic Fibrosis	Drug: VX-121;Drug: TEZ;Drug: VX-561;Drug: TEZ/IVA;Drug: IVA;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	87	Phase 2	United States;Germany;Netherlands;Portugal;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
36	NCT03911713	April 17, 2019	4 November 2019	A Phase 2 Study to Evaluate Efficacy and Safety of VX-561 in Subjects Aged 18 Years and Older With Cystic Fibrosis	A Phase 2, Randomized, Double-blind Study to Evaluate the Efficacy and Safety of VX-561 in Subjects Aged 18 Years and Older With Cystic Fibrosis	Cystic Fibrosis	Drug: VX-561;Drug: IVA;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Recruiting	18 Years	N/A	All	88	Phase 2	United States;Australia;Belgium;Germany;Ireland;United Kingdom
37	NCT03795363	April 10, 2019	7 October 2019	Orkambi Treatment in 2 to 5 Year Old Children With CF	Nutritional Impact of Orkambi Treatment in 2 to 5 Year Old Children Homozygous for F508del Mutations	Cystic Fibrosis	Drug: Orkambi	Children's Hospital of Philadelphia	Vertex Pharmaceuticals Incorporated	Recruiting	2 Years	5 Years	All	32	Phase 2	United States
38	NCT03870841	April 3, 2019	26 August 2019	The Effect of PC945 on Aspergillus Fumigatus Lung Infection in Patients With Cystic Fibrosis	An Open-label Study to Assess the Safety, Pharmacokinetics and Pharmacodynamics of Inhaled PC945 in Adult Cystic Fibrosis (CF) Patients With Persistent Pulmonary Aspergillus Fumigatus Infection	Aspergillosis;Cystic Fibrosis	Drug: PC945	Pulmocide Ltd		Recruiting	18 Years	N/A	All	18	Phase 2	United Kingdom
39	NCT03710538	April 1, 2019	17 June 2019	The Effect of a Pre-meal Snack and/or Exercise on Post-prandial Glycemic Excursions in Adults With Cystic Fibrosis	The Effect of a Pre-meal Snack and/or Exercise on Post-prandial Glycemic Excursions in Adults With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Snack beverage;Behavioral: Exercise	Institut de Recherches Cliniques de Montreal		Recruiting	18 Years	N/A	All	14	N/A	Canada
40	NCT03724955	April 2019	29 April 2019	Pilot E2 for Hypogonadal Women With CFBD	Pilot Study Evaluating the Effects of Estradiol for Hypogonadal Women With Cystic Fibrosis and Osteopenia	Cystic Fibrosis Related Bone Disease	Drug: Estradiol 2 mg;Other: Placebo	Emory University		Not recruiting	18 Years	N/A	Female	0	Phase 4	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
41	NCT03894657	April 2019	8 April 2019	Validation of Respiratory Epithelial Functional Assessment to Predict Clinical Efficacy of Orkambi®.	Validation of Respiratory Epithelial Functional Assessment to Predict Clinical Efficacy of Orkambi®. Pathway to Personalized Therapy in Cystic Fibrosis	Cystic Fibrosis;Homozygous F508del Mutation	Diagnostic Test: Nasal brushing;Other: Sputum sample;Other: blood sample;Drug: Orkambi	Assistance Publique - Hôpitaux de Paris		Not recruiting	12 Years	N/A	All	104	N/A	France
42	EUCTR2018-003831-31-HU	28/03/2019	30 April 2019	MS1819-SD phase II clinical trial for Exocrine Pancreatic Insufficiency caused by Cystic Fibrosis.	A multicenter, open-label Phase 2 study with escalating doses of MS1819-SD on top of a stable dose of PPEs, to investigate the efficacy and safety of this combination for the compensation of severe exocrine pancreatic insufficiency in CF patients not fully compensated with only PPEs - MS1819/18/02	Exocrine Pancreatic Insufficiency MedDRA version: 20.0 Level: LLT Classification code 10033628 Term: Pancreatic insufficiency System Organ Class: 100000004856 ;Therapeutic area: Diseases [C] - Digestive System Diseases [C06]	Product Name: MS1819-SD Pharmaceutical Form: Capsule, hard	AzurRx		Authorised			Female: yes Male: yes	24	Phase 2	Hungary
43	NCT03902236	March 1, 2019	15 April 2019	Reaction Time and Postural Control in Individuals With Cystic Fibrosis and Bronchiectasis	Evaluation of Reaction Time and Postural Control in Individuals With Cystic Fibrosis and Bronchiectasis	Cystic Fibrosis;Bronchiectasis	Other: Reaction Time and Postural Control;Other: Muscle oxygenation;Other: Exercise capacity;Other: Balance	Hacettepe University		Recruiting	6 Years	18 Years	All	60	N/A	Turkey
44	NCT03632525	February 22, 2019	29 April 2019	Intravenous Iron in Adults With Cystic Fibrosis	A Pilot Trial of Intravenous Iron for the Treatment of Iron Deficiency in Adult Patients With Cystic Fibrosis	Cystic Fibrosis;Iron-deficiency	Drug: Ferric carboxymaltose	University of Oxford		Recruiting	18 Years	N/A	All	20	Phase 4	United Kingdom
45	NCT03783286	February 6, 2019	7 October 2019	Ivacaftor Treatment in 6 Month to 2 Year Old CF Subjects	Nutritional Impact of Ivacaftor Treatment in 6 Month to 2 Year Old Children With CF Gating Mutations	Cystic Fibrosis	Drug: Ivacaftor	Children's Hospital of Philadelphia	Vertex Pharmaceuticals Incorporated	Recruiting	1 Year	2 Years	All	18	Phase 4	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
46	NCT03746483	January 10, 2019	20 May 2019	OPTION: A Trial to Assess the Safety & Efficacy of MS1819 in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	A Phase 2, Open-Label, Multicenter, 2x2 Crossover Trial to Assess the Safety and Efficacy of MS1819-SD in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	Exocrine Pancreatic Insufficiency (EPI);Cystic Fibrosis (CF)	Drug: MS1819-SD;Drug: Porcine PERT	AzurRx BioPharma, Inc.		Recruiting	18 Years	N/A	All	30	Phase 2	United States;Poland
47	NCT03647228	December 13, 2018	2 September 2019	A Phase 1/2a Study to Assess the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Single and Multiple Doses of IONIS-ENaCRx in Healthy Volunteers and Patients With Cystic Fibrosis	A Double-Blind, Placebo-Controlled, Dose-Escalation, Phase 1/2a Study to Assess the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Single and Multiple Doses of ION-827359, an Antisense Oligonucleotide Inhibitor of ENaC, Administered to Healthy Volunteers and Patients With Cystic Fibrosis	Healthy Subjects;Cystic Fibrosis	Drug: IONIS-ENaCRx;Drug: Placebo	Ionis Pharmaceuticals, Inc.		Recruiting	18 Years	50 Years	All	88	Phase 1	United Kingdom
48	NCT03669614	December 7, 2018	26 August 2019	SAD and MAD of Inhaled AR-501 in Health Adults and P. Aeruginosa Infected Cystic Fibrosis Subjects	A P1/2a Randomized, Double-Blind, Two-Part, Dose-Ascending, Multicenter Study of the Safety and PK of AR-501 (Gallium Citrate), Administered Via Inhalation, in Healthy Adult and P. Aeruginosa Infected Cystic Fibrosis Subjects	Cystic Fibrosis	Drug: inhaled AR-501;Drug: inhaled AR-501 placebo	Aridis Pharmaceuticals, Inc.		Recruiting	18 Years	49 Years	All	96	Phase 1/Phase 2	United States
49	NCT02323100	December 2, 2018	29 July 2019	Glycerol Phenylbutyrate Corrector Therapy For CF (Cystic Fibrosis)	A Double Blind, Placebo Controlled, Dose Escalation Trial of Glycerol Phenylbutyrate Corrector Therapy for Cystic Fibrosis	Cystic Fibrosis	Drug: Ravicti low dose;Drug: Ravicti high dose;Drug: Placebo	National Jewish Health	University of Alabama at Birmingham;Children's Hospital of Philadelphia;Johns Hopkins University;Horizon Pharma Ireland, Ltd., Dublin Ireland	Recruiting	18 Years	N/A	All	36	Phase 1/Phase 2	United States
50	NCT03624101	December 1, 2018	27 May 2019	Novel Therapeutic Approaches for Treatment of CF Patients With W1282X Premature Termination Codon Mutations	Novel Therapeutic Approaches for Treatment of CF Patients With W1282X Premature Termination Codon Mutations	Cystic Fibrosis	Drug: Tezacaftor/Ivacaftor	University of Alabama at Birmingham		Recruiting	18 Years	N/A	All	5	Phase 4	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
51	NCT03756922	November 27, 2018	26 August 2019	A DDI Study of FDL169 and FDL176 in Healthy Subjects	A Phase 1/2, Drug-Drug Interaction Study of FDL169 and FDL176 in Healthy Subjects and in Cystic Fibrosis Subjects Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: FDL169;Drug: FDL176	Flatley Discovery Lab LLC		Recruiting	18 Years	55 Years	All	78	Phase 1/Phase 2	United Kingdom
52	EUCTR2017-003723-29-DE	19/11/2018	26 November 2018	A study of safety and efficacy of lenabasum in Cystic Fibrosis	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial to Evaluate Efficacy and Safety of Lenabasum in Cystic Fibrosis - A Phase 2 safety and efficacy study of Lenabasum in Cystic Fibrosis	Cystic Fibrosis (CF) MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Lenabasum CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Lenabasum CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 20- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Corbus Pharmaceuticals, Inc.		Authorised			Female: yes Male: yes	415	Phase 2	Serbia;Portugal;United States;Slovakia;Greece;Spain;Réunion;Austria;Russian Federation;Italy;United Kingdom;France;Hungary;Czech Republic;Canada;Poland;Belgium;Romania;Bulgaria;Netherlands;Germany;Sweden
53	NCT03748199	November 8, 2018	3 December 2018	Clinical Study to Investigate Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of POL6014 in Patients With CF	Phase-Ib/IIa Study to Investigate Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Orally Inhaled Multiple Doses of POL6014 in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: POL6014;Drug: Placebo	Santhera Pharmaceuticals		Recruiting	18 Years	55 Years	All	40	Phase 1/Phase 2	Germany
54	NCT03617718	November 1, 2018	21 January 2019	Project 2 Airway Potential Hydrogen (pH) in Asthma	Methods to Identify and Treat Severe Asthma Patients Project 2: Airway pH Phenotyping	Cystic Fibrosis;Asthma;Severe Persistent Asthma;Healthy	Drug: Glycine Buffer	University Hospitals Cleveland Medical Center	National Institutes of Health (NIH)	Recruiting	18 Years	50 Years	All	75	Phase 1/Phase 2	United States
55	EUCTR2018-000185-11-CZ	15/10/2018	28 February 2019	A Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy	A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous or Heterozygous for the F508del Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: 100 mg VX-445/50 mg TEZ /75 mg IVA FDC Product Code: VX-445/TEZ/IVA Pharmaceutical Form: Film-coated tablet INN or Proposed INN: VX-445 CAS Number: 2216712-66-0 Current Sponsor code: VX-445 Other descriptive name: VX-445 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: Tezacaftor CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	460	Phase 3	United States;Greece;Austria;United Kingdom;Italy;France;Czech Republic;Canada;Belgium;Australia;Germany;Netherlands;Sweden
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
56	NCT03265288	October 10, 2018	30 September 2019	Study of LAU-7b in the Treatment of Cystic Fibrosis in Adults	APPLAUD: A Double-Blind, Randomized, Placebo-Controlled, Phase II Study of the Efficacy and Safety of LAU-7b in the Treatment of Cystic Fibrosis in Adults	Cystic Fibrosis	Drug: LAU-7b;Drug: Placebo oral capsule	Laurent Pharmaceuticals Inc.	Cystic Fibrosis Foundation	Recruiting	18 Years	N/A	All	136	Phase 2	United States;Canada
57	NCT03525574	October 9, 2018	30 September 2019	A Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy	A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous or Heterozygous for the F508del Mutation	Cystic Fibrosis	Drug: VX-445;Drug: TEZ;Drug: IVA	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	507	Phase 3	United States;Australia;Austria;Belgium;Canada;Czechia;France;Germany;Greece;Italy;Netherlands;Sweden;United Kingdom
58	NCT03691779	October 2, 2018	11 November 2019	Evaluation of VX 445/TEZ/IVA in Cystic Fibrosis Subjects 6 Through 11 Years of Age	A Phase 3 Study Evaluating the Pharmacokinetics, Safety, and Tolerability of VX-445/TEZ/IVA Triple Combination Therapy in Cystic Fibrosis Subjects 6 Through 11 Years of Age	Cystic Fibrosis	Drug: VX-445;Drug: TEZ;Drug: IVA	Vertex Pharmaceuticals Incorporated		Recruiting	6 Years	11 Years	All	56	Phase 3	United States;Australia
59	NCT03956589	October 1, 2018	3 June 2019	Functional Respiratory Imaging and Orkambi in CF	Functional Respiratory Imaging (FRI) to Assess the Short-term Effect of the Product ORKAMBI (Lumacaftor/ Ivacaftor) on Lung Function in ORKAMBInaive Patients With Cystic Fibrosis Homozygous for Phe508del	Cystic Fibrosis	Drug: Orkambi	University Hospital, Antwerp		Recruiting	12 Years	N/A	All	20	Phase 4	Belgium
60	EUCTR2018-002579-16-SE	26/08/2018	3 September 2018	Measurement of the posaconazole concentration in exhaled breath in CF patients after a single dose posaconazole to correlate to the concentration in blood and saliva.	Evaluation of the possible use of analysis of posaconazole in exhaled breath as a surrogate marker for the lung to monitor adequate dosages of posaconazole in CF patients treated for Aspergillus spp. related lung-disease. Part 1. Pharmacokinetic single center study.	Cystic Fibrosis (CF) is the most common life–limiting autosomal recessive disease among people of European heritage. The condition is a result of a mutation in the cystic fibrosis transmembrane conductance regulator (cftr) gene on chromosome seven, which encodes a chloride channel. In the lung defective channel activity leads to thick, viscous secretion and impaired mucociliary clearance. This causes trapping of mucus, colonization with bacteria and fungi, and a persistent inflammatory response. MedDRA version: 20.0 Level: PT Classification code 10074549 Term: Cystic fibrosis respiratory infection suppression System Organ Class: 10042613 - Surgical and medical procedures ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Noxafil® Pharmaceutical Form: Tablet	Karolinska University Hospital, Stockholm CF center		Authorised			Female: yes Male: yes	12	Phase 2	Sweden
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
61	NCT03591094	August 21, 2018	29 April 2019	Study Assessing PTI-428 Safety, Tolerability, Pharmacokinetics and Effect in Subjects With Cystic Fibrosis	A Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Assess the Safety, Tolerability, Pharmacokinetics, and Effect of PTI-428 in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: PTI-428;Drug: Placebo	Proteostasis Therapeutics, Inc.		Not recruiting	18 Years	N/A	All	40	Phase 2	United States
62	NCT03551691	August 7, 2018	25 March 2019	PPIs and Fat Absorption in CF and EPI	Proton Pump Inhibitors and Fat Absorption in Subjects With Cystic Fibrosis and Pancreatic Insufficiency	Pancreatic Insufficiency;Cystic Fibrosis	Drug: Omeprazole 40mg Capsule;Drug: Placebo oral capsule	Children's Hospital of Philadelphia	Chiesi USA	Recruiting	12 Years	N/A	All	24	Phase 2	United States
63	NCT03525548	August 3, 2018	18 March 2019	A Study of VX-445 Combination Therapy in CF Subjects Homozygous for F508del (F/F)	A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del Mutation (F/F)	Cystic Fibrosis	Drug: VX-445;Drug: TEZ;Drug: IVA;Drug: TEZ/IVA	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	113	Phase 3	United States;Belgium;Netherlands;United Kingdom
64	NCT03633526	August 3, 2018	2 September 2019	Evaluation of VX-659/TEZ/IVA in Cystic Fibrosis Subjects 6 Through 11 Years of Age	A Phase 3 Study Evaluating the Pharmacokinetics, Safety, and Tolerability of VX-659/TEZ/IVA Triple Combination Therapy in Cystic Fibrosis Subjects 6 Through 11 Years of Age	Cystic Fibrosis	Drug: VX-659;Drug: TEZ;Drug: IVA	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	11 Years	All	18	Phase 3	United States
65	NCT03655249	August 1, 2018	30 September 2019	Effects of AD on VI in Patients With CF	Effects of Autogenic Drainage on Ventilation Inhomogeneity in Patients With Cystic Fibrosis	Cystic Fibrosis	Other: Autogenic drainage;Drug: Aerosoltherapy	Cliniques universitaires Saint-Luc- Université Catholique de Louvain		Recruiting	6 Years	N/A	All	30	N/A	Belgium
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
66	NCT03593434	July 30, 2018	20 August 2018	Airway Clearance Therapy on Hyperpolarized 129Xenon and MRI	The Effect of Airway Clearance Therapy on Hyperpolarized 129Xenon MRI Compared With Lung Clearance Index and Spirometry in Cystic Fibrosis	Cystic Fibrosis	Drug: hyperpolarized Xenon gas	Children's Hospital Medical Center, Cincinnati	National Heart, Lung, and Blood Institute (NHLBI)	Recruiting	6 Years	21 Years	All	20	Phase 2	United States
67	NCT03447262	July 13, 2018	18 March 2019	A Study Evaluating the Long Term Safety and Efficacy of VX-659 Combination Therapy	A Phase 3, Open-label Study Evaluating the Long Term Safety and Efficacy of VX-659 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous or Heterozygous for the F508del Mutation	Cystic Fibrosis	Drug: VX-659;Drug: TEZ;Drug: IVA	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	484	Phase 3	United States;Australia;Canada;Denmark;Germany;Ireland;Israel;Poland;Spain;Switzerland;United Kingdom
68	NCT03565692	July 1, 2018	18 December 2018	Lum-Iva-biota: Exploring the Respiratory Mycobiota and Microbiota Profile in French CF Patients Taking Lumacaftor-Ivacaftor	Lum-Iva-biota: Exploring the Respiratory Mycobiota and Microbiota Profile in French CF Patients Taking Lumacaftor-Ivacaftor	Cystic Fibrosis	Biological: the lung mycobiota and microbiota profile;Biological: the gut mycobiota and microbiota profile	University Hospital, Bordeaux	Société Française de la Mucoviscidose	Recruiting	6 Years	N/A	All	250	Phase 1	France
69	EUCTR2017-003761-99-DE	27/06/2018	14 January 2019	A Study of the Effects of Lumacaftor/Ivacaftor on Disease Progression in Subjects Aged 2 Through 5 Years With Cystic Fibrosis, Homozygous for F508del	An Exploratory Phase 2, 2-part, Randomized, Double blind, Placebo controlled Study With a Long term, Open label Period to Explore the Impact of Lumacaftor/Ivacaftor on Disease Progression in Subjects Aged 2 Through 5 Years With Cystic Fibrosis, Homozygous for F508del	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor / ivacaftor Product Code: VX-809 / VX-770 Pharmaceutical Form: Granules INN or Proposed INN: LUMACAFTOR CAS Number: 936727-05-8 Current Sponsor code: VX-809 Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Granules Route of administration of the placebo: Oral use Product Name: lumacaftor / ivacaftor Product Code: VX-809 / VX-770 Pharmaceutical Form: Granules INN or Proposed INN: LUMACAFTOR CAS Number: 936727-05-8 Current Sponsor code: VX-809 Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 188- Pharmaceutical form of the placebo: Granules Route of administration of the placebo: Oral use Trade Name: Orkambi 100 mg/125 mg film-coated tablets Product Name: LUM/IVA fixed-dose combination Pharmaceutical Form: Film-coated tablet INN or Proposed INN: LUMACAFTOR CAS Number: 936727-05-8 Current Sponsor code: VX-809 Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Other descriptive name: IVACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	50	Phase 2	Germany
70	EUCTR2016-004479-35-BE	26/06/2018	28 February 2019	A Study to Evaluate Efficacy and Safety of TEZ/IVA in Subjects Aged 6 through 11 Years With Cystic Fibrosis	A Phase 3, Double-blind, Parallel-group Study to Evaluate the Efficacy and Safety of Tezacaftor in Combination With Ivacaftor in Subjects Aged 6 Through 11 Years With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor 50 mg/ 75 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: VX-661/ivacaftor 100 mg/ 150 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s)	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	65	Phase 3	France;Poland;Belgium;Ireland;Denmark;Australia;Germany;United Kingdom;Switzerland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
71	EUCTR2017-002968-40-BE	26/06/2018	7 January 2019	A study lasting 96 weeks to assess a combined treatment called Tezacaftor and Ivacaftor in children aged 6 to 11 years who have Cystic Fibrosis	A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Tezacaftor in Combination With Ivacaftor in Subjects With Cystic Fibrosis Aged 6 Years and Older, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor 50 mg/ 75 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Product Name: VX-661/ivacaftor 100 mg/ 150 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg/g milligram(s)/gram Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Trade Name: Kalydeco 150mg Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	121	Phase 3	France;United States;Canada;Poland;Belgium;Ireland;Denmark;Australia;Germany;United Kingdom;Switzerland
72	NCT03525444	June 15, 2018	30 September 2019	A Phase 3 Study of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Heterozygous for the F508del Mutation and a Minimal Function Mutation (F/MF)	A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Minimal Function Mutation (F/MF)	Cystic Fibrosis	Drug: VX-445;Drug: TEZ;Drug: IVA;Drug: Matched Placebos	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	405	Phase 3	United States;Australia;Austria;Belgium;Canada;Czechia;France;Germany;Greece;Italy;Netherlands;Sweden;United Kingdom
73	EUCTR2018-001573-24-BE	11/06/2018	27 August 2018	Evaluation of the short-term effect of ORKAMBI on lung function in patients with cystic fibrosis who have two copies of the F508del mutation using functional respiratory imaging	Functional Respiratory Imaging (FRI) to assess the short-term effect of the product ORKAMBI (lumacaftor/ ivacaftor) on lung function in ORKAMBI-naive patients with Cystic Fibrosis Homozygous for Phe508del.	Cystic Fibrosis Homozygous (homozygous for the F508del mutation) MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Orkambi Pharmaceutical Form: Film-coated tablet INN or Proposed INN: LUMACAFTOR Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125-	Antwerp University Hospital		Authorised			Female: yes Male: yes	20	Phase 4	Belgium
74	EUCTR2017-003723-29-SE	07/06/2018	28 February 2019	A study of safety and efficacy of lenabasum in Cystic Fibrosis	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial to Evaluate Efficacy and Safety of Lenabasum in Cystic Fibrosis - A Phase 2 safety and efficacy study of Lenabasum in Cystic Fibrosis	Cystic Fibrosis (CF) MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule, hard INN or Proposed INN: LENABASUM CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule, hard INN or Proposed INN: LENABASUM CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 20- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Corbus Pharmaceuticals, Inc.		Authorised			Female: yes Male: yes	415	Phase 2	Serbia;Portugal;United States;Slovakia;Greece;Spain;Réunion;Austria;Russian Federation;Italy;United Kingdom;France;Hungary;Czech Republic;Canada;Poland;Belgium;Romania;Bulgaria;Netherlands;Germany;Sweden
75	EUCTR2017-003319-21-GB	04/06/2018	30 April 2019	Study Assessing the Safety, Tolerability and Pharmacokinetics of PTI-808 in Healthy Adult Subjects and in Adults with Cystic Fibrosis	A Phase 1 / 2 Study to Evaluate the Safety, Tolerability, and Pharmacokinetics of PTI-808 in Healthy Adult Subjects and in Adults with Cystic Fibrosis	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: N-(5-hydroxy-2,4-bis(trimethylsilyl)phenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide Product Code: PTI-808 Pharmaceutical Form: Capsule, hard INN or Proposed INN: None CAS Number: Not assigned Current Sponsor code: PTI-808 Other descriptive name: PTI-808 Concentration unit: mg milligram(s) Concentration type: range Concentration number: 10-150 Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Name: Sodium (R)-8-methyl-2-(3-methylbenzofuran-2-yl)-5-(1-(tetrahydro-2H-pyran-4-yl)ethoxy)quinoline-4-ca Product Code: PTI-801 Pharmaceutical Form: Capsule, hard INN or Proposed INN: None CAS Number: Not assigned Current Sponsor code: PTI-801 Other descriptive name: PTI-801 Concentration unit: mg milligram(s) Concentration type: range Concentration number: 50-100 Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Name: N-(trans-3-(5-((R)-1-hydroxyethyl)-1,3,4-oxadiazol-2-yl)cyclobutyl)-3-phenylisoxazole-5-carboxamide Product Code: PTI-428 Pharmaceutical Form: Capsule, hard INN or Proposed INN: None CAS Number: Not assigned Current Sponsor code: PTI-428 Other descriptive name: PTI-428 Concentration unit: mg milligram(s) Concentration type: range Concentration number: 5-100 Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Proteostasis Therapeutics, Inc.		Authorised			Female: yes Male: yes	240	Phase 1;Phase 2	France;United States;Canada;Belgium;Denmark;Germany;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
76	NCT03540524	May 31, 2018	15 April 2019	A Study Looking at the Safety, Tolerability and Efficacy of the Combination of the Study Drugs GLPG2451 and GLPG2222 With or Without GLPG2737 in Patients With Cystic Fibrosis.	Assessment of Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Multiple Oral Doses of the Combination of GLPG2451 and GLPG2222, With or Without GLPG2737, in Adult Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: GLPG2451 dose regimen A;Drug: GLPG2451 dose regimen B;Drug: GLPG2222;Drug: GLPG2737	Galapagos NV		Not recruiting	18 Years	N/A	All	10	Phase 1	Belgium;Bulgaria;Germany;Greece;Netherlands;Serbia;Sweden;United Kingdom
77	EUCTR2016-001440-18-NL	30/05/2018	11 June 2018	The influence of cytochrome P450 3A4 inhibitors on serum levels of Ivacaftor in cystic fibrosis patients and healthy subjects.	Pharmacokinetic interactions between ivacaftor and cytochrome P450 3A4 inhibitors in cystic fibrosis patients and healthy controls - IACI	cystic fibrosis and healthy subjects;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Ivacaftor Pharmaceutical Form: Film-coated tablet Trade Name: claritromycin 500mg film coated tablets Product Name: Claritromycin Pharmaceutical Form: Film-coated tablet Trade Name: Azithromycin 500 mg film-coated tablet Product Name: azithromycin Pharmaceutical Form: Film-coated tablet Trade Name: Norvir 100 mg film-coated tablets Product Name: Norvir Pharmaceutical Form: Film-coated tablet	university medical center utrecht		Authorised			Female: yes Male: yes	12	Phase 4	Netherlands
78	NCT03482960	May 29, 2018	22 October 2019	Comparison of 129Xe MRI With 19F MRI in CF Lung Disease	Comparison of 129Xe MRI With 19F MRI in CF Lung Disease	Cystic Fibrosis	Drug: Hyperpolarized Xenon gas;Drug: PFP	University of North Carolina, Chapel Hill	Cystic Fibrosis Foundation	Not recruiting	18 Years	N/A	All	8	Early Phase 1	United States
79	NCT03462056	May 17, 2018	15 July 2019	Ready to Use Therapeutic Food (RUTF) to Promote Growth in Cystic Fibrosis	Pilot Study of Ready to Use Therapeutic Food to Promote Weight Gain in Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Cystic Fibrosis Ready to Use Supplemental Food	Washington University School of Medicine	Cystic Fibrosis Foundation	Not recruiting	2 Years	12 Years	All	16	Phase 1/Phase 2	United States
80	NCT03559062	May 17, 2018	11 February 2019	A Study to Evaluate Efficacy and Safety of TEZ/IVA in Subjects Aged 6 Through 11 Years With Cystic Fibrosis	A Phase 3, Double-blind, Parallel-group Study to Evaluate the Efficacy and Safety of Tezacaftor in Combination With Ivacaftor in Subjects Aged 6 Through 11 Years With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: TEZ/IVA;Drug: IVA;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	11 Years	All	69	Phase 3	Australia;Belgium;Denmark;France;Germany;Ireland;Poland;Switzerland;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
81	NCT03375047	May 10, 2018	1 April 2019	Study to Evaluate the Safety & Tolerability of MRT5005 Administered by Nebulization in Adults With Cystic Fibrosis	A Phase 1/2, Randomized, Double-Blinded, Placebo-Controlled, Combined Single and Multiple Ascending Dose Study Evaluating the Safety, Tolerability, and Biological Activity of MRT5005 Administered by Nebulization to Adult Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: MRT5005;Drug: Normal saline	Translate Bio, Inc.		Recruiting	18 Years	N/A	All	32	Phase 1/Phase 2	United States
82	NCT03428334	May 10, 2018	14 October 2019	Roflumilast in Non-CF Bronchiectasis Study	A 4-week Single-arm Study of Roflumilast in Stable-state Non-cystic Fibrosis Bronchiectasis	Non-cystic Fibrosis Bronchiectasis	Drug: Oral roflumilast	The University of Hong Kong		Not recruiting	18 Years	N/A	All	20	Phase 2	Hong Kong
83	NCT03460990	May 1, 2018	18 December 2018	A Study of VX-659 Combination Therapy in CF Subjects Homozygous for F508del (F/F)	A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-659 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del Mutation (F/F)	Cystic Fibrosis	Drug: VX-659;Drug: TEZ;Drug: IVA;Drug: TEZ/IVA	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	116	Phase 3	United States;Australia;Germany;Ireland;Spain;United Kingdom
84	NCT03522831	May 1, 2018	4 November 2019	CF Bronchodilation	A Double-blind Placebo-controlled Crossover Study to Assess the Effects of Bronchodilation on Dyspnea, Ventilatory Responses, and Exercise Tolerance in Adults With Cystic Fibrosis	Lung Diseases;Cystic Fibrosis	Drug: Salbutamol;Drug: Placebo	University of British Columbia		Recruiting	19 Years	N/A	All	20	N/A	Canada
85	EUCTR2017-002968-40-IE	27/04/2018	28 February 2019	A study lasting 96 weeks to assess a combined treatment called Tezacaftor and Ivacaftor in children aged 6 to 11 years who have Cystic Fibrosis	A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Tezacaftor in Combination With Ivacaftor in Subjects With Cystic Fibrosis Aged 6 Years and Older, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor 50 mg/ 75 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Product Name: VX-661/ivacaftor 100 mg/ 150 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg/g milligram(s)/gram Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Trade Name: Kalydeco 150mg Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	121	Phase 3	France;United States;Canada;Poland;Belgium;Ireland;Denmark;Australia;Germany;United Kingdom;Switzerland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
86	EUCTR2017-004132-11-DE	25/04/2018	28 February 2019	A Study to Evaluate the Efficacy and Safety of VX-659 Drug in Combination With Other Drugs in Subjects With Cystic Fibrosis	A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-659 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Minimal Function Mutation (F/MF)	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-659/Tezacaftor/Ivacaftor Product Code: VX-659/TEZ/IVA Pharmaceutical Form: Film-coated tablet INN or Proposed INN: VX-659 Current Sponsor code: VX-659 Other descriptive name: VX-659 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 120- INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	360	Phase 3	United States;Canada;Poland;Spain;Ireland;Denmark;Australia;Israel;Norway;Germany;United Kingdom;Switzerland
87	NCT03492567	April 25, 2018	3 December 2018	Study of Circulating Blood Cell Monocytes as Predictive Biomarker of Bone Cystic Fibrosis Disease. Impact of CFTR Correctors.	Study of Circulating Monocytes as Predictive Biomarker of Osteoclastogenesis in Cystic Fibrosis-related Bone Loss. Impact of CFTR Correctors.	Cystic Fibrosis	Biological: Blood test	Assistance Publique - Hôpitaux de Paris	Vaincre la Mucoviscidose	Not recruiting	18 Years	N/A	All	25	N/A	France
88	EUCTR2016-004479-35-DK	18/04/2018	30 April 2019	A Study to Evaluate Efficacy and Safety of TEZ/IVA in Subjects Aged 6 through 11 Years With Cystic Fibrosis	A Phase 3, Double-blind, Parallel-group Study to Evaluate the Efficacy and Safety of Tezacaftor in Combination With Ivacaftor in Subjects Aged 6 Through 11 Years With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor 50 mg/ 75 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: VX-661/ivacaftor 100 mg/ 150 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s)	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	65	Phase 3	France;Poland;Belgium;Ireland;Australia;Denmark;Germany;United Kingdom;Switzerland
89	EUCTR2017-003723-29-HU	16/04/2018	27 August 2018	A study of safety and efficacy of lenabasum in Cystic Fibrosis	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial to Evaluate Efficacy and Safety of Lenabasum in Cystic Fibrosis - A Phase 2 safety and efficacy study of Lenabasum in Cystic Fibrosis	Cystic Fibrosis (CF) MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule INN or Proposed INN: Lenabasum CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule INN or Proposed INN: Lenabasum CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 20- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use	Corbus Pharmaceuticals, Inc.		Authorised			Female: yes Male: yes	415	Phase 2	Portugal;Serbia;United States;Slovakia;Greece;Spain;Austria;Russian Federation;Italy;United Kingdom;France;Hungary;Czech Republic;Canada;Poland;Belgium;Romania;Bulgaria;Netherlands;Germany;Sweden
90	NCT03527095	April 5, 2018	12 November 2018	A Study to Compare the Pharmacokinetics of Different Oral Formulations of FDL169 in Healthy Subjects	A Phase 1, Open-label, Randomised, Cross Over Study to Compare the Pharmacokinetics of Different Oral Formulations of FDL169 in Healthy Subjects Following Single Doses	Cystic Fibrosis	Drug: FDL169	Flatley Discovery Lab LLC		Not recruiting	18 Years	55 Years	All	11	Phase 1	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
91	NCT03489629	April 3, 2018	26 August 2019	STaph Aureus Resistance-Treat Early and Repeat (STAR-TER)	STaph Aureus Resistance-Treat Early and Repeat (STAR-TER)	Cystic Fibrosis	Drug: Trimethoprim Sulfamethoxazole (TMP/SMX);Drug: Minocycline;Drug: Mupirocin;Drug: Chlorhexidine Gluconate;Behavioral: Environmental Decontamination	University of North Carolina, Chapel Hill	University of Washington;Cook Children's Medical Center;Indiana University;National Jewish Health;University of Michigan;University of Texas Southwestern Medical Center;St. Louis Children's Hospital	Recruiting	2 Years	45 Years	All	42	Phase 2	United States
92	NCT03768089	March 19, 2018	11 June 2019	Study of VX-121 in Healthy Subjects and in Subjects With Cystic Fibrosis	A Phase 1/2 Study of VX-121 in Healthy Subjects and in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: VX-121;Drug: TEZ/IVA;Drug: IVA;Drug: Matched Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	114	Phase 1/Phase 2	Netherlands;United Kingdom
93	EUCTR2018-000126-55-NL	15/03/2018	21 May 2018	A study to investigate the safety and the movement of the study drug VX- 121 around the body in healthy people and patients with cystic fibrosis	A Phase 1/2 Study of VX-121 in Healthy Subjects and in Subjects With Cystic Fibrosis	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-121 Pharmaceutical Form: Powder for oral suspension INN or Proposed INN: Not yet assigned Current Sponsor code: VX-121 Concentration unit: mg milligram(s) Concentration type: range Concentration number: 10-720 Pharmaceutical form of the placebo: Powder for oral suspension Route of administration of the placebo: Oral use Product Name: tezacaftor/ivacaftor 100mg/150mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	120	Phase 1;Phase 2	Netherlands
94	NCT03447249	March 7, 2018	18 March 2019	A Phase 3 Study of VX-659 Combination Therapy in Subjects With Cystic Fibrosis Heterozygous for the F508del Mutation and a Minimal Function Mutation (F/MF)	A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-659 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Minimal Function Mutation (F/MF)	Cystic Fibrosis	Drug: VX-659;Drug: TEZ;Drug: IVA;Drug: Matched Placebos	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	385	Phase 3	United States;Australia;Canada;Denmark;Germany;Ireland;Israel;Poland;Spain;Switzerland;United Kingdom
95	NCT03516331	March 7, 2018	12 November 2018	A Drug-Drug Interaction Study of FDL169 and FDL176 in Healthy Subjects	A Phase 1, Open Label, Drug-Drug Interaction Study of FDL169 and FDL176 in Healthy Subjects	Cystic Fibrosis	Drug: FDL176 & FDL169 coadministration	Flatley Discovery Lab LLC		Not recruiting	18 Years	55 Years	All	16	Phase 1	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
96	NCT03367494	March 1, 2018	8 April 2019	A New Optical Sweat Test Method Based on a Citric Acid-derived Multi-halide Sensor	A New Optical Sweat Test Method Based on a Citric Acid-derived Multi-halide Sensor	Cystic Fibrosis	Diagnostic Test: Measurement of Sweat Chloride and Sweat Bromide	Milton S. Hershey Medical Center		Recruiting	18 Years	N/A	All	50	N/A	United States
97	NCT03589313	February 12, 2018	23 July 2018	Pharmacokinetics of GLPG3067 in Male Subjects With Cystic Fibrosis.	Evaluation of the Pharmacokinetics, Safety and Tolerability of a Single Dose of GLPG3067 Administered as Solid Formulation in Male Subjects With Cystic Fibrosis.	Cystic Fibrosis	Drug: GLPG3067 single dose	Galapagos NV		Not recruiting	18 Years	N/A	Male	6	Phase 1	Belgium
98	NCT03421366	February 5, 2018	12 February 2018	Modified Release Posaconazole in Patients With Cystic Fibrosis	Modified Release Posaconazole in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Posaconazole	Bayside Health		Not recruiting	18 Years	N/A	All	20	N/A
99	NCT03500263	January 30, 2018	30 September 2019	Safety, Tolerability, and Pharmacokinetics of PTI-808, PTI-801, and PTI-428 Combination Therapy in Subjects With Cystic Fibrosis	A Phase 1 / 2, Randomized, Double-Blind, Placebo-Controlled Study Designed to Evaluate the Safety, Tolerability, and Pharmacokinetics of PTI-808, PTI-801, and PTI-428 Combination Therapy in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: PTI-808;Drug: Placebo;Drug: PTI-801;Drug: PTI-428	Proteostasis Therapeutics, Inc.		Not recruiting	18 Years	N/A	All	12	Phase 1/Phase 2	United Kingdom
100	NCT03460704	January 29, 2018	11 June 2018	Trial in Non-cystic Fibrosis Bronchiectasis Patients With Chronic Lung Infections Treated With Colistimethate Sodium (PROMIS-2)	A Double-blind, Placebo-controlled, Multi-centre, Clinical Trial to Investigate the Efficacy and Safety of 24 Months of Therapy With Inhaled Colistimethate Sodium in the Treatment of Subjects With Non-cystic Fibrosis Bronchiectasis Chronically Infected With Pseudomonas Aeruginosa (P. Aeruginosa)	Non Cystic Fibrosis Bronchiectasis	Drug: Colistimethate sodium;Drug: Saline Solution	Zambon SpA		Recruiting	18 Years	90 Years	All	374	Phase 3	Poland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
101	NCT02653027	January 1, 2018	26 August 2019	Effect of Lumacaftor-ivacaftor on Glucose Handling and Tolerance in Cystic Fibrosis Phe508del	A Study of the Effect of Combination Lumacaftor and Ivacaftor on Glucose Tolerance in Persons With Cystic Fibrosis Who Are Homozygous for the Phe508del Cystic Fibrosis Transmembrane Conductance Regulator Mutation.	Diabetes;Cystic Fibrosis	Drug: Lumacaftor-ivacaftor;Other: OGTT	Massachusetts General Hospital		Not recruiting	18 Years	65 Years	All	0	N/A
102	EUCTR2018-000185-11-DE		28 February 2019	A Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy	A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous or Heterozygous for the F508del Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: 100 mg VX-445/50 mg TEZ /75 mg IVA FDC Product Code: VX-445/TEZ/IVA Pharmaceutical Form: Film-coated tablet INN or Proposed INN: VX-445 CAS Number: 2216712-66-0 Current Sponsor code: VX-445 Other descriptive name: VX-445 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: Tezacaftor CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Not Available			Female: yes Male: yes	460	Phase 3	United States;Greece;Austria;United Kingdom;Italy;France;Czech Republic;Canada;Belgium;Australia;Germany;Netherlands;Sweden
103	EUCTR2018-000185-11-SE		28 February 2019	A Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy	A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous or Heterozygous for the F508del Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: 100 mg VX-445/50 mg TEZ /75 mg IVA FDC Product Code: VX-445/TEZ/IVA Pharmaceutical Form: Film-coated tablet INN or Proposed INN: VX-445 CAS Number: 2216712-66-0 Current Sponsor code: VX-445 Other descriptive name: VX-445 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: Tezacaftor CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Not Available			Female: yes Male: yes	460	Phase 3	United States;Greece;Austria;United Kingdom;Italy;France;Czech Republic;Canada;Belgium;Australia;Germany;Netherlands;Sweden
104	EUCTR2018-002624-16-FR		28 February 2019	NA	Validation of respiratory epithelial functional assessment to predict clinical efficacy of Orkambi®. Pathway to personalized therapy in Cystic Fibrosis PREDICT-CF - PREDICT-CF	Homozygous F508del patient aged 12 years or older MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Orkambi® Product Name: Orkambi® Pharmaceutical Form: Tablet	ASSISTANC-PUBLIQUE-HOPITAUX DE PARIS (AP-HP)		Not Available			Female: yes Male: yes	104	Phase 4	France
105	NCT03451045	December 22, 2017	26 August 2019	Trial to Evaluate Efficacy and Safety of Lenabasum in Cystic Fibrosis	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial to Evaluate Efficacy and Safety of Lenabasum in Cystic Fibrosis	Cystic Fibrosis	Drug: Lenabasum 20 mg;Drug: Lenabasum 5 mg;Other: Placebo	Corbus Pharmaceuticals Inc.	Cystic Fibrosis Foundation	Recruiting	12 Years	N/A	All	415	Phase 2	United States;Austria;Belgium;Bulgaria;Canada;Czechia;France;Germany;Greece;Hungary;Italy;Netherlands;Poland;Portugal;Russian Federation;Serbia;Slovakia;Spain;Sweden;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
106	NCT03424252	December 18, 2017	12 November 2018	An Phase 1 Study to Evaluate the Pharmacokinetic (PK) Profile of FDL169 New Formulations in Healthy Subjects	A Phase 1, Open-label, Crossover, Randomised Study to Evaluate the Pharmacokinetic Profile of FDL169 Sublingual Formulations in the Fed State in Healthy Subjects	Cystic Fibrosis	Drug: FDL169	Flatley Discovery Lab LLC		Not recruiting	18 Years	55 Years	All	11	Phase 1	United Kingdom
107	NCT03218917	December 1, 2017	11 June 2019	Assessment of INS1007 in Subjects With Non-Cystic Fibrosis Bronchiectasis	Randomized, Double-Blind, Placebo-Controlled, Parallel-Group, Multi-Center Study to Assess the Efficacy, Safety & Tolerability, and PK of INS1007 Administered Once Daily for 24 Weeks in Subjects With Non-CF Bronchiectasis - The Willow Study	Non-Cystic Fibrosis Bronchiectasis	Drug: INS1007 10 mg oral tablet;Drug: INS1007 25 mg oral tablet;Drug: Placebo Oral Tablet	Insmed Incorporated		Not recruiting	18 Years	85 Years	All	240	Phase 2	United States;Australia;Belgium;Bulgaria;Denmark;Germany;Italy;Korea, Republic of;Netherlands;New Zealand;Poland;Singapore;Spain;United Kingdom;Sweden
108	NCT03474042	November 29, 2017	25 June 2018	GLPG2737 on Top of Orkambi in Subjects With Cystic Fibrosis	A Phase IIa, Randomized, Double-blind, Placebo-controlled Study to Evaluate GLPG2737 in Orkambi-treated Subjects With Cystic Fibrosis Homozygous for the F508del Mutation	Cystic Fibrosis	Drug: GLPG2737;Drug: Placebo	Galapagos NV		Not recruiting	18 Years	N/A	All	22	Phase 2	Germany
109	NCT03219164	November 28, 2017	28 October 2019	Safety and Efficacy of 2 Treatment Regimens of Aztreonam for Inhalation Solution in Children With Cystic Fibrosis and New Onset Pseudomonas Aeruginosa Infection	Randomized, Double-Blind, Phase 3B Trial to Evaluate the Safety and Efficacy of 2 Treatment Regimens of Aztreonam 75 mg Powder and Solvent for Nebulizer Solution / Aztreonam for Inhalation Solution (AZLI) in Pediatric Subjects With Cystic Fibrosis (CF) and New Onset Respiratory Tract Pseudomonas Aeruginosa (PA) Infection/Colonization	Pseudomonas Aeruginosa Respiratory Tract Infection;Cystic Fibrosis	Drug: AZLI;Drug: Placebo	Gilead Sciences		Recruiting	3 Months	18 Years	All	140	Phase 3	United States;Austria;Belgium;Denmark;France;Germany;Greece;Israel;Italy;Netherlands;Spain;United Kingdom
110	EUCTR2016-005110-22-DE	20/11/2017	25 June 2018	A clinical study to investigate safety, tolerability and dose of orally inhaled multiple doses of POL6014 in patients with Cystic Fibrosis	Phase-Ib/IIa study to investigate safety, tolerability, pharmacokinetics and pharmacodynamics of orally inhaled multiple doses of POL6014 in patients with Cystic Fibrosis	Cystic fibrosis;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: POL6014 Pharmaceutical Form: Nebuliser solution Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Santhera Pharmaceuticals (Switzerland) Ltd		Authorised			Female: yes Male: yes	40	Phase 1;Phase 2	Germany
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
111	NCT03206788	November 11, 2017	26 August 2019	Losartan and Inflammation in Cystic Fibrosis	Losartan as Anti-inflammatory Therapy to Augment F508del Cystic Fibrosis Transmembrane (CFTR) Recovery	Cystic Fibrosis	Drug: Losartan;Drug: placebo	University of Miami	University of Alabama at Birmingham;Children's Hospital Medical Center, Cincinnati;University of Kansas Medical Center	Recruiting	12 Years	N/A	All	36	Phase 2	United States
112	EUCTR2017-002181-42-DE	27/10/2017	23 July 2018	GLPG2737 on top of Orkambi in subjects with cystic fibrosis	A Phase IIa, randomized, double-blind, placebo-controlled study to evaluate GLPG2737 in Orkambi-treated subjects with cystic fibrosis homozygous for the F508del mutation	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Product Name: GLPG2737 Product Code: G1117337 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Not applicable Current Sponsor code: G1117337 Other descriptive name: GLPG2737 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use	Galapagos NV		Not Recruiting			Female: yes Male: yes	18	Phase 2	Germany
113	NCT03278314	October 9, 2017	12 November 2018	Tezacaftor/Ivacaftor Combination Therapy Expanded Access Program for Patients 12 Years of Age and Older With Cystic Fibrosis	Tezacaftor/Ivacaftor Combination Therapy Expanded Access Program for Patients 12 Years of Age and Older With Cystic Fibrosis	Cystic Fibrosis	Drug: tezacaftor/ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All		Phase 1/Phase 2
114	EUCTR2017-000797-11-NL	06/10/2017	30 April 2018	A Study of VX-445 in Healthy Subjects and Subjects with Cystic Fibrosis	A Phase 1/2 Study of VX-445 in Healthy Subjects and Subjects with Cystic Fibrosis	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-445 Pharmaceutical Form: Tablet INN or Proposed INN: VX-445 Current Sponsor code: VX-445 Other descriptive name: VX-445 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: VX-445 Current Sponsor code: VX-445 Other descriptive name: VX-445 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: tezacaftor/ivacaftor 100mg/150mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: tezacaftor Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	80	Phase 2	Netherlands;Australia;Belgium;United States
115	NCT03309358	September 28, 2017	11 June 2018	A Study of the Safety and Tolerability of Inhaled SNSP113 in Healthy Subjects and Subjects With Stable Cystic Fibrosis	A Multiple-Site, Randomized, Double-Blind, Placebo-Controlled, Single Ascending Dose (SAD) Study to Assess the Safety and Tolerability of Inhaled SNSP113 in Healthy Male Subjects (Part A) and Subjects With Stable Cystic Fibrosis (Part B)	Lung Diseases;Pulmonary Disease;Cystic Fibrosis;Cystic Fibrosis Lung;Cystic Fibrosis Pulmonary Exacerbation;Cystic Fibrosis With Exacerbation;Respiratory Tract Disease;Pulmonary Inflammation;Multi-antibiotic Resistance;Antibiotic Resistant Infection;Lung Infection;Lung Infection Pseudomonal;Lung; Infection, Atypical Mycobacterium;Burkholderia Infections;Burkholderia Cepacia Infection;Lung Inflammation	Drug: Inhaled SNSP113;Drug: Inhaled Placebo	Synspira, Inc.		Not recruiting	18 Years	50 Years	All	32	Phase 1	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
116	NCT03181932	September 26, 2017	7 October 2019	A Study of AeroVanc for the Treatment of MRSA Infection in CF Patients	A Phase III, Randomized, Double-blind, Placebo-controlled Study of AeroVanc for the Treatment of Persistent Methicillin-resistant Staphylococcus Aureus Lung Infection in Cystic Fibrosis Patients	MRSA;Cystic Fibrosis	Drug: Vancomycin inhalation powder;Drug: Placebo inhalation powder	Savara Inc.		Recruiting	6 Years	N/A	All	200	Phase 3	United States;Canada
117	EUCTR2016-002749-42-DE	22/09/2017	28 February 2019	Study of Aztreonam for Inhalation in Children with Cystic Fibrosis and New Infection of the Airways by Pseudomonas aeruginosa bacteria	Randomized, Double-Blind, Phase 3B Trial to Evaluate the Safety and Efficacy of 2 Treatment Regimens of Aztreonam 75 mg Powder and Solvent for Nebulizer Solution / Aztreonam for Inhalation Solution (AZLI) in Pediatric Subjects with Cystic Fibrosis (CF) and New Onset Respiratory Tract Pseudomonas aeruginosa (PA) Infection/Colonization - ALPINE2 (Aztreonam Lysine for Pseudomonas Infection Eradication 2)	Cystic fibrosis and new onset lower respiratory tract culture positive for Pseudomonas aeruginosa MedDRA version: 20.1 Level: LLT Classification code 10068288 Term: Cystic fibrosis pulmonary exacerbation System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Cayston Pharmaceutical Form: Powder and solvent for nebuliser solution INN or Proposed INN: AZTREONAM Current Sponsor code: AZLI Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Powder and solvent for nebuliser solution Route of administration of the placebo: Inhalation use	Gilead Sciences, Inc.		Authorised			Female: yes Male: yes	140	Phase 3	United States;Greece;Spain;Ireland;Austria;Israel;United Kingdom;Italy;France;Belgium;Denmark;Germany;Netherlands
118	NCT02769637	September 7, 2017	9 July 2018	Effect of Acid Blockade on Microbiota and Inflammation in Cystic Fibrosis (CF)	Effect of Acid Blockade on Microbiota and Inflammation in Cystic Fibrosis (CF)	Cystic Fibrosis	Drug: PPI treatment	University of Colorado, Denver	Cystic Fibrosis Foundation Therapeutics	Not recruiting	10 Years	21 Years	All	2	Phase 1	United States
119	NCT03771313	September 1, 2017	18 December 2018	Pharmacokinetic and Pharmacodynamic Analysis of Ceftaroline in Children and Adolescents With Cystic Fibrosis	Pharmacokinetic and Pharmacodynamic Analysis of Ceftaroline in Children and Adolescents With Cystic Fibrosis	Cystic Fibrosis	Drug: Ceftaroline	Children's Hospital Medical Center, Cincinnati		Recruiting	2 Years	21 Years	All	24	Phase 4	United States
120	EUCTR2016-005230-30-PT	28/08/2017	30 April 2019	A research study to find out if SPX-101 helps people with Cystic Fibrosis and to find out if it is safe.	A Randomized, Double-Blind, Placebo-Controlled Phase II Study to Evaluate the Efficacy and Safety of SPX-101 Inhalation Solution in Subjects with Cystic Fibrosis (HOPE-1 STUDY: HYDRATION FOR OPTIMAL PULMONARY EFFECTIVENESS) - HOPE-1 Study: Hydration for Optimal Pulmonary Effectiveness	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: SPX-101 Pharmaceutical Form: Inhalation solution INN or Proposed INN: SPX-101 Current Sponsor code: SPX-101 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 60- Pharmaceutical form of the placebo: Inhalation solution Route of administration of the placebo: Inhalation use Product Name: SPX-101 Pharmaceutical Form: Inhalation solution INN or Proposed INN: SPX-101 Current Sponsor code: SPX-101 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 60- Pharmaceutical form of the placebo: Inhalation solution Route of administration of the placebo: Inhalation use	Spyryx Biosciences, Inc.		Authorised			Female: yes Male: yes	90	Phase 2	France;Portugal;Canada;Australia;Italy;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
121	EUCTR2017-000797-11-BE	28/08/2017	7 January 2019	A Study of VX-445 in Healthy Subjects and Subjects with Cystic Fibrosis	A Phase 1/2 Study of VX-445 in Healthy Subjects and Subjects with Cystic Fibrosis	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-445 Pharmaceutical Form: Tablet INN or Proposed INN: VX-445 Current Sponsor code: VX-445 Other descriptive name: VX-445 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: VX-445 Current Sponsor code: VX-445 Other descriptive name: VX-445 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: tezacaftor/ivacaftor 100mg/150mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: tezacaftor Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: Tezacaftor Product Code: VX-661 Pharmaceutical Form: Tablet INN or Proposed INN: Tezacaftor (TEZ) Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: Deuterated Ivacaftor Product Code: VX-561 (CTP-656) Pharmaceutical Form: Tablet INN or Proposed INN: VX-561 Current Sponsor code: VX-561 Other descriptive name: VX-561	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	104	Phase 2	United States;Belgium;Australia;Netherlands
122	NCT03093714	August 23, 2017	11 June 2018	A Study to Evaluate Safety, PK and PD of FDL169 in Cystic Fibrosis Subjects	A Randomized, Double-Blind, Placebo-Controlled, Parallel Study to Evaluate Safety, Pharmacokinetics (PK) and Pharmacodynamics(PD) of FDL169 in Cystic Fibrosis (CF) Subjects Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: FDL169;Drug: Placebo	Flatley Discovery Lab LLC		Not recruiting	18 Years	85 Years	All	27	Phase 1	Australia;Czechia;Germany;United Kingdom
123	NCT03277196	August 16, 2017	6 May 2019	A Study to Evaluate the Safety of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have an Approved Ivacaftor-Responsive Mutation	A Phase 3, 2-Arm, Open-label Study to Evaluate the Safety and Pharmacodynamics of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have an Approved Ivacaftor-Responsive Mutation	Cystic Fibrosis	Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated		Recruiting	N/A	24 Months	All	75	Phase 3	United States;Australia;Canada;Germany;Ireland;United Kingdom
124	EUCTR2016-004558-13-PL	14/08/2017	30 April 2019	A double-blind, placebo controlled, multicentre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled colistimethate sodium in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa)	A double-blind, placebo controlled, multicentre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled colistimethate sodium in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa) - PROMIS II	Non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa MedDRA version: 20.1 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Promixin, 1 million International Units (IU) Powder for Nebuliser Solution Product Name: Colistimethate sodium Pharmaceutical Form: Powder for nebuliser solution INN or Proposed INN: COLISTIMETHATE SODIUM CAS Number: 8068-28-8 Other descriptive name: Colistimethate Sodium Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 1000000- Pharmaceutical form of the placebo: Powder for nebuliser solution Route of administration of the placebo: Inhalation use	Zambon S.p.A.		Authorised			Female: yes Male: yes	420	Phase 3	France;United States;Canada;Argentina;Poland
125	EUCTR2016-003585-11-GB	10/08/2017	10 September 2018	Study to Evaluate the Safety and Efficacy of VX-659 drug in combination with other drugs in Subjects Aged 18 Years and Older With Cystic Fibrosis	A Phase 2, Randomized, Double-blind, Controlled Study to Evaluate the Safety and Efficacy of VX-659 Combination Therapy in Subjects Aged 18 Years and Older With Cystic Fibrosis	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-659 Product Code: VX-659 Pharmaceutical Form: Tablet INN or Proposed INN: VX-659 Current Sponsor code: VX-659 Other descriptive name: VX-659 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 80- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: Tezacaftor/Ivacaftor 100mg/150mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Tezacaftor Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: Tezacaftor Product Code: VX-661 Pharmaceutical Form: Tablet INN or Proposed INN: TEZACAFTOR Current Sponsor code: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: Deuterated Ivacaftor Product Code: VX-561 (CTP-656) Pharmaceutical Form: Tablet INN or Proposed INN: Deuterated Ivacaftor Other descriptive name: VX-561 (CTP-656) Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	105	Phase 2	United States;Ireland;Israel;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
126	NCT03172793	August 8, 2017	4 November 2019	Telavancin Pharmacokinetics in Cystic Fibrosis Patients	Pharmacokinetics and Tolerability of Telavancin at Differing Dosing Regimens in Cystic Fibrosis Adults Admitted With Acute Pulmonary Exacerbations	Cystic Fibrosis	Drug: Telavancin Injection	Joseph L. Kuti, PharmD	Cumberland Pharmaceuticals, Inc.	Not recruiting	18 Years	N/A	All	18	Phase 4	United States
127	NCT03224351	August 8, 2017	11 March 2019	A Study Evaluating the Safety and Efficacy of VX-659 Combination Therapy in Subjects With Cystic Fibrosis	A Phase 2, Randomized, Double-blind, Controlled Study to Evaluate the Safety and Efficacy of VX-659 Combination Therapy in Subjects Aged 18 Years and Older With Cystic Fibrosis	Cystic Fibrosis	Drug: TEZ/IVA;Drug: VX-659;Drug: IVA;Drug: Matched Placebos;Drug: TEZ;Drug: VX-561	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	124	Phase 2	United States;Ireland;Israel;United Kingdom
128	NCT03229252	August 1, 2017	3 June 2019	An Efficacy and Safety Study of SPX-101 Inhalation Solution in Subjects With Cystic Fibrosis	A Randomized, Double-blind, Placebo-controlled Phase II Study to Evaluate the Efficacy and Safety of SPX-101 Inhalation Solution in Subjects With Cystic Fibrosis (HOPE-1 Study: Hydration for Optimal Pulmonary Effectiveness)	Cystic Fibrosis	Drug: Placebo Inhalation Solution;Drug: SPX-101	Spyryx Biosciences, Inc.		Not recruiting	18 Years	50 Years	All	91	Phase 2	Canada;France;Italy;Portugal;United Kingdom
129	NCT03258424	July 28, 2017	11 June 2018	Study Assessing PTI-428 Safety, Tolerability, and Pharmacokinetics in Subjects With Cystic Fibrosis on KALYDECO® as Background Therapy	A Phase I, Randomized, Placebo-Controlled, Study Designed to Assess the Safety, Tolerability, and Pharmakinetics of PTI-428 in Subjects With Cystic Fibrosis.	Cystic Fibrosis	Drug: PTI-428;Drug: Placebo	Proteostasis Therapeutics, Inc.		Recruiting	18 Years	N/A	All	16	Phase 1	United Kingdom
130	EUCTR2016-001214-24-DE	27/07/2017	26 March 2018	A Multi-center, Randomized, Placebo-Controlled Phase I/II Study Designed to Assess the Safety, Tolerability, and how the body breaks down the drug PTI-428 in Subjects with Cystic Fibrosis	A Phase I/II Multi-center, Randomized, Placebo-Controlled, Study Designed to Assess the Safety, Tolerability, and Pharmacokinetics of PTI-428 in Subjects with Cystic Fibrosis - Proteostasis PTI428	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Code: PTI-428 Pharmaceutical Form: Capsule, hard Current Sponsor code: PTI-428 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Code: PTI-428 Pharmaceutical Form: Capsule, hard Current Sponsor code: PTI-428 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Proteostasis Therapeutics		Not Recruiting			Female: yes Male: yes	132	Phase 1;Phase 2	France;United States;Czech Republic;Canada;Denmark;Germany;Italy;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
131	NCT02819856	July 21, 2017	25 March 2019	SPI-1005 for Prevention and Treatment of Tobramycin Induced Ototoxicity	A Phase 2, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety and Efficacy of SPI-1005 in Cystic Fibrosis (CF) Patients With Acute Pulmonary Exacerbation (APE) Receiving IV Tobramycin at Risk for Ototoxicity	Ototoxicity	Drug: Placebo;Drug: SPI-1005 Ebselen 200mg Capsule x1;Drug: SPI-1005 Ebselen 200mg Capsule x2;Drug: SPI-1005 Ebselen 200mg Capsule x3	Sound Pharmaceuticals, Incorporated	Medical University of South Carolina;Cystic Fibrosis Foundation	Recruiting	18 Years	N/A	All	80	Phase 2	United States
132	NCT03068312	July 18, 2017	28 January 2019	A Study to Evaluate Efficacy of Ivacaftor in Subjects With Cystic Fibrosis Who Have a 3849 + 10KB C?T or D1152H CFTR Mutation	A Randomized, Double-blind, Placebo-controlled, Crossover Study to Evaluate the Efficacy of Ivacaftor in Subjects With Cystic Fibrosis Who Are 6 Years of Age and Older and Have Either a 3849 + 10KB C?T or D1152H-CFTR Mutation	Cystic Fibrosis	Drug: Ivacaftor;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	N/A	All	38	Phase 3	Israel
133	NCT03251092	July 17, 2017	28 October 2019	Study Designed to Assess the Safety, Tolerability and PK of PTI-808 in Healthy Volunteers and in Adults With Cystic Fibrosis	A Phase 1/2 Study to Evaluate the Safety, Tolerability, and Pharmacokinetics of PTI-808 in Healthy Adult Subjects and in Adults With Cystic Fibrosis	Healthy Volunteer - Complete;Cystic Fibrosis	Drug: PTI-808;Drug: Placebo;Drug: PTI-428;Drug: PTI-801	Proteostasis Therapeutics, Inc.		Recruiting	18 Years	99 Years	All	180	Phase 1/Phase 2	United States;Belgium;Canada;Denmark;France;Germany;New Zealand;United Kingdom
134	EUCTR2016-001214-24-DK	10/07/2017	30 April 2018	A Multi-center, Randomized, Placebo-Controlled Phase I/II Study Designed to Assess the Safety, Tolerability, and how the body breaks down the drug PTI-428 in Subjects with Cystic Fibrosis	A Phase I/II Multi-center, Randomized, Placebo-Controlled, Study Designed to Assess the Safety, Tolerability, and Pharmacokinetics of PTI-428 in Subjects with Cystic Fibrosis - Proteostasis PTI428	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Code: PTI-428 Pharmaceutical Form: Capsule Current Sponsor code: PTI-428 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use Product Code: PTI-428 Pharmaceutical Form: Capsule Current Sponsor code: PTI-428 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 10- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use Product Code: PTI-428 Pharmaceutical Form: Capsule Current Sponsor code: PTI-428 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use Product Code: PTI-428 Pharmaceutical Form: Capsule Current Sponsor code: PTI-428 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use	Proteostasis Therapeutics		Not Recruiting			Female: yes Male: yes	144	Phase 1;Phase 2	France;United States;Czech Republic;Canada;Denmark;Germany;Italy;United Kingdom
135	NCT03450720	June 28, 2017	11 June 2018	Pharmacokinetics of GLPG2737 in Male Subjects With Cystic Fibrosis	Evaluation of the Pharmacokinetics, Safety and Tolerability of a Single Dose of GLPG2737 Administered as Oral Suspension in Male Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: GLPG2737 single dose	Galapagos NV		Not recruiting	18 Years	N/A	Male	6	Phase 1	Belgium
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
136	NCT03173573	June 27, 2017	17 September 2018	A Study to Assess the Safety, Tolerability and PK Profile of FDL176 in Healthy and CF Participants	A Five Part Phase 1 Study to Assess the Safety, Tolerability and Pharmacokinetic (PK) Profile of Single and Repeat Oral Doses of FDL176 in Healthy and Cystic Fibrosis (CF) Participants	Cystic Fibrosis	Drug: FDL176;Drug: Placebo	Flatley Discovery Lab LLC		Not recruiting	18 Years	55 Years	All	109	Phase 1	Australia
137	EUCTR2016-004033-25-ES	19/06/2017	3 July 2017	Randomized clinical trial to assess the effect of nebulizad bicarbonate on bacterial infections in patients with cystic fibrosis	Efect of nebulized bicarbonate on bacterial infections in patients with cystic fibrosis. Randomized clinical trial	Cystic Fibrosis MedDRA version: 20.0 Level: LLT Classification code 10074550 Term: Preventive antimicrobial therapy in cystic fibrosis System Organ Class: 100000004865 MedDRA version: 20.0 Level: LLT Classification code 10011764 Term: Cystic fibrosis NOS System Organ Class: 100000004850 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Sodium bicarbonate Pharmaceutical Form: Solution for injection INN or Proposed INN: Sodium Bicarbonate CAS Number: 144-55-8 Other descriptive name: SODIUM BICARBONATE BP Concentration unit: mol/l mole(s)/litre Concentration type: equal Concentration number: 1- Pharmaceutical form of the placebo: Solution for injection Route of administration of the placebo: Inhalation use Product Name: sodium chloride Pharmaceutical Form: Concentrate for solution for injection INN or Proposed INN: Sodium chloride CAS Number: 7647-14-5 Other descriptive name: SODIUM CHLORIDE Concentration unit: g/ml gram(s)/millilitre Concentration type: equal Concentration number: 0.2- Product Name: sodium chloride Pharmaceutical Form: Concentrate for solution for injection INN or Proposed INN: Sodium chloride CAS Number: 7647-14-5 Other descriptive name: SODIUM CHLORIDE Concentration unit: g/ml gram(s)/millilitre Concentration type: equal Concentration number: 0.2- Product Name: Water for injections Pharmaceutical Form: Solvent for parenteral use INN or Proposed INN: water for injections CAS Number: 7789-20-0 Other descriptive name: WATER FOR INJECTIONS, EP Concentration unit: ml millilitre(s) Concentration type: equal Concentration number: 10-	Fundació Parc Taulí		Authorised			Female: yes Male: yes		Phase 2	Spain
138	NCT03078088	June 15, 2017	27 August 2018	Airway Alkalinization and Nasal Colonization	Airway Alkalinization and Nasal Colonization	Healthy Subjects;Cystic Fibrosis	Drug: Tham;Drug: Saline	Lakshmi Durairaj		Recruiting	16 Years	N/A	All	64	Phase 1	United States
139	NCT03020719	June 14, 2017	27 August 2018	The Role of Oral Glutathione on Growth Parameters in Children With Cystic Fibrosis	A Multi Center Placebo Controlled Double Blind Randomized Study Evaluating the Role of Oral Glutathione on Growth Parameters in Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Oral Glutathione;Drug: Placebo	University of Minnesota - Clinical and Translational Science Institute	Cystic Fibrosis Foundation Therapeutics	Not recruiting	2 Years	11 Years	All	60	Phase 2	United States
140	NCT03066453	June 13, 2017	4 March 2019	Evaluation of Short Antibiotic Combination Courses Followed by Aerosols in Cystic Fibrosis	Evaluation of an Antibiotic Treatment With 14 Days of Intravenous Tobramycin Versus the Same Antibiotic Associated With 5 Days of Intravenous Tobramycin Followed by Tobramycin Aerosol for 9 Days in Cystic Fibrosis.	Cystic Fibrosis	Drug: Tobi Inhalant Product;Drug: Nebcin	University Hospital, Lille		Recruiting	8 Years	N/A	All	97	Phase 3	France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
141	NCT03093974	June 1, 2017	15 October 2018	Long Term Efficacy and Safety of Inhaled Colistimethate Sodium in Bronchiectasis Subjects With Chronic Pseudomonas Aeruginosa Infection.	A Double-blind, Placebo-controlled, Multi-centre, Clinical Trial to Investigate the Efficacy and Safety of 12 Months of Therapy With Inhaled Colistimethate Sodium in the Treatment of Subjects With Non-cystic Fibrosis Bronchiectasis Chronically Infected With Pseudomonas Aeruginosa (P. Aeruginosa)	Non Cystic Fibrosis Bronchiectasis	Drug: Colistimethate Sodium;Drug: Saline Solution	Zambon SpA		Recruiting	18 Years	N/A	All	464	Phase 3	Belgium;Germany;Italy;Portugal;Spain;United Kingdom
142	NCT03056989	May 31, 2017	16 December 2017	A Safety Study of SPX-101 Inhalation Solution in Subjects With Cystic Fibrosis	A Phase 1, Single-Center, Open-Label Safety Study of SPX-101 Inhalation Solution in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: SPX-101	Spyryx Biosciences, Inc.		Not recruiting	18 Years	50 Years	All	5	Phase 1	Canada
143	EUCTR2016-004558-13-FR	30/05/2017	2 October 2017	A double-blind, placebo controlled, multicentre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled Promixin® (colistimethate sodium) in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa)	A double-blind, placebo controlled, multicentre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled Promixin® (colistimethate sodium) in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa) - PROMIS II	Non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa MedDRA version: 19.1 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Promixin, 1 million International Units (IU) Powder for Nebuliser Solution Product Name: Colistimethate sodium Pharmaceutical Form: Powder for nebuliser solution INN or Proposed INN: COLISTIMETHATE SODIUM CAS Number: 8068-28-8 Other descriptive name: Colistimethate Sodium Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 1000000- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Zambon S.p.A.		Authorised			Female: yes Male: yes	264	Phase 3	United States;France;Canada;Poland
144	NCT02950805	May 30, 2017	23 July 2018	A Study to Assess the Effect of AZD5634 on Mucociliary Clearance, Safety, Tolerability and Pharmacokinetic Parameters in Patients With Cystic Fibrosis	A Phase 1b Randomized Blinded Placebo-Controlled, Cross-Over Study to Assess the Effect of AZD5634 on Mucociliary Clearance as Well as Safety, Tolerability, and Pharmacokinetic Parameters Following Single Inhaled Dose Administration to Patients With Cystic Fibrosis.	Pulmonary/Respiratory Diseases	Drug: Placebo;Drug: AZD5634	AstraZeneca	Parexel	Not recruiting	18 Years	60 Years	All	9	Phase 1	United States
145	EUCTR2015-002743-33-DE	29/05/2017	28 February 2019	A clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled colistimethate sodium in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa)	A double-blind, placebo-controlled, multi-centre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled colistimethate sodium in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa) - PROMIS I	Non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa MedDRA version: 20.1 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Promixin/Tadim Product Name: Colistimethate sodium Pharmaceutical Form: Powder for nebuliser solution INN or Proposed INN: COLISTIMETHATE SODIUM CAS Number: 8068-28-8 Other descriptive name: Colistimethate Sodium Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 1000000- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Zambon S.p.A.		Authorised			Female: yes Male: yes	420	Phase 3	Portugal;Greece;Spain;Israel;Switzerland;United Kingdom;Italy;France;Belgium;Australia;Germany;Netherlands;New Zealand
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
146	EUCTR2016-004477-40-GB	26/05/2017	30 April 2019	A study looking at the safety, tolerability and efficacy of the study drug GLPG2222 in patients with cystic fibrosis who have the F508del CFTR mutation on both alleles	A Phase IIa, randomized, double-blind, placebo-controlled study to evaluate multiple doses of GLPG2222 in subjects with Cystic Fibrosis who are homozygous for the F508del mutation	Cystic Fibrosis MedDRA version: 19.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Product Name: GLPG2222 Product Code: G957389 Pharmaceutical Form: Tablet INN or Proposed INN: Not applicable Current Sponsor code: G957389 Other descriptive name: GLPG2222 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: GLPG2222 Product Code: G957389 Pharmaceutical Form: Tablet INN or Proposed INN: Not applicable Current Sponsor code: G957389 Other descriptive name: GLPG2222 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: GLPG2222 Product Code: G957389 Pharmaceutical Form: Tablet INN or Proposed INN: Not applicable Current Sponsor code: G957389 Other descriptive name: GLPG2222 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Galapagos NV		Not Recruiting			Female: yes Male: yes	50	Phase 2	Serbia;United States;Belgium;Spain;Netherlands;United Kingdom
147	NCT03150719	May 24, 2017	30 September 2019	A Study to Evaluate Safety, Efficacy, and Tolerability of TEZ/IVA in Orkambi® (Lumacaftor/Ivacaftor) -Experienced Subjects With Cystic Fibrosis (CF)	Phase 3b, Randomized, Double-blind, Placebo-controlled, Parallel Group Study to Assess the Safety, Efficacy, and Tolerability of Tezacaftor/Ivacaftor (TEZ/IVA) in an Orkambi-experienced Population Who Are Homozygous for the F508del CFTR Mutation	Cystic Fibrosis	Drug: Tezacaftor/Ivacaftor;Drug: Ivacaftor;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	98	Phase 3	United States;France;Germany
148	NCT03125395	May 12, 2017	26 August 2019	A Rollover Safety Study of Lumacaftor/Ivacaftor in Subjects Aged 2 Years and Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	A Phase 3, Rollover Study to Evaluate the Safety of Long-term Treatment With Lumacaftor/Ivacaftor Combination Therapy in Subjects Aged 2 Years and Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: Lumacaftor;Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	2 Years	N/A	All	57	Phase 3	United States;Canada
149	EUCTR2016-004996-33-ES	03/05/2017	3 July 2017	A Pilot Study to Evaluate the Use of Smart Adherence Technology to Measure Lumacaftor/Ivacaftor Adherence in CF Subjects Homozygous for the F508del-CFTR Mutation	A Phase 4, Open-label Treatment, Randomized, Multicenter, 2-arm, Parallelgroup, Pilot Study of Adherence to Lumacaftor/Ivacaftor in CF Subjects Homozygous for the F508del-CFTR Mutation	Cystic fibrosis subjects Homozygous for the F508del-CFTR Mutation MedDRA version: 19.1 Level: SOC Classification code 10010331 Term: Congenital, familial and genetic disorders System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Orkambi Product Name: Orkambi Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Lumacaftor Current Sponsor code: VX-809; VRT-826809; VRT-0826809 Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200 - INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770, VRT-813077 Other descriptive name: IVACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125 -	Vertex Pharmaceuticals Incoporated		Not Recruiting			Female: yes Male: yes	75	Phase 4	United Kingdom;Australia;Spain;Canada;United States
150	NCT03070522	May 1, 2017	15 July 2019	Prednisone in Cystic Fibrosis Pulmonary Exacerbations	Randomized Controlled Trial of Prednisone in Cystic Fibrosis Pulmonary Exacerbations	Cystic Fibrosis Pulmonary Exacerbation	Drug: Prednisone;Drug: Placebos	The Hospital for Sick Children	Canadian Cystic Fibrosis Foundation	Recruiting	N/A	N/A	All	84	Phase 3	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
151	NCT03051490	April 28, 2017	11 June 2018	RESULT: Reliable, Emergent Solution Using Liprotamase Treatment	A Phase 3, Randomized, Open-Label, Assessor-Blind, Non-Inferiority, Active-Comparator Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Exocrine Pancreatic Insufficiency;Cystic Fibrosis	Drug: Liprotamase;Drug: porcine PERT	Anthera Pharmaceuticals		Not recruiting	7 Years	N/A	All	140	Phase 3	United States;Hungary;Israel;Lithuania;Poland;Spain;United Kingdom
152	NCT03140527	April 10, 2017	30 September 2019	Study Assessing the Safety, Tolerability, Pharmacokinetics, Food Effect, and Drug-Drug Interactions of PTI-801 in Healthy Volunteers, and Safety, Tolerability, and Pharmacokinetics of PTI-801 in Subjects With Cystic Fibrosis	A Multi-Center, Randomized, Placebo-Controlled, Phase 1, Two-Part Study Designed to Assess the Safety, Tolerability, Pharmacokinetics, Food Effect, and Drug-Drug Interactions of PTI-801 in Healthy Volunteers, and Safety, Tolerability, and Pharmacokinetics of PTI-801 in Subjects With Cystic Fibrosis	Healthy Volunteer;Cystic Fibrosis	Drug: PTI-801;Drug: Placebo;Drug: PTI-808	Proteostasis Therapeutics, Inc.		Recruiting	18 Years	N/A	All	180	Phase 1	United States;Canada;Denmark;Germany
153	NCT03104855	April 3, 2017	6 May 2019	Clearance of 25-hydroxyvitamin D in Cystic Fibrosis	Clearance of 25-hydroxyvitamin D in Cystic Fibrosis	Cystic Fibrosis	Drug: d6-25-hydroxyvitamin D3	University of Washington	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	Not recruiting	18 Years	N/A	All	10	Phase 1	United States
154	NCT03584841	March 31, 2017	22 July 2019	Implementation of a Non-invasive Version of the Imaging ß-adrenergic-dependent Sweat Secretion Test	Implementation of a Non-invasive Version of the Imaging ß-adrenergic-dependent Sweat Secretion Test: Value for Diagnosis and Efficacy of Target Therapies for Cystic Fibrosis	Mucoviscidosis Involving the Lung	Diagnostic Test: Iontophoresis sessions in forearm of subjects with pilocarpine	Cliniques universitaires Saint-Luc- Université Catholique de Louvain		Recruiting	18 Years	100 Years	All	120	N/A	Belgium
155	EUCTR2016-001619-19-NL	27/03/2017	8 May 2017	Genistein as an add-on treatment for CF?	Genistein as an add-on treatment for CF? -	Cystic Fibrosis ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: Genistein Pharmaceutical Form: Capsule INN or Proposed INN: genistein CAS Number: 446-72-0 Other descriptive name: GENISTEIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 48.5- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use	UMC Utrecht		Authorised			Female: yes Male: yes		Phase 2	Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
156	NCT03119649	March 18, 2017	16 December 2017	A Study to Evaluate Multiple Doses of GLPG2222 in Adult Subjects With Cystic Fibrosis	A Phase IIa, Randomized, Double-blind, Placebo-controlled Study to Evaluate Multiple Doses of GLPG2222 in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del Mutation	Cystic Fibrosis	Drug: GLPG2222 dose 1 for cohort A;Drug: GLPG2222 dose 2 for cohort A;Drug: Placebo 1;Drug: GLPG2222 dose 1 for cohort B;Drug: GLPG2222 dose 2 for cohort B;Drug: Placebo 2	Galapagos NV		Not recruiting	18 Years	99 Years	All	59	Phase 2	United States;Belgium;Netherlands;Serbia;Spain;United Kingdom
157	NCT03256799	March 17, 2017	16 December 2017	Evaluation of Ivacaftor in Patients Using Ataluren for Nonsense Mutations	An Open Label Study to Investigate the Role of Ivacaftor for the Treatment of Cystic Fibrosis in Combination With Ataluren (PTC124) in Cystic Fibrosis Patients Using Ataluren for Nonsense Mutations	Cystic Fibrosis	Drug: Ivacaftor	University of Alabama at Birmingham		Not recruiting	19 Years	N/A	All	1	Phase 4	United States
158	NCT02950116	March 9, 2017	16 December 2017	Lung Clearance Index (LCI) in Pediatric Patients With Obstructive Lung Disease	Lung Clearance Index in Pediatric Patients With Obstructive Lung Disease	Asthma;Bronchiectasis;Cystic Fibrosis	Device: Perform three multiple breath nitrogen washout tests (N2-MBW-test)	Universitair Ziekenhuis Brussel		Not recruiting	6 Years	17 Years	All	27	N/A	Belgium
159	NCT02498535	February 22, 2017	2 September 2019	Efficacy and Safety of Inhaled Nitric Oxide (NO) in Cystic Fibrosis (CF) Patients	Prospective, Randomized, Placebo Controlled Trial of the Efficacy and Safety of Inhaled Nitric Oxide (NO) in Cystic Fibrosis (CF) Patients	Cystic Fibrosis	Drug: Nitric Oxide 160 ppm	Novoteris, LLC	Cystic Fibrosis Foundation;Mallinckrodt	Recruiting	18 Years	N/A	All	60	Phase 2	United States;Canada
160	EUCTR2015-002743-33-GB	21/02/2017	28 February 2019	A double-blind, placebo controlled, multicentre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled colistimethate sodium in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa)	A double-blind, placebo controlled, multi-centre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled colistimethate sodium in the treatment of subjects with non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa (P. aeruginosa) - PROMIS I	Non-cystic fibrosis bronchiectasis chronically infected with Pseudomonas aeruginosa MedDRA version: 20.1 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Promixin/Tadim Product Name: Colistimethate sodium Pharmaceutical Form: Powder for nebuliser solution INN or Proposed INN: COLISTIMETHATE SODIUM CAS Number: 8068-28-8 Other descriptive name: Colistimethate Sodium Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 1000000- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Zambon S.p.A.		Authorised			Female: yes Male: yes	420	Phase 3	Portugal;Greece;Spain;Israel;United Kingdom;Switzerland;Italy;France;Belgium;Australia;Germany;Netherlands;New Zealand
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
161	NCT02888730	February 16, 2017	25 February 2019	Tobramycin Delivered by Nebulized Sonic Aerosol for Chronic Rhinosinusitis Treatment of Cystic Fibrosis Patients	Efficacy of Antibiotic (Tobramycin) Delivered by Nebulized Sonic Aerosol for Chronic Rhinosinusitis Treatment of Cystic Fibrosis Patients: A Multicenter Double-blind Randomized Controlled Trial	Cystic Fibrosis;Rhinosinusitis;Lung Diseases	Drug: Tobramycin nebulized nasally;Drug: Physiologic serum nebulized nasally	Virginie ESCABASSE	Henri Mondor University Hospital	Recruiting	7 Years	N/A	All	86	Phase 3	France
162	NCT02976519	February 15, 2017	17 September 2018	BI 443651 Multiple Rising Dose in Healthy Volunteers Followed by a Cross-over in CF Subjects	A Phase Ib, Multicentre, Double Blind, Randomized, Two-part Study, First Part Multiple Rising Dose and Second Part Two-way Cross-over, to Assess Safety, Tolerability, Efficacy and Pharmacokinetics of BI 443651 Compared to Placebo Via Respimat® in Healthy Volunteers and CF Subjects.	Cystic Fibrosis	Drug: Placebo;Drug: BI 443651	Boehringer Ingelheim		Not recruiting	18 Years	55 Years	All	64	Phase 1	Germany;United Kingdom
163	NCT02919995	February 8, 2017	20 May 2019	A Study of RPL554 in Patients With Cystic Fibrosis	A Phase IIa, Randomised, Double Blind, Placebo Controlled, Three Way Crossover Study to Assess the Pharmacokinetics of RPL554 Administered to Adult Patients With Cystic Fibrosis.	Cystic Fibrosis	Drug: RPL554;Drug: Placebo	Verona Pharma plc	Cystic Fibrosis Trust	Not recruiting	18 Years	N/A	All	10	Phase 2	United Kingdom
164	NCT03256968	January 27, 2017	28 January 2019	PTC Study to Evaluate Ataluren in Combination With Ivacaftor	An Open Label N of 1 Study to Evaluate the Study and Efficacy of Long-Term Treatment With Ivacaftor in Combination With Ataluren (PTC124) in Subjects With Nonsense Mutation Cystic Fibrosis	Cystic Fibrosis	Drug: Ataluren	University of Alabama at Birmingham		Not recruiting	6 Years	N/A	All	1	Phase 4	United States
165	NCT03227471	January 23, 2017	8 April 2019	A Study of VX-445 in Healthy Subjects and Subjects With Cystic Fibrosis	A Phase 1/2 Study of VX-445 in Healthy Subjects and Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: IVA;Drug: TEZ/IVA;Drug: VX-445;Drug: Matched Placebo;Drug: TEZ;Drug: VX-561	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	230	Phase 1/Phase 2	United States;Australia;Belgium;Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
166	EUCTR2015-004986-99-IT	11/01/2017	19 February 2018	A study to identify whether it is safe, if it works, and how much and how often cysteamine should be given to adult patients with Cystic Fibrosis (CF) who are being treated for a worsening of CF associated lung disease.	A Randomized, Double-Blind, Parallel Group, Placebo-Controlled Study Investigating the Optimal Dose Regimen, Efficacy, and Safety of Adding Oral Cysteamine in Adult Patients with Cystic Fibrosis (CF) Being Treated for an Exacerbation of CF-associated Lung Disease.	exacerbation of Cystic Fibrosis MedDRA version: 19.0 Level: PT Classification code 10070608 Term: Infective pulmonary exacerbation of cystic fibrosis System Organ Class: 10021881 - Infections and infestations ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Product Name: cysteamine bitartrate Pharmaceutical Form: Capsule, hard Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	NovaBiotics, Ltd		Authorised			Female: yes Male: yes	120	Phase 2	United States;Netherlands;United Kingdom;Italy
167	EUCTR2016-000454-36-AT	03/01/2017	9 October 2017	A Study Evaluating the Safety and Efficacy of VX-440 Combination Therapy in Subjects With Cystic Fibrosis	A Phase 2, Randomized, Double-blind, Controlled Study to Evaluate the Safety and Efficacy of VX-440 Combination Therapy in Subjects Aged 12 Years and Older With Cystic Fibrosis	Cystic fibrosis MedDRA version: 19.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-440 Pharmaceutical Form: Tablet INN or Proposed INN: VX-440 Other descriptive name: VX-440 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: tezacaftor/ivacaftor 100mg/150mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: tezacaftor Current Sponsor code: VX-661 Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: Tezacaftor Product Code: VX-661 Pharmaceutical Form: Tablet INN or Proposed INN: Tezacaftor (TEZ) Other descriptive name: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco 150 mg film-coated tablets Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	198	Phase 2	United States;Canada;Belgium;Spain;Denmark;Australia;Austria;Netherlands;Germany;Italy;United Kingdom;Sweden
168	NCT02955888	January 3, 2017	12 March 2018	Study of Safety, Tolerability & Efficacy in Cystic Fibrosis Patients With Abnormal Glucose Tolerance	A Phase 2, Double-Blind, Placebo Controlled Study to Evaluate the Safety & Tolerability of PBI-4050 and Its Effects on Pancreatic and Pulmonary Function in Cystic Fibrosis Patients With Abnormal Glucose Tolerance	Cystic Fibrosis	Drug: PBI4050;Drug: Placebo	ProMetic Pharma SMT Limited	ProMetic BioSciences Inc.	Not recruiting	18 Years	N/A	All	11	Phase 2	Canada
169	NCT02730793	January 2017	15 July 2019	Aztreonam Aerosol to Treat Cystic Fibrosis Nasal Disease	Aztreonam Aerosol to Treat Cystic Fibrosis Nasal Disease	Cystic Fibrosis	Drug: Oral Aztreonam;Drug: Nasal Aztreonam;Drug: Nasal Placebo	Virginia Commonwealth University	Eastern Virginia Medical School	Not recruiting	7 Years	100 Years	All	30	Phase 2	United States
170	NCT02894684	January 2017	22 October 2019	Aztreonam for Inhalation Solution (AZLI) for the Treatment of Exacerbations of Cystic Fibrosis	Aztreonam for Inhalation Solution (AZLI) for the Treatment of Exacerbations of Cystic Fibrosis. An Randomised, Crossover Pilot Study of AZLI Plus Intravenous Colistin® Versus Standard Dual Intravenous Therapy	Cystic Fibrosis;Infection;Pseudomonas	Drug: Aztreonam;Drug: Standard Care	Liverpool Heart and Chest Hospital NHS Foundation Trust	University of Liverpool	Not recruiting	16 Years	65 Years	Male	16	Phase 4	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
171	NCT02947126	January 2017	19 November 2018	Multilevel Models of Therapeutic Response in the Lungs	Multilevel Models of Therapeutic Response in the Lungs	Cystic Fibrosis	Drug: Hypertonic Saline;Drug: Isotonic Saline;Drug: Indium-DTPA;Drug: Technetium Sulfur Colloid	University of Pittsburgh	National Heart, Lung, and Blood Institute (NHLBI)	Recruiting	12 Years	N/A	All	62	N/A	United States
172	NCT03045523	January 2017	16 December 2017	A Study to Evaluate GLPG2222 in Ivacaftor-treated Subjects With Cystic Fibrosis	A Phase IIa, Randomized, Double-blind, Placebo-controlled Study to Evaluate GLPG2222 in Ivacaftor-treated Subjects With Cystic Fibrosis Harbouring One F508del CFTR Mutation and a Second Gating (Class III) Mutation	Cystic Fibrosis	Drug: GLPG2222 150 mg q.d.;Drug: GLPG2222 300 mg q.d.;Drug: Placebo	Galapagos NV		Not recruiting	18 Years	99 Years	All	37	Phase 2	Australia;Belgium;Czechia;Germany;Ireland;United Kingdom
173	EUCTR2017-001379-21-DE		30 April 2019	A study to assess the safety and pharmacodynamics of long-term ivacaftor treatment in children less than 24 months of age with cystic fibrosis (a rare hereditary disease that affects the lungs, digestive system and other organs).	A Phase 3, 2-Arm, Open-label Study to Evaluate the Safety and Pharmacodynamics of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have an Approved Ivacaftor-Responsive Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25- Trade Name: Kalydeco Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Trade Name: Kalydeco Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75-	Vertex Pharmaceuticals Incorporated		Not Available			Female: yes Male: yes	75	Phase 3	United States;Canada;Ireland;Germany;United Kingdom
174	EUCTR2017-001379-21-IE		29 January 2018	A study to assess the safety and pharmacodynamics of long-term ivacaftor treatment in children less than 24 months of age with cystic fibrosis (a rare hereditary disease that affects the lungs, digestive system and other organs).	A Phase 3, 2-Arm, Open-label Study to Evaluate the Safety and Pharmacodynamics of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have an Approved Ivacaftor-Responsive Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25- Trade Name: Kalydeco Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Trade Name: Kalydeco Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75-	Vertex Pharmaceuticals Incorporated		Not Available			Female: yes Male: yes	75	Phase 3	United States;Canada;Ireland;Germany;United Kingdom
175	EUCTR2017-003723-29-FR		28 February 2019	A study of safety and efficacy of lenabasum in Cystic Fibrosis	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial to Evaluate Efficacy and Safety of Lenabasum in Cystic Fibrosis - A Phase 2 safety and efficacy study of Lenabasum in Cystic Fibrosis	Cystic Fibrosis (CF) MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Lenabasum CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Name: Lenabasum Product Code: JBT-101 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Lenabasum CAS Number: 137945-48-3 Current Sponsor code: JBT-101 Other descriptive name: resunab, ajulemic acid, anabasum Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 20- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Corbus Pharmaceuticals, Inc.		Not Available			Female: yes Male: yes	415	Phase 2	Serbia;Portugal;United States;Slovakia;Greece;Spain;Réunion;Austria;Russian Federation;Italy;United Kingdom;France;Hungary;Czech Republic;Canada;Poland;Belgium;Romania;Australia;Bulgaria;Netherlands;Germany;Sweden
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
176	EUCTR2016-001585-29-NL	21/12/2016	29 January 2018	Lumacaftor/Ivacaftor Combination Therapy in Subjects With Cystic Fibrosis Who Have an A455E-CFTR Mutation	A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Crossover Study to Evaluate the Efficacy of Lumacaftor/Ivacaftor Combination Therapy in Subjects With Cystic Fibrosis Who Have an A455E-CFTR Mutation	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Orkambi 200 mg/125 mg film-coated tablets Product Name: LUM/IVA fixed-dose combination Pharmaceutical Form: Film-coated tablet INN or Proposed INN: LUMACAFTOR Current Sponsor code: VX-809 Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	20	Phase 2	Netherlands
177	NCT03205904	December 12, 2016	20 August 2018	Nutritional Intervention and Glycemic Improvement in Patients With Pre-diabetic Cystic Fibrosis.	Nutritional Intervention and Glycemic Improvement in Patients With Pre-diabetic Cystic Fibrosis.	Cystic Fibrosis-related Diabetes;Cystic Fibrosis	Dietary Supplement: Diet	Hospital de Clinicas de Porto Alegre		Recruiting	10 Years	N/A	All	38	N/A	Brazil
178	EUCTR2015-004263-36-GB	07/12/2016	4 September 2017	Study to assess the effects of inhaled RPL554 in adults with cystic fibrosis.	A Phase IIa, randomised, double blind, placebo controlled, three way crossover study to assess the pharmacokinetics of RPL554 administered to adult patients with Cystic Fibrosis. - To assess the effects of inhaled RPL554 in adults with cystic fibrosis	Cystic Fibrosis (CF) MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Product Name: RPL554 Product Code: RPL554 Pharmaceutical Form: Nebuliser suspension INN or Proposed INN: RPL554 CAS Number: 298680-25-8 Current Sponsor code: RPL554 Other descriptive name: RPL554 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: range Concentration number: 0.5-6 Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Verona Pharma plc		Authorised			Female: yes Male: yes	10	Phase 2	United Kingdom
179	EUCTR2016-002837-31-GB	05/12/2016	28 February 2019	A study looking at the safety and tolerability of the drug GLPG2222 in patients with cystic fibrosis with the F508del CFTR mutation and a second gating (class III) mutation	A phase IIa, randomized, double-blind, placebo-controlled study to evaluate GLPG2222 in ivacaftor-treated subjects with Cystic Fibrosis harbouring one F508del CFTR mutation and a second gating (class III) mutation	Cystic Fibrosis MedDRA version: 19.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Product Name: GLPG2222 Product Code: G957389 Pharmaceutical Form: Oral suspension INN or Proposed INN: Not Applicable Current Sponsor code: G957389 Other descriptive name: GLPG2222 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Oral suspension Route of administration of the placebo: Oral use Product Name: GLPG2222 Product Code: G957389 Pharmaceutical Form: Oral suspension INN or Proposed INN: Not Applicable Current Sponsor code: G957389 Other descriptive name: GLPG2222 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Oral suspension Route of administration of the placebo: Oral use	Galapagos NV		Not Recruiting			Female: yes Male: yes	35	Phase 2	Czech Republic;Belgium;Ireland;Australia;Germany;United Kingdom
180	EUCTR2016-002837-31-IE	02/12/2016	20 August 2018	A study looking at the safety and tolerability of the drug GLPG2222 in patients with cystic fibrosis with the F508del CFTR mutation and a second gating (class III) mutation	A phase IIa, randomized, double-blind, placebo-controlled study to evaluate GLPG2222 in ivacaftor-treated subjects with Cystic Fibrosis harbouring one F508del CFTR mutation and a second gating (class III) mutation	Cystic Fibrosis MedDRA version: 19.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Product Name: GLPG2222 Product Code: G957389 Pharmaceutical Form: Oral suspension INN or Proposed INN: Not Applicable Current Sponsor code: G957389 Other descriptive name: GLPG2222 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Oral suspension Route of administration of the placebo: Oral use Product Name: GLPG2222 Product Code: G957389 Pharmaceutical Form: Oral suspension INN or Proposed INN: Not Applicable Current Sponsor code: G957389 Other descriptive name: GLPG2222 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Oral suspension Route of administration of the placebo: Oral use	Galapagos NV		Authorised			Female: yes Male: yes	35	Phase 2	Czech Republic;Belgium;Ireland;Australia;Germany;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
181	NCT02971839	December 2016	27 August 2018	Study to Evaluate the Safety and Efficacy of CTP-656 in Patients With Cystic Fibrosis With CFTR Gating Mutations	A Phase 2, Randomized, Parallel-Group, Double-Blind, Placebo Controlled Study to Evaluate the Safety and Efficacy of CTP-656 With an Open-Label Active Comparator in Patients With Cystic Fibrosis With CFTR Gating Mutations.	Cystic Fibrosis	Drug: CTP-656, 20 mg (QD);Drug: CTP-656, 100 mg (QD);Drug: CTP-656, 150 mg (QD);Drug: Placebo (QD);Drug: Kalydeco, 150 mg Tablet (BID; open-label)	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	11	Phase 2	United States
182	NCT03000348	December 2016	9 July 2018	A Study of the Dosing, Efficacy, and Safety of Oral Cysteamine in Adult Patients With Cystic Fibrosis Exacerbations	A Randomized, Double-Blind, Parallel Group, Placebo-Controlled Study Investigating the Optimal Dose Regimen, Efficacy, and Safety of Adding Oral Cysteamine in Adult Patients Being Treated for an Exacerbation of CF-associated Lung Disease	Cystic Fibrosis	Drug: Cysteamine;Drug: Placebo Oral Capsule	NovaBiotics Ltd.	Agility Clinical, Inc.;PSR Group B.V.	Not recruiting	18 Years	N/A	All	91	Phase 2	United States;Italy;United Kingdom
183	NCT02759562	November 4, 2016	20 August 2018	Effect of Andecaliximab on FEV1 in Adults With Cystic Fibrosis	A Phase 2b, Dose-Ranging Study of the Effect of GS-5745 on FEV1 in Adult Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: Andecaliximab;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	N/A	All	6	Phase 2	Australia;France;Germany;Spain;United Kingdom;Belgium;Canada;Ireland;United States
184	NCT02934698	November 1, 2016	16 December 2017	An Efficacy and Safety Study of Ivacaftor in Patients With Cystic Fibrosis and Two Splicing Mutations	An Efficacy and Safety Study of Ivacaftor in Patients With Cystic Fibrosis and Two Splicing Mutations	Cystic Fibrosis	Drug: Ivacaftor	Medical University of South Carolina	Vertex Pharmaceuticals Incorporated	Not recruiting	18 Years	N/A	Female	2	Phase 3
185	NCT02951195	November 2016	21 January 2019	A Study Evaluating the Safety of VX-152 Combination Therapy in Adults With Cystic Fibrosis	A Phase 2, Randomized, Double Blind, Controlled Study to Evaluate the Safety of VX-152 Combination Therapy in Adults With Cystic Fibrosis	Cystic Fibrosis	Drug: VX-152;Drug: Tezacaftor;Drug: Ivacaftor;Drug: Triple Placebo;Drug: Placebo for VX-152	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	80	Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
186	NCT03029455	November 2016	16 December 2017	A Study to Evaluate Safety and Pharmacokinetics of VX-659 in Healthy Subjects and in Adults With Cystic Fibrosis	A Phase 1, Randomized, Double Blind, Placebo Controlled, Dose Escalation, and Bioavailability Study Evaluating the Safety and Pharmacokinetics of VX-659 in Healthy Subjects and in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: VX-659;Drug: Tezacaftor;Drug: Ivacaftor;Drug: VX-659 Matching Placebo;Drug: Triple Combination (TC) Matching Placebos	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	163	Phase 1	United Kingdom
187	NCT03056326	November 2016	16 December 2017	A Study to Investigate Safety, Tolerability and Pharmacokinetics of Single and Repeat Doses of CHF6333 in Healthy Subjects	A Phase I, Randomised, Double-blind, Placebo-controlled Study to Investigate the Safety, Tolerability and Pharmacokinetics of Inhaled CHF 6333 After Single and Repeated Ascending Doses in Healthy Male Subjects	Non-Cystic Fibrosis Bronchiectasis;Cystic Fibrosis	Drug: CHF6333 (Part 1 - SAD);Drug: Placebo (Part 1 - SAD);Drug: CHF6333 (Part 2 - MAD);Drug: Placebo (Part 2 - MAD)	Chiesi Farmaceutici S.p.A.		Not recruiting	18 Years	55 Years	Male	72	Phase 1	Belgium
188	EUCTR2015-004986-99-GB	31/10/2016	3 April 2017	A study to identify whether it is safe, if it works, and how much and how often cysteamine should be given to adult patients with Cystic Fibrosis (CF) who are being treated for a worsening of CF associated lung disease.	A Randomized, Double-Blind, Parallel Group, Placebo-Controlled Study Investigating the Optimal Dose Regimen, Efficacy, and Safety of Adding Oral Cysteamine in Adult Patients with Cystic Fibrosis (CF) Being Treated for an Exacerbation of CF-associated Lung Disease.	exacerbation of Cystic Fibrosis;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Product Name: cysteamine bitartrate Pharmaceutical Form: Capsule, hard Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	NovaBiotics, Ltd		Authorised			Female: yes Male: yes	120	Phase 2	United States;Netherlands;Italy;United Kingdom
189	NCT02677701	October 21, 2016	11 June 2019	Testing the Effect of Adding Chronic Oral Azithromycin to Inhaled Tobramycin in People With CF	TEACH Trial: Testing the Effect of Adding CHronic Azithromycin to Inhaled Tobramycin. A Randomized, Placebo-controlled, Double-blinded Trial of Azithromycin 500mg Thrice Weekly in Combination With Inhaled Tobramycin	Cystic Fibrosis	Drug: azithromycin;Drug: placebo (for azithromycin);Drug: inhaled tobramycin	Seattle Children's Hospital	National Heart, Lung, and Blood Institute (NHLBI);Cystic Fibrosis Foundation;CF Therapeutics Development Network Coordinating Center	Recruiting	12 Years	N/A	All	140	Phase 4	United States
190	NCT02912637	October 2016	11 February 2019	Hyperpolarized Xenon Imaging in Patients With Cystic Fibrosis	Hyperpolarised 129Xe MRI for Non-invasive Assessment of Ventilation, Perfusion and the Alveolar Membrane - a Physiological Study in Healthy Volunteers & Cystic Fibrosis Patients	Cystic Fibrosis	Other: Hyperpolarized Xenon MRI	Papworth Hospital NHS Foundation Trust	University of Sheffield	Not recruiting	18 Years	70 Years	All	30	Phase 3	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
191	NCT02934139	October 2016	16 December 2017	MAD Study Evaluating the Safety, Tolerability, and Pharmacokinetics of Cavosonstat (N91115) in Healthy Subjects (SNO-9)	A Phase 1, Randomized, Double-Blind, Placebo-Controlled, Multiple Ascending Dose Study to Evaluate the Safety, Tolerability, and Pharmacokinetics of N91115 in Healthy Subjects	Cystic Fibrosis	Drug: Cavosonstat;Other: Placebo	Nivalis Therapeutics, Inc.		Not recruiting	18 Years	55 Years	All	32	Phase 1	United States
192	NCT02951182	October 2016	27 August 2018	A Study Evaluating the Safety and Efficacy of VX-440 Combination Therapy in Subjects With Cystic Fibrosis	A Phase 2, Randomized, Double-blind, Controlled Study to Evaluate the Safety and Efficacy of VX-440 Combination Therapy in Subjects Aged 12 Years and Older With Cystic Fibrosis	Cystic Fibrosis	Drug: Tezacaftor;Drug: Ivacaftor;Drug: VX-440;Drug: Matched Placebos	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	74	Phase 2	United States;Australia;Austria;Belgium;Canada;Denmark;Germany;Italy;Spain;United Kingdom
193	NCT02953314	October 2016	15 October 2018	A Study to Evaluate the Pharmacokinetics, Safety, and Tolerability of VX-661/Ivacaftor in Pediatric Subjects With Cystic Fibrosis	A Phase 3, Open Label Study to Evaluate the Pharmacokinetics, Safety, and Tolerability of VX-661 in Combination With Ivacaftor in Subjects 6 Through 11 Years of Age With Cystic Fibrosis, Homozygous or Heterozygous for the F508del CFTR Mutation	Cystic Fibrosis	Drug: VX-661;Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	11 Years	All	70	Phase 3	United States;Canada
194	EUCTR2015-003040-39-ES	29/09/2016	17 October 2016	Dose-finding Study to Assess the Efficacy, Safety and Tolerability of Tobramycin Inhalation Powder in Patients With Non-Cystic Fibrosis Bronchiectasis and Pulmonary P. Aeruginosa Infection	A randomized, blinded, parallel group, multi-center dose-finding study, to assess the efficacy, safety and tolerability of different doses of tobramycin inhalation powder in patients with Non-Cystic Fibrosis Bronchiectasis and pulmonary P. aeruginosa infection	Pseudomonas aeruginosa infection in patients with non-cystic fibrosis bronchiectasis MedDRA version: 19.0 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders MedDRA version: 19.0 Level: PT Classification code 10070295 Term: Infective exacerbation of bronchiectasis System Organ Class: 10021881 - Infections and infestations ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: TOBI Podhaler Product Name: Tobramycin inhalation powder Product Code: TBM100 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Novartis Pharma Services AG		Authorised			Female: yes Male: yes	180	Phase 2	France;Belgium;Spain;Ireland;Netherlands;Germany;Italy;United Kingdom;Switzerland
195	EUCTR2016-002578-11-GB	16/09/2016	3 April 2017	A trial to determine the safest and most effective antibiotic strategy for infants diagnosed with CF.	The cystic fibrosis (CF) anti-staphylococcal antibiotic prophylaxis trial (CF START); a randomised registry trial to assess the safety and efficacy of flucloxacillin as a longterm prophylaxis agent for infants with CF. - CF START	Cystic fibrosis MedDRA version: 19.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Flucloxacillin 250mg/5ml Sugar-Free Powder for Oral Solution Product Name: Flucloxacillin Product Code: na Pharmaceutical Form: Powder for oral solution INN or Proposed INN: Flucloxacillin CAS Number: 5250-39-5 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: range Concentration number: 25-50-mg/ml	Alder Hey Children's NHS Foundation Trust		Authorised			Female: yes Male: yes	480	Phase 4	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
196	NCT02730208	September 2016	4 November 2019	A Study to Evaluate the Effect of VX-661 in Combination With Ivacaftor on Chest Imaging Endpoints in Subjects With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	A Phase 2, Randomized, Placebo-Controlled, Double-blind Study to Evaluate the Effect of VX-661 in Combination With Ivacaftor on Chest Imaging Endpoints in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic Fibrosis	Drug: Tezacaftor/Ivacaftor;Drug: Ivacaftor;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	41	Phase 2	Australia
197	NCT02823964	September 2016	11 June 2018	EASY: Extended Access to Sollpura Over Years	An Open-Label Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	Exocrine Pancreatic Insufficiency;Cystic Fibrosis	Drug: Liprotamase	Anthera Pharmaceuticals		Not recruiting	7 Years	N/A	All	25	Phase 4	United States;Czechia;Hungary;Israel;Poland;Spain;Czech Republic
198	NCT02875366	September 2016	16 December 2017	A Study of the Effects of Lumacaftor/Ivacaftor on Exercise Tolerance in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	A Phase 4, Randomized, Double-Blind, Placebo-Controlled, Parallel-Design Study of the Effect of Lumacaftor/Ivacaftor Combination Therapy on Exercise Tolerance in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: LUM/IVA;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	70	Phase 4	Australia;United Kingdom
199	EUCTR2016-001785-29-IE	15/08/2016	29 August 2016	Study of the effects of combined treatment with the CFTR corrector ivacaftor and IV antibiotics on infection in CF.	Combined Effect of CFTR Modifiers and Intensive Antibiotic Treatment	Cystic fibrosis;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Kalydeco Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: range Concentration number: 300-300 Trade Name: Ciprofloxacin Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Ciprofloxacin CAS Number: 86393-32-0 Other descriptive name: CIPROFLOXACIN HYDROCHLORIDE MONOHYDRATE Concentration unit: mg milligram(s) Concentration type: range Concentration number: 1500-1500 Product Name: Flucloxicillin Pharmaceutical Form: Powder for injection INN or Proposed INN: FLUCLOXACILLIN SODIUM CAS Number: 1847-24-1 Concentration unit: mg milligram(s) Concentration type: range Concentration number: 4000-8000 Trade Name: Fortum Pharmaceutical Form: INN or Proposed INN: CEFTAZIDIME CAS Number: 72558-82-8 Concentration unit: g gram(s) Concentration type: range Concentration number: 4-8 Product Name: Tobramycin Pharmaceutical Form: Powder and solvent for solution for injection INN or Proposed INN: TOBRAMYCIN SULFATE CAS Number: 79645-27-5 Concentration unit: mg/kg milligram(s)/kilogram Concentration type: range Concentration number: 10-20 Trade Name: Meropenem Pharmaceutical Form: INN or Proposed INN: Meropenem CAS Number: 96036-03-2 Other descriptive name: MEROPENEM Concentration unit: g gram(s) Concentration type: range Concentration number: 3-8 Trade Name: Colistin Pharmaceutical Form: INN or Proposed INN: COLISTIMETHATE SODIUM CAS Number: 8068-28-8 Concentration unit: Munit million units Concentration type: range Concentration number: 2-8	St. Vincent's University Hospital		Authorised			Female: yes Male: yes		Phase 4	Ireland
200	EUCTR2016-000166-35-NL	08/08/2016	22 August 2016	Inhaled nebulized tobramycin in non-CF bronchiectasis	Effects of long term ToBrAmycin InhalaTion SoluTion (TIS) once daiLy on Exacerbation rate in patients with non-cystic fibrosis bronchiectasis. A double blind, randomized, placebo and TIS twice daily (open label) controlled trial. The BATTLE study.	Patients with non-cystic fibrosis bronchiectasis MedDRA version: 19.0 Level: PT Classification code 10070295 Term: Infective exacerbation of bronchiectasis System Organ Class: 10021881 - Infections and infestations ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Tobramycin Steri-Neb 300 mg/5 ml, inhalation solution Pharmaceutical Form: Inhalation solution Pharmaceutical form of the placebo: Inhalation solution Route of administration of the placebo: Inhalation use	Noordwest Ziekenhuisgroep		Authorised			Female: yes Male: yes		Phase 2;Phase 3	Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
201	NCT02858843	August 1, 2016	11 June 2018	The Effect of Combination Lumacaftor and Ivacaftor on Markers of Hyperglycemia	A Study of the Effect of Combination Lumacaftor and Ivacaftor on Markers of Hyperglycemia in Persons With Cystic Fibrosis	Cystic Fibrosis;Diabetes	Drug: lumacaftor-ivacaftor	Massachusetts General Hospital		Not recruiting	18 Years	65 Years	All	1	N/A
202	NCT02657473	August 2016	16 December 2017	Inhaled Nebulized Tobramycin in Non-cystic Fibrosis Bronchiectasis	Long-term Inhaled Nebulized Tobramycin in Patients With Non-cystic Fibrosis Bronchiectasis. A Randomized Placebo Controlled Trial. The BATTLE Study Bronchiectasis And Tobramycin SoluTion InhaLation ThErapy.	Non-CF Bronchiectasis	Drug: tobramycin inhalation solution;Drug: Saline 0.9% inhalation solution	Medical Center Alkmaar		Recruiting	18 Years	N/A	All	52	Phase 2/Phase 3	Netherlands
203	NCT02866721	August 2016	25 February 2019	Safety and Tolerability Study of Allogeneic Mesenchymal Stem Cell Infusion in Adults With Cystic Fibrosis	A Phase I, Single Center, Open Label, Single Dose, Dose Escalation Study Assessing the Safety and Tolerability of AllogeneiC MEsenchymAl Stem CEll Infusion in Adults With Cystic Fibrosis-CEASE CF	Cystic Fibrosis	Biological: Mesenchymal Stem Cells	Erica Roesch	Case Western Reserve University;Cystic Fibrosis Foundation	Recruiting	18 Years	N/A	All	15	Phase 1	United States
204	NCT02918409	August 2016	25 March 2019	IV Colistin for Pulmonary Exacerbations: Improving Safety and Efficacy	IV Colistin for Pulmonary Exacerbations: Improving Safety and Efficacy	Cystic Fibrosis	Drug: Colistin;Drug: Tobramycin	National Jewish Health		Recruiting	18 Years	N/A	All	270	Phase 4	United States
205	NCT02950883	August 2016	16 September 2019	Saline Hypertonic in Preschoolers + CT	Saline Hypertonic in Preschoolers With Cystic Fibrosis and Lung Structure as Measured by Computed Tomography (CT)	Cystic Fibrosis	Drug: Control Group 0.9% Isotonic Saline;Drug: Active Treatment Group 7% Hypertonic Saline	University of Washington, the Collaborative Health Studies Coordinating Center	Cystic Fibrosis Foundation	Recruiting	3 Years	5 Years	All	120	Phase 2/Phase 3	United States;Australia;Belgium;Canada;Denmark;France;Italy;Netherlands;Spain
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
206	NCT02750501	July 20, 2016	20 August 2018	Absorption and Safety With Sustained Use of RELiZORB Evaluation (ASSURE) Study	Absorption and Safety With Sustained Use of RELiZORB Evaluation (ASSURE) Study in Patients With Cystic Fibrosis Receiving Enteral Feeding	Cystic Fibrosis	Device: RELiZORB (immobilized lipase) cartridge;Other: Impact Peptide 1.5	Alcresta Therapeutics, Inc.	Cystic Fibrosis Foundation Therapeutics	Not recruiting	4 Years	N/A	All	49	N/A	United States
207	NCT03486236	July 20, 2016	11 June 2018	A Study Evaluating Safety and Pharmacokinetics of VX-440 in Combination With Tezacaftor/Ivacaftor in Healthy Adult Subjects	A Phase 1, Randomized, Double Blind, Placebo Controlled, Multiple Dose Escalation Study Evaluating Safety and Pharmacokinetics of VX-440 in Combination With VX-661/Ivacaftor in Healthy Adult Subjects	Cystic Fibrosis	Drug: VX-440;Drug: TEZ;Drug: IVA;Drug: Matched Placebos	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	All	16	Phase 1	United Kingdom
208	NCT02718495	July 19, 2016	1 April 2019	Study Assessing PTI-428 Safety, Tolerability, and Pharmacokinetics in Subjects With Cystic Fibrosis	A Phase I/II, Multi-center, Randomized, Placebo-Controlled, Study Designed to Assess the Safety, Tolerability, and Pharmacokinetics of PTI-428 in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: PTI-428;Drug: Placebo	Proteostasis Therapeutics, Inc.		Not recruiting	18 Years	N/A	All	56	Phase 1/Phase 2	United States;Canada;Denmark;France;Germany;Czechia;United Kingdom
209	NCT02555059	July 15, 2016	28 January 2019	Special Drug Use Investigation of Ciproxan Injection in Pediatrics	Special Drug Use Investigation of Ciproxan® Injection in Pediatrics	Cystitis / Pyelonephritis / Cystic Fibrosis / Anthrax	Drug: Cipro (Ciprofloxacin, BAYQ3939)	Bayer		Not recruiting	N/A	14 Years	All	48	Phase 4	Japan
210	NCT02992080	July 12, 2016	25 February 2019	Small Circulating RNA as Molecular Markers of Lung Disease in Cystic Fibrosis	Determination of Circulating miRNAs as Diagnostic Markers of Lung Disease in Cystic Fibrosis	Cystic Fibrosis	Biological: miRNAs isolation from blood samples of patients and control	University Hospital, Montpellier		Recruiting	12 Months	65 Years	All	80	N/A	France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
211	NCT02810691	July 2016	20 August 2018	The Effect of Soluble Fiber to Reduce Post-prandial Glycemic Excursion in Adults With Cystic Fibrosis	The Effect of Soluble Fiber to Reduce Post-prandial Glycemic Excursion in Adults With Cystic Fibrosis	Cystic Fibrosis-related Diabetes;Cystic Fibrosis	Dietary Supplement: Soluble fiber supplementation;Dietary Supplement: Placebo (for fiber supplementation)	Institut de Recherches Cliniques de Montreal	Canadian Cystic Fibrosis Foundation;Université de Montréal	Not recruiting	18 Years	N/A	All	14	N/A	Canada
212	NCT02807415	June 1, 2016	11 March 2019	ICM to Evaluate the Activation of p.Phe508del-CFTR by Lumacaftor in Combination With Ivacaftor	Intestinal Current Measurements (ICM) to Evaluate the Activation of Mutant CFTR in Subjects With Cystic Fibrosis Aged 12 Years and Older, Homozygous for the p.Phe508del-CFTR Mutation, Treated With Lumacaftor in Combination With Ivacaftor	Cystic Fibrosis	Drug: Lumacaftor plus Ivacaftor	Hannover Medical School	Heidelberg University;University of Giessen	Recruiting	6 Years	N/A	All	100	N/A	Germany
213	NCT02734810	June 2016	11 June 2018	SIMPLICITY: Studying Impacts on Malabsorption With Liprotamase in Cystic Fibrosis	A Phase 3, Open-Label Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Exocrine Pancreatic Insufficiency;Cystic Fibrosis	Drug: Liprotamase Powder for Oral Solution	Anthera Pharmaceuticals		Not recruiting	N/A	N/A	All	15	Phase 3	United States
214	NCT02781610	June 2016	17 June 2019	Standardized Treatment of Pulmonary Exacerbations II	Standardized Treatment of Pulmonary Exacerbations II (STOP2)	Pulmonary Cystic Fibrosis	Drug: Standard of care IV antibiotic(s)	Chris Goss	Cystic Fibrosis Foundation;CF Therapeutics Development Network Coordinating Center;Medical University of South Carolina;University of Washington	Recruiting	18 Years	N/A	All	1300	Phase 4	United States;Canada
215	NCT02823470	June 2016	16 December 2017	A Pilot Study to Evaluate the Use of Smart Adherence Technology to Measure Lumacaftor/Ivacaftor Adherence in CF Subjects Homozygous for the F508del CFTR Mutation	A Phase 4, Open-label Treatment, Randomized, Multicenter, 2-arm, Parallel-group, Pilot Study of Adherence to Lumacaftor/Ivacaftor in CF Subjects Homozygous for the F508del CFTR Mutation	Cystic Fibrosis	Drug: LUM/IVA;Device: activated smart device;Device: de-activated smart device	Vertex Pharmaceuticals Incorporated		Not recruiting	16 Years	N/A	All	24	Phase 4	United States;Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
216	EUCTR2016-000354-35-DK	12/05/2016	13 March 2017	High dose antioxidant treatment of patients with cystic fibrosis evaluated by measuring desmosine/isodesmosine in urine	High dose antioxidant treatment of patients with cystic fibrosis evaluated by measuring desmosine/isodesmosine in urine	cystic fibrosis MedDRA version: 19.0 Level: LLT Classification code 10068288 Term: Cystic fibrosis pulmonary exacerbation System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Mucolysin Pharmaceutical Form: Effervescent tablet INN or Proposed INN: ACETYLCYSTEINE SODIUM Concentration unit: mg milligram(s) Concentration type: range Concentration number: 200-6oo Trade Name: AAC Hexal Pharmaceutical Form: Tablet INN or Proposed INN: ACETYLCYSTEINE SODIUM Concentration unit: mg milligram(s) Concentration type: range Concentration number: 200-6oo	Aarhus University Hospital		Not Recruiting			Female: yes Male: yes		Phase 2	Denmark
217	NCT02722122	May 2016	10 October 2016	Study to Evaluate the Safety,Tolerability, Pharmacokinetics and Exploratory Efficacy Parameters of AIR DNase™in Patients With Cystic Fibrosis Previously Treated With Pulmozyme®	A Proof-of-Concept, Open Label Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Exploratory Efficacy Parameters of AIR DNase™in Patients With Cystic Fibrosis Previously Treated With Pulmozyme®	Cystic Fibrosis	Drug: AIR DNase™	Protalix		Recruiting	12 Years	N/A	Both	15	Phase 2	Israel
218	NCT02797132	May 2016	16 December 2017	Safety and Pharmacokinetic Study of Lumacaftor/Ivacaftor in Subjects Aged 2 Through 5 Years With Cystic Fibrosis, Homozygous for F508del	A Phase 3, 2-Part, Open-label Study to Evaluate the Safety and Pharmacokinetics of Lumacaftor/Ivacaftor Combination Therapy in Subjects Aged 2 Through 5 Years With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic Fibrosis	Drug: lumacaftor/ ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	2 Years	5 Years	All	60	Phase 3	United States;Canada
219	NCT02850692	April 18, 2016	26 August 2019	Portal Hypertension and Systemic Endothelial Function	Portal Hypertension and Systemic Endothelial Function: Investigation of Systemic Endothelial Dysfunction in Case of Portal Hypertension Associated With Cystic Fibrosis.	Cystic Fibrosis;Portal Hypertension	Other: measure of endothelial function;Biological: Blood sample;Other: Hepatic elastography;Diagnostic Test: Injected abdominal CT	Hopital Foch		Recruiting	18 Years	N/A	All	60	N/A	France
220	NCT02724527	April 2016	28 November 2016	Study of Cavosonstat (N91115) in CF Patients Who Are Heterozygous for F508del-CFTR and a Gating Mutation and Being Treated With Ivacaftor	A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study of N91115 for Efficacy and Safety in Patients With CF Heterozygous for F508del-CFTR + Gating Mutation Being Treated With Ivacaftor	Cystic Fibrosis	Drug: Cavosonstat;Drug: Placebo	Nivalis Therapeutics, Inc.		Not recruiting	18 Years	N/A	Both	19	Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
221	NCT02767297	April 2016	31 October 2016	Bioavailability and Pharmacokinetics Study of FDL169 in Healthy Subjects and Subjects With Cystic Fibrosis	A Three-Part Phase 1b Bioavailability and Pharmacokinetics Study of Two Formulations of FDL169 in Healthy Subjects and Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: FDL169	Flatley Discovery Lab LLC		Not recruiting	18 Years	N/A	Both	46	Phase 1/Phase 2	United Kingdom
222	EUCTR2016-000398-19-BE	08/03/2016	30 April 2018	Pilot study: pharmacokinetics of beta-lactam antibiotics, piperacillin-tazobactam, ceftazidim and meropenem in blood and sputum of cystic fibrosis patients.	Pilot study: pharmacokinetics of beta-lactam antibiotics, piperacillin-tazobactam, ceftazidim and meropenem in blood and sputum of cystic fibrosis patients. - pharmacokinetics of beta-lactam antibiotics, piperacillin-tazobactam, ceftazidim and meropenem	Cystic fibrosis;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Meropenem Fresenius Kabi 1 g poeder voor oplossing voor injectie of infusie Pharmaceutical Form: Powder for solution for injection/infusion INN or Proposed INN: Meropenem Fresenius Kabi Other descriptive name: MEROPENEM TRIHYDRATE Concentration unit: g gram(s) Concentration type: equal Concentration number: 1- Trade Name: Ceftazidim Fresenius Kabi 2000 mg poeder voor oplossing voor injectie of infusie Pharmaceutical Form: Powder for solution for injection/infusion INN or Proposed INN: Ceftazidim Fresenius Kabi Other descriptive name: CEFTAZIDIME PENTAHYDRATE Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 2000- Trade Name: Piperacilline/Tazobactam Fresenius Kabi 2 g/0,25 g poeder voor oplossing voor infusie Pharmaceutical Form: Powder for solution for injection/infusion INN or Proposed INN: Piperacilline/Tazobactam Fresenius Kabi 2 g/0,25 g poeder voor oplossing voor infusie Other descriptive name: PIPERACILLIN SODIUM Concentration unit: g gram(s) Concentration type: equal Concentration number: 2- INN or Proposed INN: Piperacilline/Tazobactam Fresenius Kabi 2 g/0,25 g poeder voor oplossing voor infusie Other descriptive name: TAZOBACTAM SODIUM Concentration unit: g gram(s) Concentration type: equal Concentration number: 0.25- Trade Name: Piperacilline/Tazobactam Fresenius Kabi 4 g/0,5 g poeder voor oplossing voor infusie Pharmaceutical Form: Powder for solution for injection/infusion INN or Proposed INN: Piperacilline/Tazobactam Fresenius Kabi 4 g/0,5 g poeder voor oplossing voor infusie Other descriptive name: PIPERACILLIN SODIUM Concentration unit: g gram(s) Concentration type: equal Concentration number: 4- INN or Proposed INN: Piperacilline/Tazobactam Fresenius Kabi 4 g/0,5 g poeder voor oplossing voor infusie Other descriptive name: TAZOBACTAM SODIUM Concentration unit: g gram(s) Concentration type: equal Concentration number: 0.5-	Ghent University		Not Recruiting			Female: yes Male: yes	45	Phase 4	Belgium
223	NCT02354859	March 2016	1 April 2019	A Phase 2 IV Gallium Study for Patients With Cystic Fibrosis (IGNITE Study)	A Phase 2, Multi-Center, Randomized, Placebo-Controlled Study of IV Gallium Nitrate in Patients With Cystic Fibrosis (IGNITE Study)	Cystic Fibrosis	Drug: Gallium nitrate;Drug: Normal Saline	University of Washington	Cystic Fibrosis Foundation	Not recruiting	18 Years	N/A	All	119	Phase 2	United States
224	NCT02725567	March 2016	28 January 2019	A Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age and Have a CFTR Gating Mutation	A Phase 3, 2 Part, Open-Label Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age and Have a CFTR Gating Mutation	Cystic Fibrosis	Drug: ivacaftor	Vertex Pharmaceuticals Incorporated		Recruiting	N/A	24 Months	All	35	Phase 3	United States;Australia;Canada;Ireland;United Kingdom
225	NCT02848560	March 2016	18 June 2018	Monitoring Response to Orkambi in Cystic Fibrosis Lung Disease by Inhaled Xenon MRI	Validation of MRI as a Sensitive Tool to Longitudinally Monitor CF Lung Disease Progression and Response to CFTR Modulator Therapy in Young Children With CF	Cystic Fibrosis	Drug: Hyperpolarized Xenon	Children's Hospital Medical Center, Cincinnati	National Heart, Lung, and Blood Institute (NHLBI)	Recruiting	6 Years	12 Years	All	38	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
226	NCT02649751	February 22, 2016	18 December 2018	Evaluation of (R)-Roscovitine Safety and Effects in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	A Phase II, Dose Ranging, Multicenter, Double-blind, Placebo Controlled Study to Evaluate Safety and Effects of (R)-Roscovitine in Adults Subjects With Cystic Fibrosis, Carrying 2 Cystic Fibrosis Causing Mutations With at Least One F508del-CFTR Mutation and Chronically Infected With Pseudomonas Aeruginosa, a Study Involving 36 CF Patients (24 Treated, 12 Controls). ROSCO-CF.	Cystic Fibrosis	Drug: Roscovitine;Drug: Placebo	University Hospital, Brest	ManRos Therapeutics;Cyclacel Pharmaceuticals, Inc.	Not recruiting	18 Years	N/A	All	49	Phase 2	France
227	NCT02679729	February 11, 2016	16 December 2017	To Assess the Safety, Tolerability and Pharmacokinetics of AZD5634 Following Inhaled and Intravenous (IV)Dose Administration	A Phase I, Randomized, Single-Blind, Placebo-Controlled Study to Assess the Safety, Tolerability and Pharmacokinetics of AZD5634 Following Single-Ascending Inhaled Doses (Part A) and After Single Inhaled and Intravenous Doses (Part B) in Healthy Subjects	Cystic Fibrosis	Drug: AZD5634 for inhalation;Drug: AZD5634 for infusion;Other: Placebo	AstraZeneca		Not recruiting	18 Years	50 Years	All	63	Phase 1	United States
228	NCT03512119	February 11, 2016	11 June 2018	Observational Study of Glucose Tolerance Abnormalities in Patient With Cystic Fibrosis Homozygous for Phe 508 Del CFTR Treated by Lumacaftor-Ivacaftor	Observational Study of Glucose Tolerance Abnormalities in Patient With Cystic Fibrosis Homozygous for Phe 508 Del CFTR Treated by Lumacaftor-Ivacaftor	Cystic Fibrosis Homozygous for Phe 508 Del CFTR;Glucose Intolerance or Newly Diagnosis Diabetes	Drug: Lumacaftor-Ivacaftor treatment	University Hospital, Strasbourg, France		Recruiting	12 Years	N/A	All	100	N/A	France
229	EUCTR2015-002677-38-GB	04/02/2016	28 February 2019	An investigation of the effect of CTX-4430 on lung inflammation in patients with cystic fibrosis	A Phase 2, Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study to Evaluate the Efficacy, Safety, and Tolerability of CTX-4430 Administered Orally Once-Daily for 48 Weeks in Adult Patients with Cystic Fibrosis - EMPIRE CF	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011763 Term: Cystic fibrosis lung System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: CTX-4430 Product Code: CTX-4430 Pharmaceutical Form: Capsule, hard INN or Proposed INN: acebilustat CAS Number: 943764-99-6 Current Sponsor code: CTX-4430 Other descriptive name: CRC3357, ZK-355322, Leukoton Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Name: CTX-4430 Product Code: CTX-4430 Pharmaceutical Form: Capsule, hard INN or Proposed INN: acebilustat CAS Number: 943764-99-6 Current Sponsor code: CTX-4430 Other descriptive name: CRC3357, ZK-355322, Leukoton Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Celtaxsys Inc.		Not Recruiting			Female: yes Male: yes	195	Phase 2	France;United States;Canada;Belgium;Germany;Italy;United Kingdom
230	NCT02625246	February 4, 2016	9 September 2019	Safety and Potential Efficacy of Human Mesenchymal Stem Cells in Non-Cystic Fibrosis Bronchiectasis	A Phase I, Trial to Evaluate the Safety, Tolerability, and Potential Efficacy of Allogeneic Human Mesenchymal Stem Cell (hMSC) Infusion in Patients With Non-Cystic Fibrosis Bronchiectasis	Bronchiectasis	Biological: hMSCs	Marilyn Glassberg		Not recruiting	30 Years	87 Years	All	6	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
231	EUCTR2015-003291-77-GB	02/02/2016	2 May 2016	A study looking at the safety of the drug GLPG1837 in patients with cystic fibrosis and the G551D mutation	A phase IIa, open label study of multiple doses of GLPG1837 in subjects with cystic fibrosis and the G551D mutation	Cystic fibrosis with the G551D mutation MedDRA version: 18.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Product Name: GLPG1837 Product Code: GLPG1837 Pharmaceutical Form: Tablet INN or Proposed INN: Not Applicable CAS Number: 1654725-02-6 Current Sponsor code: GLPG1837 Other descriptive name: GLPG1837 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 250- Product Name: GLPG1837 Product Code: GLPG1837 Pharmaceutical Form: Tablet INN or Proposed INN: Not Applicable CAS Number: 1654725-02-6 Current Sponsor code: GLPG1837 Other descriptive name: GLPG1837 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Product Name: GLPG1837 Product Code: GLPG1837 Pharmaceutical Form: Tablet INN or Proposed INN: Not Applicable CAS Number: 1654725-02-6 Current Sponsor code: GLPG1837 Other descriptive name: GLPG1837 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 62.5-	Galapagos NV		Authorised			Female: yes Male: yes	32	Phase 2a	Czech Republic;Ireland;Australia;Germany;United Kingdom
232	NCT02508207	February 2016	16 December 2017	A Phase 2 Study to Evaluate Effects of VX-661/Ivacaftor on Lung and Extrapulmonary Systems in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	A Phase 2, Randomized, Double-Blind, Placebo Controlled, Parallel-Group, Exploratory Study to Evaluate Effects of VX-661 in Combination With Ivacaftor on Lung and Extrapulmonary Systems in Subjects Aged 18 Years and Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: VX-661/ivacaftor;Drug: ivacaftor;Drug: VX-661/ ivacaftor matching placebo;Drug: ivacaftor matching placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	34	Phase 2	United States
233	NCT02684552	February 2016	12 September 2016	Non Invasive Ventilation During Physical Training in Cystic Fibrosis	Physiotherapist in Charge of the Study	Cystic Fibrosis	Device: Non invasive ventilation;Device: Oxygen	Karolinska University Hospital		Not recruiting	18 Years	90 Years	Both	8	N/A	Sweden
234	NCT02694393	February 2016	24 September 2018	Inhaled Sodium Nitrite as an Antimicrobial for Cystic Fibrosis	Phase I/II Study of Inhaled Sodium Nitrite as an Antimicrobial for Pseudomonas Infection in Cystic Fibrosis	Cystic Fibrosis	Drug: sodium nitrite	Schmidhofer, Mark, MD	Mast Therapeutics, Inc.;Cystic Fibrosis Foundation Therapeutics	Recruiting	18 Years	N/A	All	35	Phase 1/Phase 2	United States
235	NCT02709109	February 2016	15 October 2018	A Study to Evaluate the Safety and Efficacy of VX-371 in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation	A Phase 2a, Randomized, Double-blind, Placebo-controlled, Incomplete Block, Crossover Study to Evaluate the Safety and Efficacy of VX-371 in Subjects Aged 12 Years or Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation, and Being Treated With Orkambi	Cystic Fibrosis	Drug: VX-371;Drug: Saline;Drug: Placebo;Drug: Orkambi	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	142	Phase 2	United States;France;Ireland;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
236	NCT02840136	February 2016	25 June 2018	LC-MS/MS Based Method Development for the Monitoring of Antibiotic Concentrations in Sputum of Cystic Fibrosis Patients	Optimisation, Valorisation and Application of UPLC-MS/MS Based Monitoring of Antibiotic Concentrations in Sputum of Cystic Fibrosis Patients - Part 3: Non-blank Sputum Samples for Method Optimisation and Validation	Cystic Fibrosis	Drug: Piperacillin-tazobactam combination product;Drug: Meropenem;Drug: Ceftazidime	University Ghent	King Baudouin Foundation;Belgische Vereniging voor Strijd tegen Mucoviscidose	Not recruiting	12 Years	N/A	All	40	N/A	Belgium
237	EUCTR2015-001644-11-GB	22/01/2016	7 January 2019	Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment with Lumacaftor in Combination with Ivacaftor.	A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 6 Years and Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor/ivacaftor 100mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX 809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Product Name: lumacaftor/ivacaftor 200mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- INN or Proposed INN: Lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX 809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	256	Phase 3	France;United States;Canada;Belgium;Denmark;Australia;Germany;United Kingdom;Sweden
238	NCT03475381	January 22, 2016	11 June 2018	Real-life Follow-up of Cystic Fibrosis Patients Treated With Ivacaftor+Lumacaftor (Orkambi*)	Real-life Follow-up of Cystic Fibrosis Patients Treated With Ivacaftor+Lumacaftor (Orkambi*)	Cystic Fibrosis	Drug: Ivacaftor+lumacaftor	Assistance Publique - Hôpitaux de Paris	Effi-stat;Société Française de la Mucoviscidose	Not recruiting	12 Years	N/A	All	685	N/A	France
239	EUCTR2015-003292-30-BE	18/01/2016	8 August 2016	A study looking at the safety of the drug GLPG1837 in patients with cystic fibrosis and the S1251N mutation	A phase IIa, open-label study of two doses of GLPG1837 in subjects with cystic fibrosis and the S1251N mutation.	Cystic fibrosis with the S1251N mutation MedDRA version: 18.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Product Name: GLPG1837 Product Code: GLPG1837 Pharmaceutical Form: Tablet INN or Proposed INN: Not Applicable CAS Number: 1654725-02-6 Current Sponsor code: GLPG1837 Other descriptive name: GLPG1837 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 62.5- Product Name: GLPG1837 Product Code: GLPG1837 Pharmaceutical Form: Tablet INN or Proposed INN: Not Applicable CAS Number: 1654725-02-6 Current Sponsor code: GLPG1837 Other descriptive name: GLPG1837 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125-	Galapagos NV		Not Recruiting			Female: yes Male: yes	6	Phase 2	Belgium;Netherlands
240	NCT02646995	January 2016	18 June 2018	Lipid Formulation to Increase the Bioavailability of Fatty Acids in Cystic Fibrosis (CF) Patients		Cystic Fibrosis	Dietary Supplement: modified lipid formulation;Dietary Supplement: Fish oil	Nestlé		Not recruiting	5 Years	18 Years	All	16	N/A	Switzerland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
241	NCT02661438	January 2016	18 April 2016	Summative Usability Study of Ciprofloxacin Dry Powder for Inhalation Using Placebo	Multicenter Summative Usability Study of Ciprofloxacin Dry Powder for Inhalation in Subjects With Non-Cystic Fibrosis Bronchiectasis (NCFB) or Chronic Obstructive Pulmonary Disease (COPD) Using Matching Placebo	Bronchiectasis;Pulmonary Disease, Chronic Obstructive	Drug: Placebo to Ciprofloxacin DPI (BAYQ3939)	Bayer	Novartis	Not recruiting	40 Years	N/A	Both	46	N/A	United States
242	EUCTR2016-001840-20-Outside-EU/EEA		23 May 2016	Study to Validate the Instructions for Use of TOBI® Podhaler™ in Cystic Fibrosis Patients	A Multi-center, Human Factors Engineering (HFE) Usability Study in Cystic Fibrosis Patients to Validate the Approved Instructions for Use (IFU) of TOBI® Podhaler™ (Tobramycin Inhalation Powder) Using Placebo Capsules.	cystic fibrosis MedDRA version: 19.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: TOBI Podhaler Pharmaceutical Form: Inhalation powder, hard capsule Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Novartis Pharmaceuticals Corporation		Not Available			Female: yes Male: yes	45	Phase 4	United States
243	EUCTR2016-004318-82-Outside-EU/EEA		22 May 2017	A study comparing Tobramycin Inhalation Powder (TIP) administered once daily continuously versus TIP administered BID in 28 day on / 28 day off cycles for the treatment of pulmonary Pseudomonas aeruginosa in patients with cystic fibrosis	Tobramycin Inhalation Powder (TIP) Administered Once Daily Continuously Versus TIP Administered BID in 28 Day on / 28 Day Off Cycles	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 20.0 Level: LLT Classification code 10021860 Term: Infection Pseudomonas aeruginosa System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: TOBI Podhaler Pharmaceutical Form: Capsule INN or Proposed INN: Tobramycin Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 112-	Novartis Pharmaceuticals Corporation		Not Available			Female: yes Male: yes	200	Phase 4	United States
244	EUCTR2016-004479-35-IE		28 February 2019	A Study to Evaluate Efficacy and Safety of TEZ/IVA in Subjects Aged 6 through 11 Years With Cystic Fibrosis	A Phase 3, Double-blind, Parallel-group Study to Evaluate the Efficacy and Safety of Tezacaftor in Combination With Ivacaftor in Subjects Aged 6 Through 11 Years With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor 50 mg/ 75 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: VX-661/ivacaftor 100 mg/ 150 mg Pharmaceutical Form: Film-coated tablet INN or Proposed INN: TEZACAFTOR CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Other descriptive name: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s)	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	65	Phase 3	France;Poland;Belgium;Ireland;Denmark;Australia;Germany;United Kingdom;Switzerland
245	EUCTR2016-004786-80-DE		26 November 2018	Tolerability and Efficacy of Anakinra in patients with cystic fibrosis.	A phase IIa, randomized, placebo-controlled, double-blind, cross-over study to evaluate safety and efficacy of subcutanous administration of anakinra in patients with cystic fibrosis. - ANAKIN	Cystic fibrosis MedDRA version: 20.0 Level: LLT Classification code 10028141 Term: Mucoviscidosis System Organ Class: 100000004850 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Kineret Pharmaceutical Form: Solution for injection in pre-filled syringe INN or Proposed INN: ANAKINRA CAS Number: 143090-92-0 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Solution for injection in pre-filled syringe Route of administration of the placebo: Subcutaneous use	Ruprecht-Karls-University Heidelberg, Medical Faculty		Not Available			Female: yes Male: yes	52	Phase 2	Germany
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
246	NCT02589444	December 2015	16 December 2017	Vitamin D and Microbiota in Cystic Fibrosis	Pilot Study Evaluating the Role of Vitamin D Repletion on Gut and Lung Microbiota in Cystic Fibrosis	Vitamin D Deficiency;Cystic Fibrosis	Dietary Supplement: High-Dose Vitamin D3;Other: Stool Sample;Other: Sputum Sample;Other: Sham Comparator;Procedure: Blood draw	Emory University		Not recruiting	18 Years	N/A	All	41	N/A	United States
247	NCT02605590	December 2015	30 May 2016	Safety, Tolerability and Pharmacokinetics Study of AIR DNAse Administered by Inhalation to Healthy Adult Volunteers	A Randomized, Double-Blind, Placebo-Controlled, Single Ascending Dose and Multiple Ascending Dose Study of the Safety, Tolerability, and Pharmacokinetics of AIR-DNase When Administered by Inhalation to Healthy Adult Subjects	Cystic Fibrosis (CF)	Drug: AIR DNase;Drug: Placebo	Protalix		Not recruiting	18 Years	55 Years	Male	18	Phase 1	Israel
248	NCT02626182	December 2015	29 July 2019	Evaluation and Treatment of Pulmonary Vascular Disease in Moderate to Severe CF	Evaluation and Treatment of Pulmonary Vascular Disease in Moderate to Severe Cystic Fibrosis Lung Disease	Cystic Fibrosis	Drug: sildenafil;Drug: placebo	National Jewish Health		Not recruiting	18 Years	N/A	All	14	Phase 1/Phase 2	United States
249	NCT02680418	December 2015	11 April 2016	Pharmacokinetics of FDL169 in Healthy Female Subjects	A Phase I Dose Escalation Study to Assess the Pharmacokinetics (PK) of FDL169 in Healthy Female Volunteers	Cystic Fibrosis	Drug: FDL169	Flatley Discovery Lab LLC		Not recruiting	18 Years	55 Years	Female	8	Phase 1	United Kingdom
250	EUCTR2015-003662-87-DK	13/11/2015	2 October 2017	Pseudomonas Aeruginosa - Inhalation treatment, biomarkers and quality of life	Pseudomonas Aeruginosa - Inhalation treatment, biomarkers and quality of life - Pseudomonas, Tazocin or Colistin	Chronic pulmonary disease patients, except cystic fibrosis patients, with confirmed pulmonary infection by sputum sample positive for Pseudomonas Aeruginosa. MedDRA version: 18.1 Level: LLT Classification code 10051190 Term: Pneumonia Pseudomonas aeruginosa System Organ Class: 100000004862 MedDRA version: 18.1 Level: LLT Classification code 10050700 Term: Chronic respiratory disease NOS System Organ Class: 100000004855 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: colistimethate sodium (promixin) Pharmaceutical Form: Inhalation vapour, powder INN or Proposed INN: COLISTIMETHATE SODIUM CAS Number: 8068-28-8 Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 1000000- Trade Name: Piperacillin with Tazobactam (tazocin) Pharmaceutical Form: Powder and solvent for solution for infusion INN or Proposed INN: PIPERACILLIN Other descriptive name: PIPERACILLIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 4000- INN or Proposed INN: TAZOBACTAM Other descriptive name: TAZOBACTAM Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 500-	Aarhus University Hospital, Department of Respiratory Medicine		Not Recruiting			Female: yes Male: yes		Phase 2	Denmark
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
251	NCT02589236	November 2015	16 December 2017	Study of Cavosonstat (N91115) in Patients With CF Homozygous for the F508del-CFTR Mutation	A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study of N91115 to Evaluate Efficacy and Safety in Patients With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation Treated With Lumacaftor/Ivacaftor	Cystic Fibrosis	Drug: Cavosonstat;Drug: Placebo	Nivalis Therapeutics, Inc.	Medidata Solutions	Not recruiting	18 Years	N/A	All	138	Phase 2	United States
252	NCT02598999	November 2015	13 May 2019	Dose Escalation Study of ALX-009 in Healthy Men and Cystic Fibrosis (CF) and Non-CF Bronchiectasis Patients	Randomized, Double Blind, Placebo-controlled Study of the Safety, Tolerability and Pharmacokinetics After Single Ascending Doses or Multiple Ascending Doses of OSCN-, bLF or ALX-009 in Healthy Male and CF and Non-CF Bronchiectasis Patients	Cystic Fibrosis;Bronchiectasis	Drug: ALX-009;Drug: OSCN-;Drug: bLF;Drug: Placebo	Alaxia SAS		Recruiting	18 Years	50 Years	All	103	Phase 1	France
253	NCT02443688	October 30, 2015	26 August 2019	EMPIRE CF: A Phase 2 Study to Evaluate the Efficacy, Safety, and Tolerability of CTX-4430 in Adult Cystic Fibrosis (CF) Patients	A Phase 2, Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study to Evaluate the Efficacy, Safety, and Tolerability of CTX-4430 Administered Orally Once-Daily for 48 Weeks in Adult Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: CTX-4430;Drug: Placebo	Celtaxsys, Inc.		Not recruiting	18 Years	30 Years	All	200	Phase 2	United States;Belgium;Canada;France;Germany;Italy;United Kingdom
254	NCT01132482	October 2015	16 December 2017	Effects of Sildenafil on CFTR-dependent Ion Transport Activity	Phase II Study of the Effects of Sildenafil on CFTR-dependent Ion Transport Activity	Cystic Fibrosis	Drug: Sildenafil;Drug: Placebo	National Jewish Health		Not recruiting	18 Years	N/A	All	23	Phase 2	United States
255	NCT02518672	October 2015	16 December 2017	Pro-resolving Effect of MAG-DHA in Cystic Fibrosis (PREMDIC)	Role of DHA Monoglyceride (MAG-DHA) in the Resolution of Pulmonary Inflammation of Patients With Cystic Fibrosis.	Cystic Fibrosis	Dietary Supplement: MAG-DHA;Dietary Supplement: Placebo	SCF Pharma	Centre de recherche du Centre hospitalier universitaire de Sherbrooke;Solutex (Spain)	Not recruiting	18 Years	N/A	All	11	Phase 2	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
256	NCT02465450	September 29, 2015	11 June 2018	Safety, Tolerability, Pharmacokinetics, and Efficacy of JBT-101 (Lenabasum) in Cystic Fibrosis	A Phase 2, Double-blind, Randomized, Placebo-controlled Multicenter Study to Evaluate Safety, Tolerability, Pharmacokinetics, and Efficacy of JBT-101 in Cystic Fibrosis	Cystic Fibrosis	Drug: JBT-101 (lenabasum);Other: Placebo	Corbus Pharmaceuticals Inc.		Not recruiting	18 Years	65 Years	All	85	Phase 2	United States;Belgium;France;Germany;Italy;Poland;United Kingdom
257	EUCTR2014-004787-37-AT	21/09/2015	5 September 2016	A study in people with Cystic Fibrosis (a rare hereditary lung disease) to assess the efficacy and safety of a combination of two experimental drugs.	A Phase 3, Randomized, Double-blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Heterozygous for the F508del-CFTR Mutation and With a Second CFTR Mutation That Is Not Likely to Respond to VX-661 and/or Ivacaftor Therapy (F508del/NR)	Cystic Fibrosis MedDRA version: 19.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor 100mg/150mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Not yet assigned CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 VRT-0893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco Product Name: ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	300	Phase 3	France;United States;Canada;Spain;Australia;Austria;Israel;Germany
258	EUCTR2015-002911-13-FR	15/09/2015	7 January 2019	A Phase II, dose ranging, multicenter, double-blind, placebo controlled study to evaluate safety and efficacy of (R)-roscovitine in subjects with Cystic Fibrosis.	A Phase II, dose ranging, multicenter, double-blind, placebo controlled study to evaluate safety and efficacy of (R)-roscovitine in subjects with Cystic Fibrosis, homozygous for the F508del-CFTR mutation and chronically infected with Pseudomonas aeruginosa, a study involving 36 CF patients (24 treated, 12 controls). - ROSCO-CF	Cystic Fibrosis treated by 'roscovitine' in subjects homozygous for the F508del-CFTR mutation and chronically infected with Pseudomonas aeruginosa. MedDRA version: 18.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: SELICICLIB Product Code: CYC202 Pharmaceutical Form: Capsule Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use	CHRU de Brest		Not Recruiting			Female: yes Male: yes	36	Phase 2	France
259	EUCTR2014-004827-29-IT	01/09/2015	21 September 2015	A study to assess the efficacy and safety of a combination of two experimental drugs in people with cystic fibrosis (a rare hereditary lung disease)	A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long term Treatment With VX 661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del CFTR Mutation	Cystic Fibrosis MedDRA version: 18.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor (VX-770) 100mg/150 mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- INN or Proposed INN: Not yet assigned CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Trade Name: Kalydeco Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	1375	Phase 3	United States;Spain;Ireland;Austria;Israel;Italy;Switzerland;United Kingdom;France;Canada;Belgium;Australia;Denmark;Netherlands;Germany;Sweden
260	NCT02605538	September 1, 2015	27 August 2018	Hepatitis B in Cystic Fibrosis and Latent Tuberculosis Respectively	Hepatitis B in Cystic Fibrosis and Latent Tuberculosis Respectively	Cystic Fibrosis;Hepatitis A;Hepatitis B;Tuberculosis	Biological: Vaccination with vaccine against hepatitis A and B	Karolinska University Hospital	Karolinska Institutet	Recruiting	1 Year	N/A	All	250	N/A	Sweden
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
261	NCT02421120	September 2015	16 December 2017	Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients	A Prospective, Multicenter, Open-Label Study to Assess Population Pharmacokinetics and Safety of Intravenous Ceftolozane/Tazobactam in Adult Cystic Fibrosis Patients Admitted With Acute Pulmonary Exacerbation	Cystic Fibrosis;Cystic Fibrosis Pulmonary Exacerbation;Pseudomonas Aeruginosa Infection	Drug: Ceftolozane/Tazobactam	Joseph Kuti	Cubist Pharmaceuticals LLC;Indiana University Health;University of North Carolina;St. Christopher's Hospital for Children	Not recruiting	18 Years	N/A	All	21	Phase 4	United States
262	NCT02504827	September 2015	11 June 2018	Steady-state Pharmacokinetics of Ceftazidime/Avibactam in Cystic Fibrosis	Steady-state Pharmacokinetics of Ceftazidime/Avibactam in CF	Cystic Fibrosis	Drug: Ceftazidime/avibactam	University of Southern California		Not recruiting	18 Years	N/A	All	12	Phase 4	United States
263	NCT02564354	September 2015	21 November 2016	Exploratory Study to Evaluate QR-010 in Subjects With Cystic Fibrosis ?F508 CFTR Mutation	Open-Label, Exploratory Study to Evaluate the Effects of QR-010 on Nasal Potential Difference in Subjects With CF With the ?F508 CFTR Mutation	Cystic Fibrosis	Drug: QR-010	ProQR Therapeutics	European Commission	Not recruiting	18 Years	N/A	Both	18	Phase 1	United States;Belgium;France
264	NCT03497117	August 25, 2015	22 October 2019	19F MRI of Ventilation in Subjects With Cystic Fibrosis Undergoing Treatment for Pulmonary Exacerbation	19F Magnetic Resonance Imaging of Ventilation in Subjects With Cystic Fibrosis Undergoing Treatment for Pulmonary Exacerbation	Cystic Fibrosis	Device: Lung Clearance Index;Drug: MRI with PFP	University of North Carolina, Chapel Hill	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	Recruiting	18 Years	99 Years	All	16	Early Phase 1	United States
265	NCT02496780	August 2015	11 November 2019	The Impact of Insulin Therapy on Protein Turnover in Pre-Diabetic Cystic Fibrosis Patients	The Impact of Insulin Therapy on Protein Turnover in Pre-Diabetic Cystic Fibrosis Patients	Cystic Fibrosis	Drug: novolog insulin;Drug: levemir insulin;Drug: placebo	University of Minnesota	Children's Hospitals and Clinics of Minnesota	Recruiting	10 Years	25 Years	All	80	Phase 2/Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
266	NCT02531984	August 2015	7 September 2015	The Efficacy of Azithromycin in Treating Children With Non Cystic Fibrosis Bronchiectasis	Open Trial With Randomized Withdrawal of Treatment, to Evaluate the Efficacy of Azithromycin in the Treatment of Children With Non Cystic Fibrosis Bronchiectasis ( AZI-STOP Study )	Non Cystic Fibrosis Bronchiectasis in Children	Drug: Azithromycin	Assistance Publique Hopitaux De Marseille		Not recruiting	3 Years	17 Years	Both	100	Phase 3
267	NCT02535130	August 2015	29 August 2016	Evaluation of Nebulization and Positive Expiratory Pressure Combination		Cystic Fibrosis;Healthy Subjects	Device: Positive expiratory pressure;Drug: amikacine nebulization	Cliniques universitaires Saint-Luc- Université Catholique de Louvain		Recruiting	16 Years	55 Years	Both	15	N/A	Belgium
268	NCT02544451	August 2015	9 October 2018	Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor	A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 6 Years and Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: Ivacaftor;Drug: Lumacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	N/A	All	240	Phase 3	United States;Australia;Belgium;Canada;Denmark;France;Germany;Sweden;United Kingdom
269	NCT02565914	August 2015	22 October 2019	A Study to Evaluate the Safety and Efficacy of Long Term Treatment With VX-661 in Combination With Ivacaftor in Subjects With Cystic Fibrosis Who Have an F508del-CFTR Mutation	A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long Term Treatment With VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: VX-661/ivacaftor;Drug: ivacaftor	Vertex Pharmaceuticals Incorporated		Recruiting	12 Years	N/A	All	1116	Phase 3	United States;Australia;Austria;Belgium;Canada;Denmark;France;Germany;Ireland;Israel;Italy;Netherlands;Spain;Sweden;Switzerland;United Kingdom
270	NCT02740868	August 2015	15 April 2019	Xenon-129 Lung Magnetic Resonance Imaging: Study of Healthy Volunteers and Participants With Pulmonary Disease	Development of Hyperpolarized Xenon-129 Lung Magnetic Resonance Imaging: Comparative Pilot Study of Healthy Volunteers and Participants With Pulmonary Disease	Cystic Fibrosis;Asthma	Drug: Xenon-129;Device: Magnetic Resonance Imaging;Device: Lung Clearance Index	The Hospital for Sick Children		Recruiting	8 Years	N/A	All	30	Phase 2	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
271	NCT02310789	July 31, 2015	11 June 2018	(Study: Vertex IIS) Does Ivacaftor Alter Wild Type CFTR-Open Probability In The Sweat Gland Secretory Coil?	(Study: Vertex IIS) A Study To Access the Effects of Ivacaftor on Wild Type CFTR-Open Probability (PO) In The Sweat Gland Secretory Coil	Cystic Fibrosis	Drug: Ivacaftor;Drug: ß-Adrenergic cocktail;Drug: Pilocarpine Nitrate 5%;Device: Macroduct sweat stimulator	Richard Barry Moss		Not recruiting	18 Years	N/A	All	8	N/A	United States
272	NCT02456103	July 31, 2015	4 February 2019	Extension Study of Ataluren for Nonsense Mutation Cystic Fibrosis	A Phase 3 Extension Study of Ataluren (PTC124) in Patients With Nonsense Mutation Cystic Fibrosis	Cystic Fibrosis	Drug: Ataluren	PTC Therapeutics		Not recruiting	6 Years	N/A	All	246	Phase 3	United States;Argentina;Australia;Belgium;Brazil;Bulgaria;Canada;France;Germany;Greece;Israel;Italy;Netherlands;Poland;Spain;United Kingdom
273	EUCTR2015-001317-28-NL	30/07/2015	22 May 2017	A B2-agonist as a CFTR activator in CF - Part 2	A B2-agonist as a CFTR activator in CF - Part 2 - ABBA 2	Cystic fibrosis;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Salbutamol Pharmaceutical Form: Tablet	UMC Utrecht		Not Recruiting			Female: yes Male: yes		Phase 2	Netherlands
274	NCT02444234	July 2015	11 June 2018	Pharmacokinetics of Tedizolid Phosphate in Cystic Fibrosis	Steady-State Pharmacokinetics of Tedizolid in Plasma and Sputum of Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Tedizolid PO/IV;Drug: Tedizolid IV/PO	University of Southern California	Merck Sharp & Dohme Corp.	Not recruiting	18 Years	N/A	All	11	Phase 4	United States
275	NCT02514473	July 2015	16 December 2017	A Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects With CF, Homozygous for the F508del-CFTR Mutation	A Phase 3, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects Aged 6 Through 11 Years With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: VX-809;Drug: Placebo;Drug: VX-770	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	11 Years	All	206	Phase 3	United States;Australia;Belgium;Canada;Denmark;France;Germany;Sweden;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
276	EUCTR2015-000543-16-GB	19/06/2015	13 June 2016	A study in children aged 6 Through 11 Years With Cystic Fibrosis to assess the efficacy and safety of a combination of two experimental drugs	A Phase 3, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects Aged 6 Through 11 Years With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic Fibrosis;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor/ivacaftor 100mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: LUMACAFTOR Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	200	Phase 3	France;United States;Canada;Belgium;Denmark;Australia;Germany;United Kingdom;Sweden
277	NCT02239458	June 2015	28 April 2015	DPP-IV Inhibition Prior to Development of Diabetes in Patients With Cystic Fibrosis		Cystic Fibrosis	Drug: Saxagliptin	Ram Weiss		Not recruiting	18 Years	65 Years	Both	60	N/A	Israel
278	NCT02279498	June 2015	20 August 2018	SOLUTION: Study of Oral Liprotamase Unit-Matched Therapy Of Non-Porcine Origin in Patients With Cystic Fibrosis	A Phase 3, Randomized, Open-Label, Assessor-Blind, Noninferiority, Active-Comparator Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Exocrine Pancreatic Insufficiency;Cystic Fibrosis	Drug: Liprotamase;Drug: porcine (pig) PERT	Anthera Pharmaceuticals		Not recruiting	7 Years	N/A	All	128	Phase 3	United States;Canada;Czechia;Hungary;Israel;Poland;Spain;Czech Republic
279	NCT02532764	June 2015	16 December 2017	Dose Escalation Study of QR-010 in Homozygous ?F508 Cystic Fibrosis Patients	Phase 1b, Randomized, Double-blind, Placebo-controlled, Dose Escalation Study to Evaluate the Safety, Tolerability and Pharmacokinetics of QR-010 in Subjects With Homozygous ?F508 Cystic Fibrosis	Cystic Fibrosis	Drug: QR-010;Drug: Placebo	ProQR Therapeutics	European Commission	Not recruiting	18 Years	60 Years	All	64	Phase 1/Phase 2	United States;Belgium;Canada;Czechia;Denmark;France;Germany;Italy;Spain;United Kingdom;Czech Republic;Netherlands
280	EUCTR2014-004838-25-IT	20/05/2015	19 February 2018	A study in people with Cystic Fibrosis ( a rare hereditary lung disease) to assess the efficacy and safety of a combination of two experimental drugs	A Phase 3, Randomized, Double-Blind, Ivacaftor-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Heterozygous for the F508del-CFTR Mutation and a Second CFTR Allele With a Gating Defect That Is Clinically Demonstrated to be Ivacaftor Responsive	Cystic Fibrosis MedDRA version: 17.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: VX-661/ivacaftor 100mg/150mg Product Code: VX-661/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Not yet assigned CAS Number: 1152311-62-0 Current Sponsor code: VX-661 Other descriptive name: VRT-893661 VRT-0893661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Trade Name: Kalydeco Product Name: ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	200	Phase 3	France;United States;Canada;Belgium;Ireland;Austria;Germany;United Kingdom;Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
281	NCT02449031	May 5, 2015	14 January 2019	Observational Study in Cystic Fibrosis Patients Using TOBI® Podhaler™ or Other FDA Approved Inhaled Antipseudomonal Antibacterial Drugs	A Prospective Observational Study in Cystic Fibrosis Patients With Chronic Respiratory Pseudomonas Aeruginosa Infection Treated With TOBI® Podhaler™ (Tobramycin Inhalation Powder) or Other FDA Approved Inhaled Antipseudomonal Antibacterial Drugs	Pseudomonas Aeruginosa in Cystic Fibrosis	Drug: TOBI Podhaler;Drug: TOBI;Drug: Bethkis;Drug: Cayston	Mylan Inc.	Cystic Fibrosis Foundation	Not recruiting	6 Years	N/A	All	260	N/A	United States
282	NCT02468908	May 2015	16 December 2017	Inhaled Molgramostim (rhGM-CSF) in Healthy Adult Subjects	A Randomized, Double-Blind, Placebo-Controlled, Single-Centre, Single Ascending Dose and Multiple Ascending Dose Study of the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of Molgramostim When Administered by Inhalation to Healthy Adult Subjects	Pulmonary Alveolar Proteinosis;Bronchiectasis;Cystic Fibrosis;Acute Respiratory Distress Syndrome	Drug: Molgramostim;Drug: Placebo	Savara Inc.	Celerion	Not recruiting	18 Years	55 Years	All	42	Phase 1	United Kingdom
283	NCT02343445	April 2015	22 August 2016	Clearing Lungs With ENAC Inhibition in Cystic Fibrosis	A Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Safety and Efficacy of P-1037 Solution for Inhalation in Patients With Cystic Fibrosis (CF)	Cystic Fibrosis	Drug: P-1037;Drug: Hypertonic Saline;Drug: Saline	Vertex Pharmaceuticals Incorporated	Parion Sciences	Not recruiting	12 Years	80 Years	Both	142	Phase 2	United States
284	NCT02445053	April 2015	26 August 2019	Observational Study of Outcomes in Cystic Fibrosis Patients With Selected Gating Mutations on a CFTR Allele (The VOCAL Study)	Observational Study of Outcomes in Cystic Fibrosis Patients With Selected Gating Mutations on a CFTR Allele (The VOCAL Study)	Cystic Fibrosis	Drug: ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	N/A	All	90	N/A	Italy;Netherlands;United Kingdom
285	NCT02690064	April 2015	22 October 2019	Mechanisms for Vascular Dysfunction and Exercise Tolerance in CF	Mechanisms for Vascular Dysfunction and Exercise Tolerance in CF	Cystic Fibrosis	Dietary Supplement: Acute Antioxidant;Dietary Supplement: Chronic Antioxidant;Other: Placebo	Augusta University		Recruiting	7 Years	N/A	All	80	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
286	NCT02325362	March 17, 2015	11 June 2018	Effect of Miglustat on the Nasal Potential Difference in Patients With Cystic Fibrosis Homozygous for the F508del Mutation	Single Center, Double-blind, Randomized, Placebo-controlled, Two-period/Two-treatment Crossover, Proof-of-mechanism Study Investigating the Effect of Miglustat on the Nasal Potential Difference in Adult Patients With Cystic Fibrosis Homozygous for the F508del Mutation	Cystic Fibrosis	Drug: Miglustat ; placebo;Drug: Placebo ; Miglustat	Assistance Publique - Hôpitaux de Paris	Actelion;CRCM (Centres de Ressources et de Compétences de la Mucoviscidose)	Not recruiting	18 Years	N/A	All	16	Phase 2/Phase 3	France
287	NCT02378467	March 1, 2015	11 February 2019	Saline Hypertonic in Preschoolers	Saline Hypertonic in Preschoolers	Cystic Fibrosis	Drug: 7% Hypertonic Saline (HS);Drug: 0.9% Isotonic Saline (IS)	University of Washington, the Collaborative Health Studies Coordinating Center	Cystic Fibrosis Foundation	Not recruiting	3 Years	5 Years	All	150	N/A	United States;Canada
288	NCT02390219	March 2015	16 December 2017	Study to Evaluate Lumacaftor and Ivacaftor Combination Therapy in Subjects 12 Years and Older With Advanced Lung Disease	A Phase 3b, Open-Label Study to Evaluate Lumacaftor and Ivacaftor Combination Therapy in Subjects 12 Years and Older With Cystic Fibrosis and Advanced Lung Disease, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis;Advanced Lung Disease	Drug: Lumacaftor;Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	46	Phase 3	United States
289	NCT02392234	March 2015	26 February 2018	A Phase 3 Study to Evaluate the Efficacy and Safety of Ivacaftor and VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Heterozygous for the F508del-CFTR Mutation	A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Crossover Study to Evaluate the Efficacy and Safety of Ivacaftor and VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Heterozygous for the F508del-CFTR Mutation, and a Second Allele With a CFTR Mutation Predicted to Have Residual Function	Cystic Fibrosis	Drug: VX-661/ivacaftor;Drug: ivacaftor;Drug: VX-661/ ivacaftor placebo;Drug: ivacaftor placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	246	Phase 3	United States;Australia;Belgium;Canada;France;Germany;Israel;Italy;Netherlands;United Kingdom;Switzerland
290	NCT02275936	February 2015	14 November 2016	Study of N91115 in Patients With Cystic Fibrosis Homozygous F508del-CFTR Mutation	A Phase 1b, Randomized, Double-Blind, Placebo-Controlled, Parallel, Group Study of N91115 to Evaluate Safety and Pharmacokinetics in Patients With Cystic Fibrosis Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: N91115	Nivalis Therapeutics, Inc.		Not recruiting	18 Years	80 Years	Both	51	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
291	NCT02453789	February 2015	11 June 2018	A Study of OligoG in Cystic Fibrosis Subjects With Burkholderia Spp. Infection	A Randomized Double-blind, Placebo-controlled Cross-over Study of Inhaled Alginate Oligosaccharide (OligoG) for 28 Days in Subjects With Cystic Fibrosis Using Aztreonam Due to Chronic Colonization With Burkholderia Spp.	Cystic Fibrosis;Burkholderia Infection	Drug: Alginate oligosaccharide;Drug: Placebo	AlgiPharma AS		Not recruiting	18 Years	N/A	All	15	Phase 2	Germany
292	NCT02489955	February 2015	3 August 2015	Antibiotic Nephrotoxicity in Adult Patients With Cystic Fibrosis	Prospective Randomised Trial of 'Area Under the Curve' (AUC) Dosing Strategy for Intravenous Tobramycin Versus Standard Trough Dosing for Pulmonary Exacerbations in Adult Patients With Cystic Fibrosis (CF)	Cystic Fibrosis	Drug: Amikacin;Drug: Tobramycin;Drug: Colomycin	The Leeds Teaching Hospitals NHS Trust		Recruiting	18 Years	N/A	Both		N/A	United Kingdom
293	ChiCTR-IPC-15005915	2015-01-31	18 April 2017	A Randomized, Controlled Study of Combined the therapy of bronchoalveolar lavage and Amikacin injection in Patients with Acute Exacerbation of Bronchiectasis	A Randomized, Controlled Study of Combined the therapy of bronchoalveolar lavage and Amikacin injection in Patients with Acute Exacerbation of Bronchiectasis	non-cystic fibrosis bronchiectasis	Experimental group:Routine treatment combining with the therapy of bronchoalveolar lavage and local drug injection (Amikacin);	Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine		Recruiting	18	80	Both	Experimental group:50;	New Treatment Measure Clinical Study
294	NCT02248922	January 27, 2015	16 December 2017	Evaluation of Lung Clearance Index in Cystic Fibrosis (CF) Patients, Infected With P.Aeruginosa	An 8 Week Open-label Interventional Multicenter Study to Evaluate the Lung Clearance Index as Endpoint for Clinical Trials in Cystic Fibrosis Patients = 6 Years of Age, Chronically Infected With Pseudomonas Aeruginosa	Chronic Lung Infection With Pseudomonas Aeruginosa in Cystic Fibrosis	Drug: TIS or TIP	Novartis Pharmaceuticals		Not recruiting	6 Years	N/A	All	17	Phase 4	Germany
295	EUCTR2014-004519-35-HU	09/01/2015	4 August 2015	A Phase II, Multicenter, Parallel-Group, Active-Controlled, Randomized, Double-blind, Dose-Ranging Study to Evaluate the Efficacy and Safety of Different Doses of Creon IR in Subjects with Pancreatic Exocrine Insufficiency due to Cystic Fibrosis	A Phase II, Multicenter, Parallel-Group, Active-Controlled, Randomized, Double-blind, Dose-Ranging Study to Evaluate the Efficacy and Safety of Different Doses of Creon IR in Subjects with Pancreatic Exocrine Insufficiency due to Cystic Fibrosis	Pancreatic exocrine Insufficiency due to Cystic Fibrosis;Therapeutic area: Diseases [C] - Immune System Diseases [C20]	Product Name: CREON IR Pharmaceutical Form: Capsule, hard INN or Proposed INN: NOT APPLICABLE CAS Number: 8049-47-6 Current Sponsor code: CREON IR Other descriptive name: PANCREATIN (Pancreas Powder) Concentration unit: U unit(s) Concentration type: range Concentration number: 4000-30000 Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Trade Name: Kreon 25 000 Product Name: Creon® 25,000 Pharmaceutical Form: Capsule, hard INN or Proposed INN: not assigned CAS Number: 8049-47-6 Other descriptive name: PANCREATIN (Pancreas Powder) Concentration unit: U unit(s) Concentration type: equal Concentration number: 25000- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Abbott Laboratories GmbH		Not Recruiting			Female: yes Male: yes	78	Phase 2	Czech Republic;Hungary;Spain;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
296	NCT02347657	January 2015	26 February 2018	A Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of VX-661 in Combination With Ivacaftor	A Phase 3, Randomized, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of VX-661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic Fibrosis	Drug: VX-661/ ivacaftor;Drug: ivacaftor;Drug: VX-661/ ivacaftor placebo;Drug: ivacaftor placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	509	Phase 3	United States;Canada;Denmark;France;Germany;Ireland;Italy;Netherlands;Spain;Sweden;Switzerland;United Kingdom
297	NCT02359357	January 2015	18 April 2016	FTIH - Single and Repeat Oral Doses of FDL169 in Healthy Volunteers	A Phase 1, Randomised, Double-Blind, Placebo-Controlled, Dose-Escalation First-Time-in-Human Study to Assess the Safety, Tolerability and Pharmacokinetic Profile of Single and Repeat Oral Doses of FDL169 in Healthy Volunteers	Cystic Fibrosis	Drug: FDL169;Drug: Placebo	Flatley Discovery Lab LLC		Not recruiting	18 Years	45 Years	Male	130	Phase 1	United Kingdom
298	NCT02507843	January 2015	3 August 2015	Vitamin D as an Adjunctive Treatment in Patients With Non-Cystic Fibrosis Bronchiectasis	Vitamin D as an Adjunctive Treatment in Patients With Non-Cystic Fibrosis Bronchiectasis: a Double-blind Randomised Controlled Trial	Bronchiectasis	Drug: Cholecalciferol;Drug: Placebo	Shanghai Pulmonary Hospital, Shanghai, China		Recruiting	18 Years	N/A	Both	200	Phase 4	China
299	NCT02526004	January 2015	31 August 2015	Cystic Fibrosis Microbiome-determined Antibiotic Therapy Trial in Exacerbations: Results Stratified	Cystic Fibrosis Microbiome-determined Antibiotic Therapy Trial in Exacerbations: Results Stratified	Cystic Fibrosis	Drug: Ceftazidime;Drug: Tobramycin	University College Cork	Queen's University, Belfast;Paris Descartes University;University of Dundee;University of Washington;University Hospital Heidelberg;Teagasc;Clininfo;GABO:mi;Papworth Hospital;Katholieke Universiteit Leuven;Assistance Publique - Hôpitaux de Paris;European Union	Recruiting	16 Years	80 Years	Both	252	N/A	Ireland
300	NCT04122547	January 2015	22 October 2019	Efficacy of Roflumilast on Exacerbations in Patients With Non-cystic Fibrosis Bronchiectasis	Efficacy of Roflumilast on Exacerbations in Patients With Non-cystic Fibrosis Bronchiectasis: a Randomized Double-blind Placebo-controlled Trial	Exacerbation Copd;Bronchiectasis;Lung Function Decreased	Drug: Roflumilast;Drug: Placebo	Prince of Songkla University		Not recruiting	18 Years	80 Years	All	40	Phase 3	Thailand
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
301	EUCTR2015-000395-97-Outside-EU/EEA		16 February 2015	A Clinical Trial to Evaluate the Effects of the drug Aztreonam Lysine for Inhalation in Patients with Cystic Fibrosis, Mild Lung Disease, and infection with the bacteria Pseudomonas aeruginosa (AIR-CF4)	A Double-Blind, Multicenter, Multinational, Randomized, Placebo-Controlled Trial Evaluating Aztreonam Lysine for Inhalation in Patients with Cystic Fibrosis, Mild Lung Disease, and P. aeruginosa (AIR-CF4)	Cystic Fibrosis with Mild Lung Disease and P. aeruginosa (AIR-CF4);Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Cayston INN or Proposed INN: AZTREONAM CAS Number: 78110-38-0 Current Sponsor code: AZLI Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Powder and solvent for nebuliser solution Route of administration of the placebo: Inhalation use	Gilead Sciences, Inc.	Gilead Sciences Pty Ltd;Gilead Sciences Canada, Inc.	Not Available			Female: yes Male: yes	157		United States;Australia;Canada
302	EUCTR2015-000396-26-Outside-EU/EEA		16 February 2015	Program to Give the Drug Aztreonam Lysine for Inhalation to Patients with Cystic Fibrosis and infection by the Bacteria Pseudomonas aeruginosa Who Have Limited Treatment Options and are at Risk for Disease Progression	Expanded Access Program for Aztreonam Lysine for Inhalation in Patients with Cystic Fibrosis and Pseudomonas aeruginosa Airway Infection Who Have Limited Treatment Options and are at Risk for Disease Progression	Adult and paediatric cystic fibrosis (CF) patients with pulmonary Pseudomonas aeruginosa (PA) infection.;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Cayston INN or Proposed INN: AZTREONAM CAS Number: 78110-38-0 Current Sponsor code: AZLI Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75-	Gilead Sciences, Inc.		Not Available			Female: yes Male: yes	603		United States
303	EUCTR2015-000397-36-Outside-EU/EEA		16 February 2015	Program to Give the Drug Aztreonamn Lysine for Inhalation to Canadian Patients with Cystic Fibrosis and infection by the Bacteria Pseudomonas aeruginosa Who Have Limited Treatment Options and are at Risk for Disease Progression	Expanded Access Program for Aztreonam Lysine for Inhalation in Canadian Patients with Cystic Fibrosis and Pseudomonas aeruginosa Airway Infection Who Have Limited Treatment Options and are at Risk for Disease Progression	Adult and paediatric cystic fibrosis (CF) patients with pulmonary Pseudomonas aeruginosa (PA) infection. MedDRA version: 17.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Cayston INN or Proposed INN: AZTREONAM CAS Number: 78110-38-0 Current Sponsor code: AZLI Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75-	Gilead Sciences, Inc.		Not Available			Female: yes Male: yes	45		Canada
304	EUCTR2015-001219-11-Outside-EU/EEA		7 April 2015	A study of the efficacy and tolerability of pancrelipase microtablet (MT) capsules for the treatment of cystic fibrosis-dependent exocrine pancreatic insufficiency	A randomized double-blind (withdrawal) phase 3 study to evaluate the efficacy and tolerability of pancrelipase MT capsules compared with placebo in the treatment of subjects with cystic fibrosis-dependent exocrine pancreatic insufficiency	Cystic fibrosis MedDRA version: 17.1 Level: LLT Classification code 10011765 Term: Cystic fibrosis pancreas System Organ Class: 100000004850 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: PANCREASE MT Product Name: PANCREASE MT Pharmaceutical Form: Capsule INN or Proposed INN: pancrelipase Other descriptive name: PANCRELIPASE AMYLASE Concentration unit: Other Concentration type: up to Concentration number: 10000- Pharmaceutical form of the placebo: Capsule Route of administration of the placebo: Oral use	Johnson & Johnson Pharmaceutical Research and Development, L.L.C.		Not Available			Female: yes Male: yes	40	Phase 3	United States;Canada
305	EUCTR2015-001225-16-Outside-EU/EEA		10 July 2015	A Study to Evaluate the Pharmacokinetics and Safety of Doripenem in Children and Adolescents with Cystic Fibrosis	An Open-Label Study to Evaluate the Single-Dose Pharmacokinetics and Safety of Doripenem in Pediatric Subjects 6 to 17 Years of age, Inclusive, With Cystic Fibrosis	Cystic fibrosis MedDRA version: 18.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: DORIBAX Product Name: DORIBAX Pharmaceutical Form: Powder for solution for infusion INN or Proposed INN: DORIPENEM HYDRATE CAS Number: 364622-82-2 Other descriptive name: DORIPENEM HYDRATE Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 500-	Johnson & Johnson Pharmaceutical Research & Development L.L.C		Not Available			Female: yes Male: yes	22		United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
306	EUCTR2015-001267-39-FR		2 October 2017	A study to assess the efficacy and safety of ivacaftor in children aged 3 to 5 with Cystic Fibrosis (a rare hereditary disease that affects the lungs, digestive system and other organs).	A Phase 3b, 2-part, Randomized, Double-blind, Placebo-controlled Crossover Study With a Long term Open-label Period to Investigate Ivacaftor in Subjects With Cystic Fibrosis Aged 3 Through 5 Years Who Have a Specified CFTR Gating Mutation	Cystic Fibrosis MedDRA version: 18.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Granules Route of administration of the placebo: Oral use Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Granules Route of administration of the placebo: Oral use Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Trade Name: Kalydeco Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Not Available			Female: yes Male: yes	50	Phase 3	France;Canada;Australia;United Kingdom
307	EUCTR2015-001644-11-SE		18 June 2018	Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment with Lumacaftor in Combination with Ivacaftor.	A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 6 Years and Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor/ivacaftor 100mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX 809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Product Name: lumacaftor/ivacaftor 200mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- INN or Proposed INN: Lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX 809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200-	Vertex Pharmaceuticals Incorporated		Not Available			Female: yes Male: yes	256	Phase 3	France;United States;Canada;Belgium;Denmark;Australia;Germany;United Kingdom;Sweden
308	EUCTR2015-001997-16-GB		28 February 2019	A study to assess the safety, pharmacokinetics, and pharmacodynamics of ivacaftor in children less than 24 months of age with cystic fibrosis (a rare hereditary disease that affects the lungs, digestive system and other organs).	A Phase 3, 2-Part, Open-label Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have a CFTR Gating Mutation	Cystic Fibrosis MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25- Trade Name: Kalydeco Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Trade Name: Kalydeco Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules in sachet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75-	Vertex Pharmaceuticals Incorporated		Not Available			Female: yes Male: yes	35	Phase 3	United States;Canada;Ireland;Germany;United Kingdom
309	EUCTR2015-004841-13-FR		28 February 2019	A Study to Evaluate the Safety and Efficacy of VX-371 in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation	A Phase 2a, Randomized, Double-blind, Placebo-controlled, Incomplete Block, Crossover Study to Evaluate the Safety and Efficacy of VX-371 in Subjects Aged 12 Years or Older With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation, and Being Treated With Orkambi	Cystic Fibrosis MedDRA version: 19.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Code: VX-371 in hypertonic saline Pharmaceutical Form: Inhalation solution INN or Proposed INN: Not yet assigned Current Sponsor code: VX-371 Other descriptive name: VX-371 Concentration unit: µg/µl microgram(s)/microlitre Concentration type: up to Concentration number: 30- Product Code: VX-371 in saline Pharmaceutical Form: Inhalation solution INN or Proposed INN: Not yet assigned Current Sponsor code: VX-371 Other descriptive name: VX-371 Concentration unit: µg/ml microgram(s)/millilitre Concentration type: up to Concentration number: 30- Pharmaceutical form of the placebo: Inhalation solution Route of administration of the placebo: Inhalation use Trade Name: Orkambi Product Name: lumacaftor/ivacaftor 200mg/125mg tablets Product Code: VX-809/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Other descriptive name: Ivacaftor Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- INN or Proposed INN: Lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Other descriptive name: LUMACAFTOR Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- Product Name: Hypertonic saline Pharmaceutical Form: Inhalation solution INN or Proposed INN: 4.2% NaCl/inhalation solution	Vertex Pharmaceuticals Inc.		Not Recruiting			Female: yes Male: yes	150	Phase 2	United States;France;Czech Republic;Ireland;United Kingdom
310	EUCTR2014-002125-35-DE	18/12/2014	20 August 2018	A phase IIb study of OligoG in subjects with cystic fibrosis colonized with Burkholderia spp.	A randomized double-blind, placebo-controlled cross-over study of inhaled alginate oligosaccharide (OligoG) for 28 days in subjects with Cystic Fibrosis using aztreonam due to chronic colonization with Burkholderia spp. - A phase IIb study of OligoG in subjects with cystic fibrosis colonized with Burkholderia spp.	Cystic Fibrosis;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: OligoG CF-5/20 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Oligomer of Sodium Alginate Current Sponsor code: OligoG Other descriptive name: OLIGOG CF-5/20 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 35- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Algipharma AS		Not Recruiting			Female: yes Male: yes	12	Phase 2	Germany
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
311	NCT02262871	December 2014	11 June 2018	HFN Versus NIV in Cystic Fibrosis. The HIFEN Study	Randomized Cross-over Physiologic Study of High Flow Nasal Oxygen Cannula Versus Non-invasive Ventilation in Cystic Fibrosis. The HIFEN Study	Cystic Fibrosis	Device: High flow nasal oxygen cannula;Device: Noninvasive ventilation	St. Michael's Hospital, Toronto		Not recruiting	18 Years	N/A	All	15	N/A	Canada
312	NCT02273661	November 19, 2014	6 May 2019	Evaluation of a Therapeutic Strategy Including Nebulised Liposomal Amphotericin B (Ambisome®) in Maintenance Treatment of Allergic Bronchopulmonary Aspergillosis (Cystic Fibrosis Excluded).	Evaluation of a Therapeutic Strategy Including Nebulised Liposomal Amphotericin B (Ambisome®) in Maintenance Treatment of Allergic Bronchopulmonary Aspergillosis (Cystic Fibrosis Excluded)	Allergic Bronchopulmonary Aspergillosis	Drug: Liposomal amphotericin B (Ambisome®);Drug: placebo	Poitiers University Hospital		Not recruiting	18 Years	N/A	All	174	Phase 2	France
313	EUCTR2014-001204-21-DE	17/11/2014	2 October 2017	An open-label clinical trial that evaluates the lung clearance index in cystic fibrosis patients = 6 years of age, chronically infected with Pseudomonas aeruginosa	An 8 week open-label interventional multicenter study to evaluate the lung clearance index as endpoint for clinical trials in cystic fibrosis patients = 6 years of age, chronically infected with Pseudomonas aeruginosa	Chronic lung infection with Pseudomonas aeruginosa in cystic fibrosis patients MedDRA version: 18.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 18.0 Level: LLT Classification code 10021860 Term: Infection Pseudomonas aeruginosa System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: TOBI Podhaler Product Name: TOBI Podhaler Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28- Pharmaceutical Form: Nebulisation solution INN or Proposed INN: Tobramycin CAS Number: 3232986-56-4 Current Sponsor code: TBM100 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300-	Novartis Pharma GmbH		Not Recruiting			Female: yes Male: yes		Phase 4	Germany
314	NCT03312140	November 6, 2014	16 December 2017	Examination of the Lipid Metabolism of the Liver After Choline Substitution in Cystic Fibrosis	Examination of the Lipid Metabolism of the Liver After Choline Substitution in Cystic Fibrosis	Cystic Fibrosis Liver Disease	Drug: Choline Chloride	University Hospital Tuebingen		Not recruiting	18 Years	N/A	Male	10	N/A
315	EUCTR2013-005357-79-CZ	15/10/2014	8 August 2016	Long Term Administration of Inhaled Mannitol in Cystic Fibrosis – A Safety and Efficacy Trial in Adult Cystic Fibrosis Subjects	Long Term Administration of Inhaled Mannitol in Cystic Fibrosis – A Safety and Efficacy Trial in Adult Cystic Fibrosis Subjects	Cystic Fibrosis MedDRA version: 19.0 Level: LLT Classification code 10011764 Term: Cystic fibrosis NOS System Organ Class: 100000004850 ;Therapeutic area: Diseases [C] - Digestive System Diseases [C06]	Trade Name: Bronchitol Product Name: Bronchitol Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Mannitol Other descriptive name: MANNITOL Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 40- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Pharmaxis Limited		Not Recruiting			Female: yes Male: yes	440	Phase 3	United States;Slovakia;Greece;Spain;Ukraine;Russian Federation;Israel;Italy;United Kingdom;France;Czech Republic;Hungary;Mexico;Canada;Argentina;Poland;Belgium;Romania;Bulgaria;South Africa;Sweden
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
316	NCT02134353	October 2014	11 June 2018	A Safety and Efficacy Trial of Inhaled Mannitol in Adult Cystic Fibrosis Subjects	Long Term Administration of Inhaled Mannitol in Cystic Fibrosis - A Safety and Efficacy Trial in Adult Cystic Fibrosis Subjects	Cystic Fibrosis	Drug: Inhaled mannitol;Drug: Placebo Comparator: Arm B - Control	Pharmaxis		Not recruiting	18 Years	99 Years	All	423	Phase 3	United States;Argentina;Australia;Belgium;Canada;Czechia;Hungary;Israel;Italy;Mexico;New Zealand;Poland;Romania;Russian Federation;Slovakia;South Africa;Spain;Ukraine;Czech Republic;France
317	NCT02157922	October 2014	11 June 2018	A Phase IIb Study of OligoG in Subjects With Cystic Fibrosis	A Double-blind, Randomized, Placebo-controlled Cross Over Study of Inhaled Alginate Oligosaccharide (OligoG) Administered for 28 Days in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: alginate oligosaccharide	AlgiPharma AS	Eurostars;Smerud Medical Research International AS	Not recruiting	18 Years	N/A	All	65	Phase 2	Denmark;Germany;Norway;Sweden;United Kingdom
318	NCT02194881	October 2014	16 December 2017	Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation	Ivacaftor in French Patients With Cystic Fibrosis and a G551D Mutation - Efficacy and Safety Results After the First Year of Treatment in the Real Life Setting.	Cystic Fibrosis	Drug: CF patients with a G551D mutation and treated with Ivacaftor	Assistance Publique - Hôpitaux de Paris	Vaincre la Mucoviscidose	Not recruiting	6 Years	N/A	All	57	N/A	France
319	NCT02227888	October 2014	14 November 2016	PK Study of N91115 in Cystic Fibrosis Patients	A Phase 1, Open-Label Study Evaluating the Pharmacokinetics of N91115 in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: N91115	Nivalis Therapeutics, Inc.		Not recruiting	18 Years	N/A	Both	6	Phase 1	United States
320	NCT02372383	October 2014	8 August 2016	Improving Treatment of Nontuberculous Mycobacterial Infection in Cystic Fibrosis	Pharmacokinetic Evaluation of Nontuberculous Mycobacterial Antibiotics in Cystic Fibrosis Versus Controls	Cystic Fibrosis	Drug: Ethambutol;Drug: Rifampin;Drug: Azithromycin;Drug: Pancrelipase	University of Colorado, Denver	Cystic Fibrosis Foundation Therapeutics;Colorado Clinical & Translational Sciences Institute	Not recruiting	16 Years	45 Years	Both	32	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
321	EUCTR2014-000844-13-DK	25/09/2014	8 August 2016	A phase IIb study of OligoG in subjects with cystic fibrosis	A double-blind, randomized, placebo-controlled cross over study of inhaled alginate oligosaccharide (OligoG) administered for 28 days in subjects with Cystic Fibrosis. - A phase IIb study of OligoG in subjects with cystic fibrosis	Cystic Fibrosis MedDRA version: 17.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: OligoG CF-5/20 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Oligomer of Sodium Alginate Current Sponsor code: OligoG Other descriptive name: OLIGOG CF-5/20 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 35- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Algipharma AS		Not Recruiting			Female: yes Male: yes	76	Phase 2	Denmark;Germany;Sweden
322	EUCTR2013-004595-35-GB	11/09/2014	11 June 2018	Safety and tolerability of BAY63-2521 in Cystic Fibrosis patients	Multi-center phase 2 study to assess the safety, tolerability and early signs of efficacy of tid orally administered BAY63-2521 in adult deltaF508 homozygous Cystic Fibrosis patients - Early signs of efficacy study with BAY63-2521 in adult homozygous deltaF508 Cystic Fibrosis patients	Cystic Fibrosis MedDRA version: 19.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Adempas Product Name: Adempas 0.5 mg Product Code: BAY63-2521 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Riociguat CAS Number: 625115-55-1 Current Sponsor code: BAY63-2521 Other descriptive name: RIOCIGUAT Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 0.5- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Trade Name: Adempas Product Name: Adempas 1.0 mg Product Code: BAY63-2521 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Riociguat CAS Number: 625115-55-1 Current Sponsor code: BAY63-2521 Other descriptive name: RIOCIGUAT Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 1- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Trade Name: Adempas Product Name: Adempas 2.0 mg Product Code: BAY63-2521 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Riociguat CAS Number: 625115-55-1 Current Sponsor code: BAY63-2521 Other descriptive name: RIOCIGUAT Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 2- Product Name: Adempas 0.125 mg Product Code: BAY63-2521 Pharmaceutical Form: Film-coated tablet	Bayer AG		Not Recruiting			Female: yes Male: yes	63	Phase 2	France;United States;Canada;Belgium;Netherlands;Germany;Italy;United Kingdom
323	NCT02212587	September 2014	20 June 2016	Tobramycin Inhalation Powder (TIP) in Cystic Fibrosis Patients Infected With Burkholderia Cepacia Complex	Pilot Study of the in Vivo Efficacy of Tobramycin Inhalation Powder (TIP) in Cystic Fibrosis Patients Infected With Burkholderia Cepacia Complex	Cystic Fibrosis;Burkholderia Cepacia Infection	Drug: TOBI	St. Michael's Hospital, Toronto		Not recruiting	6 Years	N/A	Both	10	Phase 1	Canada
324	NCT02803944	September 2014	27 June 2016	Continuous Azithromycin in Cystic Fibrosis Patients Beyond Two Years	Long Term Effects of Azithromycin in Patients With Cystic Fibrosis Using the National Database	Cystic Fibrosis	Drug: Azithromycin	Hospices Civils de Lyon		Not recruiting	7 Years	N/A	Both	2055	N/A	France
325	EUCTR2013-004987-80-BE	28/08/2014	7 October 2014	Colistin dosage through blood samples and sputum in Cystic Fibrosis (CF) population	Colistin use in Cystic Fibrosis patients: pharmacokinetic studies (intravenous administration) in relation with pharmacodynamics, tolerance profile and risk of selection of resistance.	Colistin use in Cystic Fibrosis patients MedDRA version: 16.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: COLISTINEB 2MUI Pharmaceutical Form: Powder for solution for injection/infusion INN or Proposed INN: Colistineb CAS Number: 8068-28-8 Other descriptive name: COLISTIMETHATE SODIUM (A COMPONENT) Concentration unit: million IU million international units Concentration type: equal Concentration number: 2-	CUB - Hôpital Erasme		Authorised			Female: yes Male: yes			Belgium
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
326	EUCTR2014-000817-30-NL	14/08/2014	8 August 2016	Comparing the effect of curcumin and genistein to treatment with Ivacaftor in CF patients with a class III mutation	Comparing the effect of curcumin and genistein to treatment with Ivacaftor in CF patients with a class III mutation - TICTAC-study	Cystic Fibrosis;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Ivacaftor/Kalydeco Pharmaceutical Form: Film-coated tablet Product Name: Curcuma Longa Extract cpl formaat 00 23,2 x 8,5 mm 723 mg Pharmaceutical Form: Capsule INN or Proposed INN: curcumin CAS Number: 458-37-7 Other descriptive name: CURCUMIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 600- Product Name: Genisteine Plus 150 mg cpl formaat 1 18,7 x 6,4 mm 438 mg Pharmaceutical Form: Capsule INN or Proposed INN: genistein CAS Number: 446-72-0 Other descriptive name: GENISTEIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 48.5-	University Medical Centre Utrecht		Not Recruiting			Female: yes Male: yes		Phase 2	Netherlands
327	EUCTR2014-002387-32-GB	05/08/2014	20 August 2018	Trial of Rosuvastatin for the Prevention of Kidney Toxicity caused by Tobramycin in Children with Cystic Fibrosis	Phase IIa, Randomised, Controlled, Open-Label Trial of Rosuvastatin for the Prevention of Aminoglycoside-Induced Kidney Toxicity in Children with Cystic Fibrosis - PROteKT	Aminoglycoside-induced nephrotoxicity MedDRA version: 18.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 18.0 Level: PT Classification code 10069022 Term: Kidney injury molecule-1 System Organ Class: 10022891 - Investigations MedDRA version: 18.0 Level: LLT Classification code 10067571 Term: Nephrotoxicity System Organ Class: 100000004857 ;Therapeutic area: Analytical, Diagnostic and Therapeutic Techniques and Equipment [E] - Therapeutic techniques [E02]	Trade Name: Crestor Product Name: Crestor Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Rosuvastatin calcium CAS Number: 287714-41-4 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 10-	University of Liverpool		Not Recruiting			Female: yes Male: yes	50	Phase 2	United Kingdom
328	NCT03391414	August 2014	8 January 2018	Effects of Inhaled Bicarbonate on Airway pH in Cystic Fibrosis	Effects of Inhaled Bicarbonate on Airway pH in Cystic Fibrosis	Cystic Fibrosis	Drug: hypertonic bicarbonate;Drug: sodium chloride	Joseph Pilewski	Cystic Fibrosis Foundation Therapeutics	Not recruiting	12 Years	N/A	All	12	Phase 1	United States
329	EUCTR2014-001401-41-NL	29/07/2014	11 August 2014	Once daily deep inhalation of tobramycin with smart nebulizer more effective to treat small airways disease in cystic fibrosis?	Targeting Antibiotics to Pseudomonas Aeruginosa in Small airways (TAPAS) study in patients with cystic fibrosis - TAPAS study in patients with CF	Cystic Fibrosis and chronic infection with Pseudomonas aeruginosa;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Bramitob 300mg/4ml Nebuliser Solution Pharmaceutical Form: Nebulisation solution			Authorised			Female: yes Male: yes	26		Netherlands
330	EUCTR2014-000284-40-GB	05/06/2014	10 April 2017	Cysteamine in Cystic Fibrosis	An open label investigation of the tolerability and pharmacokinetics of oral cysteamine in adults with Cystic Fibrosis. - Cysteamine in Cystic Fibrosis (2)	Cystic Fibrosis MedDRA version: 16.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Cystagon (cysteamine bitartrate) immediate-release capsules Product Name: Cystagon (cysteamine bitartrate) immediate-release capsules Pharmaceutical Form: Capsule, hard INN or Proposed INN: cysteamine bitartrate Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	University of Aberdeen	NHS Grampian	Not Recruiting			Female: yes Male: yes	12	Phase 1;Phase 2	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
331	NCT01576315	June 2014	25 April 2016	ATCF (Azole Therapy in Cystic Fibrosis)	Efficacy of Itraconazole and of Voriconazole in Patients With Cystic Fibrosis and Presenting With Persistent Positive Sputums for Aspergillus.	Cystic Fibrosis;Aspergillus Infections	Drug: Itraconazole/voriconazole	Rennes University Hospital		Not recruiting	12 Years	N/A	Both	11	Phase 2	France;United Kingdom
332	NCT02139306	June 2014	7 December 2015	Study of Ataluren in Nonsense Mutation Cystic Fibrosis (ACT CF)	A Phase 3 Efficacy and Safety Study of Ataluren (PTC124®) in Patients With Nonsense Mutation Cystic Fibrosis	Cystic Fibrosis	Drug: Ataluren (PTC124®);Drug: Placebo	PTC Therapeutics	Cystic Fibrosis Foundation Therapeutics;European Cystic Fibrosis Society-Clinical Trial Network (ECFS-CTN)	Not recruiting	6 Years	N/A	Both	208	Phase 3	United States;Argentina;Australia;Belgium;Brazil;Bulgaria;Canada;France;Germany;Greece;Israel;Italy;Netherlands;Poland;Spain;United Kingdom
333	NCT02141191	June 2014	16 December 2017	A Study of Lung Clearance After Hypertonic Saline Delivery Using the tPAD Device	A Randomized Crossover Mucociliary Clearance Study of Aerosolized 7% NaCl Solution Administered Overnight by the tPAD Device to Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: inhaled hypertonic saline (7%)	University of Pittsburgh	Parion Sciences	Not recruiting	18 Years	N/A	All	12	Phase 1	United States
334	NCT02107859	May 31, 2014	4 February 2019	Study of Ataluren (PTC124) in Cystic Fibrosis	An Open-Label Safety and Efficacy Study for Patients With Nonsense Mutation Cystic Fibrosis Previously Treated With Ataluren (PTC124)	Cystic Fibrosis	Drug: Ataluren (PTC124)	PTC Therapeutics		Not recruiting	6 Years	N/A	All	61	Phase 3	United States;Belgium;France;Israel;Italy;Spain;Sweden;Germany
335	EUCTR2013-005481-19-DK	27/05/2014	23 May 2016	High Dose Antioxidant Treatment for Patients with Cystic Fibrosis	High Dose Antioxidant Treatment for Patients with Cystic Fibrosis	Cystic Fibrosis MedDRA version: 17.0 Level: LLT Classification code 10068288 Term: Cystic fibrosis pulmonary exacerbation System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Mucolysin Pharmaceutical Form: Effervescent tablet INN or Proposed INN: ACETYLCYSTEINE Other descriptive name: ACETYLCYSTEINE Concentration unit: mg milligram(s) Concentration type: range Concentration number: 200-600 Trade Name: Mucomyst Pharmaceutical Form: Effervescent tablet INN or Proposed INN: ACETYLCYSTEINE Other descriptive name: ACETYLCYSTEINE Concentration unit: mg milligram(s) Concentration type: range Concentration number: 200-600 Trade Name: ACC Pharmaceutical Form: Tablet INN or Proposed INN: ACETYLCYSTEINE Other descriptive name: ACETYLCYSTEINE Concentration unit: mg milligram(s) Concentration type: range Concentration number: 200-600 Trade Name: Tad Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: GLUTATHIONE CAS Number: 8000050-16-0 Other descriptive name: GLUTATHIONE SODIUM Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 600-	Aarhus University Hospital		Not Recruiting			Female: yes Male: yes		Phase 2	Denmark
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
336	NCT02144246	May 2014	18 January 2016	Contraceptive Hormones and Women With Cystic Fibrosis	Contraceptive Hormones and Women With Cystic Fibrosis: Satisfaction and Effects on Disease	Cystic Fibrosis Exacerbations While on and Off Hormonal Contraception	Other: Non-hormonal period;Drug: Ortho-cyclen, Ethinyl estradiol/norgestimate, 0.035 mg/0.250 mg	University of Pennsylvania	Society of Family Planning	Not recruiting	18 Years	40 Years	Female	5	Phase 1	United States
337	EUCTR2013-004295-35-AT	30/04/2014	27 October 2014	Lung Clearance Index as an OUTcome parameter to detect the efficacy f Aztreonam Lysine Inhalation in cystic fibrosis patients with near normal spirometry - an observational proof-of concept study	Lung Clearance Index as an OUTcome parameter to detect the efficacy f Aztreonam Lysine Inhalation in cystic fibrosis patients with near normal spirometry - an observational proof-of concept study - LCI-OUT	Chronic lung P. Aeruginosa Infection MedDRA version: 16.1 Level: PT Classification code 10061229 Term: Lung infection System Organ Class: 10021881 - Infections and infestations ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Cayston 75 mg Pulver und Lösungsmittel zur Herstellung einer Lösung für einen Vernebler Product Name: Cayston 75 mg Pulver und Lösungsmittel zur Herstellung einer Lösung für einen Vernebler Pharmaceutical Form: Powder and solvent for nebuliser solution INN or Proposed INN: aztreonam CAS Number: 827611-49-4 Other descriptive name: AZTREONAM LYSINE Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75-75 Trade Name: Colistin Forest - Trockenstechampullen mit Lösungsmittel Product Name: Colistin Forest - Trockenstechampullen mit Lösungsmittel Pharmaceutical Form: Powder and solvent for nebuliser solution INN or Proposed INN: COLISTIMETHATE SODIUM CAS Number: 8068-28-8 Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 1.000.000-1.000.000 Trade Name: TOBI Pharmaceutical Form: Nebuliser solution INN or Proposed INN: TOBRAMYCIN SULFATE CAS Number: 79645-27-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300-300	Medical University Innsbruck - Department für Kinder- und Jugendheilkunde (Pädiatrie III)		Authorised			Female: yes Male: yes			Austria
338	EUCTR2014-000057-37-NL	30/04/2014	8 August 2016	Bronchodilcation as a CFTR activator in CF	A B2-agonist as a CFTR activator in CF - ABBA study	Cystic Fibrosis;Therapeutic area: Body processes [G] - Metabolic Phenomena [G03]	Product Name: Salbutamol Product Code: R03AC02 Pharmaceutical Form: Inhalation vapour INN or Proposed INN: SALBUTAMOL CAS Number: 18559-94-9 Concentration unit: µg microgram(s) Concentration type: up to Concentration number: 400- Product Name: Salbutamol Product Code: R03CC02 Pharmaceutical Form: Tablet INN or Proposed INN: SALBUTAMOL CAS Number: 18559-94-9 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 4-	University Medical Centre Utrecht		Not Recruiting			Female: yes Male: yes		Phase 2	Netherlands
339	EUCTR2013-005366-19-GB	29/04/2014	3 April 2017	A Multi-centre, Randomised, Double-Blind, Placebo-Controlled Study to evaulate the Safety and Efficacy of Pulmaquin® (ARD-3150, Dual Release Coprofloxacin for Inhalation) in subjects who have a lung infection that includes the bacteria Pseudomonas aeruginosa due to the non-cystic fibrosis bronchiectasis	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety and Efficacy of Pulmaquin® in the Management of Chronic Lung Infections with Pseudomonas aeruginosa in Subjects with Non-Cystic Fibrosis Bronchiectasis, including 28 Day Open-Label Extension (ORBIT-4) - Pulmaquin® with Non-Cystic Fibrosis Bronchiectasis (Orbit 4)	Chronic lung infections with Pseudomonas aeruginosa in subjects with non-cystic fibrosis bronchiectasis MedDRA version: 16.1 Level: LLT Classification code 10057582 Term: Lung infection pseudomonal System Organ Class: 100000004862 MedDRA version: 16.1 Level: LLT Classification code 10006446 Term: Bronchiectasis NOS System Organ Class: 100000004855 ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Pulmaquin® Product Code: ARD-3150 Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Ciprofloxacin Hydrochloride CAS Number: 86483-48-9 Current Sponsor code: Ciprofloxacin for Inhalation (CFI) Other descriptive name: CIPROFLOXACIN HYDROCHLORIDE Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 50- INN or Proposed INN: Ciprofloxacin Hydrochloride CAS Number: 86483-48-9 Current Sponsor code: Free Ciprofloxacin for Inhalation (FCI) Other descriptive name: CIPROFLOXACIN HYDROCHLORIDE Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 20- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Aradigm Corporation		Not Recruiting			Female: yes Male: yes	255	Phase 3	United States;Spain;Israel;United Kingdom;Italy;France;Hungary;Canada;Poland;Romania;Australia;Netherlands;Germany;New Zealand
340	EUCTR2013-004659-19-DE	23/04/2014	22 May 2017	Ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis (non–CF BE)	Randomized, double-blind, placebo-controlled, multicenter study comparing Ciprofloxacin DPI 32.5 mg BID intermittently administered for 28 days on / 28 days off or 14 days on / 14 days off versus placebo to evaluate the time to first pulmonary exacerbation and frequency of exacerbations in subjects with non–cystic fibrosis bronchiectasis. - Respire 2	non-CF bronchiectasis MedDRA version: 19.0 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Ciprofloxacin DPI Product Code: BAYQ3939 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Ciprofloxacin Current Sponsor code: BAYq3939 Other descriptive name: CIPROFLOXACIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 32.5- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Bayer AG		Not Recruiting			Female: yes Male: yes	492	Phase 3	Serbia;United States;Portugal;Philippines;Taiwan;Hong Kong;Slovakia;Thailand;Russian Federation;Australia;South Africa;Latvia;Netherlands;China;Korea, Republic of;Turkey;Lithuania;Austria;Czech Republic;Hungary;Canada;Argentina;Brazil;Poland;Romania;Bulgaria;Germany;New Zealand
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
341	EUCTR2013-004659-19-NL	23/04/2014	5 December 2016	Ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis (non–CF BE)	Randomized, double-blind, placebo-controlled, multicenter study comparing Ciprofloxacin DPI 32.5 mg BID intermittently administered for 28 days on / 28 days off or 14 days on / 14 days off versus placebo to evaluate the time to first pulmonary exacerbation and frequency of exacerbations in subjects with non–cystic fibrosis bronchiectasis. - Respire 2	non-CF bronchiectasis MedDRA version: 19.0 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Ciprofloxacin DPI Product Code: BAYQ3939 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Ciprofloxacin Current Sponsor code: BAYq3939 Other descriptive name: CIPROFLOXACIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 32.5- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Bayer AG		Not Recruiting			Female: yes Male: yes	492	Phase 3	Portugal;Serbia;United States;Philippines;Hong Kong;Taiwan;Slovakia;Thailand;Russian Federation;Australia;South Africa;Latvia;Netherlands;China;Korea, Republic of;Lithuania;Turkey;Austria;Czech Republic;Argentina;Brazil;Poland;Romania;Bulgaria;Germany
342	NCT01515007	April 2014	29 January 2018	Phase 3 Study With Ciprofloxacin Dispersion for Inhalation in Non-CF Bronchiectasis (ORBIT-3)	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety and Efficacy of Pulmaquin® in the Management of Chronic Lung Infections With Pseudomonas Aeruginosa in Subjects With Non-Cystic Fibrosis Bronchiectasis, Including 28 Day Open-Label Extension and Pharmacokinetic Substudy (ORBIT-3)	Non Cystic Fibrosis Bronchiectasis	Drug: Ciprofloxacin dispersion for inhalation;Drug: Placebo	Aradigm Corporation	Grifols Therapeutics Inc.	Not recruiting	18 Years	N/A	All	278	Phase 3	United States;Australia;Canada;Germany;Hungary;Ireland;Israel;Italy;Korea, Republic of;Latvia;Poland;Romania;South Africa;Spain;Taiwan;United Kingdom;New Zealand
343	NCT01991028	April 2014	19 February 2015	A Study to Investigate Lung Deposition of Radiolabelled OligoG	An Open Label, Randomised, Two-way Crossover Scintigraphic Study to Investigate Lung Deposition of Radiolabelled OligoG Delivered as a Dry Powder and as a Nebulised Solution in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Radiolabelled OligoG CF-5/20 DPI;Drug: Radiolabelled OligoG CF-5/20 6% Solution	Bio-Images Research Ltd	AlgiPharma AS	Not recruiting	18 Years	N/A	Both	10	Phase 2	United Kingdom
344	NCT02057458	April 2014	21 January 2019	Blood Flow and Vascular Function in Cystic Fibrosis	Role of Blood Flow and Vascular Function on Exercise Capacity in Cystic Fibrosis	Cystic Fibrosis	Drug: Acute (1 hour) Sildenafil;Drug: Sub-Chronic (4 weeks) Sildenafil;Drug: Placebo	Augusta University	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	Not recruiting	18 Years	N/A	All	50	Phase 2	United States
345	NCT02113397	April 2014	11 June 2018	Evaluation of Inhaled Antibiotics on Bacterial Diversity and Richness in the Cystic Fibrosis Lung	Comparative Evaluation of Bacterial Diversity and Richness in CF Lung in Patients Who Use Cycled Every Other Month Tobramycin Inhalation Powder (TOBI™ Podhaler™) Or Continuous Alternating Therapy With Tobramycin Inhalation Powder (TOBI™ Podhaler™) and Inhaled Colistimethate	Cystic Fibrosis	Drug: TOBI™ Podhaler™ 112 mg inhaled twice daily;Drug: Colistimethate 75 mg inhaled two times daily	Dartmouth-Hitchcock Medical Center	Novartis Pharmaceuticals	Not recruiting	12 Years	75 Years	All	1	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
346	NCT02141958	April 2014	21 July 2016	Safety Study of Fenretinide in Adult Patients With Cystic Fibrosis	An Adaptive Phase I Intra-patient Dose Escalation Study of Fenretinide in Adult Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Fenretinide;Drug: Placebo	Elias Matouk		Not recruiting	18 Years	N/A	Both	15	Phase 1	Canada
347	NCT03341741	March 11, 2014	16 December 2017	Combined Dry Powder Tobramycin and Nebulized Colistin Inhalation in CF Patients	Combined Dry Powder Tobramycin and Nebulized Colistin Inhalation in CF Patients	Cystic Fibrosis With Pulmonary Manifestations	Drug: Tobramycin Powder;Drug: Colistin	University Hospital Tuebingen		Not recruiting	12 Years	N/A	All	26	Phase 3
348	NCT02070744	March 2014	11 June 2018	Study to Evaluate Safety and Efficacy of VX-661 in Combination With Ivacaftor in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation With an Open-Label Expansion	A Phase 2, Randomized, Multicenter, Double Blind, Placebo Controlled Study to Evaluate Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of VX-661 in Combination With Ivacaftor for 12 Weeks in Subjects With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation With an Open-Label Extension	Cystic Fibrosis	Drug: VX-661;Drug: Ivacaftor;Drug: Placebo matched to VX-661;Drug: Placebo matched to Ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	40	Phase 2	United States
349	NCT02081963	March 2014	29 April 2019	Combined Administration of Nebulized Amikacin in Patients With Acute Exacerbation of Non-Cystic Fibrosis Bronchiectasis	A Randomized, Controlled Study of Combined Administration of Nebulized Amikacin in Patients With Acute Exacerbation of Non-Cystic Fibrosis Bronchiectasis	Non-Cystic Fibrosis Bronchiectasis	Drug: Amikacin;Drug: Normal saline	Qilu Hospital of Shandong University		Not recruiting	18 Years	80 Years	All	178	Phase 4	China
350	NCT02084043	March 2014	29 June 2015	In Vitro Assessment of a Breath-synchronized Vibrating Mesh Nebulizer During Non Invasive Ventilation	In Vitro Comparison of Continuous and Breath-synchronized Vibrating Mesh Nebulizer During Non Invasive Ventilation: Analysis of Inhaled and Lost Doses.	Respiratory Diseases;Lung Diseases;Cystic Fibrosis;COPD;Asthma	Drug: Nebulization of Amikacin during NIV (RR: 15 cycles/minute);Drug: Nebulization of Amikacin during NIV (RR: 25 cycles/minute)	University Hospital St Luc, Brussels	Université Catholique de Louvain;University of Applied Sciences of Western Switzerland;School of Gestion and Engineering Vaud, Switzerland	Not recruiting	N/A	N/A	Both	3	N/A	Belgium;Switzerland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
351	NCT02088216	March 2014	8 August 2016	Effect of Long-term, High-dose N-acetylcysteine on Exacerbations of Bronchiectaisis	Effect of Long-term, High-dose N-acetylcysteine on Acute Exacerbations of Patients With Non-cystic Fibrosis Bronchiectasis: the BENE Randomized, Controlled Trial	Non-Cystic Fibrosis Bronchiectasis	Drug: N-acetylcysteine;Drug: Placebo	Qilu Hospital		Not recruiting	18 Years	80 Years	Both	150	Phase 4	China
352	NCT02148978	March 2014	19 February 2015	Cystic Fibrosis Related Diabetes	DPP-IV Inhibition in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Saxagliptin	Ram Weiss		Recruiting	18 Years	65 Years	Both	30	N/A	Israel
353	NCT01746784	February 2014	19 October 2017	Safety and Pharmacokinetic Study of N6022 in Subjects With Cystic Fibrosis Homozygous for the F508del-CFTR Mutation	A Phase 1b, Randomized, Double-Blind, Placebo-Controlled, Dose Escalation Study of N6022 to Evaluate Safety and Pharmacokinetics in Subjects With Cystic Fibrosis Homozygous for the F508del-CFTR Mutation (SNO1)	Cystic Fibrosis	Drug: N6022;Drug: Normal saline	Nivalis Therapeutics, Inc.		Not recruiting	18 Years	N/A	All	66	Phase 1	United States
354	NCT01937325	February 2014	8 August 2016	CPET in CF Patients With One G551D Mutation Taking VX770	Airway Infection, Inflammatory Markers and Exercise Capacity in Patients With Cystic Fibrosis and at Least One G551D Mutation Taking VX770 (Ivacaftor)	Cystic Fibrosis	Drug: ivacaftor;Drug: placebo	The Alfred		Not recruiting	16 Years	70 Years	Both	20	Phase 4	Australia
355	EUCTR2013-004488-30-NL	31/01/2014	10 February 2014	Targeting Antibiotics to Pseudomonas Aeruginosa in Small airways (TAPAS) study in patients with cystic fibrosis: pharmacokinetics (PK)	Targeting Antibiotics to Pseudomonas Aeruginosa in Small airways (TAPAS) study in patients with cystic fibrosis: pharmacokinetics (PK) - TAPAS-PK study in patients with CF	Cystic fibrosis;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Bramitob Pharmaceutical Form: Inhalation vapour, liquid INN or Proposed INN: TOBRAMYCIN CAS Number: 32986-56-4 Current Sponsor code: TAPAS-PK-2014 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: up to Concentration number: 600-	Haga Hospital		Authorised			Female: yes Male: yes			Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
356	EUCTR2013-000604-41-IT	21/01/2014	13 June 2016	A study in people with Cystic Fibrosis (a rare hereditary pulmonary disease) to assess the long-term efficacy and safety of a combination of two experimental drugs	A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic fibrosis in patients homozygous or heterozygous for the F508del-CFTR Mutation MedDRA version: 16.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor/ivacaftor 200mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: lumacaftor/ivacaftor 200mg/83mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 83- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: ivacaftor 125mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	1000	Phase 3	United States;Spain;Ireland;Austria;United Kingdom;Italy;France;Czech Republic;Canada;Belgium;Australia;Denmark;Netherlands;Germany;Sweden
357	EUCTR2013-003774-27-GB	20/01/2014	3 April 2017	A study to investigate lung deposition of radiolabelled OligoG(v1.0)	An open label, randomised, two-way crossover scintigraphic study to investigate lung deposition of radiolabelled OligoG delivered as a dry powder and as a nebulised solution in cystic fibrosis patients - A study to investigate lung deposition of radiolabelled OligoG (v1.0)	Cystic fibrosis (CF) is an autosomal, recessive inheritable disease caused by a homozygote defect at the long arm of Chromosome 7. This mutation causes absence or defect of the cystic fibrosis transmembrane conductance regulator, an ion channel transporting chloride and bicarbonate ions across the cell membrane in exocrine glands. Decreased chloride transport leads to dehydration of the mucus layer, and decreased bicarbonate to increased mucus adhesion. Mucus stagnation results.;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Radiolabelled OligoG CF-5/20 Dry Powder for Inhalation (DPI) Pharmaceutical Form: Inhalation powder INN or Proposed INN: OligoG CAS Number: 9005-38-3 Current Sponsor code: OligoG CF-5/20 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 96- Product Name: Radiolabelled OligoG CF-5/20 6% Solution for Nebulisation Pharmaceutical Form: Nebuliser solution INN or Proposed INN: OligoG CAS Number: 9005-38-3 Current Sponsor code: OligoG CF-5/20 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 90-	AlgiPharma AS		Not Recruiting			Female: yes Male: yes	12	Phase 2	United Kingdom
358	NCT02015507	January 2014	19 February 2015	An Open-Label, Phase 1 Study in Healthy Adult Subjects to Examine the Effects of Multiple-Dose Ciprofloxacin on Ivacaftor and VX-661 in Combination With Ivacaftor	An Open-Label, Phase 1 Study in Healthy Adult Subjects to Examine the Effects of Multiple-Dose Ciprofloxacin on the Multiple-Dose Pharmacokinetics of Ivacaftor and on the Multiple-Dose Pharmacokinetics of VX-661 Administered in Combination With Ivacaftor	Cystic Fibrosis	Drug: ivacaftor;Drug: VX-661;Drug: ciprofloxacin	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	34	Phase 1
359	NCT02015663	January 2014	16 December 2017	Tobramycin Inhalation Powder (TIP) Administered Once Daily Continuously Versus TIP Administered BID in 28 Day on / 28 Day Off Cycles	A 24-week, Open-label, Parallel-group, Interventional Phase IV Study Comparing Tobramycin Inhalation Powder (TIP) Administered Once Daily Continuously Versus TIP Administered BID in 28 Day on / 28 Day Off Cycles for the Treatment of Pulmonary Pseudomonas Aeruginosa in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Tobramycin Inhalation Powder	Novartis Pharmaceuticals		Not recruiting	6 Years	N/A	All	32	Phase 4	United States
360	NCT02043717	January 2014	16 December 2017	Increased Vitamin D Reduces Pulmonary Exacerbations in CF	Increasing Vitamin D Serum Levels Reduces Pulmonary Exacerbations in Patients With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Vitamin D	Hadassah Medical Organization		Not recruiting	4 Years	N/A	All	90	N/A	Israel
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
361	EUCTR2014-003023-22-FR		2 October 2017	Open trial with randomized withdrawal of treatment, to evaluate the efficacy of azithromycin in the treatment of bronchiectasis excluding cystic fibrosis in children (AZI-STOP study)	Open trial with randomized withdrawal of treatment, to evaluate the efficacy of azithromycin in the treatment of bronchiectasis excluding cystic fibrosis in children (AZI-STOP study) - AZI-STOP study	bronchiectasis excluding cystic fibrosis in children;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: ZITHOMAX 40 mg/ml ENFANTS Product Name: zithromax Pharmaceutical Form: Powder for oral suspension INN or Proposed INN: AZITHROMYCIN DIHYDRATE Current Sponsor code: azithromycine Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 41,92- Trade Name: ZITHROMAX 250 mg Product Name: ZITHROMAX Pharmaceutical Form: Tablet INN or Proposed INN: AZITHROMYCIN DIHYDRATE Current Sponsor code: azithromycine Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 262-	ASSISTANCE PUBLIQUE HOPITAUX DE MARSEILLE		Not Available			Female: yes Male: yes		Phase 3	France
362	EUCTR2014-003882-10-FR		2 October 2017	Evaluation of the efficacy of antibiotic treatments associated with the Nebcine® as intravenous injection only and / or monitoring of aerosols of Tobi® in order to optimize the therapeutic management of exacerbations in patients with cystic fibrosis .	Evaluation of the effectiveness of a treatment involving one (or several ) antibiotic (s) with 14-day tobramycin ( Nebcine® ) by intravenous injection versus the same antibiotic treatment (s ) associated with only 5 days of tobramycin ( Nebcine® ) by intravenous injection followed tobramycin aerosol ( Tobi® ) for 9 days in the context of cystic fibrosis	mucoviscidosis or cystic fibrosis MedDRA version: 18.0 Level: LLT Classification code 10068288 Term: Cystic fibrosis pulmonary exacerbation System Organ Class: 100000004862 ;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Trade Name: NEBCINE Product Name: NEBCINE Pharmaceutical Form: Solution for injection CAS Number: 32986-56-4 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Trade Name: NEBCINE Product Name: NEBCINE Pharmaceutical Form: Solution for injection CAS Number: 32986-56-4 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100-0 Trade Name: NEBCINE Product Name: NEBCINE Pharmaceutical Form: Solution for injection CAS Number: 32986-56-4 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Trade Name: NEBCINE Product Name: NEBCINE Pharmaceutical Form: Solution for injection CAS Number: 32986-56-4 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Trade Name: TOBI Product Name: TOBI Pharmaceutical Form: Nebuliser solution CAS Number: 32986-56-4 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300-	CHRU de Lille		Not Available			Female: yes Male: yes		Phase 2	France
363	NCT01946412	December 2013	19 October 2017	Roll-Over Study of Ivacaftor in Cystic Fibrosis Pediatric Subjects With a CF Transmembrane Conductance Regulator Gene (CFTR) Gating Mutation	A Phase 3, 2-Arm, Roll-Over Study to Evaluate the Long-term Safety and Pharmacodynamics of Ivacaftor Treatment in Pediatric Subjects With Cystic Fibrosis and a CFTR Gating Mutation	Cystic Fibrosis	Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	2 Years	N/A	All	33	Phase 3	United States;Canada;United Kingdom
364	NCT01958944	December 2013	13 June 2016	Evaluation of the Safety and Tolerability of Inhaled Nitric Oxide to Subjects With Cystic Fibrosis	Phase II Prospective, Open Labeled, Multi-Center, Evaluation of the Safety and Tolerability of Nitric Oxide Given Intermittently Via Inhalation to Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: Nitric oxide	Advanced Inhalation Therapies Ltd	Soroka University Medical Center;Schneider Children's Medical Center, Israel	Not recruiting	10 Years	N/A	Both	9	Phase 1/Phase 2	Israel
365	NCT01975259	December 2013	14 September 2015	Investigating the Incretin Effect in Cystic Fibrosis	Investigating the Incretin Effect in Cystic Fibrosis	Cystic Fibrosis	Drug: Oral Glucose Tolerance test (75g 2-hour);Drug: Modified Oral Glucose Tolerance Test (50g 4-hours);Drug: Matched isoglycemic clamp;Drug: Hyperglycemic clamp with concurrent GLP-1 infusion;Drug: Hyperglycemic Clamp with concurrent GIP infusion;Drug: Hyperglycemic clamp with placebo infusion;Other: Liquid Meal Test (Carbohydrate-rich);Other: Liquid Meal Test (Fat-rich);Other: Liquid Meal Test (Mixed);Device: Continuous Glucose Monitoring	Liverpool Heart and Chest Hospital NHS Foundation Trust		Not recruiting	17 Years	50 Years	Both	50	N/A	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
366	NCT02036879	December 2013	11 February 2019	Gender Disparity and Hormones in Cystic Fibrosis	Gender Disparity and Hormones in Cystic Fibrosis	Cystic Fibrosis (CF)	Drug: Loestrin (norethindrone acetate and ethinyl estradiol)	University of Texas Southwestern Medical Center		Not recruiting	18 Years	N/A	All	55	N/A	United States
367	NCT02096315	December 2013	16 December 2017	Safety, Efficacy and PK/PD of POL7080 in Patients With Exacerbation of Non-cystic Fibrosis Bronchiectasis.	A Phase II, Open-label, Multicenter Study to Assess the Tolerance, Safety, Efficacy and Pharmacokinetics/Pharmacodynamics (PK/PD) of POL7080 in the Treatment of Patients With Acute Exacerbation of Non-cystic Fibrosis Bronchiectasis Due to Pseudomonas Aeruginosa Infection Requiring Intravenous Treatment	Bronchiectasis;Lower Respiratory Infection	Drug: POL7080	Polyphor Ltd.		Not recruiting	18 Years	79 Years	All	20	Phase 2	Spain;United Kingdom
368	EUCTR2013-002819-10-HU	29/11/2013	27 October 2014	A Double-blind, Randomized, Multicenter, Cross-over Study to Compare the Effect of Creon N and Creon® on Fat Digestion in Subjects = 12 years of Age with Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	A Double-blind, Randomized, Multicenter, Cross-over Study to Compare the Effect of Creon N and Creon® on Fat Digestion in Subjects = 12 years of Age with Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Pancreatic exocrine Insufficiency due to Cystic Fibrosis;Therapeutic area: Diseases [C] - Immune System Diseases [C20]	Product Name: CREON N 25000 Pharmaceutical Form: Capsule, hard INN or Proposed INN: not applicable CAS Number: 8049-47-6 Current Sponsor code: CREON N Other descriptive name: PANCREATIN Concentration unit: IU/kg international unit(s)/kilogram Concentration type: equal Concentration number: 25000- Trade Name: Creon 25000 Pharmaceutical Form: Capsule, hard INN or Proposed INN: not assigned CAS Number: 8049-47-6 Other descriptive name: PANCREATIN Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 25000-	Abbott Laboratories GmbH		Not Recruiting			Female: yes Male: yes	40		Hungary;Spain;Russian Federation
369	NCT00924547	November 2013	3 August 2015	Oral Docosahexanoic Acid Supplementation in Cystic Fibrosis	Oral Docosahexanoic Acid Supplementation in Cystic Fibrosis: Effects on Exhaled Pro-inflammatory Isoprostanes and Analysis of Its Esterification Sites in Plasma	Cystic Fibrosis	Dietary Supplement: Docosahexanoic Acid Supplement;Dietary Supplement: Placebo	Vanderbilt University		Not recruiting	6 Years	N/A	Both	17	Phase 2	United States
370	NCT01702415	October 2013	2 November 2015	Zoledronic Acid in Cystic Fibrosis	Randomised, Double Blind, Placebo Controlled Trial to Ascertain the Efficacy and Safety of Intravenous Zoledronic Acid in Adult Patients With Cystic Fibrosis.	Cystic Fibrosis	Drug: Zoledronic acid;Drug: Placebo	Papworth Hospital NHS Foundation Trust	Novartis Pharmaceuticals	Not recruiting	18 Years	N/A	Both	0	Phase 4	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
371	NCT01784419	October 2013	19 February 2015	Short Term Effects of Ivacaftor in Non-G551D Cystic Fibrosis Patients	Short Term Effects of Ivacaftor in Non-G551D Cystic Fibrosis Patients	Cystic Fibrosis	Drug: ivacaftor	University of California, San Francisco		Recruiting	6 Years	N/A	Both	30	N/A	United States
372	NCT01880346	October 2013	7 December 2015	Comparison of Absorption of Vitamin D in Cystic Fibrosis	Vehicles for the Absorption of Vitamin D in Cystic Fibrosis: Comparison of Powder vs Oil	Cystic Fibrosis	Dietary Supplement: Vitamin D Powder;Dietary Supplement: Vitamin D Oil	Emory University		Not recruiting	18 Years	59 Years	Both	17	Phase 4	United States
373	NCT01931839	October 2013	16 December 2017	A Phase 3 Rollover Study of Lumacaftor in Combination With Ivacaftor in Subjects 12 Years and Older With Cystic Fibrosis	A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Drug: Lumacaftor Plus Ivacaftor Combination;Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	1164	Phase 3	United States;Australia;Austria;Belgium;Canada;Czech Republic;Denmark;France;Germany;Ireland;Italy;Netherlands;Spain;Sweden;United Kingdom
374	NCT02035488	October 2013	19 February 2015	Pharmacokinetic Evaluation and Tolerability of Dry Powder Tobramycin by a Novel Device in Patients With Non Cystic Fibrosis Bronchiectasis	Pharmacokinetic Evaluation and Tolerability of Dry Powder Tobramycin by a Novel Device in Patients With Non Cystic Fibrosis Bronchiectasis	Bronchiectasis	Drug: Tobramycin	University Medical Centre Groningen		Not recruiting	18 Years	N/A	Both	8	Phase 1/Phase 2	Netherlands
375	EUCTR2013-001258-82-IT	12/09/2013	18 January 2016	Phase II clinical study for the therapy of cystic fibrosis patients with a specific mutation	A phase II pilot clinical study of experimental research to evaluate the functional rescue of CFTR protein through proteostasis regulators - Proteostasis regulators effect in cystic fibrosis therapy	Cystic fibrosis patients with F508del-CFTR in homozygous or compound heterozygous with Class I or II mutations;Therapeutic area: Body processes [G] - Genetic Phenomena [G05]	Trade Name: Cystagon Product Name: Cysteamine bitartrate (Cystagon) Product Code: EMEA/H/C/000125 Pharmaceutical Form: Capsule	European Institute for Cystic Fibrosis Research (IERFC)		Not Recruiting			Female: yes Male: yes		Phase 2	Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
376	EUCTR2013-002202-31-GB	11/09/2013	14 April 2014	Study conducted in several hospitals to verify the tolerance, safety and efficacy to cure of the study medication (POL7080) and its distribution in the body when given to patients with bronchiectasis caused by bacterium Pseudomonas aeruginosa,requiring intravenous treatment.	A phase II, open-label, multi-center study to assess the tolerance, safety, efficacy and pharmacokinetics/pharmacodynamics (PK/PD) of POL7080 in the treatment of patients with acute exacerbation of non-cystic fibrosis bronchiectasis due to Pseudomonas aeruginosa infection requiring intravenous treatment. - POL7080-002	Acute exacerbation of non-cystic fibrosis bronchiectasis due to Pseudomonas aeruginosa infection.;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Product Name: POL7080 Product Code: POL7080 Pharmaceutical Form: Solution for infusion INN or Proposed INN: POL7080 CAS Number: 944252-63-5 Current Sponsor code: POL7080 Other descriptive name: POL7080 Concentration unit: mg/kg milligram(s)/kilogram Concentration type: equal Concentration number: 7.5-	Polyphor Ltd.		Authorised			Female: yes Male: yes	20	Phase 2	Spain;United Kingdom
377	NCT01944735	September 2013	16 March 2015	Phase 1 Study Assessing the Safety and Tolerability of CTX-4430 in Cystic Fibrosis Patients	A Phase 1, Randomized, Double-Blind, Placebo-Controlled, Ascending Multiple-Dose Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of CTX-4430 When Administered Orally to Cystic Fibrosis Patients for Fifteen Days	Cystic Fibrosis	Drug: CTX-4430;Drug: Placebo	Celtaxsys, Inc.	Celerion	Not recruiting	18 Years	55 Years	Both	17	Phase 1	United Kingdom
378	NCT01953367	September 2013	19 February 2015	Bioequivalence and Safety of Vantobra and TOBI in Healthy Subjects	Bioequivalence and Safety Study of Vantobra and TOBI Nebulizer Solutions in Healthy Subjects	Cystic Fibrosis	Drug: Vantobra (tobramycin);Drug: TOBI (tobramycin)	Pari Pharma GmbH		Not recruiting	18 Years	50 Years	Both	72	Phase 1	Germany
379	NCT02048592	September 2013	19 February 2015	Impact of Immunonutrition on the Patients With Cystic Fibrosis	Impact of Immunonutrition on the Patients With Cystic Fibrosis	Cystic Fibrosis;Malnutrition;Oxidative Stress	Dietary Supplement: Impact-Nutridrink;Dietary Supplement: Nutridrink-Impact	University Hospital, Motol		Recruiting	18 Years	40 Years	Both	30	Phase 4	Czech Republic
380	NCT02288429	September 2013	15 July 2019	Evaluation of Pharmacokinetic and Pharmacodynamic Properties of Intravenous Colistimethate Sodium	Evaluation of Steady-state Pharmacokinetic and Pharmacodynamic Properties of Intravenous Colistimethate Sodium in Cystic Fibrosis and Critically Ill Patients	Colistin	Drug: colistimethate sodium	University of Colorado, Denver		Not recruiting	18 Years	89 Years	All	16	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
381	NCT01885169	August 2013	19 February 2015	LAIV (Flumist®) Administration in CF Patients	Safety of Live-attenuated Influenza Vaccine (LAIV, Flumist®) in Patients With Cystic Fibrosis (CF)	Cystic Fibrosis	Biological: Flumist®	McGill University Health Center	Ministere de la Sante et des Services Sociaux;Canadian Cystic Fibrosis Foundation;PHAC/CIHR Influenza Research Network	Not recruiting	2 Years	18 Years	Both	170	N/A	Canada
382	NCT01916577	August 2013	19 February 2018	Autologous CD117+ Progenitor Cell Mobilization for Lung Transplantation	Autologous CD117+ Progenitor Cell Mobilization for Lung Transplantation	COPD;Cystic Fibrosis;Pulmonary Fibrosis	Drug: Plerixafor mobilization of autologous CD117 stem cells	University of Colorado, Denver	Sanofi	Not recruiting	18 Years	70 Years	All	15	Phase 1	United States
383	EUCTR2012-000386-20-GB	31/07/2013	28 September 2015	A Roll-over study in children with Cystic Fibrosis (a rare hereditary pulmonary disease) to assess the long-term safety and pharmacodynamics of ivacaftor	A Phase 3, 2-Arm, Roll-Over Study to Evaluate the Long-term Safety and Pharmacodynamics of Ivacaftor Treatment in Pediatric Subjects With Cystic Fibrosis and a CFTR Gating Mutation	cystic fibrosis MedDRA version: 18.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: Ivacaftor Product Code: VX-770 Pharmaceutical Form: Granules INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Trade Name: Kalydeco Product Name: ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	35	Phase 3	United States;Canada;United Kingdom
384	EUCTR2013-000219-25-IT	18/07/2013	30 April 2018	MRSA study.	Early eradication of S. Aureus (MRSA) in patients with cystic fibrosis: a randomized multicenter study.	Patients affected by cystic fibrosis and who present an initial infection of MRSA.;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Product Name: Rifampin Pharmaceutical Form: Tablet INN or Proposed INN: RIFAMPICIN CAS Number: 13292-46-1 Product Name: Trimethoprim-sulfamethoxazole Pharmaceutical Form: Tablet INN or Proposed INN: SULFAMETHOXAZOLE CAS Number: 723-46-6 Other descriptive name: TRIMETHOPRIM Product Name: minocycline Pharmaceutical Form: Tablet CAS Number: 13614-98-7 Other descriptive name: MINOCYCLINE HYDROCHLORIDE	AOU Meyer		Authorised			Female: yes Male: yes	100	Phase 3	Italy
385	NCT01155752	July 2013	16 December 2017	Pulmozyme in Cystic Fibrosis With Sinusitis	Z4770s, Use of Recombinant Human DNASE in Cystic Fibrosis Patients With Chronic Sinusitis to Prevent Acute Sinusitis Exacerbations and Improve Symptoms and Outcomes - A Pilot Study	Sinusitis;Cystic Fibrosis	Drug: Pulmozyme single use ampule;Drug: placebo	Milton S. Hershey Medical Center	Genentech, Inc.	Not recruiting	12 Years	65 Years	All	0	Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
386	NCT01897233	July 2013	16 December 2017	Study of Lumacaftor in Combination With Ivacaftor in Subjects 6 Through 11 Years of Age With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	A Phase 3, Open-label Study to Evaluate the Pharmacokinetics, Safety, and Tolerability of Lumacaftor in Combination With Ivacaftor in Subjects 6 Through 11 Years of Age With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: Lumacaftor;Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	11 Years	All	62	Phase 3	United States;Canada
387	NCT01899105	July 2013	19 February 2015	A Phase 1 Study to Investigate the Food Effect of Lumacaftor in Combination With Ivacaftor	A Phase 1, Randomized, Single-Dose, Open-Label Crossover Study to Investigate the Effect of Food on the Relative Bioavailability of 2 Fixed-Dose Combinations of Lumacaftor and Ivacaftor Tablet Formulations in Healthy Adult Subjects	Cystic Fibrosis	Drug: lumacaftor;Drug: ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	28	Phase 1	United States
388	EUCTR2012-001565-33-GB	21/06/2013	17 November 2014	An open-label clinical trial that compares how long it takes in total for a patient with cystic fibrosis to take a daily dose of tobramycin dry power versus nebulised forms of tobramycin or colistin	An open-label, crossover, interventional Phase IV study to compare the ease of use of tobramycin inhalation powder with tobramycin inhalation solution and nebulized colistimethate for the treatment of pulmonary Pseudomonas aeruginosa in patients with cystic fibrosis	Chronic lung infection with Pseudomonas aeruginosa in cystic fibrosis patients MedDRA version: 17.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 17.1 Level: LLT Classification code 10021860 Term: Infection Pseudomonas aeruginosa System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: TOBI Podhaler Product Name: TOBI Podhaler Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28- Pharmaceutical Form: Nebulisation solution INN or Proposed INN: Tobramycin CAS Number: 3232986-56-4 Current Sponsor code: TBM100 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300- Pharmaceutical Form: Powder and solvent for nebuliser solution INN or Proposed INN: Colistimethate CAS Number: 30387-39-4 Other descriptive name: COLISTIMETHATE Concentration unit: million IU million international units Concentration type: equal Concentration number: 1-	Novartis Pharma Services AG		Authorised			Female: yes Male: yes	67	Phase 4	Spain;Germany;Switzerland;United Kingdom
389	EUCTR2012-003990-24-DE	20/06/2013	30 June 2014	A study in people with Cystic Fibrosis ( a rare hereditary pulmonary disease) to assess the efficacy and safety of a combination of two experimental drugs	A Phase 3, Randomized, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic fibrosis in patients homozygous for the F508del-CFTR Mutation MedDRA version: 16.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor/ivacaftor 200mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: lumacaftor/ivacaftor 200mg/83mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 83- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: ivacaftor 125mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	501	Phase 3	United States;France;Canada;Spain;Belgium;Denmark;Austria;Australia;Germany;United Kingdom
390	EUCTR2012-003990-24-GB	10/06/2013	28 February 2019	A study in people with Cystic Fibrosis ( a rare hereditary pulmonary disease) to assess the efficacy and safety of a combination of two experimental drugs	A Phase 3, Randomized, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic fibrosis in patients homozygous for the F508del-CFTR Mutation MedDRA version: 20.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor/ivacaftor 200mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: lumacaftor/ivacaftor 200mg/83mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 83- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: ivacaftor 125mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	501	Phase 3	France;United States;Canada;Belgium;Spain;Denmark;Austria;Australia;Germany;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
391	NCT01859390	June 2013	16 December 2017	Effects of an Antioxidant-Enriched Multivitamin Supplement on Inflammation and Oxidative Stress in Cystic Fibrosis	A Multi-Center, Randomized, Controlled, Double-Blind Study of the Effects of an Antioxidant-Enriched Multivitamin Supplement on Inflammation and Oxidative Stress in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: AquADEKs-2;Dietary Supplement: control multivitamin	University of Colorado, Denver	Cystic Fibrosis Foundation Therapeutics;Yasoo Health	Not recruiting	10 Years	N/A	All	73	Phase 2	United States
392	NCT01879228	June 2013	30 September 2019	Effect of Chronic Incretin-based Therapy in Cystic Fibrosis	A Randomized, Double-blind, Placebo Controlled Study of the Effectiveness of Chronic Incretin-based Therapy on Insulin Secretion in Cystic Fibrosis	Cystic Fibrosis;Pancreatic Insufficiency	Drug: Sitagliptin	University of Pennsylvania	Children's Hospital of Philadelphia	Not recruiting	18 Years	N/A	All	26	N/A	United States
393	NCT01887197	June 2013	19 November 2018	Repeatability and Response Study of Absorptive Clearance Scans	Repeatability and Response Study of Absorptive Clearance Scans	Cystic Fibrosis	Other: Absorptive clearance scan;Drug: inhaled hypertonic saline (7%);Drug: mannitol inhalation powder	Tim Corcoran		Not recruiting	18 Years	N/A	All	24	Phase 1	United States
394	NCT01910415	June 2013	19 February 2015	Phase 1, QT/QTC Interval Study in Healthy Subjects	A Phase 1, Randomized, Placebo and Active Controlled, Double-Blind, Parallel, Electrocardiogram Study to Evaluate the Effect of Lumacaftor in Combination With Ivacaftor on the QT/QTc Interval in Healthy Subjects	Cystic Fibrosis	Drug: Lumacaftor;Drug: Lumacaftor Placebo;Drug: Ivacaftor;Drug: Ivacaftor Placebo;Drug: moxifloxacin hydrochloride	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	200	Phase 1	Netherlands
395	EUCTR2012-004437-16-NL	15/05/2013	3 June 2013	Pharmacokinetic evaluation and local tolerability of dry powder tobramycin by a novel device in patients with non cystic fibrosis bronchiectasis	Pharmacokinetic evaluation and tolerability of dry powder tobramycin by a novel device in patients with non cystic fibrosis bronchiectasis - Tobra-02	Bronchiectasis Lung infections;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Dry powder tobramycin Product Code: J01GB01 Pharmaceutical Form: Inhalation powder INN or Proposed INN: Tobramycin dry powder CAS Number: 32986-56-4 Other descriptive name: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 30-	University Medical Center Groningen		Authorised			Female: yes Male: yes			Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
396	EUCTR2012-003989-40-SE	07/05/2013	24 July 2017	A study in people with Cystic Fibrosis ( a rare hereditary pulmonary disease) to assess the efficacy and safety of a combination of two experimental drugs	A Phase 3, Randomized, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic fibrosis in patients homozygous for the F508del-CFTR Mutation MedDRA version: 16.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: lumacaftor/ivacaftor 200mg/125mg tablets Product Code: VX-809 / VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: lumacaftor/ivacaftor 200mg/83mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 83- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: ivacaftor 125mg tablet Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	501	Phase 3	United States;France;Czech Republic;Canada;Ireland;Australia;Germany;Netherlands;United Kingdom;Italy;Sweden
397	NCT01710644	May 2013	13 June 2016	Efficacy and Tolerability of NM-BL in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	A Randomized, Double-blind, Multicenter, Two-period Crossover Study to Assess the Efficacy and Tolerability of NM-BL (Burlulipase) in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	Pancreatic Insufficiency;Cystic Fibrosis;Digestive System Diseases;Lung Diseases;Respiratory Tract Diseases	Drug: Burlulipase;Drug: Placebo (Caramel in sterile water)	Nordmark Arzneimittel GmbH & Co. KG	Parexel	Not recruiting	12 Years	N/A	Both	35	Phase 1/Phase 2	United States
398	NCT01807923	May 2013	19 October 2017	A Study of Lumacaftor in Combination With Ivacaftor in Cystic Fibrosis Subjects Aged 12 Years and Older Who Are Homozygous for the F508del-CFTR Mutation	A Phase 3, Randomized, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Drug: Lumacaftor Plus Ivacaftor Combination;Drug: Ivacaftor;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	559	Phase 3	United States;Australia;Canada;Czech Republic;France;Germany;Ireland;Italy;Netherlands;Sweden;United Kingdom
399	NCT01840735	May 2013	19 February 2015	Phase 1b Safety, Tolerability, and PK Study to Assess GS-5737 in Subjects With CF	A Phase 1b, Randomized, Double-Blind, Placebo-Controlled, Single Dose Study to Assess the Safety, Tolerability, and Pharmacokinetics of GS-5737 in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: GS-5737;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	N/A	Both	8	Phase 1	United States
400	NCT01851694	May 2013	23 April 2019	Beta-cell Response to Incretin Hormones in Cystic Fibrosis	Determination of Beta-cell Responsiveness to the Incretin Hormones GLP-1 and GIP in Cystic Fibrosis	Cystic Fibrosis;Pancreatic Insufficiency	Drug: GLP-1;Drug: GIP	University of Pennsylvania	Children's Hospital of Philadelphia	Recruiting	18 Years	N/A	All	45	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
401	NCT01852448	May 2013	26 August 2019	Genetics of Insulin and Incretins in Cystic Fibrosis	Evaluation of the Enteroinsular Axis in Cystic Fibrosis	Cystic Fibrosis	Genetic: Blood or Saliva Sample Collection;Other: Glucose -potentiated arginine (GPA) stimulation tests	Children's Hospital of Philadelphia	University of Pennsylvania	Recruiting	2 Years	N/A	All	350	Phase 1	United States
402	NCT01863238	May 2013	5 September 2016	An Ocular Safety Study of Ivacaftor-Treated Pediatric Patients 11 Years of Age or Younger With Cystic Fibrosis	An Ocular Safety Study of Ivacaftor-Treated Pediatric Patients 11 Years of Age or Younger With Cystic Fibrosis	Cystic Fibrosis	Other: Ophthalmologic examinations;Drug: Ivacaftor Exposed	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	6 Years	11 Years	Both	95	N/A	United States
403	NCT01807949	April 2013	19 October 2017	A Study of Lumacaftor in Combination With Ivacaftor in Cystic Fibrosis Subjects Aged 12 Years and Older Who Are Homozygous for the F508del-CFTR Mutation	A Phase 3, Randomized, Double Blind, Placebo Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of Lumacaftor in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Cystic Fibrosis, Homozygous for the F508del CFTR Mutation	Drug: Placebo;Drug: Lumacaftor Plus Ivacaftor Combination;Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	65 Years	All	563	Phase 3	United States;Australia;Austria;Belgium;Canada;Denmark;France;Germany;Spain;United Kingdom
404	EUCTR2011-003821-93-DE	13/03/2013	6 February 2017	A Phase 2, Multicenter, Double Blinded, Placebo Controlled Study to Evaluate Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of VX-661 Monotherapy and VX-661/Ivacaftor Cotherapy in Subjects with Cystic Fibrosis, Homozygous or Heterozygous for the F508del CFTR Mutation	A Phase 2, Multicenter, Double Blinded, Placebo Controlled Study to Evaluate Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of VX-661 Monotherapy and VX-661/Ivacaftor Cotherapy in Subjects with Cystic Fibrosis, Homozygous or Heterozygous for the F508del CFTR Mutation	Cystic Fibrosis MedDRA version: 16.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: VX-661 Product Code: VX-661, VRT-893661 Pharmaceutical Form: Tablet INN or Proposed INN: Not yet assigned Current Sponsor code: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 10- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: VX-661 Product Code: VX-661, VRT-893661 Pharmaceutical Form: Tablet INN or Proposed INN: Not yet assigned Current Sponsor code: VX-661 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: Ivacaftor Product Code: VX-770, VRT-813077 Pharmaceutical Form: Tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	220	Phase 2	United Kingdom;Germany;Canada;United States
405	NCT01746095	March 2013	19 February 2015	Efficacy and Safety Study of AeroVanc for the Treatment of Persistent MRSA Lung Infection in Cystic Fibrosis Patients	A Phase 2, Randomized, Double Blind, Placebo-controlled Study of AeroVanc for the Treatment of Persistent Methicillin-resistant Staphylococcus Aureus Lung Infection in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Vancomycin inhalation powder;Drug: Placebo inhalation powder	Savara Inc.	Synteract, Inc.;Cystic Fibrosis Foundation Therapeutics	Not recruiting	12 Years	N/A	Both	87	Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
406	NCT01837355	March 2013	11 June 2018	Modulation of Intestinal and Pulmonary Inflammation by Lactobacillus Diet Supplementation in Pediatric Cystic Fibrosis	Modulation of Intestinal and Pulmonary Inflammation by Lactobacillus Rhamnosus Diet Supplementation in Pediatric Cystic Fibrosis (MoHuM-1)	Pulmonary Inflammation;Cystic Fibrosis;Microbiota	Dietary Supplement: Lactobacillus rhamnosus;Dietary Supplement: Placebo	Cantonal Hospital of St. Gallen	Swiss Federal Institute of Technology;University Hospital Inselspital, Berne	Recruiting	6 Years	20 Years	All	68	N/A	Switzerland
407	NCT01775137	February 2013	19 October 2017	Ext. Long-term Safety Study in CF Patients: Single Arm TIP	A 48 Week Extension to CTBM100C2401, a Single Arm, Open-label, Multicenter, Phase IV Extension Trial to Assess Long Term Safety of Tobramycin Inhalation Powder (TIP) in Patients With Cystic Fibrosis Who Completed Participation in CTBM100C2401.	Long-term Safety of TIP	Drug: TBM100	Novartis Pharmaceuticals		Not recruiting	6 Years	N/A	All	45	Phase 4	United States;Argentina;Australia;Canada;Germany;Hungary;Italy;Mexico;Spain
408	EUCTR2012-004491-18-GB	30/01/2013	11 March 2013	Safety and Efficacy of Oral BAY 85-8501 in Patients with inflammation of the tubes in the lungs.	A Phase IIa, Randomized, Double-blind, Placebo-controlled, Parallel Group Study to Assess the Safety and Efficacy of 28 Day Oral Administration of BAY 85-8501 in Patients with non-Cystic Fibrosis Bronchiectasis	Non-Cystic Fibrosis Bronchiectasis MedDRA version: 15.1 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: BAY 85-8501 Product Code: BAY 85-8501 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: N/A CAS Number: N/A Current Sponsor code: BAY 85-8501 Other descriptive name: BAY 85-8501 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 0.5- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Bayer HealthCare AG		Authorised			Female: yes Male: yes	90	Phase 2a	Spain;United Kingdom
409	EUCTR2012-002699-14-GB	28/01/2013	4 February 2013	Trial of inhaled mannitol in children with cystic fibrosis	A randomised, multicentre, double-blind, placebo-controlled, crossover trial determining the efficacy of dry powder mannitol in improving lung function in subjects with Cystic Fibrosis aged six to seventeen years - DPM-CF-204 mannitol in CF aged 6-17 years	Cystic fibrosis in children aged 6 to 17 years MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Bronchitol Product Name: Bronchitol Product Code: N/A Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: mannitol CAS Number: 69-95-8 Current Sponsor code: N/A Other descriptive name: N/A Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 400- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Pharmaxis Ltd.		Authorised			Female: yes Male: yes	160		Canada;Argentina;United Kingdom
410	EUCTR2012-002880-86-GB	25/01/2013	4 February 2013	Resting the pancreas in Cystic Fibrosis	PRESERVING ?ETA-CELLS: ‘RESTING THE PANCREAS’ IN CYSTIC FIBROSIS	Cystic Fibrosis Related Diabetes (CFRD) MedDRA version: 14.1 Level: LLT Classification code 10022468 Term: Insulin System Organ Class: 100000004848 ;Therapeutic area: Body processes [G] - Physiological processes [G07]	Trade Name: Levemir FlexPen Product Name: Levemir FlexPen Pharmaceutical Form: Solution for injection in pre-filled pen	Liverpool Heart and Chest Hospital NHS Trust		Authorised			Female: yes Male: yes			United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
411	NCT00928135	January 22, 2013	11 June 2018	Aerosolized Hypertonic Xylitol Versus Hypertonic Saline in Cystic Fibrosis (CF) Subjects	Randomized Controlled Study of Aerosolized Hypertonic Xylitol Versus Hypertonic Saline in Hospitalized Patients With Exacerbation of Cystic Fibrosis	Cystic Fibrosis	Drug: Xylitol;Drug: Saline	Joseph Zabner		Not recruiting	12 Years	N/A	All	63	Phase 1/Phase 2	United States
412	EUCTR2011-004208-39-DE	21/01/2013	8 August 2016	Ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis (non–CF BE)	Randomized, double-blind, placebo-controlled, multicEnter Study comParing CIprofloxacin DPI 32.5 mg BID intermittently administered for 28 days on / 28 days off or 14 days on / 14 days off versus placebo to evaluate the time to fiRst pulmonary exacErbation and frequency of exacerbations in subjects with non–cystic fibrosis bronchiectasis. - RESPIRE 1	bronchiectasis MedDRA version: 18.1 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Body processes [G] - Circulatory and Respiratory Physiological Phenomena [G09]	Product Name: Ciprofloxacin DPI Product Code: BAYq3939 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Ciprofloxacin Current Sponsor code: BAYq3939 Other descriptive name: CIPROFLOXACIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 32.5- Pharmaceutical form of the placebo: Inhalation powder Route of administration of the placebo: Inhalation use	Bayer HealthCare AG		Not Recruiting			Female: yes Male: yes	400	Phase 3	Serbia;United States;Slovakia;Spain;Israel;United Kingdom;Italy;France;Argentina;Brazil;Australia;Denmark;Latvia;Germany;New Zealand;Japan
413	NCT01705145	January 2013	19 October 2017	Study of Ivacaftor in Cystic Fibrosis Subjects 2 Through 5 Years of Age With a CFTR Gating Mutation	A Phase 3, 2-Part, Open-Label Study to Evaluate the Safety, Pharmacokinetics and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are 2 Through 5 Years of Age and Have a CFTR Gating Mutation	Cystic Fibrosis	Drug: Ivacaftor	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	2 Years	5 Years	All	35	Phase 3	United States;Canada;United Kingdom
414	NCT01768663	January 2013	19 February 2015	A Phase 1 Study to Examine the Drug-Drug Interaction of Ciprofloxacin, Itraconazole, and Rifampin on the Combination of Lumacaftor With Ivacaftor in Healthy Adult Subjects	A Phase 1, Open-Label Study to Examine the Effect of Ciprofloxacin, Itraconazole, and Rifampin on the Pharmacokinetics of Lumacaftor in Combination With Ivacaftor in Healthy Adult Subjects	Cystic Fibrosis	Drug: Lumacaftor;Drug: Ivacaftor;Drug: Ciprofloxacin;Drug: Itraconazole;Drug: Rifampin	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	80	Phase 1	United States
415	EUCTR2012-002503-17-NL	21/12/2012	7 January 2013	Tobramycin nebulisation with I-neb (TONI) study in children with cystic fibrosis: pharmacokinetics and safety	Tobramycin nebulisation with I-neb (TONI) study in children with cystic fibrosis: pharmacokinetics and safety - TONI study in children with CF	Cystic fibrosis MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Bramitob Product Name: Bramitob Product Code: 033841 Pharmaceutical Form: Inhalation solution INN or Proposed INN: TOBRAMYCIN CAS Number: 32986-56-4 Current Sponsor code: TONI-2012 Concentration unit: mg milligram(s) Concentration type: up to Concentration number: 600 -	Erasmus University Medical Centre		Authorised			Female: yes Male: yes			Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
416	EUCTR2012-003532-23-HU	19/12/2012	23 February 2015	An extension of a clinical study to investigate long term safety of tobramycin inhalation powder (TIP) in patients with Cystic Fibrosis	A 48 week extension to CTBM100C2401, a single arm open-label, multicenter, phase IV trial, to assess long term safety of tobramycin inhalation powder (TIP) in patients with Cystic Fibrosis	Chronic lung infection with Pseudomonas aeruginosa in cystic fibrosis patients MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 14.1 Level: LLT Classification code 10021860 Term: Infection pseudomonas aeruginosa System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: TOBI Podhaler Product Name: TOBI Podhaler Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28-	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	120	Phase 4	France;United States;Hungary;Mexico;Canada;Argentina;Spain;Australia;Germany;Italy
417	NCT01641822	December 2012	19 October 2017	Phase 3 Study of Aztreonam for Inhalation Solution (AZLI) in a Continuous Alternating Therapy Regimen for the Treatment of Chronic Pseudomonas Aeruginosa Infection in Patients With CF	A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Multicenter Study of Aztreonam for Inhalation Solution (AZLI) in a Continuous Alternating Therapy (CAT) Regimen of Inhaled Antibiotics for the Treatment of Chronic Pulmonary Pseudomonas Aeruginosa Infection in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: AZLI;Drug: Placebo to match AZLI;Drug: Tobramycin inhalation solution	Gilead Sciences		Not recruiting	6 Years	N/A	All	107	Phase 3	United States
418	NCT01695343	December 2012	19 February 2015	Study to Evaluate the Effect of KB001-A on Time-to-Need for Antibiotic Treatment	A Phase 2, Randomized, Double-blind, Placebo-controlled, Repeat-dose Study of KB001-A in Subjects With Cystic Fibrosis Infected With Pseudomonas Aeruginosa	Cystic Fibrosis	Biological: KB001-A;Drug: Placebo Comparator	KaloBios Pharmaceuticals		Not recruiting	12 Years	50 Years	Both	182	Phase 2	United States;Australia;Israel;New Zealand
419	NCT01712334	December 2012	19 October 2017	A Study of the Comparable Efficacy and Safety of Pulmozyme (Dornase Alfa) Delivered by the eRapid Nebulizer System in Patients With Cystic Fibrosis	A Phase IV Multicenter, Randomized, Open Label, Two-Period, Crossover Study in Patients With Cystic Fibrosis to Evaluate the Comparable Efficacy and Safety of Pulmozyme Delivered by the eRapid Nebulizer System	Cystic Fibrosis	Drug: dornase alfa [Pulmozyme®]	Genentech, Inc.		Not recruiting	6 Years	N/A	All	99	Phase 4	United States
420	NCT01684683	November 2012	23 March 2015	The Effect of Theophylline in the Treatment of Bronchiectasis	Clinical Efficacy and Safety of Theophylline in the Treatment of Non-Cystic Fibrosis(NCF) Bronchiectasis	Bronchiectasis	Drug: Theophylline;Drug: Placebo(for Theophylline)	The First Affiliated Hospital of Guangzhou Medical University		Not recruiting	18 Years	70 Years	Both	100	Phase 4	China
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
421	NCT01721382	November 2012	20 June 2016	Treatment of Dysglycemia Using Sitagliptin in Adolescents With Cystic Fibrosis	Treatment of Dysglycemia Using Sitagliptin in Adolescents With Cystic Fibrosis.	Cystic Fibrosis	Drug: Sitagliptin	Nemours Children's Clinic		Not recruiting	13 Years	20 Years	Both	6	Phase 1	United States
422	EUCTR2012-000389-39-GB	25/10/2012	28 September 2015	Rollover Study of Ivacaftor in Subjects With Cystic Fibrosis and a Non-G551D CFTR Mutation	A Phase 3, Two-Arm, Rollover Study to Evaluate the Safety of Long-Term Ivacaftor Treatment in Subjects 6 Years of Age and Older with Cystic Fibrosis and a Non-G551D CFTR Mutation	cystic fibrosis MedDRA version: 18.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Kalydeco Product Name: ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	120	Phase 3	France;United States;Belgium;United Kingdom
423	NCT01316276	October 5, 2012	26 August 2019	Extension Study of Liposomal Amikacin for Inhalation in Cystic Fibrosis (CF) Patients With Chronic Pseudomonas Aeruginosa (Pa) Infection	Long Term Safety and Tolerability Study of Open-Label Liposomal Amikacin for Inhalation (ARIKACE™) in Cystic Fibrosis Patients With Chronic Infection Due to Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: Liposomal amikacin for inhalation	Insmed Incorporated		Not recruiting	6 Years	N/A	All	206	Phase 3	Austria;Belgium;Bulgaria;Canada;Denmark;France;Germany;Greece;Hungary;Ireland;Italy;Netherlands;Poland;Serbia;Slovakia;Spain;United Kingdom
424	NCT01586728	October 2012	29 June 2015	Oxygen Therapy in Cystic Fibrosis	Indication and Benefits of Nocturnal Oxygen Therapy in Cystic Fibrosis	Cystic Fibrosis	Other: Air - oxygen;Other: Oxygen - Air	Assistance Publique - Hôpitaux de Paris	Vaincre la Mucoviscidose	Not recruiting	6 Years	N/A	Both	8	N/A	France
425	NCT01594827	October 2012	22 January 2018	Persistent Methicillin Resistant Staphylococcus Aureus Eradication Protocol (PMEP)	Persistent MRSA Eradication Protocol (PMEP)	Cystic Fibrosis	Drug: Inhaled Vancomycin;Drug: Placebo (Sterile Water);Drug: Rifampin;Drug: Trimethoprim/Sulfamethoxazole (TMP/SMX);Drug: Doxycycline;Drug: Mupirocin Intranasal Creme;Drug: 4% chlorhexidine gluconate liquid skin cleanser	Johns Hopkins University	Case Western Reserve University;Cystic Fibrosis Foundation Therapeutics	Not recruiting	12 Years	N/A	All	29	Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
426	NCT01793649	October 2012	19 February 2015	A Phase 1 Study to Characterize the Effect of GS-5737 Enhancement of Mucociliary Clearance (MCC) in Healthy Subjects	A Phase 1, Randomized, Double-Blind, Cross-Over, Vehicle-Controlled, Single-Dose Study To Characterize The Effect of GS-5737 Enhancement of Mucociliary Clearance (MCC) In Healthy Subjects	Cystic Fibrosis;Chronic Obstructive Pulmonary Disease	Drug: GS-5737	Gilead Sciences		Not recruiting	18 Years	45 Years	Both	7	Phase 1	United States
427	ChiCTR-TRC-12002470	2012-09-15	18 April 2017	The effect of theophylline in the treatment of bronchiectasis, a random, double blind study	The effect of theophylline in the treatment of bronchiectasis, a random, double blind study.	bronchiectasis not due to cystic fibrosis	1:theophylline;2:theophylline;	The first affiliated hospital of Guangzhou Medical University		Not Recruiting	15	65	Both	1:46;2:46;	Post-market	China
428	NCT01667094	September 2012	16 December 2017	A Study Comparing Continuous Infusion Antibiotics to Standard Treatment for Lung Infections in Cystic Fibrosis	Continuous-infusion Anti-pseudomonal ß-lactams for the Treatment of Acute, Infective Pulmonary Exacerbations in Cystic Fibrosis	Cystic Fibrosis	Drug: Intermittent, short infusion Ceftazidime;Drug: Continuous infusion Ceftazidime;Drug: Intermittent, short infusion Meropenem;Drug: Continuous infusion Meropenem;Drug: Intermittent, short infusion Ticarcillin-clavulanate;Drug: Continuous infusion Ticarcillin-clavulanate;Drug: Intermittent, short infusion Cefepime;Drug: Continuous infusion Cefepime;Drug: Continuous infusion Piperacillin tazobactam;Drug: Intermittent, short infusion Piperacillin tazobactam	The Alfred		Not recruiting	18 Years	N/A	All	50	Phase 4	Australia
429	NCT01685801	September 2012	19 October 2017	Pilot Study Testing the Effect of Ivacaftor on Lung Function in Subjects With Cystic Fibrosis and Residual CFTR Function	A Pilot Study Testing the Effect of Ivacaftor on Lung Function in Subjects With Cystic Fibrosis, Residual CFTR Function, and FEV1 =40% Predicted	Cystic Fibrosis	Drug: Ivacaftor;Drug: Placebo-matched-to-ivacaftor tablet	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	24	Phase 2	United States
430	NCT01694069	September 2012	19 February 2015	Continuous Infusion Piperacillin-tazobactam for the Treatment of Cystic Fibrosis	Continuous Infusion Piperacillin-Tazobactam for the Treatment of Pulmonary Exacerbations in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Piperacillin-tazobactam combination product	West Virginia University		Not recruiting	8 Years	N/A	Both	6	Phase 4	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
431	NCT02201082	September 2012	7 September 2015	Evaluation of the Timing of the Nebulization Related to the Physiotherapy Session		Cystic Fibrosis	Procedure: Airway clearance technique;Drug: Amikacin nebulization	Cliniques universitaires Saint-Luc- Université Catholique de Louvain		Not recruiting	16 Years	50 Years	Both	15	N/A	Belgium
432	NCT01194232	August 2012	19 February 2015	Sildenafil Trial in Children and Young Adults With CF	Randomized Controlled Study of Sildenafil in Children and Young Adults With Mild to Moderate Cystic Fibrosis Lung Disease	Cystic Fibrosis With Mild to Moderate Lung Disease;CMRI of Lung Perfusion;Lung Perfusion;Lung Vascularization	Drug: Sildenafil	Children's Hospital Medical Center, Cincinnati		Not recruiting	8 Years	21 Years	Both	0	Phase 1/Phase 2	United States
433	NCT01684410	August 2012	19 October 2017	Safety and Tolerability Trial of Inhaled Alpha1-Proteinase Inhibitor (Human), Hydrophobic Chromatography Process (Alpha-1 HC) in Subjects With Cystic Fibrosis	A Three Week Dose Escalation, Randomized, Double-Blind, Placebo-Controlled Trial to Assess the Safety and Tolerability of 100 mg or 200 mg of Inhaled Alpha-1 HC, Once a Day in Subjects With Cystic Fibrosis.	Cystic Fibrosis	Biological: Alpha-1 HC 100 mg;Biological: Placebo;Biological: Alpha-1 HC 200 mg	Grifols Therapeutics Inc.		Not recruiting	18 Years	N/A	All	30	Phase 2	United States
434	NCT01652157	July 31, 2012	24 September 2018	Long-term Study in US Cystic Fibrosis Patients Receiving Digestive Enzyme Supplements to Assess Narrowing of the Large Intestine Causing Adverse Intestinal Symptoms (Fibrosing Colonopathy)	A Long-Term Prospective Observational Safety Study of the Incidence of and Risk Factors for Fibrosing Colonopathy in US Patients With Cystic Fibrosis Treated With Pancreatic Enzyme Replacement Therapy: A Harmonized Protocol Across Sponsors(The CF-FC Study)	Fibrosing Colonopathy	Biological: CREON;Biological: ZENPEP;Biological: ULTRESA;Biological: PANCREAZE;Biological: PERTZYE;Biological: other non-sponsor pancreatic enzyme replacement therapy;Other: No pancreatic enzyme replacement therapy	AbbVie (prior sponsor, Abbott)	Cystic Fibrosis Foundation Therapeutics;Digestive Care, Inc.;Janssen Research & Development, LLC;Forest Research Institute, Inc., an Allergan affiliate;VIVUS, Inc.	Recruiting	N/A	99 Years	All	50	Phase 3	United States
435	NCT02190604	July 31, 2012	15 April 2019	Safety, Tolerability, Pharmacokinetics, and Preliminary Pharmacodynamics of QBW251 in Healthy Subjects and Cystic Fibrosis Patients	A Randomized, Double Blind Placebo-controlled Study to Assess the Safety, Tolerability, Pharmacokinetics, and Preliminary Pharmacodynamics of Single and Multiple Ascending Doses of QBW251 in Healthy Subjects and Multiple Doses in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Placebo;Drug: QBW251	Novartis Pharmaceuticals		Not recruiting	18 Years	65 Years	All	153	Phase 1/Phase 2	United States;Belgium;France;Germany;Ireland;Romania;United Kingdom;Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
436	EUCTR2012-000388-26-BE	23/07/2012	21 August 2017	Study of Ivacaftor in Subjects With Cystic Fibrosis Who Have a Non-G551D CFTR Gating Mutation	A Phase 3, Two-Part, Randomized, Double-Blind, Placebo-Controlled, Crossover Study With an Open-Label Period to Evaluate the Efficacy and Safety of Ivacaftor in Subjects With Cystic Fibrosis Who Have a Non-G551D CFTR Gating Mutation	Cystic Fibrosis MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Kalydeco Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	40	Phase 3	France;United States;Belgium
437	EUCTR2011-005085-37-GB	20/07/2012	7 October 2014	Safety, tolerability, pharmacokinetics and preliminary pharmacodynamics of QBW251 in healthy subjects and cystic fibrosis patients.	A randomized, double blind placebo-controlled study to assess the safety, tolerability, pharmacokinetics and preliminary pharmacodynamics of single and multiple ascending doses of QBW251 in healthy subjects and multiple doses in cystic fibrosis patients.	cystic fibrosis MedDRA version: 17.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Code: QBW251 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Not assigned CAS Number: Not assigned Current Sponsor code: QBW251 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Product Code: QBW251 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Not assigned CAS Number: Not assigned Current Sponsor code: QBW251 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Novartis Pharma Services AG		Authorised			Female: yes Male: yes	156		France;United States;Ireland;Romania;Netherlands;Germany;United Kingdom
438	EUCTR2011-005790-23-GB	17/07/2012	28 August 2012	Comparison of recovery of adrenal gland function following short and long term steroid treatment	Comparison of adrenal recovery following short and long-term glucocorticoid therapy. - Adrenal recovery following short and long-term glucocorticoid therapy	Adrenal recovery following long and short term steroid treatment. MedDRA version: 14.1 Level: LLT Classification code 10066550 Term: Chronic arthritis System Organ Class: 10028395 - Musculoskeletal and connective tissue disorders MedDRA version: 14.1 Level: LLT Classification code 10000842 Term: Acute lymphatic leukaemia System Organ Class: 10029104 - Neoplasms benign, malignant and unspecified (incl cysts and polyps) MedDRA version: 14.1 Level: LLT Classification code 10008953 Term: Chronic liver disease System Organ Class: 10019805 - Hepatobiliary disorders MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Body processes [G] - Physiological processes [G07]	Product Name: Prednisolone Pharmaceutical Form: Tablet INN or Proposed INN: Prednisolone CAS Number: 53-03-2 Current Sponsor code: N/A Concentration unit: mg/m2 milligram(s)/square meter Concentration type: equal Concentration number: 2.5- Product Name: Hydrocortisone Pharmaceutical Form: Tablet INN or Proposed INN: Hydrocortisone CAS Number: 50-23-7 Current Sponsor code: N/A Concentration unit: mg/m2 milligram(s)/square meter Concentration type: equal Concentration number: 5-10	Birmingham Children's Hospital NHS Foundation Trust		Authorised			Female: yes Male: yes	60		United Kingdom
439	NCT01614457	July 2012	19 October 2017	Study of Ivacaftor in Subjects With Cystic Fibrosis (CF) Who Have the R117H-CF Transmembrane Conductance Regulator (CFTR) Mutation (KONDUCT)	A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Ivacaftor in Subjects With Cystic Fibrosis Who Have the R117H-CFTR Mutation	Cystic Fibrosis	Drug: Ivacaftor;Drug: Placebo	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	6 Years	N/A	All	70	Phase 3	United States;United Kingdom;Ireland
440	NCT01614470	July 2012	19 October 2017	Study of Ivacaftor in Subjects With Cystic Fibrosis (CF) Who Have a Non-G551D CF Transmembrane Conductance Regulator (CFTR) Gating Mutation	A Phase 3, Two-Part, Randomized, Double-Blind, Placebo-Controlled, Crossover Study With an Open-Label Period to Evaluate the Efficacy and Safety of Ivacaftor in Subjects With Cystic Fibrosis Who Have a Non-G551D CFTR Gating Mutation	Cystic Fibrosis	Drug: Ivacaftor;Drug: Placebo	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	6 Years	N/A	All	39	Phase 3	United States;Belgium;France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
441	NCT02014181	July 2012	19 February 2015	Flaxseed Modulates Inflammation and Oxidative Stress in CF	Flaxseed Modulates Oxidative Stress and Inflammatory Biomarkers in Stable Patients With Cystic Fibrosis and Healthy Controls	Cystic Fibrosis;Oxidative Stress;Inflammation	Dietary Supplement: finely ground flaxseed powder	University of Pennsylvania		Not recruiting	18 Years	64 Years	Both	10	Phase 1	United States
442	EUCTR2012-000387-19-GB	11/06/2012	17 March 2014	Study of Ivacaftor in Subjects With Cystic Fibrosis Who Have the R117H-CFTR Mutation	A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of Ivacaftor in Subjects With Cystic Fibrosis who Have the R117H-CFTR Mutation	cystic fibrosis MedDRA version: 16.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Trade Name: Kalydeco Product Name: ivacaftor Product Code: VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: IVACAFTOR CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	80	Phase 3	United States;United Kingdom
443	NCT01619657	June 2012	16 December 2017	Preventive Inhalation of Hypertonic Saline in Infants With Cystic Fibrosis	Randomized, Double-blind, Controlled Pilot Study on Safety of Hypertonic Saline as Preventive Inhalation Therapy in Newborns and Infants With Cystic Fibrosis	Cystic Fibrosis Lung Disease	Drug: 6% Hypertonic Saline (HS), 4mL;Drug: 0.9% Isotonic Saline (IS), 4mL	Heidelberg University	German Center for Lung Research	Not recruiting	N/A	4 Months	All	42	Phase 2	Germany
444	NCT01747330	June 2012	19 October 2017	Evaluation of Safety and Tolerability of Creon Micro in Children Younger Than Four Years With Pancreatic Exocrine Insufficiency	An Open Label, Multicenter Study Evaluating Safety and Tolerability of Creon Micro in Pediatric Subjects From at Least One Month to Less Than Four Years of Age With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Pancreatic Exocrine Insufficiency	Drug: Pancreatin	Abbott Products		Not recruiting	N/A	4 Years	All	40	Phase 3	Russian Federation
445	EUCTR2011-001916-69-NL	22/05/2012	17 June 2013	treatment with insulin in patients with cystic fibrosis without diabetes	insulin therapy in non-diabetic adults with cystic fibrosis - insulin in CF without CFRD	cystic fibrosis patients without cystic fibrosis related diabetes;Therapeutic area: Diseases [C] - Nutritional and Metabolic Diseases [C18]	Trade Name: Lantus 100 units/ml solution for injection in a cartridge Pharmaceutical Form: Solution for injection Pharmaceutical form of the placebo: Solution for injection Route of administration of the placebo: Subcutaneous use	HagaZiekenhuis, pulmonary department		Not Recruiting			Female: yes Male: yes	40		Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
446	NCT01270074	April 2012	9 May 2016	Prevention of Bronchiectasis in Infants With Cystic Fibrosis	A Phase 3 Multi-centre Randomised Placebo-controlled Study of Azithromycin in the Primary Prevention of Radiologically-defined Bronchiectasis in Infants With Cystic Fibrosis.	Cystic Fibrosis;Bronchiectasis	Drug: Azithromycin;Drug: Placebo control	Queensland Children's Medical Research Institute	Telethon Kids Institute	Recruiting	6 Weeks	6 Months	Both	130	Phase 3	Australia;New Zealand
447	NCT01572194	April 2012	11 June 2018	Predictive Value of PIIINP Urinary for the Development of Chronic Renal Failure in Patients With Cystic Fibrosis After Lung Transplantation (MUCO-IRC)	Predictive Value of PIIINP Urinary for the Development of Chronic Renal Failure in Patients With Cystic Fibrosis After Lung Transplantation.	Lung Transplantation;Cystic Fibrosis;Renal Failure	Biological: Urinary levels of PIIINP as a marker of changes in renal function	Nantes University Hospital		Not recruiting	18 Years	N/A	All	45	N/A	France
448	EUCTR2011-004761-33-GB	08/03/2012	28 February 2019	Repeated application of gene therapy in patients with cystic fibrosis	A randomised double-blind placebo-controlled Phase 2B clinical trial of repeated application of gene therapy in patients with cystic fibrosis - Repeated application of gene therapy in patients with CF v01-010204	Cystic fibrosis MedDRA version: 20.0 Level: PT Classification code 10011763 Term: Cystic fibrosis lung System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: pGM169/GL67A Pharmaceutical Form: Inhalation solution Other descriptive name: pGM169/GL67A Concentration unit: mg milligram(s) Concentration type: range Concentration number: 2.5-2.8 Pharmaceutical form of the placebo: Inhalation solution Route of administration of the placebo: Inhalation use	Imperial College		Not Recruiting			Female: yes Male: yes	130	Phase 2	United Kingdom
449	NCT01417481	March 2012	19 October 2017	Effect of Glycine in Cystic Fibrosis	Evaluation of the Capability of a Glycine Oral Supplement for Diminishing Bronchial Inflammation in Children With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Glycine;Dietary Supplement: Placebo	Instituto Nacional de Enfermedades Respiratorias	Hospital Infantil de Mexico Federico Gomez;Instituto Mexicano del Seguro Social	Not recruiting	5 Years	15 Years	All	13	Phase 2	Mexico
450	NCT03045198	March 2012	20 August 2018	Effect of Azithromycin on Fatty Acids in CF	Effect of Azithromycin on Lipoproteins and Docosahexaenoic Acid in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Azithromycin	University Childrens' Hospital (Zentrum für Kinderheilkunde des Universitätsklinikum Bonn)	University Hospital, Frankfurt;University of Bonn	Recruiting	10 Years	60 Years	All	20	Phase 4
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
451	NCT01315678	February 29, 2012	15 July 2019	Study to Evaluate Arikayce™ in CF Patients With Chronic Pseudomonas Aeruginosa Infections	Randomized, Open-Label, Active-Controlled, Multicenter Study to Assess the Efficacy, Safety and Tolerability of Arikayce™ in Cystic Fibrosis Patients With Chronic Infection Due to Pseudomonas Aeruginosa	Pseudomonas Aeruginosa Infection	Drug: Tobramycin inhalation solution using a PARI LC® Plus nebulizer.;Drug: Liposomal amikacin for inhalation (Arikayce™) using the PARI Investigational eFlow® Nebulizer.	Insmed Incorporated		Not recruiting	6 Years	N/A	All	302	Phase 3	Austria;Belgium;Bulgaria;Canada;Denmark;France;Germany;Greece;Hungary;Ireland;Italy;Netherlands;Poland;Serbia;Slovakia;Spain;Sweden;United Kingdom
452	NCT01429259	February 2012	19 October 2017	Population Pharmacokinetics of Prolonged Infusion Meropenem in Cystic Fibrosis (CF) Children	An Open Label Study to Assess the Population Pharmacokinetics, Safety, and Practicality of Administering Meropenem as a Prolonged Infusion to Cystic Fibrosis Children Admitted With an Acute Pulmonary Exacerbation	Cystic Fibrosis;Pneumonia;Pseudomonas Aeruginosa Infection	Drug: meropenem	Joseph Kuti	Thrasher Research Fund	Not recruiting	6 Years	17 Years	All	30	Phase 4	United States
453	NCT01531673	February 2012	11 June 2018	Study of VX-661 Alone and in Combination With Ivacaftor in Subjects Homozygous or Heterozygous to the F508del-Cystic Fibrosis Transmembrane Conductance Regulator(CFTR) Mutation	A Phase 2, Multicenter, Double-Blinded, Placebo Controlled Study to Evaluate Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of VX-661 Monotherapy and VX-661/Ivacaftor Cotherapy in Subjects With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: VX-661;Drug: Ivacaftor;Drug: Placebo matched to VX-661;Drug: Placebo matched to ivacaftor	Vertex Pharmaceuticals Incorporated		Not recruiting	12 Years	N/A	All	194	Phase 2	United States;Canada;Germany;United Kingdom
454	NCT01543191	February 2012	19 February 2015	A Safety and Tolerability Study OF PUR118 In Subjects With Cystic Fibrosis	A Phase 1 Dose-escalation Study TO Evaluate the Safety and Tolerability of PUR118 and Placebo in Subjects With CF	Cystic Fibrosis	Drug: PUR118	Pulmatrix Inc.	Celerion	Not recruiting	18 Years	55 Years	Both	10	Phase 1	United Kingdom
455	NCT01509339	January 2012	19 February 2015	Pharmacokinetics of Vancomycin for Inhalation in Cystic Fibrosis	Pharmacokinetics of Vancomycin for Inhalation in Cystic Fibrosis	Cystic Fibrosis;Methicillin-resistant Staphylococcus Aureus	Drug: Vancomycin	Case Western Reserve University	Cystic Fibrosis Foundation Therapeutics	Not recruiting	18 Years	N/A	Both	10	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
456	NCT01731015	January 2012	19 February 2015	Imaging Lung Function Using Oxygen Enhanced MRI	A Pilot Study for Evaluation of Regional Lung Function in Normal Subjects and Subjects With Airway and Lung Disorders Using 1H Magnetic Resonance Imaging With Oxygen as a Contrast Agent	COPD;Asthma;Cystic Fibrosis;Emphysema;Small Airways Disease	Drug: Medical Grade Oxygen	Hal C Charles		Not recruiting	18 Years	90 Years	Both	15	Phase 1	United States
457	NCT02128984	January 2012	29 July 2019	Clinical Study With an Enteral Formula With Symbiotic and DHA for Malnourished Children	Clinical Study With an Enteral Formula With Symbiotic and DHA for Malnourished Children	Cystic Fibrosis;Failure to Thrive;Malnutrition	Dietary Supplement: Symbiotic Formula with DHA and antioxidants;Dietary Supplement: Standard Formula	Laboratorios Ordesa	Quantum Experimental;Peruvian Clinical Research	Not recruiting	1 Year	N/A	All	109	N/A	Peru;Spain
458	EUCTR2011-002719-27-IT	30/12/2011	27 January 2014	EFFECT OF RECOMBINANT HUMAN INSULIN-LIKE GROWTH FACTOR-I ON GLUCOSE TOLERANCE AND AS PREVENTION TOWARDS THE DEVELOPMENT OF CYSTIC FIBROSIS RELATED DIABETES MELLITUS	GLUCOSE METABOLISM AND INFLAMMATORY PARAMETERS UNDER IGF-I TREATMENT IN CYSTIC FIBROSIS - RH-IGF-I IN CYSTIC FIBROSIS	PATIENTS WITH CYSTIC FIBROSIS MedDRA version: 14.1 Level: PT Classification code 10011766 Term: Cystic fibrosis pancreatic System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Hormonal diseases [C19]	Trade Name: *INCRELEXSC 1FL 4ML 10MG/ML Pharmaceutical Form: Solution for injection INN or Proposed INN: MECASERMIN** CAS Number: 68562-41-4 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 40-	AZIENDA OSPEDALIERA DI PARMA		Not Recruiting			Female: yes Male: yes	20		Italy
459	NCT01404234	December 2011	19 October 2017	Safety of AZLI in Children With Cystic Fibrosis (CF) and Chronic Pseudomonas Aeruginosa in the Lower Airways	Open-Label Phase 3 Trial to Evaluate the Safety of Aztreonam 75 mg Powder and Solvent for Nebuliser Solution/Aztreonam for Inhalation Solution (AZLI) in Children With Cystic Fibrosis (CF) and Chronic Pseudomonas Aeruginosa (PA) in the Lower Airways	Cystic Fibrosis;Pseudomonas Aeruginosa	Drug: AZLI	Gilead Sciences		Not recruiting	N/A	12 Years	All	61	Phase 3	United States;France;Germany;Italy;Poland;Spain
460	EUCTR2011-002000-32-HU	25/11/2011	3 February 2014	A clinical study to investigate long term safety of tobramycin inhalation powder (TIP) in patients with Cystic Fibrosis	A single arm, open-label, multicenter, Phase IV trial to assess long term safety of tobramycin inhalation powder (TIP) in patients with Cystic Fibrosis	Pseudomonas aeruginosa infection in cystic fibrosis patients MedDRA version: 14.0 Level: LLT Classification code 10021860 Term: Infection pseudomonas aeruginosa System Organ Class: 10021881 - Infections and infestations ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: TOBI Podhaler 28 mg inhalációs por, kemény kapszula Product Name: TIP (Tobramycin inhalation powder) Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Tobramycin Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28-	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	150	Phase 4	France;United States;Hungary;Mexico;Canada;Argentina;Brazil;Spain;Australia;Germany;Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
461	NCT00889434	November 1, 2011	16 December 2017	Efficacy and Safety Study of EGCG/Tocotrienol in 18 Patients With Splicing-mutation-mediated Cystic Fibrosis (CF)	Single-site, Open-label, Dose-ranging, Efficacy, and Safety Study of EGCG/Tocotrienol in 18 Patients With Splicing-mutation-mediated CF	Cystic Fibrosis	Dietary Supplement: ECGC;Dietary Supplement: Tocotrienol;Dietary Supplement: EGCG + Tocotrienol	Hadassah Medical Organization		Not recruiting	18 Years	N/A	All	7	N/A	Israel
462	NCT01537666	November 2011	19 October 2017	Inhaled Vancomycin Tolerability, Safety and Pharmacokinetics	Phase I, Reference-controlled, Dose Escalating Study to Examine the Pharmacokinetics and Safety of AeroVanc Inhalation Powder.	Healthy;Cystic Fibrosis	Drug: AeroVanc;Drug: IV vancomycin hydrochloride	Savara Inc.	INC Research Limited	Not recruiting	18 Years	50 Years	All	25	Phase 1	Australia
463	NCT02276898	November 2011	1 June 2015	A Randomized-Controlled Trial of Inhaled Hypertonic Saline (7%) to Evaluate the Lung Clearance Index	A Randomized-Controlled Trial of Inhaled Hypertonic Saline (7%) to Evaluate the Lung Clearance Index as a Short-term Pharmacodynamic Biomarker in Patients With Cystic Fibrosis.	Cystic Fibrosis	Drug: Hypertonic Saline 7%;Drug: Isotonic Saline 0.9% (Placebo)	The Hospital for Sick Children		Not recruiting	6 Years	18 Years	Both	24	Phase 2	Canada
464	EUCTR2011-006171-19-IT	27/10/2011	3 July 2012	Multicentric and randomized study to evaluate the effectiveness of the association of the 2 different treatments in the early P.aerugionsa infection in patient with cystic fibrosis disease. The study will enlist pazients over 5 years old.	randomized multicentric clinical trial upon efficacy of two different drugs combination to eradication of early p.aeruginosa infection in cystic fibrosis patients over 5 years old.	Cystic fibrosis patients with early P.aeruginosa infection. MedDRA version: 14.1 Level: SOC Classification code 10038738 Term: Respiratory, thoracic and mediastinal disorders System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: *CIPROXIN6CPR RIV 500MG Pharmaceutical Form: Tablet INN or Proposed INN: CIPROFLOXACIN HYDROCHLORIDE Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 500- Trade Name: BRAMITOBNEBUL 56F 300MG/4ML* Pharmaceutical Form: Nebuliser solution INN or Proposed INN: TOBRAMYCIN CAS Number: 32986-56-4 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300- Trade Name: *CIPROXIN6CPR RIV 500MG Pharmaceutical Form: Tablet INN or Proposed INN: CIPROFLOXACIN HYDROCHLORIDE CAS Number: 86483-48-9 Concentration unit: mg milligram(s) Concentration type: up to Concentration number: 750- Trade Name: PROMIXINNEBUL 30MONOD 1MUI* Pharmaceutical Form: Inhalation powder INN or Proposed INN: COLISTIN MESILATE SODIUM CAS Number: 2680-63-9 Concentration unit: million IU million international units Concentration type: equal Concentration number: 1000000-	AZIENDA OSPEDALIERA MEYER		Authorised			Female: yes Male: yes			Italy
465	EUCTR2011-001821-26-IT	18/10/2011	28 August 2012	Aerosol Tobramycin 300 mg single dose in adult patient with cystic fibrosis	Aerosol Tobramycin 300 mg single dose in adult patient with cystic fibrosis: pilot study of antimicrobial activity	Adult patient with cystic fibrosis MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: *BRAMITOBNEBUL 28F 300MG/4ML Pharmaceutical Form: Nebuliser liquid INN or Proposed INN: TOBRAMYCIN** CAS Number: 32986-56-4 Concentration unit: mg/g milligram(s)/gram Concentration type: equal Concentration number: 300-	FONDAZIONE IRCCS CA' GRANDA OSPEDALE MAGGIORE POLICLINICO DI MILANO		Not Recruiting			Female: yes Male: yes			Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
466	NCT01002534	October 2011	4 March 2019	Does a Nasal Instillation of Vardenafil Normalize the Nasal Potential Difference in Cystic Fibrosis Patients?	Does a Nasal Instillation of Vardenafil Normalize the Nasal Potential Difference in Cystic Fibrosis Patients Homozygous for the F508del Mutation? A Randomized, Double Blind, Placebo-controlled Study.	Cystic Fibrosis	Drug: Vardenafil;Drug: Placebo	Cliniques universitaires Saint-Luc- Université Catholique de Louvain		Not recruiting	14 Years	N/A	All	5	Phase 2	Belgium
467	NCT01426256	October 2011	16 December 2017	Vitamin D for Enhancing the Immune System in Cystic Fibrosis (DISC Study)	Vitamin D for Enhancing the Immune System in Cystic Fibrosis	Cystic Fibrosis;Respiratory Tract Infections	Dietary Supplement: Cholecalciferol	Emory University	Cystic Fibrosis Foundation Therapeutics	Not recruiting	16 Years	N/A	All	91	Phase 3	United States
468	NCT01537614	October 2011	24 October 2016	Pharmacokinetic Study of Aerosolized Colimycin in Cystic Fibrosis		Cystic Fibrosis	Drug: COLIMYCINE inhalation;Drug: COLIMYCINE injectable	Poitiers University Hospital		Not recruiting	18 Years	N/A	Both	7	Phase 1	France
469	NCT01783613	October 2011	16 December 2017	Eficacy of Long-term Suplementation With Docosahexaenoic Acid in Patients With Cystic Fibrosis	Study of the Eficacy of Long-term Suplementation With Docosahexaenoic Acid on Pulmonary, Sistemic and Intestinal Inflammation in Patients With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Docosahexaenoic acid administration: 50 mg/kg/day during 12 months;Dietary Supplement: Placebo: 50 mg/kg/day during 12 months	Fundacion para la Investigacion Biomedica del Hospital Universitario Ramon y Cajal		Not recruiting	2 Months	100 Years	All	96	N/A	Spain
470	EUCTR2011-000801-39-DE	31/08/2011	6 November 2017	Placebo controlled clinical study to evaluate efficacy and safety of an antibody derived from hens’ eggs building a barrier in the respiratory tract against the Pseudomonas germ in order to prevent infection with Pseudomonas in patients suffering from cystic fibrosis	Prospective randomized, placebo-controlled, double blind, multicenter study (phase III) to evaluate clinical efficacy and safety of avian polyclonal anti-Pseudomonas antibodies (IgY) in prevention of recurrence of Pseudomonas aeruginosa infection in cystic fibrosis patients - IMPACTT-PsAer-IgY	Cystic fibrosis (CF) is a chronic and progressive genetic disease of the body's exocrine glands. CF especially affects the respiratory system. A common effect leads to massive production of abnormal mucus of high viscosity, which clogs the airways and leads to infections. Pulmonary infections are major causes of morbidity and mortality. Pseudomonas aeruginosa (PA) infections are most common in CF patients and chronic infection with PA ultimately occurs in virtually all patients. MedDRA version: 17.1 Level: LLT Classification code 10011764 Term: Cystic fibrosis NOS System Organ Class: 100000004850 ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Product Name: avian polyclonal IgY antibody against PA Product Code: PsAer IgY Pharmaceutical Form: Gargle INN or Proposed INN: IgY Current Sponsor code: PsAer IgY Concentration unit: ELISA unit/ml enzyme-linked immunosorbent assay unit/millitre Concentration type: not less then Concentration number: 5- Pharmaceutical form of the placebo: Gargle Route of administration of the placebo: Oromucosal use	Mukoviszidose Institute gGmbH		Not Recruiting			Female: yes Male: yes	180	Phase 3	Hungary;Spain;Belgium;Ireland;Austria;Germany;Italy;Sweden
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
471	NCT01375049	August 2011	19 October 2017	Aztreonam Lysine for Pseudomonas Infection Eradication Study	Open-Label Phase 2 Trial to Evaluate the Safety and Efficacy of Aztreonam 75 mg Powder and Solvent for Nebuliser Solution/Aztreonam for Inhalation Solution (AZLI) in Pediatric Patients With Cystic Fibrosis (CF) and New Onset Lower Respiratory Tract Culture Positive for Pseudomonas Aeruginosa (PA)	Cystic Fibrosis	Drug: Aztreonam for Inhalation Solution (AZLI)	Gilead Sciences		Not recruiting	3 Months	17 Years	All	105	Phase 2	United States;Austria;Belgium;France;Germany;Italy;Netherlands;Poland;Spain;Ireland
472	NCT01772758	August 2011	15 July 2019	Cystic Fibrosis and Endothelial Function: At Rest and During Exercise	Influence of Cystic Fibrosis on Vascular Endothelial Function at Rest and During Exercise	Cystic Fibrosis	Drug: BH4 5mg;Drug: BH4 20mg;Dietary Supplement: Antioxidant Cocktail	Augusta University		Not recruiting	7 Years	N/A	All	64	Phase 2	United States
473	EUCTR2011-000130-11-IT	25/07/2011	27 January 2014	Clinical trials in rare diseases: therapeutic alternative to the use of oral corticosteroids in patients with cystic fibrosis underwent double lung transplantation.	Clinical trials in rare diseases: therapeutic alternative to the use of oral corticosteroids in patients with cystic fibrosis underwent double lung transplantation.	Double lung transplantation in patients with Cystic Fibrosis MedDRA version: 14.1 Level: PT Classification code 10011763 Term: Cystic fibrosis lung System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 14.1 Level: PT Classification code 10025127 Term: Lung transplant System Organ Class: 10042613 - Surgical and medical procedures ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Product Name: Dexamethasone sodium phosphate 250 mg/10 ml solution Product Code: NA Pharmaceutical Form: Solution for injection INN or Proposed INN: Dexamethasone Concentration unit: mg/ml milligram(s)/millilitre Concentration number: 500-	AZIENDA UNIVERSITARIA POLICLINICO UMBERTO I DI ROMA		Authorised			Female: yes Male: yes			Italy
474	NCT01381289	June 2011	19 February 2015	VX-770 Expanded Access Program	VX-770 Expanded Access Program (EAP)	Cystic Fibrosis	Drug: VX-770	Vertex Pharmaceuticals Incorporated		Not recruiting	6 Years	N/A	Both		N/A
475	NCT01755455	June 2011	16 December 2017	Does a Daily Iron Tablet Improve Anemia in Cystic Fibrosis	Iron Supplementation for the Hypoferremic Anemia of Cystic Fibrosis	Cystic Fibrosis;Anemia, Iron-Deficiency	Drug: Ferrous sulfate 325mg;Drug: Placebo	Dartmouth-Hitchcock Medical Center		Not recruiting	18 Years	70 Years	All	22	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
476	NCT02782312	June 2011	13 June 2016	Salmeterol-Fluticasone Combined Inhaled Therapy for Non-cystic Fibrosis Bronchiectasis	Salmeterol-Fluticasone Combined Inhaled Therapy for Non-cystic Fibrosis Bronchiectasis With Airway Limitation: a Prospective Study	Bronchiectasis	Drug: ICS+LABA;Other: routine therapy	Shanghai Pulmonary Hospital, Shanghai, China		Not recruiting	18 Years	N/A	Both	120	Phase 4	China
477	NCT01207245	May 2011	3 August 2015	Circadian Rhythm In Tobramycin Elimination In Cystic Fibrosis	Circadian Rhythm In Tobramycin Elimination In Cystic Fibrosis (CRITIC) A Randomized Pharmacokinetic Comparison of Tobramycin in Cystic Fibrosis	Cystic Fibrosis	Other: Tobramycin time of administration	University of Nottingham		Not recruiting	5 Years	N/A	Both	18	Phase 4	United Kingdom
478	NCT01355796	May 2011	10 September 2018	Inhaled Xylitol Versus Saline in Stable Subjects With Cystic Fibrosis	Randomized Cross Over Study of Inhaled Hypertonic Xylitol Versus Hypertonic Saline in Stable Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: Xylitol;Drug: Hypertonic saline	Joseph Zabner	Ann & Robert H Lurie Children's Hospital of Chicago;Northwestern University	Not recruiting	16 Years	N/A	All	30	Phase 1/Phase 2	United States
479	EUCTR2011-000192-13-GB	28/04/2011	26 June 2012	A 7-day open-label pharmacokinetic study to investigate the systemic absorption of colistimethate sodium after inhalation of dry powder colistimethate sodium for inhalation (Colobreathe® 125mg) in adult, adolescent and paediatric cystic fibrosis subjects with chronic pulmonary infection with Pseudomonas aeruginosa. - Colistin Systemic Exposure (COSY) study	A 7-day open-label pharmacokinetic study to investigate the systemic absorption of colistimethate sodium after inhalation of dry powder colistimethate sodium for inhalation (Colobreathe® 125mg) in adult, adolescent and paediatric cystic fibrosis subjects with chronic pulmonary infection with Pseudomonas aeruginosa. - Colistin Systemic Exposure (COSY) study	cystic fibrosis subjects with chronic pulmonary infection with Pseudomonas aeruginosa MedDRA version: 13.1 Level: PT Classification code 10011763 Term: Cystic fibrosis lung System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 13.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders	Product Name: Colobreathe Product Code: N/A Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Colistimethate sodium CAS Number: 8068288 Other descriptive name: Colistimethate sodium (BAN, USAN), Colistimethatum natricum, Colistin sodium methanesulfonate Concentration unit: mg milligram(s) Concentration type: up to Concentration number: 125-	Forest Laboratories UK Ltd.		Not Recruiting			Female: yes Male: yes	28		Germany;United Kingdom;Poland
480	NCT01313624	April 2011	19 October 2017	Safety and Effectiveness of AZLI (an Inhaled Antibiotic) in Adults With Non-Cystic Fibrosis Bronchiectasis	A Phase 3, Double-Blind, Multicenter, Randomized, Placebo-Controlled Trial Evaluating Repeated Courses of Aztreonam for Inhalation Solution in Subjects With Non-CF Bronchiectasis and Gram-Negative Endobronchial Infection	Bronchiectasis	Drug: AZLI;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	N/A	All	266	Phase 3	United States;Australia;Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
481	NCT01314716	April 2011	19 October 2017	Safety and Effectiveness of AZLI (an Inhaled Antibiotic) in Adults With Non-Cystic Fibrosis Bronchiectasis	A Phase 3, Double-Blind, Multicenter, Randomized, Placebo-Controlled Trial Evaluating Repeated Courses of Aztreonam for Inhalation Solution/Aztreonam 75 mg Powder and Solvent for Nebuliser Solution in Subjects With Non-CF Bronchiectasis and Gram-Negative Endobronchial Infection (AIR-BX2)	Bronchiectasis	Drug: AZLI;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	N/A	All	274	Phase 3	United States;Australia;Belgium;Canada;France;Germany;Italy;Netherlands;Spain;United Kingdom
482	NCT01315665	April 2011	16 December 2017	Effect of Sulforaphane in Broccoli Sprouts on Nrf2 Activation	Evaluation of the Effect of Sulforaphane in Broccoli Sprouts on Nrf2 Activation, Measures of Oxidative Stress, and Neutrophil Migration to Mucosal Surfaces in Healthy and CF Subjects	Cystic Fibrosis	Dietary Supplement: Broccoli sprouts	University Hospitals Cleveland Medical Center	Cystic Fibrosis Foundation Therapeutics	Not recruiting	18 Years	49 Years	All	15	N/A	United States
483	NCT01337219	April 2011	19 February 2015	Pharmacokinetic Bioequivalence Study of Nebcinal® 150mg/3ml Administered by Aeroneb® Idehaler® Versus Tobi® 300mg/5ml Administered by Pari LC Plus® /Pulmoaid® in Patients With Cystic Fibrosis	Pharmacokinetic Bioequivalence Study of Nebcinal® 150mg/3ml Administered by Aeroneb® Idehaler® Versus Tobi® 300mg/5ml Administered by Pari LC Plus® /Pulmoaide® in Patients With Cystic Fibrosis.	Cystic Fibrosis	Drug: Tobramycin	Erempharma	University of Lyon;Epidemiologie Pharmacologie Investigation Clinique Information medicale Mere Enfant (EPICIME);Clininfo;Hospices Civils de Lyon	Recruiting	16 Years	N/A	Both	36	Phase 1/Phase 2	France
484	NCT01347190	April 2011	19 February 2015	Safety and Tolerability Study of Liquid Alpha1 Proteinase Inhibitor (API) in Subjects With Cystic Fibrosis	A Double Blind, Randomized, Placebo Controlled, Single Dose, Phase I Study of the Safety and Tolerability of Alpha1 Proteinase Inhibitor (Human) Inhalation Solution (CR002) in Subjects With Cystic Fibrosis	Cystic Fibrosis	Biological: CR002 Liquid API;Biological: Placebo	CSL Behring		Not recruiting	18 Years	65 Years	Both	25	Phase 1	Bulgaria;Hungary;Poland;United Kingdom
485	NCT01349192	April 2011	16 December 2017	Early Methicillin-resistant Staphylococcus Aureus (MRSA) Therapy in Cystic Fibrosis (CF)	Early MRSA Therapy in CF - Culture Based vs. Observant Therapy (Treat or Observe) (Star-TOO - STaph Aureus Resistance - Treat or Observe)	Cystic Fibrosis;Methicillin-resistant Staphylococcus Aureus	Drug: Rifampin;Drug: Trimethoprim/Sulfamethoxazole;Drug: Minocycline;Drug: Mupirocin;Drug: chlorhexidine gluconate oral rinse;Drug: 2% Chlorhexidine solution wipes;Behavioral: Environmental Decontamination	University of North Carolina, Chapel Hill	CF Therapeutics Development Network Coordinating Center;Seattle Children's Hospital;Washington University School of Medicine;University of Washington;University of Colorado, Denver;Baylor College of Medicine;University of Alabama at Birmingham;Cook Children's Medical Center;University of Michigan;University of Florida;University of Texas Southwestern Medical Center;Children's Hospital Medical Center, Cincinnati;St. Louis Children's Hospital	Not recruiting	4 Years	45 Years	All	47	Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
486	EUCTR2010-023090-19-GB	29/03/2011	19 March 2012	A double-blind, randomized, placebo-controlled, cross-over study to evaluate the safety, tolerability and preliminary efficacy of alginate oligosaccharide (OligoG) administered for 28 days in subjects with Cystic Fibrosis chronically colonised with Pseudomonas aeruginosa	A double-blind, randomized, placebo-controlled, cross-over study to evaluate the safety, tolerability and preliminary efficacy of alginate oligosaccharide (OligoG) administered for 28 days in subjects with Cystic Fibrosis chronically colonised with Pseudomonas aeruginosa - A phase II cross-over study of OligoG in subjects with cystic fibrosis	Cystic Fibrosis MedDRA version: 14.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: OligoG (60 mg/ml) Product Code: OligoG Pharmaceutical Form: Nebuliser solution INN or Proposed INN: OligoG CAS Number: 9005-38-3 Current Sponsor code: OligoG Other descriptive name: OligoG-CF5/20, Alginate oligosaccharide (G-block) fragment Concentration unit: % (W/V) percent weight/volume Concentration type: equal Concentration number: 6- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	AlgiPharma AS		Authorised			Female: yes Male: yes	24	Phase 2	United Kingdom;Ireland
487	NCT01315691	March 2011	20 August 2018	Study to Evaluate Arikace™ in CF Patients With Chronic Infection Due to Pseudomonas Aeruginosa	Randomized, Placebo-Controlled, Double-Blind, Multicenter Study to Assess the Efficacy, Safety and Tolerability of Arikace™ in Cystic Fibrosis Patients With Chronic Infection Due to Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: Liposomal amikacin for inhalation;Drug: Placebo for liposomal amikacin for inhalation	Insmed Incorporated		Not recruiting	6 Years	N/A	All	0	Phase 3
488	NCT02029521	March 2011	19 October 2017	Supplementation of Oral Reduced Glutathione in Pediatric Cystic Fibrosis Patients	Supplementation of Oral Reduced Glutathione in Pediatric Cystic Fibrosis for Growth Failure	Cystic Fibrosis	Dietary Supplement: Oral reduced l-glutathione;Dietary Supplement: Placebo	Clark Bishop		Not recruiting	18 Months	10 Years	All	47	N/A	Italy
489	EUCTR2010-023529-39-GB	18/02/2011	16 October 2012	Reducing antibiotic tolerance using low dose nitric oxide in cystic fibrosis – a phase 2 pilot study - RATNO (Reducing Antibiotic Tolerance using Nitric Oxide in CF) v 1.0	Reducing antibiotic tolerance using low dose nitric oxide in cystic fibrosis – a phase 2 pilot study - RATNO (Reducing Antibiotic Tolerance using Nitric Oxide in CF) v 1.0	Cystic Fibrosis	Trade Name: INOmax 400ppm mol/mol inhalation gas Product Name: Nitric Oxide Product Code: NO Pharmaceutical Form: Inhalation gas	Southampton University Hopsitals NHS Trust		Not Recruiting			Female: yes Male: yes		Phase 2	United Kingdom
490	EUCTR2010-020413-90-BE	08/02/2011	17 August 2015	Study of Lumacaftor Monotherapy, and Lumacaftor and Ivacaftor Combination Therapy in Cystic Fibrosis (CF) Patients Homozygous or Heterozygous for the F508del-CFTR Mutation	A Phase 2, Multicenter, Double-Blind, Placebo-Controlled, Multiple-Dose Study to Evaluate the Safety, Tolerability, Efficacy, Pharmacokinetics, and Pharmacodynamics of Lumacaftor Monotherapy, and Lumacaftor and Ivacaftor Combination Therapy in Subjects With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis MedDRA version: 16.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: VX-809 Product Code: VX-809, VRT-826809 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: Kalydeco Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: Kalydeco Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: Lumacaftor/Ivacaftor Product Code: VX-809/VX-770 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Lumacaftor CAS Number: 936727-05-8 Current Sponsor code: VX-809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200- INN or Proposed INN: Ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	293	Phase 2	United States;Belgium;Ireland;Australia;Germany;United Kingdom;New Zealand
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
491	NCT01229553	January 2011	29 June 2015	Effect of Topical and Systemic Decolonization of Staphylococcus Aureus (SA) in Pediatric Cystic Fibrosis (CF) Patients	Effect of Topical and Systemic Decolonization of Staphylococcus Aureus (SA) in Pediatric Cystic Fibrosis (CF) Patients at the CF Center at SUNY Upstate Medical University, Syracuse, NY.	Cystic Fibrosis	Drug: decolonization	State University of New York - Upstate Medical University		Not recruiting	2 Months	23 Months	Both	0	N/A	United States
492	NCT01270347	January 2011	29 January 2018	Trial of Aeroquin Versus Tobramycin Inhalation Solution (TIS) in Cystic Fibrosis (CF) Patients	Phase 3, Open-label, Randomized Trial to Evaluate the Safety and Efficacy of MP-376 Inhalation Solution (Aeroquin) vs. Tobramycin Inhalation Solution (TIS) in Stable CF Patients	Cystic Fibrosis	Drug: MP-376 (Levofloxacin Solution for Inhalation);Drug: TIS (Tobramycin Inhalation Solution)	Horizon Pharma USA, Inc.	Forest Laboratories	Not recruiting	12 Years	N/A	All	267	Phase 3	United States;France;Germany;Ireland;Israel;United Kingdom
493	NCT01280994	January 2011	23 April 2019	Hyperpolarized 129Xe MRI for Imaging Pulmonary Function	Hyperpolarized 129Xe MR Imaging of the Lung Function in Healthy Volunteers and Subjects With Pulmonary Disease	Interstitial Lung Disease;Cystic Fibrosis;Pulmonary Hypertension;NSIP;Alpha 1-Antitrypsin Deficiency	Drug: Xenon	Bastiaan Driehuys		Recruiting	18 Years	80 Years	All	445	Phase 1	United States
494	NCT01494909	January 2011	6 May 2019	Development Of An Innovative Panel of Methods To Measure Intestinal Macronutrient Digestion, Absorption, and Function	Development Of An Innovative Panel of Methods To Measure Intestinal Macronutrient Digestion, Absorption, and Function	Cystic Fibrosis	Dietary Supplement: Ensure plus	Texas A&M University	Arkansas Children's Hospital Research Institute;University of Arkansas	Not recruiting	10 Years	N/A	All	31	N/A	United States
495	NCT02163681	January 2011	16 December 2017	MRI as a Means to Measure Lung Function: Non-Invasive Imaging in Neonates and Children		Healthy;Cystic Fibrosis (CF);Asthma;Bronchopulmonary Dysplasia (BPD)	Drug: Hyperpolarized Helium-3 MRI of the chest	University of Virginia	Xemed LLC	Recruiting	4 Months	65 Years	All	77	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
496	NCT00625703	December 2010	30 September 2019	Pharmacokinetics of Linezolid in Children With Cystic Fibrosis	Pharmacokinetics of Linezolid in Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Linezolid	University of Texas Southwestern Medical Center	Cystic Fibrosis Foundation	Not recruiting	N/A	18 Years	All	14	Phase 2	United States
497	NCT01100892	December 2010	15 April 2019	Cystic Fibrosis - Insulin Deficiency, Early Action	Cystic Fibrosis - Insulin Deficiency, Early Action	Cystic Fibrosis;Diabetes	Drug: Once-daily insulin detemir	Sydney Children's Hospitals Network	John Hunter Children's Hospital;Lady Cilento Children's Hospital, Brisbane;Women's and Children's Hospital, Adelaide	Recruiting	5 Years	19 Years	All	100	Phase 3	Australia
498	NCT01180634	November 2010	29 January 2018	MP-376 (Aeroquin™, Levofloxacin for Inhalation) in Patients With Cystic Fibrosis	A Phase 3, Multi-Center, Multinational, Randomized, Double-Blind, Placebo-Controlled Study To Evaluate The Efficacy And Safety Of MP-376 (Levofloxacin Inhalation Solution; Aeroquin™) In Stable Cystic Fibrosis Patients	Cystic Fibrosis	Drug: MP-376 (Levofloxacin solution for Inhalation);Drug: Placebo	Horizon Pharma USA, Inc.	Forest Laboratories	Not recruiting	12 Years	N/A	All	330	Phase 3	United States;Australia;Canada;Israel;New Zealand
499	NCT01299181	November 2010	19 February 2015	A Trial of Atorvastatin as an Anti-Inflammatory Agent in Non-Cystic Fibrosis Bronchiectasis	A Randomised Controlled Trial of Atorvastatin as an Anti-Inflammatory Agent in Non-Cystic Fibrosis Bronchiectasis	Bronchiectasis	Drug: Atorvastatin	University of Edinburgh	NHS Lothian	Not recruiting	18 Years	75 Years	Both	60	Phase 4	United Kingdom
500	NCT01299194	November 2010	16 December 2017	Atorvastatin in Bronchiectasis in Patients With Pseudomonas Aeruginosa	A Randomised Controlled Trial of Atorvastatin as an Anti-Inflammatory Agent in Non-Cystic Fibrosis Bronchiectasis in Patients With Pseudomonas Aeruginosa	BRONCHIECTASIS	Drug: ATORVASTATIN	University of Edinburgh	NHS Lothian	Not recruiting	18 Years	80 Years	All	32	Phase 4	United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
501	EUCTR2010-020546-96-GB	08/10/2010	25 September 2012	Study of the Effect of VX-770 on Lung Clearance Index in Subjects With Cystic Fibrosis and the G551D Mutation	A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Crossover Study to Evaluate the Effect of VX-770 on Lung Clearance Index in Subjects with Cystic Fibrosis, the G551D Mutation, and FEV1 >90% Predicted -	Cystic Fibrosis MedDRA version: 14.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: VX-770 Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Other descriptive name: VRT-813077 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	16	Phase 2	United States;Canada;United Kingdom
502	NCT01161537	October 2010	19 October 2017	Study of the Effect of VX-770 on Hyperpolarized Helium-3 Magnetic Resonance Imaging in Subjects With Cystic Fibrosis and the G551D Mutation	A Phase 2, Single-Blind, Placebo-Controlled Study to Evaluate the Effect of VX-770 on Hyperpolarized Helium-3 Magnetic Resonance Imaging in Subjects With Cystic Fibrosis, the G551D Mutation, and FEV1 =40% Predicted	Cystic Fibrosis	Drug: VX-770;Drug: Placebo	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	12 Years	N/A	All	13	Phase 2	United States
503	NCT01201434	October 2010	19 February 2015	Effect of Probiotics on Sputum Inflammation and Pulmonary Infections in Patients With Cystic Fibrosis	The Effect of Probiotics on Sputum Bacteria, Sputum Inflammation, and Pulmonary Infections in Patients With Cystic Fibrosis: A Double-blind Placebo-controlled Trial	Cystic Fibrosis	Dietary Supplement: Bio-25 probiotic	Sheba Medical Center		Not recruiting	5 Years	40 Years	Both	12	N/A
504	NCT01216046	October 2010	19 February 2015	Drug-Drug Interaction Study of VX-770 and VX-809 in Healthy Subjects	A Phase 1, Randomized, Double-Blind, Placebo Controlled, Multiple-Dose, Dose-Escalation, Drug-Drug Interaction Study of VX-809 and VX-770 in Healthy Subjects	Cystic Fibrosis	Drug: VX-809;Drug: VX-770;Drug: VX-809 placebo;Drug: VX-770 placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	48	Phase 1	United States
505	NCT01225211	October 2010	19 October 2017	Study of VX-809 Alone and in Combination With VX-770 in Cystic Fibrosis (CF) Patients Homozygous or Heterozygous for the F508del-CFTR Mutation	A Phase 2, Multicenter, Double-Blinded, Placebo-Controlled, Multiple-Dose Study to Evaluate Safety, Tolerability, Efficacy, Pharmacokinetics, and Pharmacodynamics of Lumacaftor Monotherapy, and Lumacaftor and Ivacaftor Combination Therapy in Subjects With Cystic Fibrosis, Homozygous or Heterozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: Lumacaftor;Drug: Ivacaftor;Drug: Lumacaftor Placebo;Drug: Ivacaftor Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	312	Phase 2	United States;Australia;Belgium;France;Germany;New Zealand;United Kingdom;Ireland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
506	EUCTR2009-012842-21-GB	20/09/2010	4 February 2013	Treatment of Exocrine Pancreatic Insufficiency in subjects with Cystic Fibrosis	Randomised, Double-Blind, Active-Controlled, Two-Treatment, Crossover, Multinational, Multicentre Study to Compare Two Pancreatic Enzyme Products in theTreatment of Exocrine Pancreatic Insufficiency in Subjects With Cystic Fibrosis	Exocrine pancreatic insufficiency associated with cystic fibrosis MedDRA version: 14.1 Level: HLGT Classification code 10015674 Term: Exocrine pancreas conditions System Organ Class: 10017947 - Gastrointestinal disorders MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Digestive System Diseases [C06]	Trade Name: Kreon 25 000 Product Name: KREON 25000 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Pancreas Powder CAS Number: 8049-47-6 Other descriptive name: PANCREATIN Concentration unit: Other Concentration type: equal Concentration number: 25000- Trade Name: Zenpep Product Name: EUR-1008 Product Code: EUR-1008 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Pancreas Powder CAS Number: 53608-75-6 Current Sponsor code: EUR-1008 Other descriptive name: PANCREASLIPASE Concentration unit: Other Concentration type: equal Concentration number: 25000-	Aptalis Pharma US Inc.		Authorised			Female: yes Male: yes	86		Bulgaria;Germany;Italy;United Kingdom
507	EUCTR2010-020516-11-IT	16/09/2010	19 March 2012	EXPERIMENTAL STUDY, RANDOMIZED, OPEN LABEL, TO EVALUATE THE EFFECT OF DOCOSAHEXAENOIC ACID (DHA) SUPPLEMENTATION VERSUS 5-METHYLTETHRAHYDROFOLATE + B12 VITAMIN AND DHA, IN CYSTIC FIBROSIS PATIENTS AND PANCREATIC INSUFFICIENCY - ND	EXPERIMENTAL STUDY, RANDOMIZED, OPEN LABEL, TO EVALUATE THE EFFECT OF DOCOSAHEXAENOIC ACID (DHA) SUPPLEMENTATION VERSUS 5-METHYLTETHRAHYDROFOLATE + B12 VITAMIN AND DHA, IN CYSTIC FIBROSIS PATIENTS AND PANCREATIC INSUFFICIENCY - ND	CYSTIC FIBROSIS AND PANCREATIC INSUFFICIENCY MedDRA version: 9.1 Level: PT Classification code 10033628 MedDRA version: 9.1 Level: PT Classification code 10011762	Trade Name: *PREFOLIC30CPR GASTROR 15MG Pharmaceutical Form: Gastro-resistant tablet INN or Proposed INN: Detoxifying agents for antineoplastic treatment Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 15- Trade Name: INDUSILOS GTT FL 30MG+FL 15ML* Pharmaceutical Form: Oral drops, solution INN or Proposed INN: Cobamamide Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 2-	AZIENDA OSPEDALIERA ISTITUTI OSPITALIERI DI VERONA		Authorised			Female: yes Male: yes			Italy
508	EUCTR2010-019802-17-PT	13/09/2010	9 October 2012		A randomised, double-blind, placebo-controlled parallel-group trial to confirm the efficacy after 12 weeks and the safety of tiotropium 5 µg administered once daily via the Respimat® device in patients with cystic fibrosis. -	Cystic Fibrosis	Trade Name: Spiriva Respimat 2.5 microgram, solution for inhalation Product Name: Spiriva Respimat 2.5 mcg Product Code: Tiotropium Respimat Pharmaceutical Form: Inhalation vapour, solution CAS Number: 411207313 Current Sponsor code: Ba 679 Br Other descriptive name: TIOTROPIUM BROMIDE MONOHYDRATE Concentration unit: µg microgram(s) Concentration type: equal Concentration number: 3.124- Pharmaceutical form of the placebo: Inhalation vapour* Route of administration of the placebo: Inhalation use	Boehringer Ingelheim France		Not Recruiting			Female: yes Male: yes	360		Hungary;Portugal;Czech Republic;United Kingdom;Germany;France;Spain;Ireland;Italy;Austria
509	NCT01090908	September 2010	19 February 2015	Evaluation of Ciprofloxacin for Inhalation to Cystic Fibrosis Patients With P. Aeruginosa	A Multi-Center, Open Label Study To Evaluate The Safety, Tolerability And Pharmacokinetics Of Ciprofloxacin For Inhalation (CFI) Given Daily For 14 Days To Stable Cystic Fibrosis Patients Ages 6 To 17 With Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: Antibiotic	Aradigm Corporation		Not recruiting	6 Years	18 Years	Both	0	N/A
510	NCT01208285	September 2010	19 February 2015	Study of VX-770 in Subjects With Moderate Hepatic Impairment and in Matched Healthy Subjects	A Phase 1 Non-Randomized, Open-Label Study to Assess the Safety and Pharmacokinetics of VX-770 in Subjects With Moderate Hepatic Impairment and in Matched Healthy Subjects	In Development for Cystic Fibrosis	Drug: VX-770	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	65 Years	Both	24	Phase 1	Czech Republic;Slovakia
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
511	NCT01222273	September 2010	11 June 2018	Open-label Vitamin D Trial for Patients With Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis	Open-label Vitamin D Trial for Patients With Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis	Cystic Fibrosis;Allergic Bronchopulmonary Aspergillosis	Dietary Supplement: cholecalciferol (Vitamin D3)	University of Pittsburgh	National Heart, Lung, and Blood Institute (NHLBI)	Not recruiting	12 Years	N/A	All	7	N/A	United States
512	NCT01223183	September 2010	19 February 2015	Absorptive Clearance After Inhaled Osmotics in Cystic Fibrosis	Absorptive Clearance After Inhaled Osmotics in Cystic Fibrosis	Cystic Fibrosis	Drug: hypertonic saline (7%);Drug: isotonic saline	University of Pittsburgh		Not recruiting	18 Years	N/A	Both	20	Phase 1	United States
513	NCT01257464	September 2010	19 February 2015	Sitagliptin in Cystic Fibrosis-Related Diabetes	The Effects of the DPPIV Inhibitor Sitagliptin in Cystic Fibrosis-related Diabetes	Cystic Fibrosis	Drug: Sitagliptin;Drug: Placebo	University of British Columbia		Not recruiting	19 Years	N/A	Both	3	Phase 2	Canada
514	EUCTR2010-018454-13-DE	31/08/2010	19 March 2012	A Phase III, Multicentre, Randomised, Placebo-Controlled, Double Blind Study of the Incidence of Recurring Pulmonary Exacerbations in Cystic Fibrosis Patients using Two Different Doses of Inhaled Nacystelyn®	A Phase III, Multicentre, Randomised, Placebo-Controlled, Double Blind Study of the Incidence of Recurring Pulmonary Exacerbations in Cystic Fibrosis Patients using Two Different Doses of Inhaled Nacystelyn®	Cystic fibrosis MedDRA version: 12.1 Level: PT Classification code 10011762 Term: Cystic fibrosis	Product Name: Nacystelyn® Product Code: NAL Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: L-Lysine-N-acetylcysteinate Current Sponsor code: NAL Other descriptive name: Nacystelyn® Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 10- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use Product Name: Nacystelyn® Product Code: NAL Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: L-Lysine-N-acetylcysteinate Current Sponsor code: NAL Other descriptive name: Nacystelyn® Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Laboratoires SMB S.A.		Not Recruiting			Female: yes Male: yes	552	Phase 3	Hungary;Czech Republic;United Kingdom;Germany;France;Spain;Italy
515	NCT01140451	August 31, 2010	15 April 2019	Extension Study of Ataluren (PTC124) in Cystic Fibrosis	A Phase 3 Extension Study of Ataluren (PTC124) in Subjects With Nonsense-Mutation-Mediated Cystic Fibrosis	Cystic Fibrosis	Drug: Ataluren (PTC124)	PTC Therapeutics	Cystic Fibrosis Foundation	Not recruiting	6 Years	N/A	All	191	Phase 3	United States;Belgium;Canada;France;Germany;Israel;Italy;Netherlands;Spain;Sweden;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
516	EUCTR2009-012997-11-FR	25/08/2010	15 May 2012	An Open-Label, Rollover Study to Evaluate the Long Term Safety and Efficacy of VX 770 in Subjects with Cystic Fibrosis - PERSIST	An Open-Label, Rollover Study to Evaluate the Long Term Safety and Efficacy of VX 770 in Subjects with Cystic Fibrosis - PERSIST	Cystic Fibrosis MedDRA version: 12.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: VX-770 Product Code: VX-770, VRT-8130077 Pharmaceutical Form: Tablet CAS Number: 873054-44-5 Current Sponsor code: VX-770 Other descriptive name: VRT-813077 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150-	Vertex Pharmaceuticals Incorporated		Authorised			Female: yes Male: yes	217		Germany;United Kingdom;Czech Republic;France;Ireland
517	NCT00782288	August 2010	25 March 2019	Phase II Study of Digitoxin to Treat Cystic Fibrosis	Phase II Study of Digitoxin to Treat Cystic Fibrosis	Cystic Fibrosis	Drug: digitoxin;Other: placebo	National Jewish Health		Not recruiting	18 Years	45 Years	All	24	Phase 2	United States
518	NCT01181622	August 2010	19 February 2015	A Safety and Tolerability Study of Denufosol in 2-4 Year Olds	A Phase 2, Multi-Center, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group, 7-Day Safety and Tolerability Study of Denufosol Tetrasodium Inhalation Solution Administered Via PARI LC® Star in Patients 2 to 4 Years of Age With Cystic Fibrosis	Cystic Fibrosis	Drug: denufosol tetrasodium Inhalation Solution;Drug: 0.9% w/v sodium chloride solution	Merck Sharp & Dohme Corp.		Not recruiting	2 Years	4 Years	Both	25	Phase 2	United States
519	NCT01116089	July 2010	19 February 2015	Pharmacokinetic Study of Bramitob® Administered for Inhalation by PARI eFlow® vs PARI LC® PLUS Nebulizer	PHARMACOKINETIC STUDY OF BRAMITOB® ADMINISTERED FOR INHALATION BY PARI eFLOW® RAPID ELECTRONIC NEBULIZER VS PARI LC® PLUS NEBULIZER COUPLED WITH THE PARI TURBO BOY® N COMPRESSOR IN CYSTIC FIBROSIS PATIENTS INFECTED WITH PSEUDOMONAS AERUGINOSA	Cystic Fibrosis	Drug: Bramitob® administered by PARI LC® PLUS nebulizer;Drug: Bramitob® administered by PARI eFlow® rapid electronic nebulizer	Chiesi Farmaceutici S.p.A.		Not recruiting	18 Years	N/A	Both	25	Phase 1	Czech Republic;Moldova, Republic of;Slovakia;Russian Federation
520	NCT01131507	July 2010	16 December 2017	PR-018: An Open-Label, Safety Extension of Study PR-011	A Multicenter, Open-Label, Safety Extension of Study PR-011 Titled: A Multicenter, Randomized, Open-Label, Crossover Study to Evaluate the Mode of Administration and Safety of EUR-1008 in Infants 1 to 12 Months of Age With Exocrine Pancreatic Insufficiency (EPI) Associated With Cystic Fibrosis (CF)	Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: EUR-1008 (APT-1008)	Forest Laboratories		Not recruiting	N/A	12 Months	All	15	Phase 4	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
521	EUCTR2010-018738-27-DE	02/06/2010	1 April 2013	A Randomized, Double Blind, Parallel Group, Placebo Controlled 28 Day Study to Investigate the Safety, Tolerability and Pharmacodynamics of SB-656933 in Patients with Cystic Fibrosis	A Randomized, Double Blind, Parallel Group, Placebo Controlled 28 Day Study to Investigate the Safety, Tolerability and Pharmacodynamics of SB-656933 in Patients with Cystic Fibrosis	Cystic fibrosis MedDRA version: 12.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: SB-656933 Tablets Product Code: SB-656933-AAA Pharmaceutical Form: Film-coated tablet Current Sponsor code: SB-656933-AAA Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 10- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use Product Name: SB-656933 Tablets Product Code: SB-656933-AAA Pharmaceutical Form: Film-coated tablet Current Sponsor code: SB-656933-AAA Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Film-coated tablet Route of administration of the placebo: Oral use	GlaxoSmithKline Research & Development Limited		Not Recruiting			Female: yes Male: yes	100		Germany
522	NCT01100606	June 2010	19 October 2017	A Study to Evaluate the Mode of Administration and Safety of EUR-1008 (APT-1008) in Infants 1 to 12 Months of Age	A Multicenter, Randomized, Open-Label, Crossover Study to Evaluate the Mode of Administration and Safety of EUR-1008 in Infants 1 to 12 Months of Age With Exocrine Pancreatic Insufficiency (EPI) Associated With Cystic Fibrosis (CF)	Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: EUR-1008 (APT-1008)	Forest Laboratories		Not recruiting	1 Month	12 Months	All	15	Phase 4	United States
523	NCT01149005	June 2010	19 February 2015	Cystic Fibrosis (CF) Exacerbation and Insulin Treatment	Evaluation of Glucose Tolerance and Insulin Treatment in Non Diabetic Patients With Cystic Fibrosis During Acute Pulmonary Exacerbation	Cystic Fibrosis;Impaired Glucose Tolerance;Pulmonary Exacerbation	Drug: novorapid / humalog short acting insulin;Drug: Novo Rapid Insulin (Novonordisk)	Hadassah Medical Organization		Not recruiting	10 Years	N/A	Both	30	N/A	Israel
524	NCT01153542	June 2010	19 February 2015	Study of VX-770 on Desipramine	An Open-Label Phase 1 Study to Examine the Effect of VX 770 on Desipramine in Healthy Subjects	In Development for Cystic Fibrosis	Drug: VX-770	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	24	Phase 1	United States
525	NCT01450267	June 2010	19 February 2015	Inhaled Glutathione (GSH) Versus Placebo in Cystic Fibrosis	Randomized, Single Blind, Controlled Trial of Inhaled Glutathione Versus Placebo in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Inhaled Reduced Glutathione;Drug: Physiological solution	Serafino A. Marsico	Federico II University	Recruiting	6 Years	45 Years	Both	150	Phase 3	Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
526	EUCTR2007-001401-15-DK	21/05/2010	5 December 2016	Treatment of patients with cystic fibrosis with N-acetylcysteine	Treatment of patients with cystic fibrosis with N-acetylcysteine	Patients with cystic fibrosis and chronic Pseudomonas lung infection MedDRA version: 12.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Trade Name: ACC600Tabs Product Name: N-acetylcystein Product Code: R05C B01 Pharmaceutical Form: Tablet INN or Proposed INN: ACETYLCYSTEINE CAS Number: 616-91-1 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 600-	Rigshospitalet		Not Recruiting			Female: yes Male: yes		Phase 4	Denmark
527	EUCTR2010-019194-15-IT	12/05/2010	19 March 2012	Esplorative study to evaluate the tolerability of Amphotericin B lipid complex (Abelcet) by aerosol in patients with Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis (ABPA) - ND	Esplorative study to evaluate the tolerability of Amphotericin B lipid complex (Abelcet) by aerosol in patients with Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis (ABPA) - ND	Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis (ABPA) MedDRA version: 9.1 Level: SOC Classification code 10021881 MedDRA version: 9.1 Level: PT Classification code 10011762	Trade Name: ABELCET Pharmaceutical Form: Concentrate for solution for infusion INN or Proposed INN: Amphotericin B Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 100-	AZIENDA OSPEDALIERA ISTITUTI OSPITALIERI DI VERONA		Authorised			Female: yes Male: yes			Italy
528	NCT00967798	May 2010	29 January 2018	Prevention of Cystic Fibrosis Diabetes	A Randomized, Double-blind, Placebo-controlled Study to Determine Whether Chronic Treatment of Cystic Fibrosis Subjects With Impaired Glucose Tolerance Using Sitagliptin (Januvia) Prevents the Development of Diabetes	Cystic Fibrosis;Prediabetes	Drug: Sitagliptin	Emory University		Not recruiting	13 Years	N/A	All	30	Phase 3	United States;Canada
529	NCT01092572	May 2010	19 February 2015	Statins To Treat Adult Cystic Fibrosis	The Effect of Simvastatin on Systemic Inflammation in Adult Cystic Fibrosis Subjects: A Pilot Study	Cystic Fibrosis;Systemic Inflammation	Drug: Simvastatin;Drug: placebo	University of British Columbia		Not recruiting	18 Years	N/A	Both	0	Phase 1/Phase 2	Canada
530	NCT01158937	May 2010	1 June 2015	Pharmacokinetic Study of Extended Infusion Meropenem in Adult Cystic Fibrosis Patients	Pharmacokinetic Study of Extended Infusion Meropenem in Adult Cystic Fibrosis Patient With Exacerbation of Pulmonary Infection	Cystic Fibrosis Pulmonary Exacerbation	Drug: Meropenem Infusion	St. Michael's Hospital, Toronto	Sunnybrook Health Sciences Centre;University of Toronto	Not recruiting	18 Years	N/A	Both	2	N/A	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
531	NCT02690857	May 2010	3 June 2019	Study of Docosahexanoic Acid in Patients With Cystic Fibrosis (CF)	Efficacy of Docosahexanoic Acid on Lipid Peroxidation in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: Docosahexaenoic acid;Drug: Sunflower Oil	Hospices Civils de Lyon		Not recruiting	6 Years	30 Years	Male	10	Phase 2
532	NCT00763477	April 2010	19 February 2015	Ghrelin in Cystic Fibrosis	The Effect of Ghrelin on Appetite and Immune Function in Patients With Cystic Fibrosis	Cystic Fibrosis	Biological: ghrelin	Papworth Hospital		Recruiting	18 Years	80 Years	Both	20	N/A	United Kingdom
533	NCT01093521	April 2010	11 February 2019	A Pharmacokinetic and Safety Study of IV Gallium Nitrate (Ganite) in Cystic Fibrosis Patients	A Pharmacokinetic and Safety Study of IV Gallium Nitrate (Ganite) in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: 100 mg/m2 dose;Drug: 200 mg/m2 dose	University of Washington	Cystic Fibrosis Foundation	Not recruiting	18 Years	55 Years	All	20	Phase 1	United States
534	NCT01321905	April 2010	19 February 2015	Prospective Intervention Study on Vitamin D in Patients With Cystic Fibrosis	5-month Pilot Intervention Study on Vitamin D in Patients With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Supplementation with vitamin D2/D3	Karolinska Institutet	Stockholm County Council, Sweden;Swedish Cystic Fibrosis Association	Recruiting	6 Years	N/A	Both	15	Phase 2	Sweden
535	NCT01460836	April 2010	19 February 2015	Indirect Comparison of Tobramycin Solution for Inhalation Versus Aztreonam Lysine for Inhalation in the Treatment of Cystic Fibrosis	Indirect Comparison of Tobramycin Solution for Inhalation Versus Aztreonam Lysine for Inhalation in the Treatment of Cystic Fibrosis	Cystic Fibrosis	Drug: Tobramycin solution for inhalation;Drug: Aztreonam lysine for inhalation	Novartis Pharmaceuticals		Not recruiting	6 Years	N/A	Both		N/A
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
536	NCT01086839	March 2010	19 February 2015	Sino-nasal Inhalation of Sodium Chloride 6,0% in Patients With Cystic Fibrosis and Chronic Rhinosinusitis	Sino-nasal Inhalation of Sodium Chloride 6,0% in Patients With Cystic Fibrosis and Chronic Rhinosinusitis. A Multicenter, Randomized, Double-blind, Placebo-controlled, Prospective Clinical Trial	Cystic Fibrosis;Rhinosinusitis	Drug: sodium chloride 6%;Drug: sodium chloride 0,9%	University of Jena		Not recruiting	8 Years	N/A	Both	69	N/A	Germany
537	NCT01348204	March 2010	19 February 2015	Nasal Potential Studies Utilizing Cystic Fibrosis Transmembrane Regulator (CFTR) Modulators	Nasal Potential Studies Utilizing CFTR Modulators (UAB Center for Clinical and Translational Science)	Cystic Fibrosis	Other: quercetin	University of Alabama at Birmingham	National Institutes of Health (NIH)	Not recruiting	8 Years	65 Years	Both	32	Phase 2	United States
538	NCT02198079	March 2010	1 June 2015	A Prospective, Multicenter, Collaborative Study to Determine the Prevalence of Nontuberculous Mycobacteria (NTM) in Pediatric Patients With Cystic Fibrosis in Florida	A Prospective, Multicenter, Collaborative Study to Determine the Prevalence of Nontuberculous Mycobacteria (NTM) in Pediatric Patients With Cystic Fibrosis in Florida	Cystic Fibrosis;Pediatrics	Biological: Sputum	University of Miami	University of Florida	Not recruiting	N/A	18 Years	Both	85	N/A	United States
539	EUCTR2009-014412-35-GB	23/02/2010	19 March 2012	An International, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy, Safety, and Tolerability of Once Daily Administration of Two Strengths of Ciprofloxacin for Inhalation Compared with Placebo for Inhalation in the Management of Pseudomonas aeruginosa in Patients with Non Cystic Fibrosis Bronchiectasis - N/A	An International, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy, Safety, and Tolerability of Once Daily Administration of Two Strengths of Ciprofloxacin for Inhalation Compared with Placebo for Inhalation in the Management of Pseudomonas aeruginosa in Patients with Non Cystic Fibrosis Bronchiectasis - N/A	Non-cystic fibrosis bronchiectasis MedDRA version: 12.0 Level: LLT Classification code 10006446 Term: Bronchiectasis NOS	Product Name: Ciprofloxacin for Inhalation Product Code: None assigned Pharmaceutical Form: Nebuliser solution INN or Proposed INN: CIPROFLOXACIN HYDROCHLORIDE CAS Number: 86483-48-9 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 50- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Aradigm Corporation		Authorised			Female: yes Male: yes	96		Germany;United Kingdom
540	NCT00889967	February 2010	16 December 2017	Safety and Efficacy Study of Ciprofloxacin for Inhalation in Patients With Non-Cystic Fibrosis Bronchiectasis ORBIT-1	An International, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy, Safety, and Tolerability of Once Daily Administration of Two Strengths of Ciprofloxacin for Inhalation Compared With Placebo for Inhalation in the Management of Pseudomonas Aeruginosa in Patients With Non Cystic Fibrosis Bronchiectasis	Non-Cystic Fibrosis Bronchiectasis	Drug: Ciprofloxacin for Inhalation;Drug: Placebo	Aradigm Corporation		Not recruiting	18 Years	80 Years	All	95	Phase 2	United States;Canada;Germany;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
541	NCT01051999	February 2010	19 February 2015	Glutamine Supplementation in Cystic Fibrosis	Glutamine Supplementation and Immunity in Adults With Cystic Fibrosis	Cystic Fibrosis;Immune Function	Dietary Supplement: Glutamine;Dietary Supplement: L-alanine	Emory University	Emmaus Medical, Inc.	Not recruiting	18 Years	N/A	Both	17	Phase 2	United States
542	NCT01059565	February 2010	19 October 2017	Safety and Efficacy Study of Aztreonam for Inhalation Solution (AZLI) in Patients With Cystic Fibrosis and Chronic Burkholderia Species Infection	Phase 3b Randomized, Double-Blind, Placebo-Controlled Two-Part Trial to Assess the Safety and Efficacy of Continuous Aztreonam for Inhalation Solution (AZLI) in Subjects With Cystic Fibrosis (CF) and Chronic Burkholderia Species Infection	Cystic Fibrosis;Burkholderia Infections	Drug: AZLI;Drug: Placebo	Gilead Sciences		Not recruiting	6 Years	N/A	All	102	Phase 3	United States;Canada
543	NCT01060566	February 2010	19 February 2015	Study of VX-770 on Midazolam and Rosiglitazone and the Effect of Fluconazole on VX-770	An Open-Label Phase 1 Study to Examine the Effect of VX-770 on Midazolam and Rosiglitazone and the Effect of Fluconazole on VX-770 in Healthy Subjects	Cystic Fibrosis	Drug: VX-770	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	24	Phase 1	United States
544	NCT01069705	February 2010	16 December 2017	Second Open Label Extension to Bridging Study CTBM100C2303	A Phase III Open-label Extension Study to Assess the Safety and Efficacy of Tobramycin Inhalation Powder After Manufacturing Process Modifications (TIPnew) in Cystic Fibrosis (CF) Patients Who Completed Participation in Study CTBM100C2303E1.	Pulmonary Infections;Pseudomonas Aeruginosa	Drug: Tobramycin inhalation powder	Novartis Pharmaceuticals		Not recruiting	6 Years	21 Years	All	49	Phase 3	Bulgaria;Estonia;Latvia;Lithuania;Romania;Russian Federation;South Africa
545	NCT01288170	February 2010	19 February 2015	Pharmacokinetic Bioequivalence Study of Nebcinal® 150mg/3ml Administered by Aeroneb® Idehaler® Versus Tobi® 300mg/5ml Administered by Pari LC Plus®	Pharmacokinetic Bioequivalence Study of Nebcinal® 150mg/3ml Administered by Aeroneb® Idehaler® Versus Tobi® 300mg/5ml Administered by Pari LC Plus®	Cystic Fibrosis	Drug: Nebcinal Tobi;Drug: Tobi Nebcinal	Erempharma	Hopitaux de Lyon;University of Lyon	Recruiting	6 Years	N/A	Both	12	N/A	France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
546	EUCTR2009-013340-36-IT	25/01/2010	27 January 2014	PREVENTION OF REPERFUSION INJURY IN HUMAN LUNG TRANSPLANTATION FOR CYSTIC FIBROSIS BY TARGETING IL-8 ACTIVITY - ND	PREVENTION OF REPERFUSION INJURY IN HUMAN LUNG TRANSPLANTATION FOR CYSTIC FIBROSIS BY TARGETING IL-8 ACTIVITY - ND	cystic fibrosis MedDRA version: 12.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: reparixin Pharmaceutical Form: Concentrate for solution for infusion INN or Proposed INN: Reparixin CAS Number: 266359-83-5 Current Sponsor code: DF 1681Y Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 33- Pharmaceutical form of the placebo: Concentrate for solution for infusion Route of administration of the placebo: Intravenous use	IST. DI RICERCHE FARMACOLOG. M. NEGRI		Not Recruiting			Female: yes Male: yes			Italy
547	EUCTR2009-016734-26-EE	08/01/2010	29 October 2012	A Phase III Open-Label Extension Study to Assess the Safety and Efficacy of Tobramycin Inhalation Powder after Manufacturing Process Modifications (TIPnew) in Cystic Fibrosis (CF) Patients Who successfully Completed Participation in Study CTBM100C2303E1.	A Phase III Open-Label Extension Study to Assess the Safety and Efficacy of Tobramycin Inhalation Powder after Manufacturing Process Modifications (TIPnew) in Cystic Fibrosis (CF) Patients Who successfully Completed Participation in Study CTBM100C2303E1.	Pseudomonas aeruginosa infection in cystic fibrosis patients MedDRA version: 12.0 Level: LLT Classification code 10021860 Term: Infection pseudomonas aeruginosa	Product Name: TIP (Tobramycin inhalation powder) Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Tobramycin Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28-	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	100	Phase 3	Estonia;Lithuania;Bulgaria;Latvia
548	NCT00996424	January 2010	19 February 2015	The Effect of Inhaled N-Acetylcysteine Compared to Normal Saline on Sputum Rheology and Lung Function	The Effect of Inhaled N-Acetylcysteine Compared to Normal Saline on Sputum Rheology and Lung Function.	Cystic Fibrosis	Drug: Acetylcysteine;Drug: normal saline	University Hospital, Ghent	BVSM	Not recruiting	6 Years	64 Years	Both	19	Phase 4	Belgium
549	NCT01044719	January 2010	19 February 2015	Duration of Antibiotics in Infective Exacerbations of Cystic Fibrosis	What Duration of Intravenous Antibiotic Therapy Should be Used in the Treatment of Infective Exacerbations of Cystic Fibrosis Chronically Colonised With Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: Ceftazidime;Drug: Tobramycin;Drug: Meropenem	Imperial College London		Not recruiting	16 Years	N/A	Both	240	Phase 4	United Kingdom
550	NCT01155115	January 2010	1 June 2015	Inflammatory and Microbiologic Markers in Sputum: Comparing Cystic Fibrosis With Primary Ciliary Dyskinesia	Inflammatory and Microbiologic Markers in Sputum in Response to Pulmonary Exacerbation: Comparing Cystic Fibrosis With Primary Ciliary Dyskinesia	Cystic Fibrosis;Primary Ciliary Dyskinesia	Procedure: Sputum Collection;Procedure: Pulmonary Function Testing;Procedure: Exhaled Nitric Oxide	The Hospital for Sick Children		Not recruiting	6 Years	18 Years	Both	46	N/A	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
551	EUCTR2010-019267-11-DE		29 May 2012	AN OPEN-LABEL, MULTICENTER, RANDOMIZED, CROSS-OVER STUDY TO COMPARE THE SAFETY AND EFFICACY OF PANZYTRAT® 25 000 TO KREON® 25 000 IN THE CONTROL OF STEATORRHEA IN SUBJECTS AGED 7 YEARS AND OLDER WITH CYSTIC FIBROSIS (CF) AND EXOCRINE PANCREATIC INSUFFICIENCY (EPI)	AN OPEN-LABEL, MULTICENTER, RANDOMIZED, CROSS-OVER STUDY TO COMPARE THE SAFETY AND EFFICACY OF PANZYTRAT® 25 000 TO KREON® 25 000 IN THE CONTROL OF STEATORRHEA IN SUBJECTS AGED 7 YEARS AND OLDER WITH CYSTIC FIBROSIS (CF) AND EXOCRINE PANCREATIC INSUFFICIENCY (EPI)	Correction of steathorrea associated with chronic exocrine pancreatic insufficiency in cystic fibrosis MedDRA version: 13.1 Level: PT Classification code 10033628 Term: Pancreatic insufficiency System Organ Class: 10017947 - Gastrointestinal disorders MedDRA version: 13.1 Level: LLT Classification code 10041968 Term: Steatorrhea System Organ Class: 10017947 - Gastrointestinal disorders MedDRA version: 13.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders	Trade Name: PANZYTRAT 25000 Product Name: PANZYTRAT 25000 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Pancreas Powder CAS Number: 8049-47-6 Other descriptive name: PANCREATIN Concentration unit: Other Concentration type: equal Concentration number: 25000- Trade Name: KREON 25000 Product Name: KREON 25000 Pharmaceutical Form: Capsule, hard CAS Number: 8049-47-6 Other descriptive name: PANCREATIN Concentration unit: Other Concentration type: equal Concentration number: 25000-	Axcan Pharma Inc.		Not Recruiting			Female: yes Male: yes	68		Germany
552	EUCTR2010-023235-41-PL		2 October 2017	A comparative, randomised, two period, multi-center, cross-over 14 weeks bioequivalence study of Tobramycin PARI (T100) versus TOBI® (Novartis) in cystic fibrosis patients with bronchopulmonary chronic Pseudomonas aeruginosa infection - T-100 BE	A comparative, randomised, two period, multi-center, cross-over 14 weeks bioequivalence study of Tobramycin PARI (T100) versus TOBI® (Novartis) in cystic fibrosis patients with bronchopulmonary chronic Pseudomonas aeruginosa infection - T-100 BE	Cystic fibrosis with bronchopulmonary chronic Pseudomonas aeruginosa infection MedDRA version: 12.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Tobramycin Product Code: T100 Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Other descriptive name: Tobramycinum Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 170/1,7- Trade Name: TOBI® Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: TOBRAMYCIN CAS Number: 32986-56-4 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 300/5,0-	PARI Pharma GmbH		Not Recruiting			Female: yes Male: yes	60	Phase 1	Poland
553	EUCTR2010-023533-34-FR		2 October 2017	Etude pharmacocinétique de l’équivalence de la biodisponibilité entre Nebcinal® 150mg/3ml administré par Aeroneb® Idehaler® et Tobi® 300mg/5ml administré par Pari LC Plus ® /Pulmoaid ® chez des patients atteints de mucoviscidose.	Etude pharmacocinétique de l’équivalence de la biodisponibilité entre Nebcinal® 150mg/3ml administré par Aeroneb® Idehaler® et Tobi® 300mg/5ml administré par Pari LC Plus ® /Pulmoaid ® chez des patients atteints de mucoviscidose.	Mucoviscidose MedDRA version: 12.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Nebcinal Product Code: Nebcinal Pharmaceutical Form: Inhalation vapour, solution Trade Name: Tobi Product Name: Tobi Pharmaceutical Form: Inhalation vapour, solution	EREMPHARMA SAS		Not Available			Female: yes Male: yes		Phase 1	France
554	EUCTR2009-016590-15-HU	29/12/2009	4 August 2015	A clinical trial to assess the treatment with nebulised tobramycin in terms of safety and ability to kill Pseudomonas bacteria in the lungs of cystic fibrosis patients aged 3 months to 6 years included	A Randomized, Double-Blind, Placebo-Controlled, Crossover Multi-Center Study to Assess the Efficacy and Safety of Inhaled Tobramycin Nebuliser Solution (TOBI®) for the Treatment of Early Infections of P. aeruginosa in Cystic Fibrosis Subjects Aged from 3 Months to less than 7 years.	Lung colonisation with Pseudomonas aeruginosa in cystic fibrosis patients MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders MedDRA version: 14.1 Level: LLT Classification code 10068292 Term: Pseudomonas colonization System Organ Class: 100000004862 MedDRA version: 14.1 Level: LLT Classification code 10068297 Term: Pseudomonas colonisation System Organ Class: 100000004862 ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: TOBI 300 mg / 5 mL nebuliser solution Product Name: TOBI Product Code: TBM100 Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 60- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	50		France;Hungary;Greece;Canada;Poland;Russian Federation;Germany;Italy;Switzerland
555	NCT00812045	December 2009	19 February 2015	Study to Assess Efficacy of AZD1236 in Patients With Cystic Fibrosis	A Randomised, Double-Blind, Placebo-Controlled, Parallel Group, Phase II Study to Assess the Efficacy of 28 Day Oral Administration of AZD1236 in Adult Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: AZD1236;Drug: Placebo	AstraZeneca		Not recruiting	18 Years	N/A	Both	44	Phase 2	Canada;Netherlands;Poland;Spain
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
556	NCT01025713	December 2009	3 August 2015	A Phase 1 Trial to Assess the Safety, Tolerability, and Pharmacokinetics of GS 9411 in Subjects With Cystic Fibrosis (CF)	A Phase 1, Randomized, Double-Blind, Placebo-Controlled Trial to Assess the Safety, Tolerability, and Pharmacokinetics of GS 9411 in Subjects With Cystic Fibrosis (CF)	Cystic Fibrosis;Mucociliary Clearance	Drug: GS-9411;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	65 Years	Both	0	Phase 1	Australia
557	NCT01035853	December 2009	19 February 2015	Sino-nasal Inhalation of Colistin in Patients With Cystic Fibrosis and Pseudomonas Aeruginosa Colonization	Sino-nasal Inhalation of Colistin Via the Pari Sinus Nebulizer in Patients With Cystic Fibrosis and Colonization of the Upper Airways With Pseudomonas Aeruginosa	Cystic Fibrosis;Pseudomonas Aeruginosa	Drug: Colistin	University of Jena		Not recruiting	6 Years	N/A	Both	10	Phase 2	Germany
558	NCT01658449	December 2009	19 February 2015	Comparison of the Tolerability of Two Formulations of Hypertonic Saline in Cystic Fibrosis Patients		Cystic Fibrosis	Other: Inhalable Hypertonic saline 7% + Hyaluronan 0.1%;Other: Inhalable Hypertonic saline 7%	Ospedale Civile Ca' Foncello		Not recruiting	8 Years	N/A	Both	40	N/A	Italy
559	EUCTR2008-008317-20-BE	13/11/2009	7 October 2014	The effect of inhaled N-Acetylcysteine compared to normal saline on sputum rheology and lung function	The effect of inhaled N-Acetylcysteine compared to normal saline on sputum rheology and lung function	Cystic Fibrosis MedDRA version: 12.0 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Lysomucil 10 % Product Name: N-acetylcysteine Pharmaceutical Form: Solution for injection INN or Proposed INN: Acetylcysteine CAS Number: 616-91-1 Concentration unit: ml millilitre(s) Concentration type: equal Concentration number: 3- Pharmaceutical form of the placebo: Solution for injection Route of administration of the placebo: Inhalation use	University Hospital Ghent		Not Recruiting			Female: yes Male: yes			Belgium
560	EUCTR2009-016662-90-FR	03/11/2009	19 March 2012	Etude de cohorte évaluant l'efficacité cliique, la tolérance et l'immunogénicité à la vaccination antigrippale pandémique chez les patients atteints de mucoviscidose et, le cas échéant, l'expression clinique de la grippe A (H1N1) et les facteurs associés à la survenue de formes sévères dans cette population. - Etude MUCOFLU	Etude de cohorte évaluant l'efficacité cliique, la tolérance et l'immunogénicité à la vaccination antigrippale pandémique chez les patients atteints de mucoviscidose et, le cas échéant, l'expression clinique de la grippe A (H1N1) et les facteurs associés à la survenue de formes sévères dans cette population. - Etude MUCOFLU	patients atteints de mucoviscidose MedDRA version: 12.0 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Oseltamivir Pharmaceutical Form: Capsule* Product Name: Vaccin grippal pandémique H1N1 Pharmaceutical Form: Solution for injection INN or Proposed INN: Vaccin grippal pandémique H1N1	Inserm		Authorised			Female: yes Male: yes			France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
561	NCT01018368	November 2009	19 February 2015	Study of VX-770 and Rifampin in Healthy Male Subjects	An Open-Label Phase 1 Study to Examine the Effect of Multiple Doses of Rifampin on the Single-Dose Pharmacokinetics of VX 770 in Healthy Subjects	Cystic Fibrosis	Drug: VX-770;Drug: Rifampin	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Male	24	Phase 1	United States
562	NCT01094704	November 2009	19 October 2017	Durability of Hypertonic Saline for Enhancing Mucociliary Clearance in Cystic Fibrosis	Durability of Hypertonic Saline for Enhancing Mucociliary Clearance in Cystic Fibrosis	Cystic Fibrosis	Drug: sodium chloride (7%)	University of North Carolina, Chapel Hill	Johns Hopkins University;Novartis Pharmaceuticals	Not recruiting	18 Years	N/A	All	16	Phase 1	United States
563	EUCTR2009-014042-28-GB	16/10/2009	19 March 2012	What duration of intravenous antibiotic therapy should be used in the treatment of infective exacerbations of cystic fibrosis in patients chronically colonised with Pseudomonas aeruginosa? - Duration of antibiotics in infective exacerbations of cystic fibrosis	What duration of intravenous antibiotic therapy should be used in the treatment of infective exacerbations of cystic fibrosis in patients chronically colonised with Pseudomonas aeruginosa? - Duration of antibiotics in infective exacerbations of cystic fibrosis	Cystic Fibrosis	Trade Name: Meropenem Product Name: Meropenem Pharmaceutical Form: Powder for infusion* INN or Proposed INN: Meropenem CAS Number: 119478-56-7 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: up to Concentration number: 50- Trade Name: Ceftazidime Product Name: Ceftazidime Pharmaceutical Form: Powder for injection* INN or Proposed INN: Ceftazidime CAS Number: 72558-82-8 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: up to Concentration number: 200- Trade Name: Tobramycin Product Name: Tobramycin Pharmaceutical Form: Solution for injection INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: up to Concentration number: 40-	Imperial College, London		Authorised			Female: yes Male: yes	240		United Kingdom
564	EUCTR2009-015875-28-IT	07/10/2009	3 April 2012	Modulation of intestinal and extraintestinal inflammation in infants with Cystic Fibrosis by early modification of intestinal microflora - Role of intestinal microflora in infants with Cystic Fibrosis	Modulation of intestinal and extraintestinal inflammation in infants with Cystic Fibrosis by early modification of intestinal microflora - Role of intestinal microflora in infants with Cystic Fibrosis	infants with cystic fibrosis MedDRA version: 9.1 Level: SOC Classification code 10017947 MedDRA version: 9.1 Level: SOC Classification code 10038738	Pharmaceutical Form: Capsule, hard INN or Proposed INN: Lactic acid producing organisms Concentration unit: CFU/g colony forming unit(s)/gram Concentration type: equal Concentration number: 20000000000- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	UNIVERSITA` DEGLI STUDI DI NAPOLI FEDERICO II		Authorised			Female: yes Male: yes			Italy
565	NCT00999531	October 2009	19 February 2015	A Randomized, Double-Blind, Placebo-Controlled Multiple Dose Trial of GS-9411 in Healthy Volunteers	A Phase 1, Randomized, Double-Blind, Placebo-Controlled Multiple Dose Trial to Assess the Safety, Tolerability, and Pharmacokinetics of GS-9411 in Healthy Volunteers	Cystic Fibrosis;Mucociliary Clearance;Airway Hydration	Drug: GS-9411;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	65 Years	Both	24	Phase 1	Australia
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
566	NCT00903201	September 28, 2009	16 December 2017	28 Day Repeat Dose in Cystic Fibrosis Patients	A Randomized, Double Blind, Parallel Group, Placebo Controlled 28 Day Study to Investigate the Safety, Tolerability and Pharmacodynamics of SB-656933 in Patients With Cystic Fibrosis.	Cystic Fibrosis	Drug: SB656933;Drug: Placebo	GlaxoSmithKline		Not recruiting	18 Years	N/A	All	146	Phase 2	United States;Canada;France;Germany;Israel
567	EUCTR2009-014050-15-BE	18/09/2009	14 October 2013	Conversion from a twice-daily tacrolimus (tac) regimen to a once-daily tacorlimus exended-release formulation (TacXL) regimen : Pharamcokinetic studies in stable cystic fibrosis (CF) and non-CF patients having received a pulmonary transplant - Tac-XL	Conversion from a twice-daily tacrolimus (tac) regimen to a once-daily tacorlimus exended-release formulation (TacXL) regimen : Pharamcokinetic studies in stable cystic fibrosis (CF) and non-CF patients having received a pulmonary transplant - Tac-XL	Stable cystic fibrosis (CF) and non-CF patients having received a pulmonary transplant MedDRA version: 12.0 Level: LLT Classification code 10025127 Term: Lung transplant	Trade Name: Prograft capsules Product Name: Tacrolimus capsules Pharmaceutical Form: Capsule, hard INN or Proposed INN: TACROLIMUS CAS Number: 104987113 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 0.5- INN or Proposed INN: TACROLIMUS CAS Number: 104987113 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 1- INN or Proposed INN: TACROLIMUS CAS Number: 104987113 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5- Trade Name: Advagraf prolonged-release hard capsules Product Name: Tacrolimus prolonged-release hard capsules Pharmaceutical Form: Capsule, hard	Hopital Erasme - Chest service		Authorised			Female: yes Male: yes			Belgium
568	EUCTR2009-013660-39-FR	10/09/2009	19 March 2012	Etude pharmacocinétique de l’équivalence de la biodisponibilité entre Nebcinal® 150mg/3ml administré par Aeroneb® Idehaler® et Tobi® 300mg/5ml administré par Pari LC Plus ®	Etude pharmacocinétique de l’équivalence de la biodisponibilité entre Nebcinal® 150mg/3ml administré par Aeroneb® Idehaler® et Tobi® 300mg/5ml administré par Pari LC Plus ®	Mucoviscidose MedDRA version: 12.0 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Nebcinal Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: tobramycine Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Trade Name: Tobi Product Name: Tobi Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: tobramycine Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300-	Erempharma SAS		Authorised			Female: yes Male: yes			France
569	EUCTR2008-002318-22-LT	07/09/2009	29 May 2012	A Randomized, Double-Blind, Placebo-Controlled, Multi-Center Phase III Study in Cystic Fibrosis (CF) Subjects to Assess Efficacy, Safety and Pharmacokinetics of Tobramycin Inhalation Powder from a Modified Manufacturing Process (TIPnew).	A Randomized, Double-Blind, Placebo-Controlled, Multi-Center Phase III Study in Cystic Fibrosis (CF) Subjects to Assess Efficacy, Safety and Pharmacokinetics of Tobramycin Inhalation Powder from a Modified Manufacturing Process (TIPnew).	Pseudomonas aeruginosa infection in cystic fibrosis patients MedDRA version: 9.1 Level: LLT Classification code 10021860 Term: Infection pseudomonas aeruginosa	Product Name: TIP (Tobramycin inhalation powder) Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: tobramycin Other descriptive name: TBM100C Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	100	Phase 3	Bulgaria;Estonia;Latvia;Lithuania
570	EUCTR2008-004764-39-LT	07/09/2009	15 May 2012	A Phase III Open-Label Extension Study to Assess the Safety and Efficacy of Tobramycin Inhalation Powder after Manufacturing Process Modifications (TIPnew) in Cystic Fibrosis (CF) Subjects Who Completed Participation in Study CTBM100C2303.	A Phase III Open-Label Extension Study to Assess the Safety and Efficacy of Tobramycin Inhalation Powder after Manufacturing Process Modifications (TIPnew) in Cystic Fibrosis (CF) Subjects Who Completed Participation in Study CTBM100C2303.	Pseudomonas aeruginosa infection in cystic fibrosis patients MedDRA version: 13.1 Level: LLT Classification code 10021860 Term: Infection pseudomonas aeruginosa System Organ Class: 10021881 - Infections and infestations	Product Name: TIP (Tobramycin inhalation powder) Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: tobramycin Current Sponsor code: TBM100C Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28-	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	100	Phase 3	Bulgaria;Estonia;Latvia;Lithuania
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
571	NCT00951522	September 2009	19 February 2015	A Trial to Assess the Safety, Tolerability, and Pharmacokinetics of GS-9411 in Healthy Male Volunteers	A Phase 1 Randomized, Double-Blind, Placebo-Controlled Trial to Assess the Safety, Tolerability, and Pharmacokinetics of GS-9411 in Healthy Male Volunteers	Cystic Fibrosis;Mucociliary Clearance;Airway Hydration	Drug: GS-9411;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	45 Years	Male	15	Phase 1	Australia
572	NCT00953706	September 2009	19 October 2017	Study of Ivacaftor in Cystic Fibrosis Subjects Aged 12 Years and Older Homozygous for the F508del-CFTR Mutation	A Phase 2, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Safety and Efficacy of VX-770 in Subjects Aged 12 Years and Older With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation	Cystic Fibrosis	Drug: Ivacaftor;Drug: Placebo	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	12 Years	N/A	All	140	Phase 2	United States
573	NCT00966602	September 2009	19 February 2015	Drug-Drug Interaction Study of VX-809 and VX-770 in Healthy Subjects	A Randomized, Double-Blind, Placebo-Controlled, Multiple-Dose, Drug-Drug Interaction Study of VX-809 and VX-770 in Healthy Subjects	Cystic Fibrosis	Drug: VX-809;Drug: VX-770;Drug: VX-809 & VX-770;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	55 Years	Both	24	Phase 1	Netherlands
574	NCT00970346	September 2009	11 April 2016	Safety and Efficacy of Inhaled OligoG CF-5/20 for the Treatment Cystic Fibrosis	A Randomised, Double-blind, Placebo-controlled Dose-escalation Phase I Study to Evaluate the Safety and Tolerability of Inhaled Aerosolised OligoG CF-5/20 (G-block Oligosaccharide Derived From Alginate Polysaccharide) in Healthy Volunteers	Cystic Fibrosis	Drug: OligoG CF-5/20	AlgiPharma AS	Smerud Medical Research International AS	Not recruiting	18 Years	65 Years	Male	26	Phase 1	United Kingdom
575	NCT00975663	September 2009	19 February 2015	Bayesian Dose Adjustment of Immunosuppressants After Lung Transplantation	Evaluation of the Interest of Therapeutic Drug Monitoring of Immunosuppressants (Tacrolimus, Mycophenolate Mofetil) Based on Bayesian Estimation During the Three First Years Following Lung Transplantation, in Patients With or Without Cystic Fibrosis	Lung and Heart-lung Transplantation	Drug: Tacrolimus and MMF	University Hospital, Limoges		Not recruiting	18 Years	N/A	Both	180	Phase 4	Belgium;France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
576	NCT00989807	September 2009	19 February 2015	Expanded Access Program for Aztreonam Lysine for Inhalation in Canadian Patients With Cystic Fibrosis	Expanded Access for Aztreonam Lysine for Inhalation in Canadian Patients With Cystic Fibrosis and Pseudomonas Aeruginosa Airway Infection Who Have Limited Treatment Options and Are at Risk for Disease Progression	Cystic Fibrosis;Pseudomonas Aeruginosa	Drug: Aztreonam lysine	Gilead Sciences		Not recruiting	6 Years	N/A	Both		N/A	Canada
577	NCT01031706	September 2009	16 December 2017	Effect of Hypertonic Saline on Mucus Clearance in Children Ages 5-12 With Cystic Fibrosis	Sustained Impact of Hypertonic Saline on Mucociliary Clearance in Young Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Hypertonic Saline;Drug: Placebo	University of North Carolina, Chapel Hill	National Heart, Lung, and Blood Institute (NHLBI)	Not recruiting	5 Years	12 Years	All	23	N/A	United States
578	NCT01111383	September 2009	11 June 2018	A Single Arm 48-Week Follow-on Safety Study to a Core Study Comparing the Efficacy and Tolerability of Tobrineb®/Actitob®/Bramitob® Versus TOBI®	A Single Arm 48-Week Follow-on Safety Study to the Core Study (A Multicentre, Multinational, Open-Label, Randomised, Parallel Group Clinical Trial of Tobrineb®/Actitob®/Bramitob® (Tobramycin Solution for Nebulisation, 300mg Twice Daily in 4mL Unit Dose Vials) Compared to TOBI® in the Treatment of Patients With Cystic Fibrosis and Chronic Infection With Pseudomonas Aeruginosa)	Cystic Fibrosis	Drug: tobramycin	Chiesi Farmaceutici S.p.A.		Not recruiting	6 Years	N/A	All	209	Phase 3	France;Poland;Ukraine
579	NCT01710449	September 2009	19 February 2015	Evaluation of Regional Ventilation Using 19F MRI of Inert Perfluorinated Gases Mixed With Oxygen	Evaluation of Regional Ventilation in Normal Subjects and Subjects With Airway and Lung Disorders (COPD, Asthma, Emphysema, Small Airway Disease and Cystic Fibrosis) Using 19F MRI of Inert Perfluorinated Gases Mixed With Oxygen.	COPD;Asthma;Cystic Fibrosis;Emphysema;Small Airways Diseases	Drug: perfluorinated gas/oxygen mixture	Hal C Charles		Not recruiting	18 Years	N/A	Both	44	Phase 1	United States
580	NCT01961661	September 2009	19 February 2015	Probiotics on Intestinal Inflammation in Cystic Fibrosis	Effect of Probiotics on Intestinal Inflammation and Microflora in Cystic Fibrosis: a Pilot Study	Cystic Fibrosis	Dietary Supplement: Lactobacillus rhamnosus GG;Dietary Supplement: placebo	Federico II University		Not recruiting	2 Years	18 Years	Both	22	Phase 3	Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
581	EUCTR2008-006446-25-NL	26/08/2009	19 March 2012	A randomized, double-blind, placebo-controlled, multiple dose study of VX-809 to evaluate safety, pharmacokinetics, and pharmacodynamics of VX-809 in cystic fibrosis subjects homozygous for the deltaF508-CFTR gene mutation - Study VX08-809-101	A randomized, double-blind, placebo-controlled, multiple dose study of VX-809 to evaluate safety, pharmacokinetics, and pharmacodynamics of VX-809 in cystic fibrosis subjects homozygous for the deltaF508-CFTR gene mutation - Study VX08-809-101	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011764 Term: Cystic fibrosis NOS	Product Name: VX-809 Product Code: VX-809 Pharmaceutical Form: Capsule, hard CAS Number: 936727-05-8 Current Sponsor code: VX-809 Other descriptive name: VRT-826809 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25-50 Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	90		Germany;Netherlands;Belgium
582	EUCTR2008-007416-15-IE	11/08/2009	10 July 2015	Study of VX-770 in Cystic Fibrosis Subjects Age 12 and Older With the G551D Mutation	A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of VX 770 in Subjects with Cystic Fibrosis and the G551D Mutation - STRIVE	Cystic Fibrosis MedDRA version: 14.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: VX-770 Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Other descriptive name: VRT-813077 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	100	Phase 3	France;United States;Czech Republic;Canada;Ireland;Australia;Germany;United Kingdom
583	EUCTR2008-007479-26-IE	11/08/2009	29 May 2012	Study of VX-770 in Cystic Fibrosis Subjects Age 6 to 11 With the G551D Mutation	A Phase 3, 2 Part, Randomized, Double-Blind, Placebo Controlled, Parallel Group Study to Evaluate the Pharmacokinetics, Efficacy and Safety of VX 770 in Subjects Aged 6 to 11 Years with Cystic Fibrosis and the G551D Mutation - ENVISION	Cystic Fibrosis MedDRA version: 14.0 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]	Product Name: VX-770 Product Code: VX-770, VRT-813077 Pharmaceutical Form: Film-coated tablet INN or Proposed INN: ivacaftor CAS Number: 873054-44-5 Current Sponsor code: VX-770 Other descriptive name: VRT-813077 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	40	Phase 3	Germany;United Kingdom;Canada;France;Ireland;United States;Australia
584	NCT00803205	July 2009	16 December 2017	Study of Ataluren (PTC124™) in Cystic Fibrosis	A Phase 3 Efficacy and Safety Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	Cystic Fibrosis	Drug: Ataluren (PTC124);Drug: Placebo	PTC Therapeutics	Cystic Fibrosis Foundation Therapeutics	Not recruiting	6 Years	N/A	All	238	Phase 3	United States;Belgium;Canada;France;Germany;Israel;Italy;Netherlands;Spain;Sweden;United Kingdom
585	NCT00910351	July 2009	19 February 2015	Cipro Inhaler for Cystic Fibrosis Children Ages 6-12	A Study to Evaluate the Safety and Pharmacokinetics of Ciprofloxacin in Adults and Children Aged 6 - 12 Years With Cystic Fibrosis Following Inhalation of Ciprofloxacin Dry Powder	Pseudomonas Infection	Drug: Ciprofloxacin (Cipro, BAYQ3939)	Bayer		Not recruiting	6 Years	12 Years	Both	19	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
586	NCT00945347	July 2009	19 February 2015	Does a Nasal Instillation of Miglustat Normalize the Nasal Potential Difference in Cystic Fibrosis Patients ?	Does a Nasal Instillation of Miglustat Normalize the Nasal Potential Difference in Cystic Fibrosis Patients Homozygous for the F508del Mutation? A Randomized, Double Blind Placebo-controlled Study.	Cystic Fibrosis	Drug: Miglustat;Drug: Placebo	Cliniques universitaires Saint-Luc- Université Catholique de Louvain		Not recruiting	14 Years	N/A	Both	10	Phase 2	Belgium
587	EUCTR2008-007877-19-DE	15/06/2009	29 April 2013	Nasale Inhalation von Colistin mit dem Pari-Sinus-Vernebler bei Patienten mit Mukoviszidose und Pseudomonasnachweis im Nasen-Nasennebenhöhlenbereich. Nasal inhalation of colistin by the Pari Sinus nebulizer im patients with cystic fibrosis and pseudomonas aeruginosa colonization in the upper airways. - colistin nasal CF pilot	Nasale Inhalation von Colistin mit dem Pari-Sinus-Vernebler bei Patienten mit Mukoviszidose und Pseudomonasnachweis im Nasen-Nasennebenhöhlenbereich. Nasal inhalation of colistin by the Pari Sinus nebulizer im patients with cystic fibrosis and pseudomonas aeruginosa colonization in the upper airways. - colistin nasal CF pilot	subjects with cystic fibrosis and pseudomonas aeruginosa colonization in the upper airways MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Colistin CF Product Name: Colistin CF Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Colistimethat-Natrium Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 1 million-	University of Jena		Not Recruiting			Female: yes Male: yes	14		Germany
588	EUCTR2009-009869-34-DE	08/06/2009	11 March 2013	Randomized, placebo-controlled, double-blind, multi center study to evaluate the safety and efficacy of ciprofloxacin inhale compared to placebo in patients with non-cystic fibrosis bronchiectasis - Bayq3939	Randomized, placebo-controlled, double-blind, multi center study to evaluate the safety and efficacy of ciprofloxacin inhale compared to placebo in patients with non-cystic fibrosis bronchiectasis - Bayq3939	Idiopathic and post-pneumonic non-Cystic Fibrosis Bronchiectasis in pulmonary stable patients (defined as FEV1 of > 35 % and < 80 percent of predicted) MedDRA version: 9.1 Level: LLT Classification code 10006445 Term: Bronchiectasis MedDRA version: 9.1 Level: PT Classification code 10006445 Term: Bronchiectasis	Product Name: Cipro Inhale Product Code: BAYq3939 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Ciprofloxacin CAS Number: 85721-33-1 Current Sponsor code: BAYQ3939 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 32,5- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Bayer HealthCare AG		Not Recruiting			Female: yes Male: yes	122		Spain;Germany;United Kingdom;Sweden
589	ChiCTR-TRC-13003979	2009-05-07	18 April 2017	Application of roxithromycin in patients with bronchiectasis in stable condition	Effect of low-dose, long-term roxithromycin on airway imflammation and remodeling of stable non-cystic fibrosis bronchiectasis	Bronchietasis	Roxithromycin group:The patients received oral roxithromycin in roxithromycin group;Control group:The patients received no drug in control group;	First Affiliated Hospital of Guangxi Medical University		Not Recruiting	18	70	Both	Roxithromycin group:26;Control group:26;	Post-market	China
590	NCT01737983	May 2009	19 February 2015	Effect of Lactobacillus Reuteri in Cystic Fibrosis	Lactobacillus Reuteri Reduces Pulmonary Exacerbations and Upper Respiratory Tract Infections in CF Patients With Mild-to-moderate Lung Disease. LR Administration Might Have a Beneficial Effect on the Disease Course of Cystic Fibrosis.	CYSTIC FIBROSIS	Dietary Supplement: Lactobacillus reuteri;Dietary Supplement: placebo	Azienda Policlinico Umberto I		Not recruiting	6 Years	42 Years	Both	61	Phase 4	Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
591	NCT01880723	May 2009	19 October 2017	Utilizing Exhaled Breathe Condensate Collection to Study Ion Regulation in Cystic Fibrosis	Modifying Genes in Cystic Fibrosis: The Beta-2 Adrenergic Receptors and Epithelial Na+ Channels	Cystic Fibrosis;Healthy	Drug: Albuterol;Drug: Placebo saline	University of Arizona	National Heart, Lung, and Blood Institute (NHLBI)	Not recruiting	15 Years	55 Years	All	32	N/A	United States
592	EUCTR2009-011289-27-IT	22/04/2009	3 April 2012	Probiotics in Cystic Fibrosis - ND	Probiotics in Cystic Fibrosis - ND	Cystic Fibrosis Patients MedDRA version: 9.1 Level: SOC Classification code 10017947	Pharmaceutical Form: Modified-release capsule, soft INN or Proposed INN: Lactic acid producing organisms Concentration unit: CFU/g colony forming unit(s)/gram Concentration type: equal Concentration number: 20000000000- Pharmaceutical form of the placebo: Modified-release capsule, soft Route of administration of the placebo: Oral use	UNIVERSITA` DEGLI STUDI DI NAPOLI FEDERICO II		Authorised			Female: yes Male: yes			Italy
593	EUCTR2008-003924-52-SE	08/04/2009	19 November 2012	A Phase 3 Efficacy and Safety Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	A Phase 3 Efficacy and Safety Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	Nonsense-Mutation-Mediated Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: PTC124 Product Code: PTC124 Pharmaceutical Form: Powder for oral suspension INN or Proposed INN: ataluren CAS Number: 775304-57-9 Current Sponsor code: PTC124 Other descriptive name: PTC-0161480; PTC-124; Compound 1a; RPS 2505; PTC-C124; 291844 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Pharmaceutical form of the placebo: Powder for oral suspension Route of administration of the placebo: Oral use Product Name: PTC124 Product Code: PTC124 Pharmaceutical Form: Powder for oral suspension INN or Proposed INN: ataluren CAS Number: 775304-57-9 Current Sponsor code: PTC124 Other descriptive name: PTC-0161480; PTC-124; Compound 1a; RPS 2505; PTC-C124; 291844 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 250- Pharmaceutical form of the placebo: Powder for oral suspension Route of administration of the placebo: Oral use Product Name: PTC124 Product Code: PTC124 Pharmaceutical Form: Powder for oral suspension INN or Proposed INN: ataluren CAS Number: 775304-57-9 Current Sponsor code: PTC124 Other descriptive name: PTC-0161480; PTC-124; Compound 1a; RPS 2505; PTC-C124; 291844 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 1000- Pharmaceutical form of the placebo: Powder for oral suspension Route of administration of the placebo: Oral use	PTC Therapeutics, Inc		Not Recruiting			Female: yes Male: yes	208	Phase 3	France;Spain;Belgium;Germany;Netherlands;Italy;United Kingdom;Sweden
594	NCT00709280	April 2009	19 February 2015	Infant Study of Inhaled Saline in Cystic Fibrosis	Infant Study of Inhaled Saline in Cystic Fibrosis	Cystic Fibrosis	Drug: 7% Hypertonic Saline (HS);Drug: 0.9% Isotonic Saline (IS)	CF Therapeutics Development Network Coordinating Center	Cystic Fibrosis Foundation Therapeutics;National Heart, Lung, and Blood Institute (NHLBI)	Not recruiting	4 Months	59 Months	Both	321	N/A	United States;Canada
595	NCT00775528	April 2009	19 October 2017	Study Investigating a Delayed-Release Pancrelipase in Patients With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	An Open Label, Multi-center, Study to Assess the Safety and Tolerability of Pancrelipase Delayed Release Capsules in Infants and Children Less Than 7 Years of Age With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Cystic Fibrosis;Pancreatic Exocrine Insufficiency	Drug: Pancrelipase Delayed Release	Solvay Pharmaceuticals		Not recruiting	1 Month	6 Years	All	19	Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
596	NCT00840333	April 2009	29 January 2018	Safety, Tolerability and Pharmacokinetics of MP-376 Administered for 14 Days to Stable Pediatric (CF) Patients	A Phase 1B, Multi-Center, Open Label Study to Evaluate the Safety, Tolerability and Pharmacokinetics of MP-376 Inhalation Solution Given Daily for 14 Days to Stable Pediatric Cystic Fibrosis Patients.	Cystic Fibrosis	Drug: MP-376 (Levofloxacin solution for Inhalation)	Horizon Pharma USA, Inc.		Not recruiting	6 Years	16 Years	All	27	Phase 1	United States
597	NCT00843817	April 2009	16 December 2017	RhDNase and Biodistribution of PMN Serine Proteases in Cystic Fibrosis Sputum	RhDNase Effect on Biodistribution of PMN Serine Proteases in Cystic Fibrosis Sputum	Cystic Fibrosis	Drug: Pulmozyme	University Hospital, Tours		Not recruiting	18 Years	N/A	All	15	Phase 4	France
598	NCT00880100	April 2009	16 December 2017	Use of Ultrase® MT12 in Young Cystic Fibrosis Children (CF)	Efficacy and Safety of Ultrase MT12 in the Control of Steatorrhea in Cystic Fibrosis (CF) and Pancreatic Insufficient (PI) Children Aged 2 to 6 Years Old	Cystic Fibrosis;Pancreatic Insufficiency	Drug: Ultrase® MT12	Forest Laboratories		Not recruiting	2 Years	6 Years	All	49	Phase 3	United States
599	NCT00885365	April 2009	19 October 2017	A Study Comparing the Efficacy and Tolerability of Tobrineb®/Actitob®/Bramitob® Versus TOBI®	A Multicentre, Multinational, Open-Label, Randomised, Parallel Group Clinical Trial of Tobrineb®/Actitob®/Bramitob® (Tobramycin Solution for Nebulisation, 300mg Twice Daily in 4mL Unit Dose Vials) Compared to TOBI® in the Treatment of Patients With Cystic Fibrosis and Chronic Infection With Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: tobramycin / Bramitob;Drug: tobramycin / TOBI	Chiesi Farmaceutici S.p.A.		Not recruiting	6 Years	N/A	All	324	Phase 3	Czech Republic;France;Germany;Hungary;Poland;Russian Federation;Spain;Ukraine
600	EUCTR2008-001530-27-GB	11/03/2009	19 March 2012	A Phase II, Randomised, Double-Blind, Placebo-Controlled, Parallel Group Study to Assess the Efficacy of 28 Day Oral Administration of AZD9668 in Patients with Cystic Fibrosis	A Phase II, Randomised, Double-Blind, Placebo-Controlled, Parallel Group Study to Assess the Efficacy of 28 Day Oral Administration of AZD9668 in Patients with Cystic Fibrosis	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: AZD9668 Product Code: AZD9668 Pharmaceutical Form: Tablet INN or Proposed INN: none CAS Number: none Current Sponsor code: AZD9668 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 30- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	AstraZeneca AB		Not Recruiting			Female: yes Male: yes	70	Phase 2	Germany;United Kingdom;Denmark;Sweden
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
601	EUCTR2009-009875-37-NL	11/03/2009	10 June 2013	A placebo-controlled trial of insulin therapy with or without adjuvant metformin in patients with cystic fibrosis-related diabetes - Insulin therapy and adjuvant metformin in CFRD	A placebo-controlled trial of insulin therapy with or without adjuvant metformin in patients with cystic fibrosis-related diabetes - Insulin therapy and adjuvant metformin in CFRD	Cystic fibrosis-related diabetes MedDRA version: 9.1 Level: HLT Classification code 10012602 Term: Diabetes mellitus (incl subtypes) MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Metformine HCL 500 PCH Pharmaceutical Form: Tablet Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Staf Longziekten		Not Recruiting			Female: yes Male: yes	25		Netherlands
602	NCT00865904	March 2009	19 October 2017	Study of VX-809 in Cystic Fibrosis Subjects With the ?F508-CFTR Gene Mutation	A Randomized, Double-Blind, Placebo-Controlled, Multiple Dose Study of VX-809 to Evaluate Safety, Pharmacokinetics, and Pharmacodynamics of VX-809 in Cystic Fibrosis Subjects Homozygous for the ?F508-CFTR Gene Mutation	Cystic Fibrosis	Drug: VX-809;Drug: Placebo	Vertex Pharmaceuticals Incorporated		Not recruiting	18 Years	N/A	All	93	Phase 2	United States;Belgium;Canada;Germany;Netherlands
603	NCT01377792	March 2009	19 February 2015	Study of Long-term Treatment With Hypertonic Saline in Patients With Cystic Fibrosis	Phase 4 Study of the Efficacy of Long-term Treatment With Hypertonic Saline on Pulmonary Exacerbations in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Hypertonic saline	Adelaida Lamas Ferreiro		Not recruiting	6 Years	N/A	Both	71	Phase 4	Spain
604	EUCTR2008-005080-33-IT	05/02/2009	3 April 2012	RANDOMIZED, SINGLE BLIND, CONTROLLED TRIAL OF INHALED GLUTATHIONE VERSUS PLACEBO IN PATIENTS WITH CYSTIC FIBROSIS. - INHALED GSH VS PLACEBO IN CYSTIC FIBROSIS	RANDOMIZED, SINGLE BLIND, CONTROLLED TRIAL OF INHALED GLUTATHIONE VERSUS PLACEBO IN PATIENTS WITH CYSTIC FIBROSIS. - INHALED GSH VS PLACEBO IN CYSTIC FIBROSIS	Cystic fibrosis. MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Trade Name: TAD 600 Pharmaceutical Form: Powder for nebuliser solution INN or Proposed INN: Glutathione Concentration unit: mg/kg milligram(s)/kilogram Concentration type: equal Concentration number: 10- Pharmaceutical form of the placebo: Powder for nebuliser solution Route of administration of the placebo: Inhalation use	AZIENDA OSPEDALIERA VINCENZO MONALDI DI NAPOLI		Authorised			Female: yes Male: yes			Italy
605	NCT00663975	February 2009	19 February 2015	Open Label Safety Study of DCI-1020 in Pediatric Cystic Fibrosis (CF) Patients	An Open-Label, Multi-Center Safety and Efficacy Study of DCI-1020 in Pediatric Cystic Fibrosis Patients With Exocrine Pancreatic Insufficiency	Digestive System Diseases;Pancreatic Disease;Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: DCI 1020	Digestive Care, Inc.		Not recruiting	N/A	2 Years	Both	24	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
606	NCT00846781	February 2009	19 February 2015	Study 08-114 Open-label Extension of Study 08-110 - A Multi-Center Study of Denufosol Tetrasodium Inhalation Solution in Patients With Cystic Fibrosis Lung Disease (P08642)		Cystic Fibrosis	Drug: Denufosol tetrasodium Inhalation Solution	Merck Sharp & Dohme Corp.		Not recruiting	5 Years	N/A	Both	308	Phase 3	United States;Australia;Canada;New Zealand
607	EUCTR2008-008228-34-GB	28/01/2009	19 March 2012	Determination of the pharmacokinetics of inhaled mannitol after single and multiple dosing in cystic fibrosis patients	Determination of the pharmacokinetics of inhaled mannitol after single and multiple dosing in cystic fibrosis patients	Cystic Fibrosis MedDRA version: 8.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: IDPM: Inhaled Dry Powder Mannitol Product Code: IDPM Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: MANNITOL CAS Number: 69658 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 400-	Pharmaxis Ltd		Not Recruiting			Female: yes Male: yes	18		United Kingdom
608	EUCTR2008-001284-11-ES	19/01/2009	19 March 2012	Estudio de la eficacia del tratamiento a largo plazo con suero salino hipertónico sobre las exacerbaciones pulmonares en pacientes con Fibrosis Quística	Estudio de la eficacia del tratamiento a largo plazo con suero salino hipertónico sobre las exacerbaciones pulmonares en pacientes con Fibrosis Quística	Fibrosis Quística MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: CLORURO SODICO 7% Product Name: CLORURO SODICO 7% Pharmaceutical Form: Nebuliser solution INN or Proposed INN: CLORURO SODICO 7% Concentration unit: ml millilitre(s) Concentration type: equal Concentration number: 10-12H Trade Name: CLORURO SODICO 7% Product Name: CLORURO SODICO 7% Pharmaceutical Form: INN or Proposed INN: CLORURO SODICO 7% Concentration unit: ml millilitre(s) Concentration type: equal Concentration number: 5-12H	ADELAIDA LAMAS FERREIRO		Authorised			Female: yes Male: yes			Spain
609	NCT03905642	January 8, 2009	15 April 2019	Multidose Safety and Tolerability Study of Dose Escalation of Liposomal Amikacin for Inhalation (ARIKACE™) - Extension Phase	Multidose Safety and Tolerability Study of Dose Escalation of Liposomal Amikacin for Inhalation (ARIKACE TM) in Cystic Fibrosis Patients With Chronic Infections Due to Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: Arikace	Insmed Incorporated		Not recruiting	6 Years	N/A	All	49	Phase 2	Belgium;Hungary;Macedonia, The Former Yugoslav Republic of;Poland;Serbia;Slovakia;Ukraine
610	EUCTR2009-011740-19-Outside-EU/EEA		16 February 2015	Clinical Trial to Assess the Safety and Efficacy of Aztreonam for Inhalation Solution (AZLI) in patients with Cystic Fibrosis (CF) and Chronic Burkholderia Species Infection	Phase 3b Randomized, Double-Blind, Placebo-Controlled Two-Part Trial to Assess the Safety and Efficacy of Continuous Aztreonam for Inhalation Solution (AZLI) in Subjects with Cystic Fibrosis (CF) and Chronic Burkholderia Species Infection	Cystic Fibrosis subjects with chronic Burkholderia spp. infection of the airways. MedDRA version: 17.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders ;Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]	Trade Name: Cayston INN or Proposed INN: AZTREONAM CAS Number: 78110-38-0 Current Sponsor code: AZLI Other descriptive name: N/A Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Powder and solvent for nebuliser solution Route of administration of the placebo: Inhalation use	Gilead Sciences, Inc.		Not Available			Female: yes Male: yes	100	Phase 3b	United States;Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
611	EUCTR2008-005045-34-GB	15/12/2008	9 May 2016	A clinical study to investigate the effect and safety of up to 6 months of treatment with inhaled Promixin in the treatment of chest infections causeed by Pseudomonas in people with a lung disease called bronchiectasis	A double-blind, vehicle-controlled, multi-centre, clinical study to investigate the efficacy and safety of up to 6 months of therapy with inhaled Promixin in the treatment of patients with non-cystic fibrosis bronchiectasis infected with Pseudomonas aeruginosa susceptible to Promixin - Inhaled Promixin in the treatment of non-CF bronchiectasis	non-CF bronchiectasis (CF = cystic fibrosis) MedDRA version: 14.0 Level: PT Classification code 10006445 Term: Bronchiectasis System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders ;Therapeutic area: Diseases [C] - Bacterial Infections and Mycoses [C01]	Trade Name: Promixin 1 million International Units (IU) Powder for Nebuliser Solution Pharmaceutical Form: Powder for nebuliser solution Other descriptive name: colistimethate sodium Concentration unit: Munit million units Concentration type: equal Concentration number: 1- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Profile Pharma Ltd		Not Recruiting			Female: yes Male: yes	144	Phase 3	Ukraine;Ireland;Russian Federation;United Kingdom
612	NCT00685971	December 2008	19 February 2015	Cholecalciferol for Vitamin D in Adult Cystic Fibrosis (CF) Patients	Randomized Placebo-Controlled Trial of Cholecalciferol for Vitamin D Deficiency in Adults With Cystic Fibrosis	Cystic Fibrosis;Vitamin D Deficiency	Dietary Supplement: 5000 IU of cholecalciferol;Dietary Supplement: placebo	St. Michael's Hospital, Toronto		Not recruiting	18 Years	N/A	Both	200	N/A	Canada
613	NCT00792714	December 2008	19 February 2015	Pharmacokinetics of Inhaled Mannitol in Cystic Fibrosis Patients	Determination of the Pharmacokinetics of Inhaled Mannitol After Single and Multiple Dosing in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Mannitol	Pharmaxis		Not recruiting	6 Years	N/A	Both	18	Phase 1	Australia;United Kingdom
614	EUCTR2008-002740-42-DE	13/11/2008	3 September 2012	Long Term Administration of Inahled Mannitol in Cystic Fibrosis - A Safety and Efficacy Study	Long Term Administration of Inahled Mannitol in Cystic Fibrosis - A Safety and Efficacy Study	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: Inhaled Dry Powder Mannitol Product Code: IDPM Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Mannitol CAS Number: 69658 Concentration unit: mg milligram(s) Concentration number: 40- Product Name: Inhaled dry powder mannitol Product Code: IDPM Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Mannitol CAS Number: 69658 Concentration unit: mg milligram(s) Concentration number: 5- Product Name: Inhaled dry powder mannitol Product Code: MTT Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Mannitol CAS Number: 69658 Concentration unit: mg milligram(s) Concentration number: 40-	Pharmaxis Pharmaceuticals Limited		Not Recruiting			Female: yes Male: yes	300		Germany;Netherlands;Belgium;France
615	EUCTR2007-006648-23-DE	12/11/2008	19 March 2012	An exploratory, randomized, double-blind, placebo controlled study to assess the efficacy of multiple doses of omalizumab in cystic fibrosis complicated with allergic bronchopulmonary aspergillosis	An exploratory, randomized, double-blind, placebo controlled study to assess the efficacy of multiple doses of omalizumab in cystic fibrosis complicated with allergic bronchopulmonary aspergillosis	Patients with cystic fibrosis complicated by allergic bronchopulmonary aspergillosis. MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung MedDRA version: 9.1 Level: LLT Classification code 10000244 Term: ABPA	Trade Name: Xolair 150 mg Pulver und Injektionslösung zur Herstellung einer Injektionslösung Product Name: Xolair Product Code: IGE025 Pharmaceutical Form: Powder and solvent for solution for injection INN or Proposed INN: Omalizumab CAS Number: 242138-07-4 Current Sponsor code: IGE025 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 150- Pharmaceutical form of the placebo: Powder for solution for injection Route of administration of the placebo: Subcutaneous use	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	60		United Kingdom;Germany;Netherlands;Belgium;Ireland;Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
616	NCT00787917	November 2008	19 October 2017	An Exploratory Study to Assess Multiple Doses of Omalizumab in Patients With Cystic Fibrosis Complicated by Acute Bronchopulmonary Aspergillosis (ABPA)	An Exploratory, Randomized, Double-blind, Placebo Controlled Study to Assess the Efficacy of Multiple Doses of Omalizumab in Cystic Fibrosis Complicated by Allergic Bronchopulmonary Aspergillosis (ABPA)	Cystic Fibrosis;Allergic Bronchopulmonary Aspergillosis	Drug: Omalizumab;Drug: Placebo;Drug: Itraconazole	Novartis Pharmaceuticals		Not recruiting	12 Years	N/A	All	14	Phase 4	Belgium;Germany;Italy;Netherlands;United Kingdom
617	NCT00789867	November 2008	29 June 2015	Single Dose of pGM169/GL67A in CF Patients	Evaluation of Safety and Gene Expression With a Single Dose of pGM169/GL67A Administered to the Nose and Lung of Individuals With Cystic Fibrosis	Cystic Fibrosis	Drug: pGM169/GL67A	Imperial College London	Royal Brompton & Harefield NHS Foundation Trust;University of Oxford;University of Edinburgh;Cystic Fibrosis Trust;University of Pennsylvania	Not recruiting	16 Years	70 Years	Both	27	Phase 1/Phase 2	United Kingdom
618	NCT00794586	November 2008	19 February 2015	Study Evaluating Fosfomycin/Tobramycin for Inhalation in Cystic Fibrosis Patients With Pseudomonas Aeruginosa Lung Infection	A Phase 2, Double-Blind, Multicenter, Randomized, Placebo-Controlled Trial Evaluating Fosfomycin/Tobramycin for Inhalation in Patients With Cystic Fibrosis and Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: FTI, AZLI;Drug: Placebo, AZLI	Gilead Sciences		Not recruiting	18 Years	N/A	Both	120	Phase 2	United States
619	NCT00800579	November 2008	19 February 2015	Trial to Assess the Safety, Tolerability and Pharmacokinetics of GS-9411 in Healthy Male Volunteers	A Randomised, Double-blind, Placebo-controlled Trial to Assess the Safety, Tolerability and Pharmacokinetics of GS-9411 in Healthy Male Volunteers	Cystic Fibrosis	Drug: GS-9411;Drug: Placebo	Gilead Sciences		Not recruiting	18 Years	45 Years	Male	12	Phase 1	Australia
620	NCT00803179	November 2008	19 October 2017	Growth Hormone Therapy for Wasting in Cystic Fibrosis	Growth Hormone Therapy for Wasting in Cystic Fibrosis	Cystic Fibrosis	Drug: Nutropin AQ	University of Massachusetts, Worcester		Not recruiting	18 Years	N/A	All	5	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
621	NCT00809094	November 2008	19 October 2017	NAC Phase IIB: A Multi-Center, Phase IIB, Randomized, Placebo-controlled, Double-Blind Study Of The Effects Of N-Acetylcysteine On Redox Changes and Lung Inflammation In Cystic Fibrosis Patients	A Multi-Center, Phase IIB, Randomized, Placebo-controlled, Double-Blind Study Of The Effects Of N-Acetylcysteine On Redox Changes and Lung Inflammation In Cystic Fibrosis Patients	Cystic Fibrosis	Drug: N-acetylcysteine (NAC);Drug: Placebo	Stanford University	Cystic Fibrosis Foundation Therapeutics	Not recruiting	7 Years	N/A	All	70	Phase 2	United States
622	NCT00846573	November 2008	19 October 2017	Hyperpolarized Noble Gas MR Imaging for Pulmonary Disorders	Hyperpolarized Noble Gas MR Imaging for Pulmonary Disorders	COPD;Cystic Fibrosis;Asthma;Healthy	Drug: Hyperpolarized Helium-3	University of Massachusetts, Worcester		Not recruiting	5 Years	N/A	All	14	N/A	United States
623	NCT01112059	November 2008	16 December 2017	Trial of Doxycycline to Reduce Sputum MMP-9 Activity in Adult Cystic Fibrosis (CF) Patients	A Randomized Trial of Doxycycline to Reduce Sputum MMP-9 Activity in Adult CF Patients Hospitalized for Pulmonary Exacerbations	Cystic Fibrosis	Drug: Doxycycline;Other: placebo	University of Alabama at Birmingham	Cystic Fibrosis Foundation Therapeutics	Not recruiting	19 Years	N/A	All	40	N/A	United States
624	EUCTR2008-004699-34-NL	28/10/2008	26 June 2012	A Randomised, Double-Blind, Placebo-Controlled, Parallel Group, Phase II study to Assess the efficacy of 28 Day Oral Administration of AZD1236 in Adult Patients with Cystic Fibrosis. - CYBER	A Randomised, Double-Blind, Placebo-Controlled, Parallel Group, Phase II study to Assess the efficacy of 28 Day Oral Administration of AZD1236 in Adult Patients with Cystic Fibrosis. - CYBER	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Code: AZD1236 Pharmaceutical Form: Tablet Current Sponsor code: AZD1236 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	AstraZeneca AB		Not Recruiting			Female: yes Male: yes	50	Phase 2	Netherlands;Spain
625	EUCTR2006-004155-38-BE	21/10/2008	7 October 2014	Biochemical effects of a long-term supplementation with omega-3 polyunsaturated fatty acids in cystic fibrosis - Omega 3 study	Biochemical effects of a long-term supplementation with omega-3 polyunsaturated fatty acids in cystic fibrosis - Omega 3 study	cystic fibrosis MedDRA version: 8.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: Omega 3 Premium Pharmaceutical Form: Capsule, soft Other descriptive name: OMEGA-3-ACID TRIGLYCERIDES Concentration unit: mg milligram(s) Concentration type: up to Concentration number: 300- Pharmaceutical form of the placebo: Capsule, soft Route of administration of the placebo: Oral use	Hôpital Universitaire Des Enfants Reine Fabiola (H.U.D.E.R.F.)		Authorised			Female: yes Male: yes	80		Belgium
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
626	NCT00757848	October 2008	19 October 2017	A Phase II , Placebo-controlled Study to Assess Efficacy of 28 Day Oral AZD9668 in Patients With Cystic Fibrosis	A Phase II, Randomised, Double-blind, Placebo-controlled, Parallel Group Study to Assess the Efficacy of 28 Day Oral Administration of AZD9668 in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: AZD9668;Drug: AZD9668 Placebo equivalent	AstraZeneca		Not recruiting	16 Years	N/A	All	56	Phase 2	Denmark;Germany;Poland;Russian Federation;Sweden;United Kingdom
627	NCT00774072	October 2008	19 February 2015	Nasal Inhalation of Tobramycin in Patients With Cystic Fibrosis and Pseudomonas Aeruginosa Colonization	Nasal Inhalation of Tobramycin by the Pari Sinus Nebulizer in Patients With Cystic Fibrosis and Pseudomonas Aeruginosa Colonization in the Upper Airways	Cystic Fibrosis;Pseudomonas Aeruginosa	Drug: Tobramycin (Gernebcin®)	University of Jena		Not recruiting	8 Years	N/A	Both	9	Phase 2	Germany
628	NCT00788138	October 2008	19 February 2015	Effects of Vitamin D Supplementation on Lung Function in an Acute Pulmonary Exacerbation of Cystic Fibrosis		Cystic Fibrosis	Dietary Supplement: Vitamin D3;Dietary Supplement: Placebo	Emory University		Not recruiting	18 Years	70 Years	Both	30	N/A	United States
629	NCT00934362	October 2008	16 December 2017	Effect of Lucinactant on Mucus Clearance in Cystic Fibrosis Lung Disease	A Double Blind, Cross-Over Study Comparing Aerosolized Lucinactant and Vehicle on Mucociliary Clearance for Cystic Fibrosis Lung Disease	Cystic Fibrosis	Drug: Lucinactant first;Drug: Placebo first	University of North Carolina, Chapel Hill	Cystic Fibrosis Foundation Therapeutics;Windtree Therapeutics	Not recruiting	14 Years	N/A	All	16	Phase 2	United States
630	EUCTR2008-001769-27-GB	29/09/2008	19 March 2012	Microbiological assessment of prophylactic ciprofloxacin therapy for children with cystic fibrosis during viral respiratory viral infection to prevent Pseudomonas aeruginosa colonisation – a randomised control study (Prevent Pseudomonas Aeruginosa Colonisation- PREPAC) - PREPAC	Microbiological assessment of prophylactic ciprofloxacin therapy for children with cystic fibrosis during viral respiratory viral infection to prevent Pseudomonas aeruginosa colonisation – a randomised control study (Prevent Pseudomonas Aeruginosa Colonisation- PREPAC) - PREPAC	Cystic Fibrosis Specifically the trial will include children aged 2-14 years with a diagnosis of cystic fibrosis and not chronically infected with pseudomonas aeruginosa in their lungs.	Trade Name: Ciproxin Suspension Product Name: Ciproxin Product Code: PL 0010/0211 Pharmaceutical Form: Oral suspension Pharmaceutical form of the placebo: Oral solution Route of administration of the placebo: Oral use	Southampton University Hospital Trust		Authorised			Female: yes Male: yes	45		United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
631	EUCTR2008-001156-43-FR	19/09/2008	10 July 2012	A randomized, double-blind, placebo-controlled parallel group study to investigate the safety and efficacy of two doses of tiotropium bromide (2.5 µg and 5 µg) administered once daily via the Respimat device for 12 weeks in patients with cystic fibrosis.	A randomized, double-blind, placebo-controlled parallel group study to investigate the safety and efficacy of two doses of tiotropium bromide (2.5 µg and 5 µg) administered once daily via the Respimat device for 12 weeks in patients with cystic fibrosis.	Cystic fibrosis. MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Spiriva Respimat 2.5 microgram, solution for inhalation Pharmaceutical Form: Inhalation vapour, solution Other descriptive name: TIOTROPIUM Concentration unit: µg microgram(s) Concentration type: equal Concentration number: 2.5- Pharmaceutical form of the placebo: Inhalation vapour, solution Route of administration of the placebo: Inhalation use Product Name: Tiotropium Respimat 1.25 microgram solution for inhalation Pharmaceutical Form: Inhalation vapour, solution Other descriptive name: TIOTROPIUM Concentration unit: µg microgram(s) Concentration type: equal Concentration number: 1.25- Pharmaceutical form of the placebo: Inhalation vapour, solution Route of administration of the placebo: Inhalation use	Boehringer Ingelheim France		Authorised			Female: yes Male: yes	596		Portugal;United Kingdom;Germany;Netherlands;Belgium;France;Italy
632	EUCTR2007-004050-85-GB	15/09/2008	26 June 2012	Evaluation of safety and gene expression with a single dose of pGM169/GL67A administered to the nose and lung of individuals with cystic fibrosis - Single dose of pGM169/GL67A in CF patients	Evaluation of safety and gene expression with a single dose of pGM169/GL67A administered to the nose and lung of individuals with cystic fibrosis - Single dose of pGM169/GL67A in CF patients	Cystic fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: pGM169/GL67A Product Code: pGM169/GL67A Pharmaceutical Form: Inhalation vapour, solution	Imperial College		Not Recruiting			Female: yes Male: yes			United Kingdom
633	EUCTR2007-006276-11-GB	01/09/2008	11 February 2013	A Phase I/II randomised, placebo-controlled, double blind trial to assess the safety, tolerability, pharmacodynamics and exploratory efficacy of heparin 25 mg inhalation powder in patients with Cystic Fibrosis (CF) - VR496/005- Orally inhaled heparin in patients with Cystic Fibrosis	A Phase I/II randomised, placebo-controlled, double blind trial to assess the safety, tolerability, pharmacodynamics and exploratory efficacy of heparin 25 mg inhalation powder in patients with Cystic Fibrosis (CF) - VR496/005- Orally inhaled heparin in patients with Cystic Fibrosis	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Heparin 25mg inhalation powder, hard capsule Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: HEPARIN SODIUM CAS Number: 9041081 Concentration unit: IU/mg international unit(s)/milligram Concentration type: not less then Concentration number: 150- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Vectura Limited		Not Recruiting			Female: yes Male: yes	64	Phase 1/2	Ireland;Italy;United Kingdom
634	NCT00630812	September 2008	19 February 2015	Long Term Administration of Inhaled Mannitol in Cystic Fibrosis	Long Term Administration of Inhaled Mannitol in Cystic Fibrosis- A Safety and Efficacy Study	Cystic Fibrosis	Drug: inhaled mannitol;Drug: Placebo comparator	Pharmaxis	ethica Clinical Research Inc.;Europe: KasaConsult bvba, Hoegaarden, Belgium;Argentina: Resolution Latin America; Buenos Aires, Argentina	Not recruiting	6 Years	N/A	Both	318	Phase 3	United States;Argentina;Belgium;Canada;France;Germany;Netherlands
635	EUCTR2007-004956-35-GB	27/08/2008	19 March 2012	The effect of intensive blood glucose control with insulin on markers of short and medium term outcomes in patients hospitalised with acute exacerbations of chronic lung disease. Pilot Study to test feasibility and safety of a clinical protocol for intensive blood glucose control with insulin on medical wards - Pilot of Intensive Blood Glucose Control with Insulin on Medical Wards	The effect of intensive blood glucose control with insulin on markers of short and medium term outcomes in patients hospitalised with acute exacerbations of chronic lung disease. Pilot Study to test feasibility and safety of a clinical protocol for intensive blood glucose control with insulin on medical wards - Pilot of Intensive Blood Glucose Control with Insulin on Medical Wards	Acute exacerbations of chronic obstructive pulmonary disease. Stress hyperglycaemia. Insulin-induced hypoglycaemia. Cystic fibrosis MedDRA version: 9.1 Level: LLT Classification code 10010953 Term: COPD exacerbation MedDRA version: 9.1 Level: LLT Classification code 10060439 Term: Stress induced hyperglycaemia MedDRA version: 9.1 Level: LLT Classification code 10022484 Term: Insulin hypoglycaemia MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Actrapid 100 IU/ml, Solution for injection in a vial Pharmaceutical Form: Solution for injection Trade Name: Levemir 100 U/ml solution for injection in a cartridge, Levemir 100 U/ml solution for injection in a pre-filled pen Pharmaceutical Form: Solution for injection Trade Name: NovoRapid 100 U/ml, solution for injection in a vial. NovoRapid Penfill 100 U/ml, solution for injection in a cartridge. NovoRapid FlexPen 100 U/ml, solution for injection in a pre-filled pen. Pharmaceutical Form: Solution for injection Trade Name: Lantus® 100 Units/ml solution for injection in a vial. Lantus® 100 Units/ml solution for injection in a cartridge. Lantus® 100 Units/ml solution for injection in a pre-filled pen Pharmaceutical Form: Solution for injection	St George's, University of London		Not Recruiting			Female: yes Male: yes			United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
636	EUCTR2008-001728-30-DE	26/08/2008	11 March 2013	A Phase 2, Multi-center, Randomized, Double-blind, Placebo-controlled Study to Evaluate the Safety, Tolerability and Efficacy of Three Dosage Regimens of MP- 376 Solution for Inhalation Given for 28 Days to Stable Cystic Fibrosis Patients	A Phase 2, Multi-center, Randomized, Double-blind, Placebo-controlled Study to Evaluate the Safety, Tolerability and Efficacy of Three Dosage Regimens of MP- 376 Solution for Inhalation Given for 28 Days to Stable Cystic Fibrosis Patients	Pseudomonas aeruginosa infection in patients suffering from stable Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung MedDRA version: 9.1 Level: LLT Classification code 10021860 Term: Infection pseudomonas aeruginosa	Product Name: MP-376 Product Code: MP-376 Pharmaceutical Form: Nebuliser solution INN or Proposed INN: levofloxacin CAS Number: 100986-85-4 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Mpex Pharmaceuticals, Inc.		Not Recruiting			Female: yes Male: yes	190	Phase 2	Netherlands;Germany
637	EUCTR2007-004507-36-DE	19/08/2008	19 November 2012	A multicenter, open label, 2 period cross-over study to evaluate the Pharmacokinetics of an 8 week continuous treatment with 1x300mg/d and 2x300mg/d TOBI® inhaled with the PARI eFlow® rapid in Cystic Fibrosis (CF) Subjects.	A multicenter, open label, 2 period cross-over study to evaluate the Pharmacokinetics of an 8 week continuous treatment with 1x300mg/d and 2x300mg/d TOBI® inhaled with the PARI eFlow® rapid in Cystic Fibrosis (CF) Subjects.	patients with cystic fibrosis and chronical infection with Pseudomonas aeruginosa MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Trade Name: Tobi Product Name: Tobi Product Code: TBM100 Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300- Trade Name: Tobi Product Name: Tobi Product Code: TBM100 Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300- Trade Name: Tobi Product Name: Tobi Product Code: TBM100 Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300- Trade Name: Tobi Product Name: Tobi Product Code: TBM100 Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Current Sponsor code: TBM100 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300-	Novartis Pharma GmbH		Not Recruiting			Female: yes Male: yes			Germany
638	EUCTR2008-000164-17-DE	13/08/2008	15 April 2013	Nasale Inhalation von Tobramycin mit dem Pari Sinus-Vernebler bei Patienten mit Mukoviszidose und Pseudomonasnachweis im Nasen-Nasennebenhöhlenbereich. Nasal inhalation of tobramycin by the Pari Sinus nebulizer in patients with cystic fibrosis and pseudomonas aeruginosa colonization in the upper airways. - tobra nasal CF pilot	Nasale Inhalation von Tobramycin mit dem Pari Sinus-Vernebler bei Patienten mit Mukoviszidose und Pseudomonasnachweis im Nasen-Nasennebenhöhlenbereich. Nasal inhalation of tobramycin by the Pari Sinus nebulizer in patients with cystic fibrosis and pseudomonas aeruginosa colonization in the upper airways. - tobra nasal CF pilot	subjects with cystic fibrosis and pseudomonas aeruginosa colonization in the upper airways	Trade Name: Gernebcin 80 mg Product Name: Gernebcin 80 mg Pharmaceutical Form: Solution for injection INN or Proposed INN: tobramycin Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 40- Pharmaceutical form of the placebo: Solution for injection Route of administration of the placebo: Inhalation use	University of Jena		Not Recruiting			Female: yes Male: yes	14		Germany
639	EUCTR2008-002352-20-BE	11/08/2008	7 October 2014	Single center, double-blind, randomized, placebo-controlled, two-period/two-treatment crossover study investigating the effect of miglustat on the nasal potential difference in patients with cystic fibrosis homozygous for the F508del mutation	Single center, double-blind, randomized, placebo-controlled, two-period/two-treatment crossover study investigating the effect of miglustat on the nasal potential difference in patients with cystic fibrosis homozygous for the F508del mutation	Cystic fibrosis homozygous for the F508del mutation MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Zavesca Pharmaceutical Form: Capsule, hard INN or Proposed INN: miglustat CAS Number: 72599-27-0 Current Sponsor code: OGT918 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use	Actelion Pharmaceuticals Ltd		Not Recruiting			Female: yes Male: yes			Belgium
640	NCT00639626	August 2008	11 June 2018	Use of Levemir® Improves Metabolic and Clinical Status in Cystic Fibrosis-related Diabetes (CFRD)	Use of Levemir® Improves Metabolic and Clinical Status in CFRD	Cystic Fibrosis Related Diabetes	Drug: insulin detemir [rDNA origin] injection	Nationwide Children's Hospital	Novo Nordisk A/S	Not recruiting	16 Years	45 Years	All	6	Phase 2/Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
641	NCT00659529	August 2008	16 December 2017	Safety and Efficacy of Sildenafil in Cystic Fibrosis (CF) Lung Disease	The Role of Phosphodiesterase Inhibitors in CF Lung Disease	Cystic Fibrosis	Drug: sildenafil	National Jewish Health	Cystic Fibrosis Foundation Therapeutics	Not recruiting	14 Years	N/A	All	36	Phase 1/Phase 2	United States
642	NCT00662675	August 2008	19 October 2017	A Study of the Efficacy and Tolerability of Pancrelipase Microtablet (MT) Capsules for the Treatment of Cystic Fibrosis-dependent Exocrine Pancreatic Insufficiency	A Randomized Double-blind (Withdrawal) Phase 3 Study to Evaluate the Efficacy and Tolerability of Pancrelipase MT Capsules Compared With Placebo in the Treatment of Subjects With Cystic Fibrosis-dependent Exocrine Pancreatic Insufficiency	Exocrine Pancreatic Insufficiency;Steatorrhea;Malabsorption Syndromes;Cystic Fibrosis	Drug: Pancrease MT 10.5, or MT 21;Drug: Placebo for Pancrease MT 10.5 or MT 21	Johnson & Johnson Pharmaceutical Research & Development, L.L.C.		Not recruiting	7 Years	60 Years	All	40	Phase 3	United States;Canada
643	NCT00742092	August 2008	19 February 2015	Miglustat in Cystic Fibrosis	Single Center, Double-blind, Randomized, Placebo-controlled, Two-period/Two-treatment Crossover Study Investigating the Effect of Miglustat on the Nasal Potential Difference in Patients With Cystic Fibrosis Homozygous for the F508del Mutation	Cystic Fibrosis	Drug: miglustat;Drug: placebo	Actelion		Not recruiting	12 Years	N/A	Both	11	Phase 2	Belgium
644	NCT00744250	August 2008	19 February 2015	Intraduodenal Aspiration Study to Assess the Bioavailability of Oral Pancrecarb® Compared to Placebo Control	Intraduodenal Aspiration Study to Assess the Bioavailability of Oral Pancrecarb® Compared to Placebo Control in Patients With Pancreatic Insufficiency	Exocrine Pancreatic Insufficiency;Chronic Pancreatitis;Cystic Fibrosis	Drug: Pancrelipase	Digestive Care, Inc.	University of North Carolina, Chapel Hill	Not recruiting	18 Years	N/A	Both	3	Phase 4	United States
645	NCT00757237	August 2008	19 October 2017	Aztreonam for Inhalation Solution vs Tobramycin Inhalation Solution in Patients With Cystic Fibrosis & Pseudomonas Aeruginosa	An Open-Label, Randomized, Phase 3 Trial to Evaluate the Efficacy and Safety of Aztreonam for Inhalation Solution (AZLI) Versus Tobramycin Inhalation Solution (TIS) in an Intermittent Aerosolized Antibiotic Regimen in Subjects With Cystic Fibrosis Followed by an Open-Label, Single Arm Extension (European Union [EU] Only)	Cystic Fibrosis	Drug: Aztreonam for Inhalation Solution (AZLI);Drug: Tobramycin Inhalation Solution (TIS)	Gilead Sciences	Chiltern International Inc.;ClinPhone, Inc.;Covance	Not recruiting	6 Years	N/A	All	274	Phase 3	United States;Austria;Belgium;Denmark;France;Germany;Ireland;Italy;Netherlands;Portugal;Spain;Switzerland;United Kingdom;Poland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
646	EUCTR2006-006215-68-CZ	29/07/2008	19 March 2012	A MULTICENTRE,MULTINATIONAL, OPEN-LABEL, RANDOMISED, PARALLEL GROUP CLINICAL TRIAL OF TOBRINEB®/ACTITOB®/ BRAMITOB® (TOBRAMYCIN SOLUTION FOR NEBULISATION, 300 MG TWICE DAILY IN 4 ML UNIT DOSE VIALS) COMPARED TO TOBI® IN THE TREATMENT OF PATIENTS WITH CYSTIC FIBROSIS AND CHRONIC INFECTION WITH PSEUDOMONAS AERUGINOSA	A MULTICENTRE,MULTINATIONAL, OPEN-LABEL, RANDOMISED, PARALLEL GROUP CLINICAL TRIAL OF TOBRINEB®/ACTITOB®/ BRAMITOB® (TOBRAMYCIN SOLUTION FOR NEBULISATION, 300 MG TWICE DAILY IN 4 ML UNIT DOSE VIALS) COMPARED TO TOBI® IN THE TREATMENT OF PATIENTS WITH CYSTIC FIBROSIS AND CHRONIC INFECTION WITH PSEUDOMONAS AERUGINOSA	cystic fibrosis and P. aeruginosa chronic infection MedDRA version: 9.1 Level: LLT Classification code 10057582 Term: Lung infection pseudomonal MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Trade Name: Bramitob Product Name: Tobrineb/Bramitob Pharmaceutical Form: Nebuliser solution INN or Proposed INN: tobramycin CAS Number: as32986-56-4 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300mg-4ml Trade Name: Tobi 300mg/5ml Nebuliser solution. Product Name: Tobi Pharmaceutical Form: Nebuliser solution INN or Proposed INN: tobramycin CAS Number: 49842-07-1 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300mg-5ml	Chiesi Farmaceutici S.p.A		Authorised			Female: yes Male: yes	320		Hungary;Germany;Czech Republic;France;Spain
647	EUCTR2007-004063-21-NL	24/07/2008	19 March 2012	An open label, exploratory study on the effect of rhBSSL on the fat absorption in patients with cystic fibrosis and pancreatic insufficiency. - N/A	An open label, exploratory study on the effect of rhBSSL on the fat absorption in patients with cystic fibrosis and pancreatic insufficiency. - N/A	Cystic fibrosis and pancreatic insufficiency MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: rhBSSL Product Code: rhBSSL Pharmaceutical Form: Powder for oral solution INN or Proposed INN: Bucelipase alfa CAS Number: 9026-00-0 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 170- Trade Name: Nexium 20 Product Name: Nexium Pharmaceutical Form: Film-coated tablet INN or Proposed INN: Esomeprazole CAS Number: 161796-78-7 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 20-	Biovitrum AB		Not Recruiting			Female: yes Male: yes	18		Netherlands
648	NCT00706004	July 2008	19 October 2017	Short Term Safety and Efficacy of Lubiprostone in Adults With Cystic Fibrosis	Short Term Safety and Efficacy of Lubiprostone in Adults With Cystic Fibrosis	Constipation;Cystic Fibrosis	Drug: lubiprostone	University of Arkansas	Takeda Pharmaceuticals North America, Inc.	Not recruiting	18 Years	N/A	All	9	N/A	United States
649	NCT01172301	July 2008	17 August 2015	Leucine-enriched Essential Amino Acid Intake to Optimize Protein Anabolism in Children With Cystic Fibrosis	Leucine-enriched Essential Amino Acid Intake to Optimize Protein Anabolism in Children With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Essential amino acid intake + Leucine vs total AA supplement	Texas A&M University	Arkansas Children's Hospital Research Institute	Not recruiting	10 Years	21 Years	Both	14	N/A	United States
650	NCT00566241	June 2008	19 February 2015	IGF-1 Therapy in Patients With Cystic Fibrosis	An Investigation Into the Use of IGF-1 Therapy in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: recombinant human IGF-1;Drug: Placebo	Stony Brook University		Not recruiting	18 Years	N/A	Both	15	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
651	NCT00677365	June 2008	29 January 2018	Safety, Tolerability and Efficacy of MP-376 Given for 28 Days to Cystic Fibrosis (CF) Patients	Phase II, Multi-Center, Randomized, Double-Blind, Placebo-Controlled, Study to Evaluate the Safety, Tolerability and Efficacy of Three Dosage Regimens of MP-376 Solution for Inhalation Given for 28 Days to Stable CF Patients	Cystic Fibrosis (CF)	Drug: MP-376;Drug: Placebo	Horizon Pharma USA, Inc.		Not recruiting	16 Years	N/A	All	151	Phase 2	United States;Germany;Netherlands
652	NCT00680316	June 2008	19 October 2017	A Study of Pulmozyme® (Dornase Alpha) in 3- to 5-Year-Old Patients With Cystic Fibrosis	A Phase IV, Multicenter, Randomized, Double-Blind, Placebo-Controlled Trial of Pulmozyme® in 3- to 5-Year-Old Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Dornase alfa;Drug: Placebo	Genentech, Inc.		Not recruiting	3 Years	5 Years	All	3	Phase 4	United States
653	NCT00690820	June 2008	19 October 2017	Study Investigating a Delayed-Release Pancrelipase in Patients With Pancreatic Exocrine Insufficiency (PEI) Due to Cystic Fibrosis (CF)	A Double-blind, Randomized, Multi-center, Placebo-controlled, Cross-over Study to Assess the Efficacy and Safety of Pancrelipase Delayed Release 12,000 Unit Capsules in Subjects Aged 7 - 11 With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Cystic Fibrosis;Pancreatic Exocrine Insufficiency	Drug: Pancrelipase Delayed Release;Drug: Placebo Comparator	Solvay Pharmaceuticals		Not recruiting	7 Years	11 Years	All	17	Phase 3	United States
654	NCT00411736	May 2008	19 February 2015	Scandinavian Cystic Fibrosis Azithromycin Study	Supplementary Oral Azithromycin in Treatment of Intermittent Pseudomonas Aeruginosa Colonization in CF-patients With Inhaled Colistin and Oral Ciprofloxacin; Postponing Next Isolate of Pseudomonas and Prevention of Chronic Infection. A Prospective, Double-blinded, Placebo-controlled Scandinavian Multi-centre Study.	Cystic Fibrosis	Drug: Study medication, azithromycin or placebo;Drug: Azithromycin or placebo tablets	Rigshospitalet, Denmark	Cystic Fibrosis Foundation Therapeutics	Not recruiting	1 Year	N/A	Both	45	Phase 4	Denmark;Norway;Sweden
655	NCT00645788	May 2008	19 October 2017	Study to Evaluate the Safety and Efficacy of Ciprofloxacin (Inhaled) in Patients With Cystic Fibrosis	Randomized, Double-blind, Placebo-controlled, Multicenter Study to Evaluate the Safety and Efficacy of Inhaled Ciprofloxacin Compared to Placebo in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: Ciprofloxacin (Cipro Inhale, BAYQ3939);Drug: Placebo	Bayer	Novartis	Not recruiting	12 Years	N/A	All	288	Phase 2	United States;Australia;Canada;Denmark;Germany;Israel;Norway;Sweden;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
656	NCT00712166	May 2008	19 October 2017	Safety and Efficacy Study of Aztreonam for Inhalation Solution (AZLI) in Patients With Cystic Fibrosis, Mild Lung Disease, and P. Aeruginosa	A Double-Blind, Multicenter, Multinational, Randomized, Placebo-Controlled Trial Evaluating Aztreonam Lysine For Inhalation in Patients With Cystic Fibrosis, Mild Lung Disease, and P. Aeruginosa (AIR-CF4)	Cystic Fibrosis;Lung Infection;Pseudomonas Aeruginosa	Drug: AZLI 75 mg three times daily (TID);Drug: Placebo three times daily (TID)	Gilead Sciences		Not recruiting	6 Years	N/A	All	160	Phase 3	United States;Australia;Canada
657	EUCTR2007-004277-26-BE	29/04/2008	10 July 2012	An Open-Label, Randomized, Phase 3 Trial to Evaluate the Efficacy and Safety of Aztreonam 75 mg Powder and Diluent for Nebuliser Solution (AZLI) versus Tobramycin Nebuliser Solution (TNS) in an Intermittent Aerosolized Antibiotic Regimen, in subjects with Cystic Fibrosis followed by an Open Label, Single Arm Extension	An Open-Label, Randomized, Phase 3 Trial to Evaluate the Efficacy and Safety of Aztreonam 75 mg Powder and Diluent for Nebuliser Solution (AZLI) versus Tobramycin Nebuliser Solution (TNS) in an Intermittent Aerosolized Antibiotic Regimen, in subjects with Cystic Fibrosis followed by an Open Label, Single Arm Extension	Adult and paediatric cystic fibrosis (CF) patients with pulmonary Pseudomonas aeruginosa (PA) infection. MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: AZLI Pharmaceutical Form: Powder for nebuliser solution INN or Proposed INN: aztreonam lysine Current Sponsor code: AZLI Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 75- Trade Name: TOBI Pharmaceutical Form: Nebuliser solution INN or Proposed INN: tobramycin Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 60-	Gilead Sciences Inc		Authorised			Female: yes Male: yes	273	Phase 3	Portugal;Germany;United Kingdom;Netherlands;Denmark;Belgium;France;Ireland;Spain;Italy;Austria
658	EUCTR2008-006502-42-IT	22/04/2008	26 June 2012	Early antibiotic treatment in pseudomonas aeruginosa eradication in cystic fibrosis patients: a randomised policentric study on two different protocols - #FFC17/2007	Early antibiotic treatment in pseudomonas aeruginosa eradication in cystic fibrosis patients: a randomised policentric study on two different protocols - #FFC17/2007	Cystic fibrosis MedDRA version: 9.1 Level: SOC Classification code 10038738	Trade Name: *TOBINEBUL 56F 1D 300MG/5ML Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300- Pharmaceutical Form: Powder and solvent for solution for infusion INN or Proposed INN: Colistin Concentration unit: million IU million international units Concentration type: equal Concentration number: 1- Pharmaceutical Form: Tablet INN or Proposed INN: Ciprofloxacin** Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 500-	AZIENDA OSPEDALIERA MEYER		Authorised			Female: yes Male: yes			Italy
659	EUCTR2007-001782-15-IT	09/04/2008	19 March 2012	Efficacy and safety of Intra-erythrocytes dexamethasone in cystic fibrosis - ND	Efficacy and safety of Intra-erythrocytes dexamethasone in cystic fibrosis - ND	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: dexamethasone Pharmaceutical Form: Solution for injection INN or Proposed INN: Dexamethasone Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 250-	ISTITUTO GIANNINA GASLINI		Authorised			Female: yes Male: yes			Italy
660	NCT00700050	April 2008	19 February 2015	Modulation by Sex Hormones of Inflammation and Susceptibility to Pseudomonas Aeruginosa in Cystic Fibrosis Airways	Modulation by Sex Hormones of Inflammation and Susceptibility to Pseudomonas Aeruginosa in Cystic Fibrosis Airways - A Pilot Study	Cystic Fibrosis	Drug: Hypertonic saline	The Hospital for Sick Children		Recruiting	N/A	22 Years	Both	32	Phase 3	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
661	NCT01323101	April 2008	16 December 2017	Doxycycline Effects on Inflammation in Cystic Fibrosis	Effect of Doxycycline on Sputum Biomarkers of Inflammation and Lung Epithelial Repair in Patients With Cystic Fibrosis.	Cystic Fibrosis	Drug: Doxycycline;Other: No doxycycline	University of Southern California		Not recruiting	18 Years	N/A	All	21	Phase 4	United States
662	NCT01759342	April 2008	3 August 2015	Comprehensive Exercise Training Program During Hospitalization for an Acute CF Exacerbation	The Efficacy of a Standardized Exercise Protocol in Inpatient Care of Patients With Cystic Fibrosis	Cystic Fibrosis	Behavioral: Aerobic exercise;Behavioral: Resistance exercise;Behavioral: Flexibility and postural exercise;Behavioral: Balance exercise	University of Alabama at Birmingham		Not recruiting	6 Years	21 Years	Both	23	N/A	United States
663	EUCTR2007-003868-22-FR	19/03/2008	19 March 2012	ASSESMENT OG EFFICACY AND TOLERABILITY OF INHABLED TOBRAMYCIN VS PLACEBO IN CYSTIC FIBROSIS PATIENTS REVEIVING ANTIBIOTHERAPY FOR 28 DAYS FOR PSEUDOMONAS AERUGINOSA PRIMO COLONISATION. - NEBCINAL 01-07	ASSESMENT OG EFFICACY AND TOLERABILITY OF INHABLED TOBRAMYCIN VS PLACEBO IN CYSTIC FIBROSIS PATIENTS REVEIVING ANTIBIOTHERAPY FOR 28 DAYS FOR PSEUDOMONAS AERUGINOSA PRIMO COLONISATION. - NEBCINAL 01-07	Assessment of microbiological efficacy and tolerability of inhaled tobramycin vs placebo in cystic fibrosis patients, primo colonised by Pseudomonas aeruginosa justified inhaled antiobiotherapeutical treatment during 28 days.	Product Code: NEB/01-07 Pharmaceutical Form: Inhalation vapour, solution Pharmaceutical form of the placebo: Inhalation vapour, liquid	Laboratoire Erempharma		Authorised			Female: yes Male: yes			France
664	NCT00628134	March 2008	16 December 2017	Self-dispersing Liquids as Aerosol Drug Carriers	Self-dispersing Liquids as Aerosol Drug Carriers	Cystic Fibrosis	Drug: calfactant;Drug: isotonic saline	University of Pittsburgh	Cystic Fibrosis Foundation Therapeutics	Not recruiting	18 Years	N/A	All	8	N/A	United States
665	NCT00635141	March 2008	19 February 2015	The Effect of Hypertonic Saline on the Lung Clearance Index in Patients With Cystic Fibrosis	The Effect of Inhaled Hypertonic Saline (7%) Versus Normal Saline (0.9%) on the Lung Clearance Index in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: hypertonic saline (7 %) and isotonic saline (0.9%)	The Hospital for Sick Children	Canadian Cystic Fibrosis Foundation	Not recruiting	6 Years	18 Years	Both	20	Phase 3	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
666	NCT00638365	March 2008	19 October 2017	Dose Escalation Study of KB001 in Cystic Fibrosis Patients Infected With Pseudomonas Aeruginosa	A Phase I/II Randomized, Double-Blind, Placebo-Controlled, Single-Dose, Dose Escalation Study of KB001 in Cystic Fibrosis Patients Infected With Pseudomonas Aeruginosa	Cystic Fibrosis	Biological: KB001;Other: Placebo	KaloBios Pharmaceuticals		Not recruiting	12 Years	N/A	All	27	Phase 1/Phase 2	United States
667	NCT00762918	March 2008	19 February 2015	Vitamin D3 for the Treatment of Low Vitamin D in Cystic Fibrosis	Vitamin D and Its Non-Classic Roles in Cystic Fibrosis	Cystic Fibrosis;Vitamin D Deficiency	Dietary Supplement: cholecalciferol	Children's Hospital of Philadelphia		Not recruiting	10 Years	25 Years	Both	0	Phase 3	United States
668	EUCTR2007-004004-12-BG	28/02/2008	19 March 2012	A STUDY TO INVESTIGATE THE EFFECT OF PANCRELIPASE DELAYED RELEASE CAPSULES ON MALDIGESTION IN PATIENTS WITH EXOCRINE PANCREATIC INSUFFICIENCY DUE TO CHRONIC PANCREATITIS AND PANCREATECTOMY	A STUDY TO INVESTIGATE THE EFFECT OF PANCRELIPASE DELAYED RELEASE CAPSULES ON MALDIGESTION IN PATIENTS WITH EXOCRINE PANCREATIC INSUFFICIENCY DUE TO CHRONIC PANCREATITIS AND PANCREATECTOMY	Pancreatic enzyme replacement therapy is the cornerstone of nutritional management of pancreatic exocrine insufficiency (PEI) due to e.g. cystic fibrosis, chronic pancreatitis, partial or complete pancreatectomy or gastrectomy. Without pancreatic enzyme replacement the patients suffer from severe symptoms of maldigestion as manifested by steatorrhea, abdominal pain, body weight loss etc. MedDRA version: 9.1 Level: LLT Classification code 10009093 Term: Chronic pancreatitis MedDRA version: 9.1 Level: LLT Classification code 10033596 Term: Pancreatectomy	Trade Name: Creon 10000 Product Name: Pancrelipase Delayed Release Capsule Pharmaceutical Form: Capsule, hard Other descriptive name: Pancrelipase/Pancreas Powder Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 12000- Pharmaceutical form of the placebo: Capsule, hard Route of administration of the placebo: Oral use Trade Name: Creon 25000 Product Name: Pancrelipase Delayed Release Capsule Pharmaceutical Form: Capsule, hard Other descriptive name: Pancrelipase/Pancreas Powder Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 24000-	Solvay Pharmaceuticals GmbH		Not Recruiting			Female: yes Male: yes	52		Bulgaria
669	NCT00605761	February 2008	19 February 2015	SD Cystic Fibrosis Study	An Open Label Dose Ascending, Single Dose Study to Investigate the Pharmacokinetics of SB-656933 in Subjects With Cystic Fibrosis.	Cystic Fibrosis	Drug: SB656933	GlaxoSmithKline		Not recruiting	16 Years	N/A	Both	9	Phase 1	United States
670	NCT00625612	February 2008	19 February 2015	Study of Denufosol Tetrasodium Inhalation Solution in Patients With Cystic Fibrosis (CF) Lung Disease	A Phase 3, International, Multi-Center, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group, Efficacy and Safety Study of Denufosol Tetrasodium Inhalation Solution in Patients With Cystic Fibrosis Lung Disease and FEV1 Greater Than or Equal to 75% Predicted But Less Than or Equal to 110% Predicted	Cystic Fibrosis	Drug: Denufosol Tetrasodium (INS37217) Inhalation Solution;Drug: Placebo	Merck Sharp & Dohme Corp.		Not recruiting	5 Years	N/A	Both	466	Phase 3	United States;Australia;Canada;New Zealand
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
671	NCT00634192	February 2008	16 December 2017	Pharmacokinetic Evaluation of an 8 -Week Treatment With Inhaled Tobramycin	A Multicenter, Open Label, 2 Period Cross-over Study to Evaluate the PK of a 8 Week Continuous Treatment With 1x300mg/d and 2x300mg/d Tobramycin Inhaled With a 'Soft Mist' Nebulizer in Cystic Fibrosis (CF) Subjects	Pseudomonas Infections	Drug: tobramycin	Novartis		Not recruiting	6 Years	N/A	All	50	Phase 3	Germany
672	NCT01983774	February 2008	28 April 2015	Effect of Proton Pump Inhibitors on CF Pulmonary Exacerbations	Effect of Proton Pump Inhibitors on CF Exacerbations	Cystic Fibrosis	Drug: Esomeprazole;Drug: Placebo	Columbia University		Not recruiting	18 Years	65 Years	Both	13	Phase 2	United States
673	NCT00557089	January 2008	19 February 2015	The Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis	Randomized, Placebo-controlled Trial on the Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: rhDNAse;Other: Placebo	The Hospital for Sick Children		Not recruiting	6 Years	18 Years	Both	17	Phase 4	Canada
674	NCT00558844	January 2008	17 October 2016	Multidose Safety and Tolerability Study of Liposomal Amikacin for Inhalation (Arikace™) In Cystic Fibrosis Patients	Phase 1b/2a Multidose Safety and Tolerability Study of Liposomal Amikacin for Inhalation (Arikace™) in Cystic Fibrosis Patient With Chronic Infections Due to Pseudomonas Aeruginosa.	Cystic Fibrosis	Drug: Placebo;Drug: Arikace™	Insmed Incorporated		Not recruiting	6 Years	N/A	Both	46	Phase 1/Phase 2	United States
675	NCT00719381	January 2008	19 February 2015	Effect of Pioglitazone on Inflammation in Cystic Fibrosis	Effect of Pioglitazone on Sputum Biomarkers of Inflammation and Lung Epithelial Repair in Cystic Fibrosis	Cystic Fibrosis	Drug: Pioglitazone	Paul Beringer		Not recruiting	18 Years	N/A	Both	24	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
676	EUCTR2008-002673-13-DE		18 April 2012	Anti-inflammatory pulmonal therapy of CF-patients with Amitriptyline and Placebo	Anti-inflammatory pulmonal therapy of CF-patients with Amitriptyline and Placebo	Deficiency of the Cftr-molecule results in accumulation of ceramide in respiratory epithelial cells. Ceramide triggers a pro-inflammatory and -apoptotic status in cystic fibrosis patients. Amitriptyline reduces ceramide levels in the lung tissue, normalises the activity of cytokines and prevents constitutive cell death of epithelial cells. Amitriptyline prevents pulmonary infections of CF-mice with P. aeruginosa. Amitriptyline may result in an improved lung function of cystic fibrosis patients.	Trade Name: Amitriptylin-CT Product Name: Amitriptyline Pharmaceutical Form: Capsule* INN or Proposed INN: Amitriptyline CAS Number: 50486 Current Sponsor code: Amitriptyline Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 12.5- Pharmaceutical form of the placebo: Capsule* Route of administration of the placebo: Oral use	PSKS		Authorised			Female: yes Male: yes			Germany
677	EUCTR2007-005346-20-GB	04/12/2007	19 March 2012	Does nebulised tobramycin (TOBI) via e-flow delivery systems cause a raised peak serum tobramycin level in children with Cystic Fibrosis? - TOBICF	Does nebulised tobramycin (TOBI) via e-flow delivery systems cause a raised peak serum tobramycin level in children with Cystic Fibrosis? - TOBICF	Children with cystic fibrosis	Trade Name: Tobi 300 mg/5 ml Nebuliser Solution Product Name: TOBI Pharmaceutical Form: Nebuliser solution INN or Proposed INN: tobramycin Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300-	Leeds Teaching Hospitals NHS Trust		Not Recruiting			Female: yes Male: yes	30		United Kingdom
678	NCT00484263	December 2007	19 February 2015	The Long Term Effect of Inhaled Hypertonic Saline (6%) in Patients With Non Cystic Fibrosis Bronchiectasis	The Long Term Effect of Inhaled Hypertonic Saline (6%) in Patients With Non-cystic Fibrosis Bronchiectasis.	Bronchiectasis	Drug: Hypertonic saline 6% -	The Alfred		Not recruiting	18 Years	N/A	Both	40	Phase 3	Australia
679	EUCTR2007-003628-39-IT	22/11/2007	19 March 2012	Pilot study to evaluate the local tollerability and efficacy of a new tobramycin 3% nasal spray formulation to reduce the bacterial density of Pseudomonas aeruginosa and/or Staphylococcus aureus, in patients affected by Cystic Fibrosis with rhinosinusal infection - ND	Pilot study to evaluate the local tollerability and efficacy of a new tobramycin 3% nasal spray formulation to reduce the bacterial density of Pseudomonas aeruginosa and/or Staphylococcus aureus, in patients affected by Cystic Fibrosis with rhinosinusal infection - ND	Rhinosinusal infection by Pseudomonas a. e/o Staphylococcus a. in patients with Cystic Fibrosis MedDRA version: 9.1 Level: HLGT Classification code 10024970 Term: Respiratory tract infections	Product Code: TNSE Pharmaceutical Form: Nasal spray, solution INN or Proposed INN: TOBRAMYCIN Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 6- Pharmaceutical form of the placebo: Nasal spray, solution Route of administration of the placebo: Nasal use	ECUPHARMA S.R.L.		Not Recruiting			Female: yes Male: yes			Italy
680	NCT00510484	November 2007	19 October 2017	Study Investigating a Delayed-Release Pancrelipase in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	A Double-blind, Randomized, Multi-center, Placebo-controlled, Cross-over Study to Assess the Efficacy and Safety of Pancrelipase Delayed Release 24,000 Unit Capsules in Subjects With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Cystic Fibrosis	Drug: Pancrelipase Delayed Release;Drug: Placebo Comparator	Solvay Pharmaceuticals		Not recruiting	12 Years	N/A	All	35	Phase 3	United States;Hungary;Israel;South Africa;Spain
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
681	NCT00537602	November 2007	19 February 2015	Miglustat / OGT 918 in the Treatment of Cystic Fibrosis	Single Center, Double-blind, Randomized, Placebo-controlled, 2-period/2-treatment Crossover Study Investigating the Effect of Miglustat on the Nasal Potential Difference in Patients With Cystic Fibrosis Homozygous for the ?F508 Mutation	Cystic Fibrosis	Drug: miglustat;Drug: placebo	Actelion		Not recruiting	12 Years	N/A	Both	6	Phase 2	Spain
682	EUCTR2007-002912-24-DE	30/10/2007	19 March 2012	Study to evaluate the safety and pharmacokinetics of ciprofloxacin following inhalation of ciprofloxacin dry powder for inhalation administered to pediatric patients with cystic fibrosis aged 12-17 years	Study to evaluate the safety and pharmacokinetics of ciprofloxacin following inhalation of ciprofloxacin dry powder for inhalation administered to pediatric patients with cystic fibrosis aged 12-17 years	long term treatment of chronic lung infections caused by Pseudomonas aeruginosa in patients with cystic fibrosis MedDRA version: 8.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Ciprofloxacin Product Code: BAY q 3939 Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: CIPROFLOXACIN CAS Number: 85721331 Current Sponsor code: BAY q 3939 Other descriptive name: 1-cyclopropyl-6-fluoro-1,4-dihydro-4-oxo-7-[1-piperazinyl]-3-quinoline carboxylic acid Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 32,5-	Bayer HealthCare AG		Not Recruiting			Female: yes Male: yes	16		Germany
683	EUCTR2007-002657-23-DE	01/10/2007	19 March 2012	A Phase 2a, Randomized, Double-Blind, Placebo-Controlled Study of VX-770 to Evaluate Safety, Pharmacokinetics, and Biomarkers of CFTR Activity in Cystic Fibrosis (CF) Subjects with Genotype G551D	A Phase 2a, Randomized, Double-Blind, Placebo-Controlled Study of VX-770 to Evaluate Safety, Pharmacokinetics, and Biomarkers of CFTR Activity in Cystic Fibrosis (CF) Subjects with Genotype G551D	cystic fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: VX-770 Product Code: VX-770, VRT-813077 Pharmaceutical Form: Tablet INN or Proposed INN: NA Current Sponsor code: VX-770 Other descriptive name: VRT-813077 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use Product Name: VX-770 Product Code: VX-770, VRT-813077 Pharmaceutical Form: Tablet INN or Proposed INN: NA Current Sponsor code: VX-770 Other descriptive name: VRT-813077 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50-	Vertex Pharmaceuticals Incorporated		Not Recruiting			Female: yes Male: yes	36	Phase 2a	Germany
684	NCT00528190	October 2007	16 December 2017	Treatment of Aspergillus Fumigatus (a Fungal Infection) in Patients With Cystic Fibrosis	Treatment of Aspergillus Fumigatus in Patients With Cystic Fibrosis: A Randomized, Double-blind, Placebo-Controlled Trial	Cystic Fibrosis	Drug: Itraconazole	Ottawa Hospital Research Institute	The Hospital for Sick Children;Canadian Cystic Fibrosis Foundation;The Physicians' Services Incorporated Foundation	Not recruiting	6 Years	N/A	All	35	Phase 4	Canada
685	NCT00541190	October 2007	16 December 2017	Absorptive Clearance in the Cystic Fibrosis Airway	Absorptive Clearance in the Cystic Fibrosis Airway	Cystic Fibrosis	Other: Technetium [Tc-99m] sulfur colloid and Indium [In-111] DTPA	University of Pittsburgh		Not recruiting	18 Years	N/A	All	21	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
686	NCT00686361	October 2007	19 February 2015	Choline Nutrition in Children With Cystic Fibrosis (CF)	To Investigate Whether Choline Supplementation in Children With CF Will Correct Biochemical Markers of Choline Deficiency and Improve Plasma Indices of Methylation Capacity and Redox Status and Result in Decreased Pro-inflammatory Cytokines	Cystic Fibrosis	Drug: Choline supplementation	University of British Columbia	Cystic Fibrosis Foundation Therapeutics	Not recruiting	5 Years	17 Years	Both	34	N/A	Canada
687	NCT01070446	October 2007	19 February 2015	Choline Nutrition in Children With Cystic Fibrosis	Choline Nutrition in Children With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: Vitamin: Choline Bitartrate (2-hydroxyethyl) trimethylammonium salt 1:1	University of British Columbia	Cystic Fibrosis Foundation (US)	Not recruiting	5 Years	17 Years	Both	34	N/A	Canada
688	EUCTR2007-000724-40-BE	27/09/2007	19 March 2012	A Phase 2 Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	A Phase 2 Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Code: PTC124 Pharmaceutical Form: Oral powder Current Sponsor code: PTC124 Concentration unit: % (W/W) percent weight/weight Concentration type: equal Concentration number: 25-	PTC Therapeutics, Inc.		Not Recruiting			Female: yes Male: yes	30	Phase 2	Belgium
689	EUCTR2007-001412-23-IE	27/09/2007	19 March 2012	Long Term Administration of Inhaled Dry Powder Mannitol In Cystic Fibrosis – A Safety and Efficacy Study	Long Term Administration of Inhaled Dry Powder Mannitol In Cystic Fibrosis – A Safety and Efficacy Study	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: Inhaled dry powder mannitol Product Code: IDPM Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Mannitol CAS Number: 69658 Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Pharmaxis Pharmaceuticals Limited		Not Recruiting			Female: yes Male: yes	250		United Kingdom;Ireland
690	NCT00221546	September 2007	19 February 2015	Influence of DHA-rich Supplement on DHA-status and Health Evolution of Patients With Cystic Fibrosis	Influence of DHA-rich Supplement on DHA-status and Health Evolution of Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Giving DHA-rich supplement	University Hospital, Ghent	Belgian Society against Cystic Fibrosis	Not recruiting	6 Years	N/A	Both	17	Phase 2	Belgium
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
691	NCT00499837	September 2007	20 June 2016	Phase II Study of the Safety and Efficacy of Inhaled Alpha-1 Antitrypsin (AAT ) in Cystic Fibrosis Patients	Double-Blind, Randomized, Placebo-Controlled, Phase II Study of the Safety and Efficacy of Inhaled Alpha-1 Antitrypsin (AAT ) in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Aerosolized, human, plasma-derived Alpha-1 Antitrypsin	Kamada, Ltd.		Not recruiting	5 Years	N/A	Both	21	Phase 2	Israel
692	NCT00531882	September 2007	16 December 2017	Pilot Mouthwash Study of Pioglitazone and Simvastatin in Healthy Volunteers	A Pilot Study Evaluating the Effect of Pioglitazone, Simvastatin, and Ibuprofen on Neutrophil Migration in Vivo in Healthy Subjects	Cystic Fibrosis	Drug: Pioglitazone;Drug: Simvastatin;Drug: Ibuprofen	University Hospitals Cleveland Medical Center		Not recruiting	18 Years	50 Years	All	25	N/A	United States
693	NCT00534079	September 2007	19 February 2015	Nasal Inhalation of Pulmozyme in Patients With Cystic Fibrosis and Chronic Rhinosinusitis	Nasal Inhalation of Dornase Alfa (Pulmozyme) in Patients With Cystic Fibrosis and Chronic Rhinosinusitis - a Double Blind Placebo-controlled Cross-over, Bicenter, Prospective Clinical Study	Cystic Fibrosis;Rhinosinusitis	Drug: Dornase alfa (Pulmozyme);Drug: isotonic saline	University of Jena		Not recruiting	5 Years	N/A	Both	23	Phase 3	Germany
694	NCT00546663	September 2007	19 February 2015	Tolerability of Inhaled Hypertonic Saline in Infants With Cystic Fibrosis	A Pilot Study to Evaluate the Tolerability of Inhaled 7% Hypertonic Saline in Infants With Cystic Fibrosis	Cystic Fibrosis	Drug: inhaled 7% hypertonic saline (HS)	CF Therapeutics Development Network Coordinating Center	Cystic Fibrosis Foundation Therapeutics	Not recruiting	12 Months	30 Months	Both	19	N/A	United States;Canada
695	NCT01851642	September 2007	26 August 2019	Lung Disease and Its Affect on the Work of White Blood Cells in the Lungs	The Role of Conformational Diseases on Macrophage Function	Alpha-1 Antitrypsin Deficiency;AAT Deficiency;AATD;Cystic Fibrosis (CF)	Procedure: History and physical exam.;Procedure: Blood draw.;Procedure: Pulmonary function testing.;Drug: Albuterol inhaler.	University of Florida		Recruiting	18 Years	N/A	All	220	Phase 1/Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
696	EUCTR2007-000959-33-DE	16/08/2007	19 March 2012	Randomized, open labelled, cross over deposition study of Tobramycin 100 PARI nebulized with eFlow® versus TOBI® nebulized with PARI LC PLUS® in subjects with CF	Randomized, open labelled, cross over deposition study of Tobramycin 100 PARI nebulized with eFlow® versus TOBI® nebulized with PARI LC PLUS® in subjects with CF	Cystic Fibrosis with Pseudomonas aeruginosa infection	Product Name: Tobramycin 100 PARI Product Code: T100 PARI Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin Current Sponsor code: T100 PARI Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 100- Trade Name: TOBI® Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin Current Sponsor code: TOBI® Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 60-	PARI GmbH		Not Recruiting			Female: yes Male: yes	25		Germany
697	NCT01018303	August 2007	19 February 2015	Safety and Efficacy of an Antioxidant-rich Multivitamin Supplement in Cystic Fibrosis	Safety and Efficacy of a Novel Antioxidant-rich Multivitamin Supplement for Persons With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: AquADEK	University of Colorado, Denver	Cystic Fibrosis Foundation Therapeutics;Yasoo Health;National Institutes of Health (NIH)	Not recruiting	10 Years	40 Years	Both	17	Phase 1/Phase 2	United States
698	EUCTR2006-002049-35-ES	22/07/2007	19 March 2012	Estudio unicéntrico, a doble ciego, aleatorizado y controlado con placebo, cruzado de 2 brazos, para investigar el efecto de miglustat sobre la diferencia de potencial nasal en pacientes con fibrosis quística homocigotos para la mutación ?F508	Estudio unicéntrico, a doble ciego, aleatorizado y controlado con placebo, cruzado de 2 brazos, para investigar el efecto de miglustat sobre la diferencia de potencial nasal en pacientes con fibrosis quística homocigotos para la mutación ?F508	fibrosis quística MedDRA version: 8.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Zavesca Product Name: miglustat Product Code: OGT 918 Pharmaceutical Form: Capsule, hard INN or Proposed INN: Miglustat CAS Number: 72599-27-0 Current Sponsor code: OGT 918 Other descriptive name: 1,5 (Butylimino)-1,5-dideoxy-D-glucitol Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 100- Pharmaceutical form of the placebo: Capsule* Route of administration of the placebo: Oral use	Actelion Pharmaceuticals Ltd		Authorised			Female: yes Male: yes	25		Spain
699	NCT00425165	July 2007	19 February 2015	Study of Denufosol Inhalation Solution in Patients With Mild to Moderate Cystic Fibrosis Lung Disease	A Randomized, Double-Blind, Two Way Crossover Evaluation of the Effects of a Single Dose of Denufosol Tetrasodium (INS37217) Inhalation Solution Versus Placebo (0.9% Sodium Chloride Solution) on Mucociliary Clearance in Patients With Mild to Moderate Cystic Fibrosis Lung Disease	Cystic Fibrosis	Drug: denufosol tetrasodium (INS37217) Inhalation Solution	Merck Sharp & Dohme Corp.		Not recruiting	10 Years	N/A	Both	6	Phase 2	United States
700	NCT00506792	July 2007	19 February 2015	Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of QAU145 in Patients With Cystic Fibrosis	A Two-part, Randomized, Double-blind, Placebo-controlled, Ascending Single-dose, Adaptive Study to Evaluate Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of QAU145 Administered Via a Nasal Spray Pump to Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: QAU145;Drug: Placebo	Novartis Pharmaceuticals		Not recruiting	18 Years	50 Years	Both	9	Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
701	NCT00513682	July 2007	16 December 2017	Efficacy and Safety of Ultrase® MT20 in Improving the Coefficient of Fat Absorption (CFA) in Children With Cystic Fibrosis (CF) and Pancreatic Insufficiency (PI)	Efficacy and Safety of Ultrase® MT20 in Improving the Coefficient of Fat Absorption (CFA%) in Children With Cystic Fibrosis (CF) and Pancreatic Insufficiency (PI)	Cystic Fibrosis;Pancreatic Insufficiency	Drug: Ultrase® MT20	Forest Laboratories		Not recruiting	7 Years	11 Years	All	9	Phase 3	United States
702	NCT01293084	July 2007	19 October 2017	Hypertonic Saline and Mucociliary Clearance in Children	Acute Inhalation of Hypertonic Saline Does Not Improve Mucociliary Clearance in All Children With Cystic Fibrosis	Cystic Fibrosis	Drug: 0.12% saline;Drug: 7% saline	Johns Hopkins University	Cystic Fibrosis Foundation Therapeutics	Not recruiting	7 Years	12 Years	All	17	Phase 2
703	EUCTR2007-002707-40-BE	28/06/2007	7 October 2014	The effect of inhalation with hypertonic saline (7%) on lung function and sputum rheology in Cystic Fibrosis patients	The effect of inhalation with hypertonic saline (7%) on lung function and sputum rheology in Cystic Fibrosis patients	Mucoviscidose MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Hypertonic saline solution Pharmaceutical Form: Solution for injection INN or Proposed INN: Sodium Chloride CAS Number: 7647-14-5 Concentration unit: % percent Concentration type: equal Concentration number: 7- Product Name: Normal saline solution Pharmaceutical Form: Solution for injection INN or Proposed INN: Sodium Chloride CAS Number: 7647-14-5 Concentration unit: % percent Concentration type: equal Concentration number: 0,9-	University Hospital Ghent		Not Recruiting			Female: yes Male: yes	60		Belgium
704	EUCTR2006-006980-22-HU	14/06/2007	19 March 2012	MULTIDOSE SAFETY AND TOLERABILITY STUDY OF DOSE ESCALATION OF LIPOSOMAL AMIKACIN FOR INHALATION (ARIKACE™) IN CYSTIC FIBROSIS PATIENTS WITH CHRONIC INFECTIONS DUE TO PSEUDOMONAS AERUGINOSA	MULTIDOSE SAFETY AND TOLERABILITY STUDY OF DOSE ESCALATION OF LIPOSOMAL AMIKACIN FOR INHALATION (ARIKACE™) IN CYSTIC FIBROSIS PATIENTS WITH CHRONIC INFECTIONS DUE TO PSEUDOMONAS AERUGINOSA	Cystic fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: Liposomal Amikacin (Arikace™) Pharmaceutical Form: Nebuliser suspension	Transave, Inc.		Not Recruiting			Female: yes Male: yes	60		Hungary;Belgium
705	NCT00449904	June 2007	19 February 2015	Open-Label Phase III Long-Term Safety Trial of Liprotamase	An Open-Label Clinical Study Evaluating the Long-Term Safety of ALTU-135 for the Treatment of Patients With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: Liprotamase	Anthera Pharmaceuticals		Not recruiting	7 Years	N/A	Both	214	Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
706	NCT00503490	June 2007	29 January 2018	Safety, Pharmacokinetic and Pharmacodynamic Study of MP-376 in Patients With Cystic Fibrosis	Phase I, Single and Multi-dose, Placebo Controlled, Randomized, Dose-Escalation Study to Evaluate the Safety, Tolerability and PK Profile of MP-376 Using the PARI eFlow Nebulizer for 14 Days to CF Patients	Cystic Fibrosis	Drug: MP-376 (Levofloxacin solution for Inhalation);Drug: placebo	Horizon Pharma USA, Inc.		Not recruiting	16 Years	N/A	All	40	Phase 1	United States
707	EUCTR2006-003275-12-DK	23/05/2007	16 August 2016	Scandinavian Cystic Fibrosis Azithromycin Study Supplementary oral azithromycin in treatment of intermittent Pseudomonas aeruginosa colonization in CF-patients with inhaled colistin and oral ciprofloxacin; postponing next isolate of pseudomonas and prevention of chronic infection. A prospective, double-blinded, placebo-controlled scandinavian multi-centre study. A investigator initiated study - Scandinavian Cystic Fibrosis Azithromycin Study	Scandinavian Cystic Fibrosis Azithromycin Study Supplementary oral azithromycin in treatment of intermittent Pseudomonas aeruginosa colonization in CF-patients with inhaled colistin and oral ciprofloxacin; postponing next isolate of pseudomonas and prevention of chronic infection. A prospective, double-blinded, placebo-controlled scandinavian multi-centre study. A investigator initiated study - Scandinavian Cystic Fibrosis Azithromycin Study	Cystic fibrosis with intermittent colonization of the airways with pseudomonas aeruginosa. MedDRA version: 8.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Trade Name: Zitromax Product Name: zitromax Product Code: zitromax Pharmaceutical Form: Film-coated tablet INN or Proposed INN: AZITHROMYCIN CAS Number: 83905015 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 250- Pharmaceutical form of the placebo: Coated tablet Route of administration of the placebo: Oral use Trade Name: Zitromax Product Name: zitromax Product Code: zitromax Pharmaceutical Form: Powder for oral suspension INN or Proposed INN: AZITHROMYCIN CAS Number: 83905015 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 40- Pharmaceutical form of the placebo: Granules for oral solution Route of administration of the placebo: Oral use	Copenhagen CF-centre		Not Recruiting			Female: yes Male: yes	250	Phase 4	Denmark
708	EUCTR2006-006693-24-AT	09/05/2007	19 March 2012	A Randomized, Double-Blind, Placebo-Controlled, Dose-finding Study to Evaluate the Efficacy and Safety of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis	A Randomized, Double-Blind, Placebo-Controlled, Dose-finding Study to Evaluate the Efficacy and Safety of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis	Cystic fibrosis is the most common fatal inherited disease in the Caucasian population, affecting about 4 in 10.000 children. In cystic fibrosis chloride transport across the respiratory epithelium is deficient, so the mucus contains less water and its viscosity is abnormally increased. Moli1901 corrects the abnormal transport of chloride thereby reducing the formation of mucus plugs and improving clearance. MedDRA version: 9.1 Level: PT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Moli1901 (2622U90, duramycin) Product Code: Moli1901 Pharmaceutical Form: Inhalation vapour, solution Pharmaceutical form of the placebo: Inhalation vapour, solution Route of administration of the placebo: Inhalation use	AOP Orphan Pharmaceuticals AG		Not Recruiting			Female: yes Male: yes	160		Hungary;Germany;Czech Republic;France;Spain;Italy;Austria;Sweden
709	NCT00449878	May 2007	19 February 2015	Liprotamase Efficacy Trial in Patients With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	A Phase III, Randomized, Double-Blind, Placebo-Controlled Clinical Study Evaluating the Efficacy and Safety of ALTU-135 Treatment in Patients With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: Liprotamase;Drug: Placebo	Anthera Pharmaceuticals		Not recruiting	7 Years	N/A	Both	163	Phase 3	United States
710	NCT00457821	May 2007	19 October 2017	Safety Study of Ivacaftor in Subjects With Cystic Fibrosis	A Phase 2a, Randomized, Double-Blind, Placebo-Controlled Study of VX-770 to Evaluate Safety, Pharmacokinetics, and Biomarkers of CFTR Activity in Cystic Fibrosis (CF) Subjects With Genotype G551D	Cystic Fibrosis	Drug: Ivacaftor 25 mg/75 mg;Drug: Ivacaftor 75 mg/150 mg;Drug: Ivacaftor 150 mg or 250 mg;Drug: Placebo	Vertex Pharmaceuticals Incorporated	Cystic Fibrosis Foundation Therapeutics	Not recruiting	18 Years	N/A	All	39	Phase 2	United States;Canada;Germany
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
711	EUCTR2007-001007-38-FR	25/04/2007	19 March 2012	STUDY OF THE LONG TERM BIOLOGICAL EFFICACY AND TOLERABILITY OF VEDROP (VITAMIN E-TPGS FORMULATION) AFTER DAILY ORAL ADMINISTRATION IN 30 PAEDIATRIC PATIENTS WITH CYSTIC FIBROSIS - 1ORP2	STUDY OF THE LONG TERM BIOLOGICAL EFFICACY AND TOLERABILITY OF VEDROP (VITAMIN E-TPGS FORMULATION) AFTER DAILY ORAL ADMINISTRATION IN 30 PAEDIATRIC PATIENTS WITH CYSTIC FIBROSIS - 1ORP2	Paediatric patient who present a cystic fibrosis	Product Name: VEDROP Product Code: VITAMIN E - TGPS Pharmaceutical Form: Oral solution INN or Proposed INN: d-a-tocopheryl PEG 1000 succinate Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 50-	ORPHAN		Authorised			Female: yes Male: yes			France
712	EUCTR2006-006362-41-GB	05/04/2007	19 March 2012	An open study on the pharmacokinetics and safety of oral voriconazole in adult patients with cystic fibrosis - Voriconazole in CF	An open study on the pharmacokinetics and safety of oral voriconazole in adult patients with cystic fibrosis - Voriconazole in CF	Adult patients with cystic fibrosis MedDRA version: 8.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Vfend Product Name: Vfend Pharmaceutical Form: Film-coated tablet INN or Proposed INN: VORICONAZOLE CAS Number: 137234629 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 200-	Leeds Teaching Hospital NHS Trust		Authorised			Female: yes Male: yes			United Kingdom
713	EUCTR2006-004078-28-GB	14/03/2007	21 August 2017	Long Term Administration of Inhaled Dry Powder Mannitol In Cystic Fibrosis – A Safety and Efficacy Study	Long Term Administration of Inhaled Dry Powder Mannitol In Cystic Fibrosis – A Safety and Efficacy Study	Cystic Fibrosis MedDRA version: 14.1 Level: PT Classification code 10011762 Term: Cystic fibrosis System Organ Class: 10010331 - Congenital, familial and genetic disorders	Product Name: IDPM: Inhaled Dry Powder Mannitol Product Code: IDPM Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: MANNITOL CAS Number: 69658 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 40- Pharmaceutical form of the placebo: Inhalation powder, hard capsule Route of administration of the placebo: Inhalation use	Pharmaxis UK Limited		Not Recruiting			Female: yes Male: yes	250	Phase 3	United Kingdom
714	NCT00434278	March 2007	16 December 2017	A Trial of Pulmozyme Withdrawal on Exercise Tolerance in Cystic Fibrosis Subjects With Severe Lung Disease (TOPIC)	A Multicenter, Randomized, Double-Blind, Placebo-Controlled Trial of Pulmozyme Withdrawal on Exercise Tolerance in Cystic Fibrosis Subjects With Severe Lung Disease	Cystic Fibrosis	Drug: Dornase alfa;Drug: placebo	Genentech, Inc.		Not recruiting	14 Years	N/A	All	27	Phase 4	United States
715	NCT00446680	March 2007	19 February 2015	Long Term Administration of Inhaled Dry Powder Mannitol In Cystic Fibrosis - A Safety and Efficacy Study	Long Term Administration of Inhaled Dry Powder Mannitol In Cystic Fibrosis - A Safety and Efficacy Study	Cystic Fibrosis	Drug: Mannitol;Drug: placebo	Pharmaxis		Not recruiting	6 Years	N/A	Both	340	Phase 3	Australia;Ireland;United Kingdom
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
716	NCT00458341	March 2007	19 February 2015	PTC124 for Pediatric Patients With Cystic Fibrosis	A Phase 2 Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	Cystic Fibrosis	Drug: PTC124	PTC Therapeutics		Not recruiting	6 Years	18 Years	Both	30	Phase 2	Belgium;France;Israel
717	NCT00431964	February 2007	19 October 2017	Effect of Azithromycin on Lung Function in 6-18 Year-olds With Cystic Fibrosis (CF) Not Infected With P. Aeruginosa	Multi-center, Multi-national, Randomized, Placebo-Controlled Trial of Azithromycin in Subjects With Cystic Fibrosis 6-18 Years Old, Culture Negative for Pseudomonas Aeruginosa	Cystic Fibrosis	Drug: azithromycin 250 mg tablets;Drug: placebo tablets	CF Therapeutics Development Network Coordinating Center	Cystic Fibrosis Foundation Therapeutics	Not recruiting	6 Years	18 Years	All	263	Phase 4	United States;Canada
718	NCT00777296	February 2007	28 November 2016	Multidose Safety and Tolerability Study of Dose Escalation of Liposomal Amikacin for Inhalation (ARIKAYCE™)	Phase 1b/2a Multidose Safety and Tolerability Study of Dose Escalation of Liposomal Amikacin for Inhalation (ARIKAYCE™) In Cystic Fibrosis Patients With Chronic Infections Due To Pseudomonas Aeruginosa.	Cystic Fibrosis	Drug: Arikayce™;Drug: Placebo	Insmed Incorporated		Not recruiting	6 Years	N/A	Both		Phase 1/Phase 2	Belgium;Hungary;Macedonia, The Former Yugoslav Republic of;Poland;Serbia;Slovakia;Ukraine;Bulgaria
719	NCT00406536	January 2007	19 February 2015	Study of LYM-X-SORB™ to Improve Fatty Acid and Choline Status in Children With CF and PI	Phase II Study: LYM-X-SORB™, an Organized Lipid Matrix: Fatty Acids and Choline in CF	Cystic Fibrosis;Pancreatic Insufficiency	Dietary Supplement: Lym-X-Sorb powder;Dietary Supplement: Placebo powder	Avanti Polar Lipids, Inc.	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	Not recruiting	6 Years	17 Years	Both	110	Phase 2	United States
720	NCT00432861	January 2007	19 February 2015	Evaluation of the Safety and Efficacy of Pancrecarb® MS-16 in Cystic Fibrosis	A Randomized, Double-Blind, Placebo-Controlled, Multi-Center, Crossover Study to Evaluate the Effectiveness and Safety of PANCRECARB® MS-16 (Pancrelipase) in Reducing Steatorrhea in Children and Adults With Cystic Fibrosis	Cystic Fibrosis;Pancreatic Insufficiency	Drug: PANCRECARB® (pancrelipase);Drug: Placebo	Digestive Care, Inc.		Not recruiting	7 Years	N/A	Both	29	Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
721	EUCTR2007-000171-41-PL		2 October 2017	A Phase III, Randomized, Double-Blind, Placebo-Controlled Clinical Study Evaluating the Efficacy and Safety of ALTU-135 Treatment in Patients with Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	A Phase III, Randomized, Double-Blind, Placebo-Controlled Clinical Study Evaluating the Efficacy and Safety of ALTU-135 Treatment in Patients with Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Patients with Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency MedDRA version: 9.1 Level: LLT Classification code 10011766 Term: Cystic fibrosis pancreatic	Product Name: ALTU-135 Product Code: ALTU-135 Pharmaceutical Form: Capsule* INN or Proposed INN: NA CAS Number: 9001-62-1 Other descriptive name: Lipase CLEC Concentration unit: U unit(s) Concentration type: equal Concentration number: 32,500 U- INN or Proposed INN: NA CAS Number: 9000-90-2 Other descriptive name: Amylase Concentration unit: U unit(s) Concentration type: equal Concentration number: 3,750 U- INN or Proposed INN: NA CAS Number: 9074-07-1 Other descriptive name: Protease Concentration unit: U unit(s) Concentration type: equal Concentration number: 25,000 U- Pharmaceutical form of the placebo: Capsule* Route of administration of the placebo: Oral use	Altus Pharmaceuticals Inc		Not Available			Female: yes Male: yes	176	Phase 3	Slovakia;Poland;Italy
722	EUCTR2007-000178-21-PL		2 October 2017	An Open-Label Clinical Study Evaluating the Long-Term Safety of ALTU-135 for the Treatment of Patients with Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	An Open-Label Clinical Study Evaluating the Long-Term Safety of ALTU-135 for the Treatment of Patients with Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency MedDRA version: 9.1 Level: LLT Classification code 10011765 Term: Cystic fibrosis pancreas	Product Name: ALTU-135 Product Code: ALTU-135 Pharmaceutical Form: Capsule* INN or Proposed INN: NA CAS Number: 9001-62-1 Other descriptive name: Lipase CLEC Concentration unit: U unit(s) Concentration type: equal Concentration number: 32,500 USP Units- INN or Proposed INN: NA CAS Number: 9000-90-2 Other descriptive name: Amylase Concentration unit: U unit(s) Concentration type: equal Concentration number: 3,750 USP Units- INN or Proposed INN: NA CAS Number: 9074-07-1 Other descriptive name: Protease Concentration unit: U unit(s) Concentration type: equal Concentration number: 25,000 USP Units-	Altus Pharmaceuticals Inc.		Not Recruiting			Female: yes Male: yes	200	Phase 3	Slovakia;Poland;Italy
723	EUCTR2007-000935-25-NL		19 March 2012	‘Peripheral targeting of inhaled rhDNase in stable CF patients.’	‘Peripheral targeting of inhaled rhDNase in stable CF patients.’	Cystic Fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Trade Name: Pulmozyme (Dornase alpha) Product Name: Pulmozyme Pharmaceutical Form: Nebuliser solution	ErasmusMC - Sophia Children's Hospital		Authorised			Female: yes Male: yes	50		Netherlands
724	EUCTR2007-001548-36-DE		2 October 2017	Nasale Inhalation von Pulmozyme bei Patienten mit Mukoviszidose und chronischer Rhinosinusitis mit dem Pari Sinus-Vernebler. - bizentrische, randomisierte, doppel-blinde, placebo-kontrollierte, prospektive klinische Studie - Nasal Inhalation of Dornase alfa (Pulmozyme) in Patients with Cystic Fibrosis and Chronic Rhinosinusitis - pulmozyme-nasal-cf	Nasale Inhalation von Pulmozyme bei Patienten mit Mukoviszidose und chronischer Rhinosinusitis mit dem Pari Sinus-Vernebler. - bizentrische, randomisierte, doppel-blinde, placebo-kontrollierte, prospektive klinische Studie - Nasal Inhalation of Dornase alfa (Pulmozyme) in Patients with Cystic Fibrosis and Chronic Rhinosinusitis - pulmozyme-nasal-cf	Cystic Fibrosis with chronic rhinosinusitis	Trade Name: Pulmozyme Product Name: Pulmozyme Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: Dornase alfa Other descriptive name: recombiant human Desoxyribonuclease ( rhDNase) Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 1- Pharmaceutical form of the placebo: Solution for injection Route of administration of the placebo: Inhalation use	University of Jena		Not Recruiting			Female: yes Male: yes	16	Phase 3	Germany
725	EUCTR2006-005180-25-DE	15/12/2006	19 March 2012	A study investigating the safety, tolerability and pharmacodynamics of bacterial lipase in patients with cystic fibrosis and pancreatic insufficiency Randomized, double-blind, placebo-controlled, single period, parallel group design	A study investigating the safety, tolerability and pharmacodynamics of bacterial lipase in patients with cystic fibrosis and pancreatic insufficiency Randomized, double-blind, placebo-controlled, single period, parallel group design	Caucasian males aged between 18 and 50 years of age (inclusive) with cystic fibrosis and pancreatic insufficiency able to discontinue their standard pancreatic enzyme therapy during the treatment phase of the study. MedDRA version: 8.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Code: LU 70274 Pharmaceutical Form: Capsule* Current Sponsor code: LU 70274 Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 90000- Pharmaceutical form of the placebo: Capsule* Route of administration of the placebo: Oral use	Nordmark Arzneimittel GmbH		Not Recruiting			Female: no Male: yes	20		Germany
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
726	NCT00351078	December 2006	8 January 2018	PTC124 for the Treatment of Cystic Fibrosis	A Phase 2b Extension Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	Cystic Fibrosis	Drug: PTC124	PTC Therapeutics		Not recruiting	18 Years	N/A	All	19	Phase 2	Israel
727	NCT00416182	December 2006	19 October 2017	Nasally Delivered Pulmozyme for Sinusitis in Cystic Fibrosis	The Use of Nasally Delivered Pulmozyme in the Treatment of Sinusitis in Cystic Fibrosis Patients: A Pilot Study	Sinusitis;Cystic Fibrosis	Drug: Pulmozyme (dornase alfa);Drug: Placebo	University of Vermont	Genentech, Inc.	Not recruiting	5 Years	N/A	All	16	Phase 2	United States
728	NCT00547053	December 2006	19 February 2015	Amiloride Solution and Tobramycin Solution for Inhalation for the Eradication of Burkholderia Dolosa in Patients With Cystic Fibrosis	A Six Month Open Label Study of Amiloride Solution for Inhalation and Tobramycin Solution for Inhalation for the Eradication of Burkholderia Dolosa in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Amiloride Solution for Inhalation	Children's Hospital Boston	Cystic Fibrosis Foundation Therapeutics	Not recruiting	6 Years	N/A	Both	25	Phase 1	United States
729	NCT00721071	December 2006	19 February 2015	Utility of Induced Sputum Using Hypertonic Saline to Evaluate Infection and Inflammation in Cystic Fibrosis	Diagnostic Utility of Induced Sputum Using Hypertonic Saline to Evaluate Airway Infection and Inflammation in Cystic Fibrosis	Cystic Fibrosis	Drug: Hypertonic Saline	The Hospital for Sick Children		Not recruiting	6 Years	18 Years	Both	95	Phase 2	Canada
730	EUCTR2006-001254-27-FR	30/11/2006	19 March 2012	Open randomised prospective comparative multi-centre intervention study of patients with cystic fibrosis and early diagnosed diabetes mellitus	Open randomised prospective comparative multi-centre intervention study of patients with cystic fibrosis and early diagnosed diabetes mellitus	Patients atteints de mucoviscidose, chez lesquels un diagnostic de diabète a été fait par hyperglycémie provoquée orale (HGPO) MedDRA version: 8.1 Level: PT Classification code 10012594 Term: DIABETES	Trade Name: NovoNorm 0.5 mg Product Name: NovoNorm 0.5 mg Pharmaceutical Form: Tablet INN or Proposed INN: Répaglinide Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 0.5- Trade Name: Actrapid Penfill 100UI/ml Product Name: Actrapid Penfill 100UI/ml Pharmaceutical Form: Solution for injection INN or Proposed INN: Insuline humaine recombinante Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 300- Trade Name: NovoNorm 1 mg Product Name: NovoNorm 1 mg Pharmaceutical Form: Tablet INN or Proposed INN: Répaglinide Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 1- Trade Name: NovoNorm 2 mg Product Name: NovoNorm 2 mg Pharmaceutical Form: Tablet INN or Proposed INN: Répaglinide Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 2-	ASSISTANCE PUBLIQUE - HOPITAUX DE PARIS (AP-HP)		Authorised			Female: yes Male: yes			France
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
731	NCT00405665	November 2006	19 February 2015	The Short Term Safety and Efficacy of Inhaled L-arginine in Patients With Cystic Fibrosis	Pilot Study of the Short Term Safety and Efficacy of Inhaled L-arginine in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: L-arginine	The Hospital for Sick Children		Not recruiting	14 Years	N/A	Both	20	Phase 2	Canada
732	NCT00408317	November 2006	16 December 2017	Safety and Efficacy Study of ULTRASE® MT20 in Participants With Cystic Fibrosis (CF) and Exocrine Pancreatic Insufficiency (PI)	A Multicenter, Randomized, Double-Blind, Crossover Study to Compare the Safety and Efficacy of Ultrase® MT20 to Placebo for the Correction of Steatorrhea in Patients With Cystic Fibrosis (CF)	Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: Ultrase® MT20;Drug: Placebo	Forest Laboratories		Not recruiting	7 Years	N/A	All	36	Phase 3	United States
733	NCT00450073	November 2006	19 October 2017	Improving Vitamin D Status In Cystic Fibrosis	Desktop Tanning Unit to Improve Vitamin D Status in Patients With Cystic Fibrosis and Short Bowel Syndrome: A Pilot Study	Cystic Fibrosis	Drug: ergocalciferol (vitamin D2);Device: Sperti Del Sol Lamp;Drug: Vitamin D3	Atlanta VA Medical Center	Emory University	Not recruiting	18 Years	60 Years	All	30	N/A	United States
734	NCT00763412	November 2006	16 December 2017	Pilot and Feasibility Study for the Treatment of Pre-diabetes in Patients With Cystic Fibrosis	Pilot and Feasibility Study for the Treatment of Pre-diabetes in Patients With Cystic Fibrosis	Cystic Fibrosis Related Diabetes;Pancreatic Insufficiency	Drug: placebo;Drug: repaglinide	Arbelaez, Ana Maria	Washington University School of Medicine;National Institutes of Health (NIH);Novo Nordisk A/S;National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	Not recruiting	12 Years	24 Years	All	31	N/A	United States
735	EUCTR2005-005594-29-HU	31/10/2006	19 March 2012	A Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis	A Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis	Cystic Fibrosis	Product Name: Moli1901 Product Code: Moli1901 Pharmaceutical Form: Nebuliser solution CAS Number: 1391-36-2 Current Sponsor code: Moli1901 Other descriptive name: Duramycin, 2622U90 Concentration unit: g/l gram(s)/litre Concentration type: equal Concentration number: 0,5- Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	AOP Orphan Pharmaceuticals AG		Authorised			Female: yes Male: yes	360		Hungary;Czech Republic
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
736	EUCTR2005-003870-88-DE	20/10/2006	27 January 2014	Randomized, placebo-controlled, double-blind study to investigate the efficacy and safety of a 24-week inhalation treatment with glutathione in cystic fibrosis patients - GSH-4	Randomized, placebo-controlled, double-blind study to investigate the efficacy and safety of a 24-week inhalation treatment with glutathione in cystic fibrosis patients - GSH-4	Cystic fibrosis (CF) is the most common autosomal recessive lethal hereditary disorder in Caucasians. The majority of cystic fibrosis patients die as a result of progressive pulmonary disease. Airway inflammation, characterized by an excessive and persistent neutrophilic infiltration, is key for the pathogenesis of CF lung disease, and ultimately leads to lung destruction.	Product Name: GSH (Glutathione) Product Code: TAD 600 Pharmaceutical Form: Inhalation vapour, liquid INN or Proposed INN: Glutathione Current Sponsor code: GSH Other descriptive name: reduced glutathione Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 600- Pharmaceutical form of the placebo: Inhalation vapour, liquid Route of administration of the placebo: Inhalation use	Mukoviszidose Institut gGmbH i. G.		Not Recruiting			Female: yes Male: yes	160		Germany
737	EUCTR2006-002259-33-DE	10/10/2006	24 April 2012	Protocol for a Phase II-study Anti-inflammatory pulmonal therapy of CF-patients with Amitriptyline and Placebo - a randomised, double-blinded, placebo-controlled, cross over study -	Protocol for a Phase II-study Anti-inflammatory pulmonal therapy of CF-patients with Amitriptyline and Placebo - a randomised, double-blinded, placebo-controlled, cross over study -	CF-patients develop a chronic pulmonary infection with Pseudomonas aeruginosa (P. aeruginosa). At present it is unknown why CF-patients are highly sensitive to P. aeruginosa infections and, most important, no curative treatment for cystic fibrosis is available. This Study should provide a novel anti-inflammatory treatment for cystic fibrosis, which reduces pulmonary complications, progression of the disease and may possibly increase the life-expectance of the patients.	Trade Name: Amitriptylin-ct Tabletten Pharmaceutical Form: Capsule* INN or Proposed INN: AMITRIPTYLINE CAS Number: 50486 Current Sponsor code: Amitriptyline Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 75- Pharmaceutical form of the placebo: Capsule* Route of administration of the placebo: Oral use Trade Name: Amitriptylin-ct Tabletten Pharmaceutical Form: Capsule* INN or Proposed INN: AMITRIPTYLINE CAS Number: 50486 Current Sponsor code: Amitriptyline Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 50- Trade Name: Amitriptylin-ct Tabletten Pharmaceutical Form: Capsule* INN or Proposed INN: AMITRIPTYLINE CAS Number: 50486 Current Sponsor code: Amitriptyline Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 25-	Paediatrisches Sekretariat fuer Klinische Studien		Authorised			Female: yes Male: yes		Phase 2	Germany
738	NCT00515229	October 2006	19 February 2015	Anti-Inflammatory Pulmonal Therapy of CF-Patients With Amitriptyline and Placebo	Protocol for a Phase II-Study Anti-Inflammatory Pulmonal Therapy of CF-Patients With Amitriptyline and Placebo - Randomised, Double-Blinded, Placebo-Controlled, Cross Over - Study -	Cystic Fibrosis;Infection;Pseudomonas Aeruginosa	Drug: amitriptyline	University Hospital Tuebingen		Not recruiting	18 Years	50 Years	Both	18	Phase 2	Germany
739	NCT00671723	October 2006	16 December 2017	Dornase Alpha Versus Hypertonic Saline for Lung Atelectasis in Non-Cystic Fibrosis Patients	Dornase Alpha Versus Hypertonic Saline for Lung Atelectasis	Atelectasis	Drug: Normal saline:;Drug: Hypertonic Saline;Drug: Dornase alpha	University of Oklahoma		Not recruiting	18 Years	90 Years	All	33	N/A	United States
740	NCT01463371	October 2006	19 February 2015	Effects of Azithromycin on Airway Oxidative Stress Markers in Patients With Bronchiectasis	Effects of Long-Term Azithromycin Treatment on Airway Oxidative Stress Markers in Patients With Stable Non-Cystic Fibrosis Bronchiectasis	Bronchiectasis	Drug: Azithromycin	Instituto de Investigacion Sanitaria La Fe	Sociedad Valenciana de Neumología	Not recruiting	16 Years	80 Years	Both	30	N/A	Spain
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
741	NCT02172534	September 2006	19 February 2015	Safety, Tolerability and Pharmacokinetics of Tiotropium in Cystic Fibrosis Patients	A Randomized, Double-blind Within Dose, Placebo-controlled Study to Investigate the Safety, Tolerability and Pharmacokinetics of Increasing Single and Multiple Doses (28-day Dosing) of Tiotropium Bromide Administered Once Daily Via the Respimat® Device in Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Tiotropium bromide low;Drug: Tiotropium bromide medium;Drug: Tiotropium bromide high;Drug: Placebo	Boehringer Ingelheim		Not recruiting	N/A	N/A	Both	113	Phase 1
742	EUCTR2006-000945-20-GB	22/08/2006	19 March 2012	To investigate the effect of vitamin K supplementation on markers of bone turnover and bone density in adolescents and adults with cystic fibrosis - Vitamin K supplementation in Cystic Fibrosis	To investigate the effect of vitamin K supplementation on markers of bone turnover and bone density in adolescents and adults with cystic fibrosis - Vitamin K supplementation in Cystic Fibrosis	Cystic Fibrosis MedDRA version: 8.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Trade Name: Menadiol Diphosphate Product Name: Menadiol Diphosphate Product Code: Menadiol Diphosphate Pharmaceutical Form: Capsule* INN or Proposed INN: Menadiol sodium diphosphate Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 10- Pharmaceutical form of the placebo: Capsule* Route of administration of the placebo: Oral use	Barts and the London NHS Trust		Authorised			Female: yes Male: yes	40		United Kingdom
743	EUCTR2005-002997-31-GB	10/08/2006	18 April 2012	A multi-centre randomised trial of insulin detemir in pre-diabetes associated with cystic fibrosis. - Randomized trial of insulin detemir in CF associated pre-diabetes	A multi-centre randomised trial of insulin detemir in pre-diabetes associated with cystic fibrosis. - Randomized trial of insulin detemir in CF associated pre-diabetes	Individuals with cystic fibrosis develop diabetes. They exhibit abnormal glucose handling (impaired glucose tolerance), poor growth and a decline in lung function before overt diabetes develops. Early treatment with insulin before diabetes develops may improve weight gain and lung function and delay progression to overt diabetes.	Product Name: Levemir Penfill Pharmaceutical Form: Solution for injection	Sheffield Children's NHS Trust		Authorised			Female: yes Male: yes	40		United Kingdom
744	NCT00357279	July 2006	19 February 2015	Study of Denufosol Inhalation Solution in Patients With Mild Cystic Fibrosis Lung Disease	A Multi-Center, Double-Blind, Placebo-Controlled Randomized, Efficacy and Safety Study of Denufosol Tetrasodium (INS37217) Inhalation Solution in Patients With Mild Cystic Fibrosis Lung Disease	Cystic Fibrosis	Drug: denufosol tetrasodium (INS37217) Inhalation Solution;Drug: Placebo - 0.9% w/v sodium chloride solution	Merck Sharp & Dohme Corp.		Not recruiting	5 Years	N/A	Both	352	Phase 3	United States;Canada
745	NCT00458614	June 2006	19 February 2015	Pharmacokinetic Study of Lansoprazole in Cystic Fibrosis	Lansoprazole Disposition in Young Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Lansoprazole	Arkansas Children's Hospital Research Institute		Not recruiting	2 Years	10 Years	Both	18	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
746	EUCTR2005-004344-30-HU	10/05/2006	19 March 2012	An Evaluation of the Safety and Tolerability of Multiple Dose Regimens of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis and Stable Lung Disease	An Evaluation of the Safety and Tolerability of Multiple Dose Regimens of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis and Stable Lung Disease	Cystic fibrosis is the most common fatal inherited disease in the Caucasian population, affecting about 4 in 10,000 children. In cystic fibrosis chloride transport across the respiratory epithelium is deficient, so the mucus contains less water and its viscosity is abnormally increased. Moli1901 corrects the abnormal transport of chloride ans water in the lungs thereby reducing the formation of mucus plugs and improving clearance.	Product Name: Moli1901 (2622U90, duramycin) Product Code: EU Orphan Designation Number EU/3/02/120 Pharmaceutical Form: Inhalation vapour, solution CAS Number: 1391-36-2 Current Sponsor code: Moli1901 Other descriptive name: Duramycin, 2622U90 Concentration unit: g/l gram(s)/litre Concentration type: equal Concentration number: 0,5- Pharmaceutical form of the placebo: Inhalation vapour, solution Route of administration of the placebo: Inhalation use	AOP Orphan Pharmaceuticals AG		Not Recruiting			Female: yes Male: yes	18		Hungary
747	EUCTR2005-004103-10-DE	03/05/2006	19 March 2012	Randomized, open labeled, multi center, active controlled, parallel 28 days safety and bioavailability study of Tobramycin 100 PARI nebulized with eFlow® versus TOBI® nebulized with PARI LC PLUS in cystic fibrosis patients with Pseudomonas Aeruginosa infections	Randomized, open labeled, multi center, active controlled, parallel 28 days safety and bioavailability study of Tobramycin 100 PARI nebulized with eFlow® versus TOBI® nebulized with PARI LC PLUS in cystic fibrosis patients with Pseudomonas Aeruginosa infections	Cystic Fibrosis with Pseudomoas aeuriginosa infection MedDRA version: 8.1 Level: LLT Classification code 10011763 Term: Cystic fibrosis lung	Product Name: Tobramycin 100 PARI Product Code: T100 PARI Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: Tobramycin Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 150-1,5 Trade Name: Tobi Product Name: TOBI® Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: Tobramycin Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 300 -5	PARI Pharma GmbH		Not Recruiting			Female: yes Male: yes	60		Germany
748	NCT00297167	May 2006	16 December 2017	Study to Evaluate the Safety and Efficacy of EUR-1008 (APT-1008) Pancreatic Enzyme Product in Participants With Cystic Fibrosis and Exocrine Pancreatic Insufficiency	A Randomized, Double-Blind, Placebo-Controlled, Two-Treatment, Crossover Study to Evaluate the Safety and Efficacy of Eurand Pancreatic Enzyme Product (PEP) in Patients With Cystic Fibrosis and Exocrine Pancreatic Insufficiency	Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: EUR-1008 (APT-1008);Drug: Placebo	Forest Laboratories		Not recruiting	7 Years	N/A	All	34	Phase 3	United States
749	NCT00399945	May 2006	19 February 2015	Tobramycin Inhalation Solution Administered by eFlow Rapid Nebulizer: Scintigraphy Study	A Phase 1, Single-Dose, Open-Label, Two-Way Crossover, Pharmacoscintigraphy Study of Aerosol Delivery Characteristics (Measured by In Vivo Lung Deposition, Nebulization Time, Serum Tobramycin Concentrations, and Pharmacokinetic Parameters) and Safety of Tobramycin Administered for Inhalation by PARI eFlow® Rapid Electronic Nebulizer (No Compressor) vs. PARI LC PLUS (TM) Jet Nebulizer (With Compressor) in Healthy Subjects and in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: Tobramycin	Novartis		Not recruiting	18 Years	65 Years	Both	12	Phase 1	United Kingdom
750	NCT00981214	May 2006	16 December 2017	Study of Pancreatic Enzyme Product in Pediatric Participants With Cystic Fibrosis and Exocrine Pancreatic Insufficiency	An Open-Label Study to Evaluate the Efficacy and Safety of Pancreatic Enzyme Product (PEP) Microtabs in Pediatric Patients With Cystic Fibrosis and Exocrine Pancreatic Insufficiency	Cystic Fibrosis;Exocrine Pancreatic Insufficiency	Drug: EUR-1008 (APT-1008)	Forest Laboratories		Not recruiting	N/A	7 Years	All	19	Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
751	NCT00322868	April 2006	5 March 2018	Safety and Efficacy of Pioglitazone as an Anti-inflammatory for the Treatment of Cystic Fibrosis (CF) Lung Disease	A Pilot Study Assessing the Safety and Efficacy of Pioglitazone as an Anti-inflammatory Agent for the Treatment of CF Lung Disease in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: pioglitazone	University Hospitals Cleveland Medical Center	Cystic Fibrosis Foundation Therapeutics	Not recruiting	18 Years	N/A	All	21	N/A	United States
752	NCT00420836	April 2006	19 February 2015	Tobramycin Administered by eFlow Rapid Nebulizer: Pharmacokinetic Study	Crossover Pharmacokinetic Study of Tobramycin Administered for Inhalation by PARI eFlow® Rapid Electronic Nebulizer (no Compressor) vs. PARI LC PLUSTM Jet Nebulizer (With Compressor) in Cystic Fibrosis Subjects	Cystic Fibrosis	Drug: Tobramycin	Novartis		Not recruiting	6 Years	N/A	Both	20	Phase 1
753	NCT00308243	March 2006	19 February 2015	Inhaled Sodium Pyruvate for the Treatment of Cystic Fibrosis.	Inhaled Sodium Pyruvate for the Treatment of Cystic Fibrosis. A Phase I, Double Blind, Placebo Controlled, Safety Study. Stage 1)	Cystic Fibrosis	Drug: Sodium Pyruvate in 0.9% Sodium Chloride Solution	Emphycorp	Cellular Sciences	Not recruiting	18 Years	N/A	Both	15	Phase 1	United States
754	NCT00311883	March 2006	19 February 2015	Hydroxychloroquine in Cystic Fibrosis	Phase 1 Study of Hydroxychloroquine in Cystic Fibrosis	Cystic Fibrosis	Drug: hydroxychloroquine	Vanderbilt University		Not recruiting	16 Years	N/A	Both	20	Phase 1	United States
755	NCT00753987	March 2006	19 February 2015	Tolerability of Hypertonic Saline in Infants With Cystic Fibrosis	Tolerability of Hypertonic Saline in Infants With Cystic Fibrosis	Cystic Fibrosis	Drug: Hypertonic Saline	The Hospital for Sick Children		Not recruiting	2 Months	2 Years	Both	13	Phase 1	Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
756	NCT00287456	February 2, 2006	21 January 2019	Use of the Insulin Pump in Cystic Fibrosis Patients With Impaired Glucose Tolerance or CFRD and in Type 1 Diabetes Patients.		Cystic Fibrosis Related Diabetes	Device: Insulin Pump;Drug: Insulin;Procedure: Oral Glucose Tolerance Test;Procedure: Whole body Protein Turnover	University of Texas Southwestern Medical Center		Not recruiting	12 Years	32 Years	All	0	N/A	United States
757	NCT00298922	February 2006	19 February 2015	Azithromycin in Patients With CF, Infected With Burkholderia Cepacia Complex	Phase II, Randomized, Double Blind, Placebo-Controlled Trial of Azithromycin in Patients With CF, Chronically Infected With Burkholderia Cepacia Complex	Cystic Fibrosis	Drug: Azithromycin;Drug: Placebo	St. Michael's Hospital, Toronto	Cystic Fibrosis Foundation Therapeutics;Pfizer	Not recruiting	19 Years	N/A	Both	45	Phase 2	Canada
758	NCT00332215	February 2006	19 February 2015	A Phase I Study of Inhaled Sodium Pyruvate for the Treatment of Cystic Fibrosis.	Inhaled Sodium Pyruvate for the Treatment of Cystic Fibrosis. A Phase I, Double Blind, Placebo Controlled, Safety Study.	Cystic Fibrosis	Drug: Inhaled Sodium Pyruvate	University of Minnesota - Clinical and Translational Science Institute	Cellular Sciences	Not recruiting	18 Years	N/A	Both	70	Phase 1	United States
759	NCT00388505	February 2006	19 October 2017	Safety of Tobramycin Inhalation Powder (TIP) vs Tobramycin Solution for Inhalation in Patients With Cystic Fibrosis	A Randomized, Open-label Multicentre Phase 3 Trial to Assess the Safety of Tobramycin Inhalation Powder Compared to Tobramycin Solution for Inhalation in Cystic Fibrosis Subjects	Cystic Fibrosis	Drug: Tobramycin Inhalation Powder;Drug: Tobramycin Solution for Inhalation	Novartis Pharmaceuticals		Not recruiting	6 Years	N/A	All	517	Phase 3	United States;Australia;Canada;Chile;Colombia;France;Germany;Hungary;Israel;Italy;Mexico;Netherlands;Spain;United Kingdom
760	NCT00483769	February 2006	19 February 2015	One Year Glargine Treatment in CFRD Children and Adolescents	One-Year Glargine-Treatment Can Ameliorate Clinical Features in Cystic Fibrosis Children and Adolescents With Glucose Derangements	Cystic Fibrosis;Glucose Metabolism Disorders	Drug: Glargine	Federico II University		Not recruiting	3 Years	20 Years	Both	20	Phase 4	Italy
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
761	EUCTR2005-003772-37-GB	06/01/2006	10 July 2015	A Randomized, Open-label, Multicenter, Phase 3 Trial to Assess the Safety of Tobramycin Inhalation Powder Compared to TOBI® in Cystic Fibrosis Subjects - TIP003	A Randomized, Open-label, Multicenter, Phase 3 Trial to Assess the Safety of Tobramycin Inhalation Powder Compared to TOBI® in Cystic Fibrosis Subjects - TIP003	Pulmonary pseudomonas aeruginosa infections in patients with cystic fibrosis Classification code 10011762	Product Name: Tobramycin Inhalation Powder (TIP) Product Code: TBM100C Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 28- Trade Name: TOBI Product Name: TOBI Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin CAS Number: 32986-56-4 Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 60-	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	500	Phase 3	Hungary;Greece;Spain;Germany;Italy;United Kingdom
762	NCT00274313	January 2006	19 February 2015	Safety Study of Inhaled 552-02 in Cystic Fibrosis Patients	A Study of the Safety and Pharmacokinetics of 552-02 Following 14 Days of Dosing By Inhalation in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: 552-02	Parion Sciences		Not recruiting	14 Years	N/A	Both	40	Phase 1/Phase 2	United States
763	NCT00287430	January 2006	25 March 2019	Growth Hormone Use in Adolescents and Adults With Cystic Fibrosis		Cystic Fibrosis	Drug: Growth Hormone;Procedure: Whole body Protein Turnover Study;Procedure: Dual Energy X-ray Absorptiometry (DEXA)	University of Texas Southwestern Medical Center	Dana S Hardin	Not recruiting	6 Years	40 Years	All	0	N/A
764	EUCTR2005-004389-17-HU	28/12/2005	19 March 2012	SAFETY AND TOLERABILITY STUDY OF SLIT AMIKACIN 500 MG ONCE DAILY FOR 14 DAYS BY INHALATION IN CYSTIC FIBROSIS STUDY SUBJECTS CHRONICALLY INFECTED WITH PSEUDOMONAS AERUGINOSA	SAFETY AND TOLERABILITY STUDY OF SLIT AMIKACIN 500 MG ONCE DAILY FOR 14 DAYS BY INHALATION IN CYSTIC FIBROSIS STUDY SUBJECTS CHRONICALLY INFECTED WITH PSEUDOMONAS AERUGINOSA	Cystic Fibrosis with mild to moderate obstructive lung disease and chronic infection with Pseudomonas aeruginosa	Product Name: SLIT Amikacin Product Code: TR02 Pharmaceutical Form: Nebuliser suspension INN or Proposed INN: amikacin Other descriptive name: amikin, amiklin, amicacin, BB-K8, Biklin, Fabianol, Kaminax, Mikavir, Novamin, Pierami Concentration unit: mg/ml milligram(s)/millilitre Concentration type: range Concentration number: 40-55	Transave, Inc		Authorised			Female: yes Male: yes	15		Hungary
765	EUCTR2005-004899-21-BE	22/12/2005	19 March 2012	Safety and tolerability study of SLIT(TM) Amikacin 500 mg once daily for 14 days by inhalation in cystic fibrosis study subjects chronically infected with Pseudomonas aeruginosa. - NA	Safety and tolerability study of SLIT(TM) Amikacin 500 mg once daily for 14 days by inhalation in cystic fibrosis study subjects chronically infected with Pseudomonas aeruginosa. - NA	Cystic Fibrosis	Product Name: SLIT(TM) Amikacin Product Code: TR02 Pharmaceutical Form: Inhalation vapour, solution INN or Proposed INN: Amikacin CAS Number: 37517-28-5 6 Current Sponsor code: NA Other descriptive name: amikin®, amiklin, amicacin, BB-K8, Biklin®, Fabianol®, Kaminax, Mikavir, Novamin®, Pierami Concentration unit: mg/ml milligram(s)/millilitre Concentration type: range Concentration number: 40-55	Transave, Inc.		Not Recruiting			Female: yes Male: yes	15		Belgium
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
766	NCT00265434	December 2005	19 February 2015	Nasal Inhalation of Dornase Alfa (Pulmozyme) in Patients With Cystic Fibrosis and Chronic Rhinosinusitis	Nasal Inhalation of Dornase Alfa (Pulmozyme) in Patients With Cystic Fibrosis and Chronic Rhinosinusitis	Cystic Fibrosis;Chronic Rhinosinusitis	Drug: Pulmozyme	University of Jena	PD Dr. Joachim Riethmöller, Tübingen;PD Dr. Assen Koitschev, Tübingen;Dr. Gerlind Schneider	Not recruiting	5 Years	N/A	Both	5	Phase 3	Germany
767	EUCTR2005-000311-98-GB	09/11/2005	28 February 2019	A randomised controlled trial (pilot study) of the use of macerated garlic oil in patients with cystic fibrosis who have pulmonary infection with Pseudomonas aeruginosa - GAP pilot study	A randomised controlled trial (pilot study) of the use of macerated garlic oil in patients with cystic fibrosis who have pulmonary infection with Pseudomonas aeruginosa - GAP pilot study	Chronic pulmonary infection with Pseudomonas aeruginosa in patients with cystic fibrosis	Product Name: Boots High Strength Garlic Odour Controlled Product Code: SE10599 Pharmaceutical Form: Capsule* Pharmaceutical form of the placebo: Capsule* Route of administration of the placebo: Oral use	University of Nottingham		Not Recruiting			Female: yes Male: yes	30	Phase 2	United Kingdom
768	NCT00117208	November 2005	19 February 2015	Comparison of Inhaled Mannitol and rhDNase in Children With Cystic Fibrosis	A Cross-Over Comparative Study of Inhaled Mannitol, Alone and in Combination With Daily rhDNase, in Children With Cystic Fibrosis	Cystic Fibrosis	Drug: mannitol;Drug: mannitol + pulmozyme;Drug: Dornase alpha	Pharmaxis		Not recruiting	8 Years	18 Years	Both	20	Phase 2	United Kingdom
769	NCT00237380	November 2005	19 February 2015	Safety and Efficacy of PTC124 for Cystic Fibrosis	A Phase 2 Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	Cystic Fibrosis	Drug: PTC124	PTC Therapeutics		Not recruiting	18 Years	N/A	Both	24	Phase 2	Israel
770	EUCTR2005-004344-30-AT	31/10/2005	10 June 2013	An Evaluation of the Safety and Tolerability of Multiple Dose Regimens of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis and Stable Lung Disease	An Evaluation of the Safety and Tolerability of Multiple Dose Regimens of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis and Stable Lung Disease	Cystic fibrosis is the most common fatal inherited disease in the Caucasian population, affecting about 4 in 10,000 children. In cystic fibrosis chloride transport across the respiratory epithelium is deficient, so the mucus contains less water and its viscosity is abnormally increased. Moli1901 corrects the abnormal transport of chloride ans water in the lungs thereby reducing the formation of mucus plugs and improving clearance.	Product Name: Moli1901 (2622U90, duramycin) Product Code: EU Orphan Designation Number EU/3/02/120 Pharmaceutical Form: Inhalation vapour, solution CAS Number: 1391-36-2 Current Sponsor code: Moli1901 Other descriptive name: Duramycin, 2622U90 Concentration unit: g/l gram(s)/litre Concentration type: equal Concentration number: 0,5- Pharmaceutical form of the placebo: Inhalation vapour, solution Route of administration of the placebo: Inhalation use	AOP Orphan Pharmaceuticals AG		Not Recruiting			Female: yes Male: yes	18		Hungary;Austria
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
771	NCT00231192	October 2005	23 March 2015	Repaglinide for Adolescents With Cystic Fibrosis-Related Diabetes	Repaglinide for Adolescents With Cystic Fibrosis-Related Diabetes	Diabetes	Drug: Repaglinide and Insulin	Children's Hospital of Philadelphia		Not recruiting	12 Years	20 Years	Both	0	N/A	United States
772	NCT00251056	October 2005	19 February 2015	Mannitol Dose Response Study in Cystic Fibrosis	A Phase IIa Randomised, Open Label, Dose Response Study to Determine the Optimum Dose of Dry Powder Mannitol Required to Generate Clinical Improvement In Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: mannitol	Pharmaxis		Not recruiting	7 Years	N/A	Both	48	Phase 2	Argentina;Canada
773	EUCTR2005-003837-42-GB	26/09/2005	18 April 2012	CIPROFLOXACIN-INDUCED PHOTOTOXICITY IN PATIENTS WITH CYSTIC FIBROSIS - ciprofloxacin phototoxicity cystic fibrosis	CIPROFLOXACIN-INDUCED PHOTOTOXICITY IN PATIENTS WITH CYSTIC FIBROSIS - ciprofloxacin phototoxicity cystic fibrosis	infection - ciprofloxacin is used to treat various infections, for example respiratory tract infections, ear, nose and throat infections, eye infections, urinary tract infections. The main indication that is relevant to this application is its use in the treatment of chest infections in patients with cystic fibrosis. I have included the ICD classification for chest infection below.	Trade Name: Ciprofloxacin (ciproxin) Product Name: N/A Product Code: N/A Pharmaceutical Form: Coated tablet	Belfast City Hospital Trust		Authorised			Female: yes Male: yes	60		United Kingdom
774	EUCTR2005-002035-28-LT	19/09/2005	19 March 2012	A Randomized, Double-Blind, Placebo-Controlled, Multicenter, Phase 3 Trial to Assess the Efficacy and Safety of Tobramycin Inhalation Powder (TIP) in Cystic Fibrosis (CF) Subjects	A Randomized, Double-Blind, Placebo-Controlled, Multicenter, Phase 3 Trial to Assess the Efficacy and Safety of Tobramycin Inhalation Powder (TIP) in Cystic Fibrosis (CF) Subjects	pulmonary P aeruginosa infection in patient with cystic fibrosis MedDRA version: 9.1 Level: LLT Classification code 10011762 Term: Cystic fibrosis	Product Name: Tobramycin Inhalation Powder Product Code: TIP Pharmaceutical Form: Inhalation powder INN or Proposed INN: Tobramycine Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 112- Pharmaceutical form of the placebo: Inhalation powder Route of administration of the placebo: Inhalation use	Novartis Pharma Services AG		Not Recruiting			Female: yes Male: yes	220	Phase 3	Lithuania
775	NCT00125346	September 2005	19 February 2015	Tobramycin Inhalation Powder (TIP) in Cystic Fibrosis Subjects	A Randomized, Double-Blind, Placebo-Controlled, Multicenter, Phase 3 Trial to Assess the Efficacy and Safety of Tobramycin Inhalation Powder (TIP) in Cystic Fibrosis (CF) Subjects	Cystic Fibrosis	Drug: Tobramycin Inhalation Powder	Novartis		Not recruiting	6 Years	21 Years	Both	98	Phase 3	United States;Argentina;Brazil;Bulgaria;Canada;Chile;Lithuania;Mexico;Former Serbia and Montenegro
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
776	NCT00234663	September 2005	19 February 2015	PTC124 for Cystic Fibrosis	A Phase 2 Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis	Cystic Fibrosis	Drug: PTC124	PTC Therapeutics	Cystic Fibrosis Foundation Therapeutics;FDA Office of Orphan Products Development	Not recruiting	18 Years	N/A	Both	24	Phase 2	United States
777	NCT00128492	August 2005	19 October 2017	Safety and Efficacy Study of Aztreonam for Inhalation Solution (AZLI) in Cystic Fibrosis (CF) Patients With Pseudomonas Aeruginosa (PA)	A Phase 3, Open-label, Follow-On Study of Multiple Courses of Aztreonam Lysinate for Inhalation (AI) in Cystic Fibrosis Patients (AIR-CF3)	Cystic Fibrosis	Drug: AZLI 75 mg two times a day (BID)/ three times a day (TID)	Gilead Sciences		Not recruiting	6 Years	N/A	All	274	Phase 3	United States;Australia;Canada;New Zealand
778	NCT00130182	August 2005	19 February 2015	A Study in Pediatric Patients With Cystic Fibrosis Lung Disease	A Double-Blind, Randomized, Placebo-Controlled, 28-Day Study of Denufosol Tetrasodium (INS37217) Inhalation Solution in Pediatric Patients With Cystic Fibrosis Lung Disease	Cystic Fibrosis	Drug: denufosol tetrasodium (INS37217)	Merck Sharp & Dohme Corp.		Not recruiting	5 Years	7 Years	Both	13	Phase 2	United States
779	EUCTR2005-002135-27-IT	06/07/2005	19 March 2012	Glargine insulin efficacy in Cystic Fibrosis patients with glucose intolerance and clinical deterioration	Glargine insulin efficacy in Cystic Fibrosis patients with glucose intolerance and clinical deterioration	Cystic Fibrosis patients with glucose intolerance MedDRA version: 6.1 Level: PT Classification code 10011762	Trade Name: *LANTUSSC 5CAR3ML100UI/ML Product Name: NA Product Code: NA Pharmaceutical Form: Solution for injection INN or Proposed INN: Insulin glargine** Concentration unit: IU international unit(s) Concentration type: equal Concentration number: 300-	ISTITUTO GIANNINA GASLINI		Not Recruiting			Female: yes Male: yes			Italy
780	NCT00217204	July 2005	19 February 2015	An Effectiveness, Safety, and Palatability Study of Pancrelipase Microtablets in Infants and Toddlers With Cystic Fibrosis and Fat Malabsorption	A Phase II, Randomized, Investigator-Blinded, Parallel-Group, Pilot Study Evaluating the Safety, Palatability and Effectiveness of Four Doses of Pancrelipase Microtablets in the Treatment of Infants and Toddlers With Cystic Fibrosis-Related Pancreatic Insufficiency and Fat Malabsorption	Cystic Fibrosis;Steatorrhea	Drug: Pancrelipase microtablets	McNeil Consumer & Specialty Pharmaceuticals, a Division of McNeil-PPC, Inc.		Not recruiting	6 Months	30 Months	Both	18	Phase 2	Belgium;Netherlands
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
781	EUCTR2004-005019-28-GB	09/05/2005	19 March 2012	Diabetes therapy to improve body mass index pulmoanry function in cystic fibrosis subjects with abnormal blood glucose	Diabetes therapy to improve body mass index pulmoanry function in cystic fibrosis subjects with abnormal blood glucose	Cystic fibrosis related diabetes mellitus	Trade Name: NovoRapid Aspartate Pharmaceutical Form: Injection* INN or Proposed INN: Novo Rapid CAS Number: 133107-64-9 Current Sponsor code: P00966 Concentration unit: IU/ml international unit(s)/millilitre Concentration type: range Trade Name: Rapeglinide Pharmaceutical Form: INN or Proposed INN: Rapeglinide CAS Number: 135062-02-1 Current Sponsor code: P00996 Concentration unit: mg milligram(s) Concentration type: range Concentration number: 0.25-1.0 Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Papworth Hospital NHS Foundation Trust		Authorised			Female: yes Male: yes	150		United Kingdom
782	NCT00112359	May 2005	19 October 2017	International Safety and Efficacy Study of Aztreonam for Inhalation Solution (AZLI) in Cystic Fibrosis Patients With P. Aeruginosa	A Phase 3, Double-Blind, Multicenter, Multinational, Randomized, Placebo-Controlled Trial Evaluating Aztreonam Lysinate for Inhalation in Cystic Fibrosis Patients With Pulmonary Pseudomonas Aeruginosa (AIR-CF1)	Cystic Fibrosis	Drug: AZLI 75 mg three times a day (TID);Drug: Placebo three times a day (TID)	Gilead Sciences		Not recruiting	6 Years	N/A	All	166	Phase 3	United States;Australia;Canada;New Zealand
783	EUCTR2004-003675-36-DK	28/04/2005	19 March 2012	A randomised, open label study to compare the efficacy and safety of a dry powder formulation of inhaled Colistimethate Sodium and nebulised TNSFI (Tobramycin nebuliser solution for inhalation, TOBI®) in cystic fibrosis patients with pseudomonas aeruginosa lung infection	A randomised, open label study to compare the efficacy and safety of a dry powder formulation of inhaled Colistimethate Sodium and nebulised TNSFI (Tobramycin nebuliser solution for inhalation, TOBI®) in cystic fibrosis patients with pseudomonas aeruginosa lung infection	Pseudomonas aeruginosa (PA) infection in patients with cystic fibrosis.	Product Name: Colobreathe Pharmaceutical Form: Inhalation powder, hard capsule INN or Proposed INN: Colistimethate sodium CAS Number: 8068-28-8 Other descriptive name: Colomycin Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 125- Product Name: TOBI® Pharmaceutical Form: Nebuliser solution INN or Proposed INN: Tobramycin Other descriptive name: TOBI® Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 300-	Forest Laboratories UK Ltd		Not Recruiting			Female: yes Male: yes	360		Belgium;Denmark
784	EUCTR2005-000313-35-GB	13/04/2005	3 April 2012	A Phase I/II Study to Investigate the Efficacy and Safety of AER 002 in Cystic Fibrosis Given at 3 mg, 10 mg, and 30 mg Doses in Single then Multiple Ascending Doses and to Determine Efficacy of the Highest Tolerable Dose in a 4-Week Proof of Concept Study	A Phase I/II Study to Investigate the Efficacy and Safety of AER 002 in Cystic Fibrosis Given at 3 mg, 10 mg, and 30 mg Doses in Single then Multiple Ascending Doses and to Determine Efficacy of the Highest Tolerable Dose in a 4-Week Proof of Concept Study	Cystic Fibrosis MedDRA version: 7.1 Level: LLT Classification code 10011762	Product Name: Recombinant truncated SPINT2 protease inhibitor Product Code: AER002 Pharmaceutical Form: Nebuliser solution Pharmaceutical form of the placebo: Nebuliser solution Route of administration of the placebo: Inhalation use	Aerovance Inc		Not Recruiting			Female: yes Male: yes	106	Phase 1/2	United Kingdom
785	EUCTR2004-004334-13-IE	08/04/2005	19 March 2012	A Phase 2 Multicenter, Randomized, Double-Blind, Placebo-Controlled Study of Talniflumate in Cystic Fibrosis Subjects	A Phase 2 Multicenter, Randomized, Double-Blind, Placebo-Controlled Study of Talniflumate in Cystic Fibrosis Subjects	Cystic Fibrosis	Trade Name: Somalgen Product Name: Talniflumate Product Code: MSI-1995 Pharmaceutical Form: Tablet INN or Proposed INN: Talniflumate CAS Number: 66898-62-2 Current Sponsor code: MSI-1995 Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 370- Pharmaceutical form of the placebo: Tablet Route of administration of the placebo: Oral use	Genaera Corporation		Authorised			Female: yes Male: yes	220	Phase 2	Ireland
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
786	NCT00198289	April 2005	19 February 2015	Aurexis® in Cystic Fibrosis Subjects Chronically Colonized With Staphylococcus Aureus in Their Lungs	A Phase IIa Dose Escalation Study to Assess Safety and Pharmacokinetics of Aurexis® in Cystic Fibrosis Subjects Chronically Colonized With Staphylococcus Aureus in Their Lungs	Staphylococcus Aureus	Drug: Aurexis® (tefibazumab)	Bristol-Myers Squibb		Not recruiting	7 Years	N/A	Both	30	Phase 2	United States
787	NCT00219882	April 2005	19 February 2015	Safety Study of Orally Administered Curcuminoids in Adult Subjects With Cystic Fibrosis	A Phase I Safety and Dose Finding Study of Orally Administered Curcuminoids in Adult Subjects With Cystic Fibrosis Who Are Homozygous for Delta F508 Cystic Fibrosis Transmembrane Conductance Regulator (?F508 CFTR) Mutation	Cystic Fibrosis	Drug: standardized turmeric root extract	Ramsey, Bonnie, MD	Seer Pharmaceuticals;CF Therapeutics Development Network Coordinating Center;Cystic Fibrosis Foundation Therapeutics	Not recruiting	18 Years	40 Years	Both	11	Phase 1	United States
788	EUCTR2004-002341-12-DE	14/02/2005	19 March 2012	Multicenter, 8-week, Randomised, Double-Blind, Placebo Controlled Study of Two Doses of Depelestat in Cystic Fibrosis Patients	Multicenter, 8-week, Randomised, Double-Blind, Placebo Controlled Study of Two Doses of Depelestat in Cystic Fibrosis Patients	Cystic fibrosis MedDRA version: 7.0 Level: PT Classification code 10011762	Product Name: Depelestat Product Code: EPI-hNE4 DX-890 Pharmaceutical Form: Powder for nebuliser solution INN or Proposed INN: Depelestat Current Sponsor code: EPIhNE4 DX-890 Other descriptive name: Engineered Protein Inhibitor of human Neutrophil Elastase Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 5.65- INN or Proposed INN: Depelestat Current Sponsor code: EPI-hNE4 DX-890 Other descriptive name: Engineerd Protein Inhibitor of human Neutrophil Elastase Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 11.3- Pharmaceutical form of the placebo: Powder for nebuliser solution Route of administration of the placebo: Inhalation use	Debiopharm SA		Not Recruiting			Female: yes Male: yes	90		United Kingdom;Germany;Belgium;Italy
789	NCT00104520	February 2005	19 October 2017	Safety and Efficacy Study of Aztreonam for Inhalation Solution (AZLI) in Cystic Fibrosis Patients With P. Aeruginosa	A Phase 3, Double-Blind, Multicenter, Randomized, Placebo-Controlled Trial With Aztreonam Lysinate for Inhalation in Cystic Fibrosis Patients With Pulmonary P. Aeruginosa Requiring Frequent Antibiotics (AIR-CF2)	Cystic Fibrosis	Drug: AZLI 75 mg two times a day (BID)/three times a day (TID);Drug: Placebo two times a day (BID)/three times a day (TID)	Gilead Sciences		Not recruiting	6 Years	N/A	All	211	Phase 3	United States
790	NCT00103714	January 2005	2 November 2015	Study of Denufosol Tetrasodium in Patients With Cystic Fibrosis Lung Disease	A Multi-Center, Double-Blind, Randomized, Placebo-Controlled, 28-Day Study of Denufosol Tetrasodium (INS37217) Inhalation Solution in Patients With Cystic Fibrosis Lung Disease	Cystic Fibrosis	Drug: denufosol tetrasodium (INS37217)	Merck Sharp & Dohme Corp.		Not recruiting	8 Years	50 Years	Both	72	Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
791	NCT00105183	January 2005	19 October 2017	EZ-2053 in the Prophylaxis of Acute Pulmonary Allograft Rejection	A Double-Blind, Placebo-Controlled, Multicenter, Dose-Ranging Study of an Anti-human-T-lymphocyte Immune Globulin (EZ-2053) in the Prophylaxis of Acute Pulmonary Allograft Rejection in Adult Recipients of Primary Pulmonary Allograft(s)	Chronic Obstructive Pulmonary;Idiopathic Pulmonary Fibrosis;Cystic Fibrosis;Bronchiectasis;Pulmonary Vascular Disease	Biological: Placebo;Biological: EZ-2053;Biological: EZ-2053 5mg/kg	Neovii Biotech		Not recruiting	18 Years	N/A	All	223	Phase 3	United States;Australia;Austria;Canada
792	NCT00179998	January 2005	19 February 2015	Effectiveness of Pulmozyme in Infants With Cystic Fibrosis	Efficacy of Pulmozyme in Infants and Young Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Recombinant Human DNase (Pulmozyme)	Nationwide Children's Hospital	Genentech, Inc.	Not recruiting	1 Month	30 Months	Both	24	Phase 2	United States
793	EUCTR2005-001438-34-BE		2 October 2017	A Phase II, Randomized, Investigator-Blinded, Parallel-Group, Pilot Study Evaluating the Safety, Palatability and Efficacy of Four Doses of Pancrelipase Microtablets in the Treatment of Infants and Toddlers with Cystic Fibrosis-Related Pancreatic Insufficiency and Fat Malabsorption	A Phase II, Randomized, Investigator-Blinded, Parallel-Group, Pilot Study Evaluating the Safety, Palatability and Efficacy of Four Doses of Pancrelipase Microtablets in the Treatment of Infants and Toddlers with Cystic Fibrosis-Related Pancreatic Insufficiency and Fat Malabsorption	Cystic Fibrosis-Related Pancreatic Insufficiency and Fat Malabsorption	Trade Name: Panzytrat® ok Product Name: Pancrelipase Microtablets Product Code: N/A Pharmaceutical Form: Tablet INN or Proposed INN: Pancreatin CAS Number: 8049-47-6 Concentration unit: IU international unit(s) Concentration type: not less then Concentration number: 450-	McNeil Consumer and Specialty Pharmaceuticals		Not Available			Female: yes Male: yes	20	Phase 2	Belgium
794	EUCTR2004-001888-21-GB	03/12/2004	21 August 2012	A cross-over comparative study of inhaled mannitol, alone and in combination with daily rhDNase, in children with cystic fibrosis - Inhaled Mannitol in Cystic Fibrosis	A cross-over comparative study of inhaled mannitol, alone and in combination with daily rhDNase, in children with cystic fibrosis - Inhaled Mannitol in Cystic Fibrosis	Cystic fibrosis (CF)	Product Name: DP Mannitol Pharmaceutical Form: Inhalation powder INN or Proposed INN: Mannitol Concentration unit: mg milligram(s) Concentration type: equal Concentration number: 40- Trade Name: Pulmozyme (dornase alfa) Product Name: Pulmozyme (dornase alfa) Pharmaceutical Form: Nebuliser liquid* INN or Proposed INN: Dornase alfa Other descriptive name: rhDNAse Concentration unit: mg/ml milligram(s)/millilitre Concentration type: equal Concentration number: 1.0-	Pharmaxis Ltd		Authorised			Female: yes Male: yes	42		United Kingdom
795	NCT00377741	December 2004	19 October 2017	A Relative Bioavailability Study of Valcyte (Valganciclovir) in Lung Transplant Recipients With or Without Cystic Fibrosis.	Relative Bioavailability Study of Ganciclovir From the Pro-drug, Valganciclovir, in Lung Transplant Recipients With or Without Cystic Fibrosis	Cytomegalovirus Infections	Drug: valganciclovir [Valcyte]	Hoffmann-La Roche		Not recruiting	14 Years	N/A	All	31	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
796	NCT00097773	September 2004	19 October 2017	Comparison of Two Treatment Regimens to Reduce PA Infection in Children With Cystic Fibrosis	Effectiveness and Safety of Intermittent Antimicrobial Therapy for the Treatment of New Onset Pseudomonas Aeruginosa Airway Infection in Young Patients With Cystic Fibrosis	Cystic Fibrosis;Pulmonary Disease, Chronic Obstructive	Drug: Tobramycin solution for inhalation (TOBI);Drug: Oral placebo;Drug: Oral ciprofloxacin	Seattle Children's Hospital	National Heart, Lung, and Blood Institute (NHLBI);Cystic Fibrosis Foundation Therapeutics;CF Therapeutics Development Network Coordinating Center	Not recruiting	1 Year	12 Years	All	304	Phase 2	United States
797	NCT00219895	August 2004	19 February 2015	Assessment of Inflammatory Mediators (AIM)	Assessment of Induced Sputum as a Tool to Evaluate Anti-Inflammatory Agents in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Ibuprofen	Ramsey, Bonnie, MD	Cystic Fibrosis Foundation Therapeutics	Not recruiting	10 Years	N/A	Both	120	N/A	United States
798	NCT00255242	July 2004	19 February 2015	Effect of Simvastatin on CF Airway Inflammation	Effect of Simvastatin on CF Airway Inflammation	Cystic Fibrosis	Drug: Simvastatin treatment for 28 days	Akron Children's Hospital	Cystic Fibrosis Foundation Therapeutics	Not recruiting	10 Years	N/A	Both	40	Phase 1	United States
799	NCT00570349	July 2004	19 October 2017	Safety and Tolerability of Inhaled Nitric Oxide in Patients With Cystic Fibrosis	Safety and Tolerability of Inhaled Nitric Oxide in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Nitric Oxide for Inhalation;Drug: Nitrogen	Mallinckrodt		Not recruiting	12 Years	N/A	All	18	Phase 1/Phase 2	United States
800	NCT00823238	July 2004	19 February 2015	Comparison of Antibiotics for Pseudomonas in Early CF	Comparison of Antibiotics for Pseudomonas in Early CF	Cystic Fibrosis	Drug: ceftazidime and tobramycin;Drug: inhaled tobramycin	University of North Carolina, Chapel Hill	Cystic Fibrosis Foundation Therapeutics	Not recruiting	3 Months	16 Years	Both	21	Phase 1	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
801	NCT00095732	June 2004	19 February 2015	Oral TheraCLEC™ - Total in Cystic Fibrosis Subjects With Exocrine Pancreatic Insufficiency	A Phase 2, Randomized, Double Blind, Parallel Dose Ranging Study of Oral TheraCLEC™ - Total in Cystic Fibrosis Subjects With Exocrine Pancreatic Insufficiency	Cystic Fibrosis;Pancreatic Insufficiency	Drug: Liprotamase;Drug: Placebo	Anthera Pharmaceuticals	Cystic Fibrosis Foundation Therapeutics	Not recruiting	7 Years	N/A	Both	129	Phase 2	United States
802	NCT02010411	June 2004	19 February 2015	Alpha1 Antitrypsin Aerosol Therapy in Cystic Fibrosis	Effects of Prolastin Aerosol Therapy on Bacterial Density in the Airways of Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Prolastin (drug)	Université de Sherbrooke		Not recruiting	14 Years	N/A	Both	17	Phase 2	Canada
803	NCT00104494	May 2004	10 August 2015	Zinc Homeostasis and Kinetics in Children With Cystic Fibrosis (CF)	Zinc Homeostasis and Kinetics in Children With CF	Cystic Fibrosis	Drug: Zinc acetate (20mg/d)	Cystic Fibrosis Foundation Therapeutics		Not recruiting	8 Years	14 Years	Both	30	N/A	United States
804	NCT00730509	April 2004	19 February 2015	Effects of 5-Methyltetrahydrofolate and Vitamin B12 Supplemetation on Red Cell Membrane in Children With Cystic Fibrosis	Preliminary Evidences of Active Form of Folic Acid and Vitamin B12 Supplementation to Ameliorate Cell Membrane in Children With Cystic Fibrosis	Cystic Fibrosis	Dietary Supplement: 5-methyltetrahydrofolate and vitamin B12	Universita di Verona		Not recruiting	3 Years	8 Years	Both	31	N/A	Italy
805	NCT00153634	March 2004	19 February 2015	Standard vs. Biofilm Susceptibility Testing in Cystic Fibrosis (CF)	Standard vs. Biofilm Susceptibility Testing in CF	Cystic Fibrosis;Chronic Bronchitis	Drug: IV amikacin;Drug: PO azithromycin;Drug: IV ceftazidime;Drug: PO ciprofloxacin;Drug: IV meropenem;Drug: IV piperacillin-tazobactam;Drug: IV ticarcillin-clavulanate;Drug: IV tobramycin	Seattle Children's Hospital	Cystic Fibrosis Foundation Therapeutics	Not recruiting	14 Years	N/A	Both	75	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
806	NCT00455130	March 2004	19 February 2015	A Phase 2 Study to Determine the Safety and Efficacy of Inhaled Dry Powder Mannitol in Cystic Fibrosis		Cystic Fibrosis	Drug: Inhaled mannitol	Pharmaxis		Not recruiting	8 Years	N/A	Both		Phase 2	Australia;New Zealand
807	NCT00163852	February 2004	19 February 2015	Treatment of Metabolic Alkalosis in Acute Exacerbations of Cystic Fibrosis	Salt Replacement for Metabolic Alkalosis in Acute Exacerbations of Cystic Fibrosis	Cystic Fibrosis	Drug: Normal saline IV, salt tablets	Bayside Health	National Health and Medical Research Council, Australia;Monash University;Cystic Fibrosis Australia	Recruiting	18 Years	75 Years	Both	40	Phase 2/Phase 3	Australia
808	NCT00728715	January 2004	19 February 2015	Efficacy of Budesonide-Formoterol in Bronchiectasis	Clinical Efficacy and Safety of Budesonide and Formoterol in the Management of Non-Cystic Fibrosis Bronchiectasis	Bronchiectasis	Drug: budesonide-formoterol single inhaler;Drug: High dose of budesonide;Drug: A;Drug: B	Hospital General de Requena	Esteve Labs (Grant)	Not recruiting	18 Years	80 Years	Both	40	N/A	Spain
809	NCT00157690	December 2003	19 February 2015	Study of Alendronate to Prevent and Treat Osteoporosis in Cystic Fibrosis Patients	A Multicentre, Double-Blind, Randomized Placebo-Controlled Study of 70mg Alendronate Once Weekly for the Prevention and Treatment of Osteoporosis in Canadian Adult Cystic Fibrosis Patients	Cystic Fibrosis;Osteoporosis;Bone Diseases, Metabolic	Drug: Alendronate;Drug: Placebo	McMaster University	Centre hospitalier de l'Université de Montréal (CHUM);London Health Sciences Centre;University of Calgary;McGill University;Laval University;Merck Frosst Canada Ltd.	Not recruiting	18 Years	N/A	Both	56	Phase 4	Canada
810	NCT00486837	December 2003	19 October 2017	Deposition of Inhaled Prolastin in Cystic Fibrosis Patients	Multicenter, Randomized, Parallel Group Study to Investigate the Optimal Deposition Site for Inhaled Prolastin® in Patients With Cystic Fibrosis (CF)	Cystic Fibrosis	Drug: Alpha1-Proteinase Inhibitor (Human)	Grifols Therapeutics Inc.		Not recruiting	8 Years	N/A	All	72	Phase 2
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
811	NCT00391976	November 2003	19 October 2017	Efficacy and Safety of 28 or 56 Day Treatment for Pseudomonas Aeruginosa in Children With Cystic Fibrosis	The Microbiologic Efficacy and Safety of Two Treatment Regimens of Inhaled Tobramycin Nebuliser Solution (TNS) for the Treatment of Early Onset Pseudomonas Aeruginosa Lower Respiratory Tract Infection in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: Tobramycin solution for inhalation 300 mg	Novartis		Not recruiting	6 Months	N/A	All	123	Phase 3
812	NCT00633191	November 2003	12 September 2016	Anti-pseudomonas IgY to Prevent Infections in Cystic Fibrosis	Post Marketing Study of Anti-pseudomonas IgY in Prevention of Recurrence of Pseudomonas Aeruginosa Infections Infections in Cystic Fibrosis (CF) Patients	Cystic Fibrosis;Infection;Pseudomonas Aeruginosa	Drug: Anti-pseudomonas IgY gargle	Immunsystem AB		Not recruiting	N/A	N/A	Both	14	Phase 1/Phase 2	Sweden
813	NCT00499720	October 2003	19 February 2015	Aztreonam Lysine for Inhalation in Patients With Cystic Fibrosis and Pseudomonas Aeruginosa Airway Infection	Expanded Access Program for Aztreonam Lysine for Inhalation in Patients With Cystic Fibrosis and Pseudomonas Aeruginosa Airway Infection Who Have Limited Treatment Options and Are at Risk for Disease Progression	Cystic Fibrosis;Pseudomonas Aeruginosa Airway Infection	Drug: Aztreonam Lysine for Inhalation	Gilead Sciences		Not recruiting	6 Years	N/A	Both		N/A	United States;Puerto Rico
814	NCT00079742	September 2003	19 February 2015	A Study to Evaluate Nutropin AQ for the Treatment of Growth Restriction in Children With Cystic Fibrosis	A Phase II, Multicenter, Randomized, Controlled, Open-Label Study of the Safety and Efficacy of Nutropin AQ [Somatropin (DNA Origin) Injection] for the Treatment of Growth Restriction in Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Nutropin AQ [somatropin (DNA origin) injection]	Genentech, Inc.		Not recruiting	5 Years	13 Years	Both	68	Phase 2	United States
815	NCT00287443	July 2003	19 February 2015	Metabolic Abnormalities in Hispanic Children With Cystic Fibrosis		Cystic Fibrosis	Procedure: Oral Glucose tolerance test;Procedure: Whole body protein turnover;Procedure: IV glucose tolerance test;Procedure: Indirect Calorimetry;Procedure: Dual Energy X-ray Absorptiometry (DEXA);Procedure: Growth Hormone Stimulation Test	University of Texas Southwestern Medical Center	Genentech, Inc.	Not recruiting	7 Years	17 Years	Both	48	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
816	NCT01055847	June 2003	19 February 2015	Aztreonam for Inhalation (AI) in Patients With Cystic Fibrosis & P. Aeruginosa Infection	A Blinded, Multicenter, Randomized, Placebo-Controlled Trial With Aztreonam for Inhalation (AI) in Cystic Fibrosis Patient With Lung Disease Due to P. Aeruginosa Infection	Cystic Fibrosis;CF;Lung Infection;Pseudomonas Aeruginosa	Drug: Aztreonam for Inhalation (AI);Drug: Placebo	Gilead Sciences	Conducted under Salus Pharma, Inc.	Not recruiting	13 Years	N/A	Both	105	Phase 2	United States
817	NCT00060801	May 2003	19 February 2015	Efficacy and Safety of 24 Weeks of Oral Treatment With BIIL 284 BS in Adult and Pediatric Patients	A Randomized, Double-blind, Placebo-controlled Study to Investigate the Efficacy and Safety of 24 Weeks of Oral Treatment With BIIL 284 BS in Adult (75 mg, 150 mg) and Pediatric (75 mg) Cystic Fibrosis Patients	Cystic Fibrosis	Drug: BIIL 283 BS (Amelubent)	Boehringer Ingelheim		Not recruiting	6 Years	N/A	Both	420	Phase 2	United States
818	NCT00056147	April 2003	19 February 2015	Study of INS37217 Inhalation Solution in Mild to Moderate Cystic Fibrosis Lung Disease	Multi-Center, Double-Blind, Randomized, Placebo-Controlled, 28-Day Study of INS37217 Inhalation Solution in Subjects With Mild to Moderate Cystic Fibrosis Lung Disease	Cystic Fibrosis	Drug: denufosol tetrasodium (INS37217)	Merck Sharp & Dohme Corp.	Cystic Fibrosis Foundation Therapeutics	Not recruiting	8 Years	50 Years	Both	90	Phase 2	United States
819	NCT00222521	April 2003	19 February 2015	Insulin Glargine Vs Standard Insulin Therapy	Comparison of Insulin Glargine Vs Standard Insulin Therapy in CFRD Without Fasting Hyperglycemia	Cystic Fibrosis Related Diabetes	Drug: Glargine insulin	University of Minnesota - Clinical and Translational Science Institute	Sanofi;Moran, Antoinette, M.D.	Not recruiting	12 Years	N/A	Both	20	Phase 3	United States
820	NCT00157183	March 2003	19 February 2015	Non-invasive Ventilation and Oxygen Therapy in Cystic Fibrosis Patients With Nocturnal Oxygen Desaturation	Non-invasive Ventilation and Oxygen Therapy in Cystic Fibrosis Patients With Nocturnal Oxygen Desaturation	Cystic Fibrosis	Device: Nocturnal oxygen , nocturnal bi-level positive pressure ventilation	Bayside Health	National Health and Medical Research Council, Australia;Monash University;Cystic Fibrosis Australia	Not recruiting	18 Years	75 Years	Both	59	Phase 1/Phase 2	Australia
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
821	NCT00530244	March 2003	11 June 2018	Use of Formula Fortified With DHA in Infants With Cystic Fibrosis	The Effect of Formula Fortified With Docosahexaenoic Acid (DHA) on Infants With Cystic Fibrosis (CF)	Cystic Fibrosis	Dietary Supplement: Docosahexaenoic acid (DHA);Dietary Supplement: Standard formula (Enfamil)	University of Massachusetts, Worcester	Cystic Fibrosis Foundation Therapeutics	Not recruiting	N/A	56 Days	All	76	N/A	United States
822	NCT00590538	February 2003	19 October 2017	Phenylbutyrate/Genistein Duotherapy in Delta F508-Heterozygotes (for Cystic Fibrosis)	A Pilot Trial of Phenylbutyrate/Genistein Duotherapy in Delta F508-Heterozygous Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Sodium 4-Phenylbutyrate;Drug: Genistein (Unconjugated Isoflavones 100);Drug: Placebo	Children's Hospital of Philadelphia	Cystic Fibrosis Foundation Therapeutics	Not recruiting	18 Years	N/A	All	9	Phase 1/Phase 2	United States
823	NCT00376428	January 2003	9 March 2015	Interest of Gentamicin-induced Readthrough in Cystic Fibrosis Patients	Application of Functional Electrophysiological Tests to Evaluate Pharmacological Treatments in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Gentamicin	Assistance Publique - Hôpitaux de Paris		Not recruiting	N/A	N/A	Both	20	Phase 2	France
824	NCT01812551	October 2002	19 February 2015	Treatment of Low Bone Density in Cystic Fibrosis.	Osteoporosis in Cystic Fibrosis: Study of Bone Mass and Bone Metabolism, and Prospective Randomized Therapeutic Trial.	Osteoporosis;Cystic Fibrosis	Drug: Alendronate;Drug: Placebo	Istituto Auxologico Italiano	Fondazione Telethon	Not recruiting	5 Years	30 Years	Both	171	Phase 3	Holy See (Vatican City State);Italy
825	NCT00043342	April 2002	19 February 2015	Study of Interferon Gamma-1b by Injection for the Treatment of Patients With Cystic Fibrosis	A Phase I/II Study of Interferon Gamma-1b by Subcutaneous Injection for the Treatment of Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: interferon gamma-1b	InterMune		Not recruiting	6 Years	N/A	Both	51	Phase 1/Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
826	NCT02269189	April 2002	19 February 2015	Safety, Tolerability and Pharmacokinetics of BIIL 284 BS in Adult and Pediatric Cystic Fibrosis (CF) Patients	A Randomized, Double-blind Within Dose, Placebo-controlled Study to Investigate the Safety, Tolerability and Pharmacokinetics of Repeated Oral Doses (15-day Dosing) of BIIL 284 BS in Adult (300 mg) and Pediatric (150 mg) Cystic Fibrosis Patients	Cystic Fibrosis	Drug: BIIL 284 BS, high dose;Drug: BIIL 284 BS, low dose;Drug: Placebo	Boehringer Ingelheim		Not recruiting	6 Years	N/A	Both	36	Phase 1
827	NCT00177645	March 2002	23 February 2016	Inhaled Bicarbonate Therapy in Cystic Fibrosis	Inhaled Bicarbonate Therapy in Cystic Fibrosis	Cystic Fibrosis	Procedure: sodium bicarbonate	University of Pittsburgh	Cystic Fibrosis Foundation Therapeutics;Children's Hospital of Pittsburgh	Not recruiting	12 Years	N/A	Both	16	Phase 1	United States
828	NCT00333385	October 2001	19 February 2015	Continuous Versus Short Infusions of Ceftazidime in Cystic Fibrosis		Cystic Fibrosis;Pseudomonas Aeruginosa;Pulmonary Exacerbation	Drug: ceftazidime	Association Nationale pour le Traitement à Domicile de l'Insuffisance Respiratoire	Vaincre la Mucoviscidose;GlaxoSmithKline;Baxter Healthcare Corporation;Roche Pharma AG	Not recruiting	8 Years	N/A	Both	120	Phase 4	France
829	NCT02265679	October 2001	19 February 2015	Safety, Tolerability and Pharmacokinetics of Increasing Doses of BIIL 284 BS in Adult and Pediatric Cystic Fibrosis (CF) Patients	A Randomized, Double-blind Within Dose, Placebo-controlled Study to Investigate the Safety, Tolerability and Pharmacokinetics of Increasing Single Oral Doses of BIIL 284 BS in Adult and Pediatric Cystic Fibrosis Patients	Cystic Fibrosis	Drug: BIIL 284 BS, low dose, pediatric patients;Drug: BIIL 284 BS, medium dose, pediatric patients;Drug: BIIL 284 BS, high dose, pediatric patients;Drug: BIIL 284 BS, low dose, adult patients;Drug: BIIL 284 BS, medium dose, adult patients;Drug: BIIL 284 BS, high dose, adult patients;Drug: Placebo	Boehringer Ingelheim		Not recruiting	6 Years	N/A	Both	45	Phase 1
830	NCT00016744	September 2001	19 February 2015	Phenylbutyrate/Genistein Duotherapy in Delta F508-Homozygous(for Cystic Fibrosis)	A Pilot Trial of Phenylbutyrate/Genistein Duotherapy in Delta F508-Homozygous Cystic Fibrosis Patients	Cystic Fibrosis	Drug: Sodium 4-Phenylbutyrate (4PBA);Drug: Unconjugated Isoflavones 100 (PTI G-4660, 87% Genistein);Drug: Placebo	Children's Hospital of Philadelphia	Cystic Fibrosis Foundation Therapeutics;National Center for Research Resources (NCRR)	Not recruiting	18 Years	N/A	Both	12	Phase 1/Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
831	NCT00662714	September 2001	19 February 2015	Early Diagnosis of Diabetes Mellitus in Patients With Cystic Fibrosis	Open Randomised Prospective Comparative Multi-centre Intervention Study of Patients With Cystic Fibrosis and Early Diagnosed Diabetes Mellitus	Cystic Fibrosis;Diabetes Mellitus	Drug: Repaglinide;Drug: short-acting Insulin (Actrapid)	Mukoviszidose Institut gGmbH	Novo Nordisk A/S;Mucoviscidose-ABCF2;Assistance Publique - Hôpitaux de Paris	Not recruiting	10 Years	N/A	Both	73	Phase 3	Austria;France;Germany;Italy
832	NCT01400750	August 2001	19 February 2015	Comparison of 2 Treatment Regimens for Eradication of P Aeruginosa Infection in Children With Cystic Fibrosis	Prospective Randomized Trial Comparing Oral Ciproxin Plus Inhaled Colistin With Tobramycin for Inhalation for Eradication of P Aeruginosa Infection in Children With Cystic Fibrosis.	Cystic Fibrosis	Drug: oral ciprofloxacin plus inhaled colistin;Drug: TOBI	Universitaire Ziekenhuizen Leuven		Not recruiting	N/A	18 Years	Both	61	Phase 4	Belgium
833	NCT00274391	July 2001	19 February 2015	Efficacy of Amiloride and Hypertonic Saline in Cystic Fibrosis	Efficacy of Amiloride and Hypertonic Saline in Cystic Fibrosis	Cystic Fibrosis	Drug: 7% NaCl;Drug: Amiloride HCl	University of North Carolina	Cystic Fibrosis Foundation Therapeutics	Not recruiting	14 Years	N/A	Both	24	Phase 2	United States
834	NCT00034515	June 2001	2 March 2015	A Study in Adult and Pediatric Patients With Cystic Fibrosis	Ph 1b/2a Study to Assess Levels of INS37217 and Placebo Treatment With Maximum Tolerated Dose Administered by Inhalation Via the Pari LC Star Nebulizer in Adult and Pediatric CF Patients	Cystic Fibrosis	Drug: denufosol tetrasodium (INS37217) Inhalation Solution	Merck Sharp & Dohme Corp.		Not recruiting	5 Years	N/A	Both	63	Phase 1/Phase 2	United States
835	NCT00072904	June 2001	19 February 2015	Diabetes Therapy to Improve BMI and Lung Function in CF	Diabetes Therapy to Improve BMI and Lung Function in CF	Cystic Fibrosis;Diabetes Mellitus	Drug: Insulin Asparte;Drug: Repaglinide	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	Cystic Fibrosis Foundation Therapeutics;Novo Nordisk A/S	Not recruiting	16 Years	N/A	Both	108	Phase 3	United States;Canada
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
836	NCT00220259	May 2001	19 February 2015	Cystic Fibrosis Withdrawal of Inhaled Steroids Evaluation Study (CF WISE Study)	Cystic Fibrosis Withdrawal of Inhaled Steroids Evaluation Study (CF WISE Study)	Cystic Fibrosis	Drug: Fluticasone or placebo	Royal Brompton & Harefield NHS Foundation Trust	Cystic Fibrosis Trust;GlaxoSmithKline	Not recruiting	6 Years	N/A	Both	240	N/A	United Kingdom
837	NCT01882400	May 2001	18 January 2018	Assessment of Response to Treatment of Osteoporosis With Oral Bisphosphonates in Patients With Muscular Dystrophy	Évaluation Multidimensionnelle de la réponse au Traitement de l'ostéoporose spontanée et Induite Par Les corticostéroïdes à l'Aide d'un Bisphosphonate à Administration Orale Chez Des Malades Porteurs d'Une Dystrophie Musculaire sévère.	Osteoporosis;Muscular Dystrophy;Cystic Fibrosis	Drug: Bisphosphonate treatment	Gilles Boire	Procter and Gamble	Not recruiting	5 Years	18 Years	All	11	Phase 4	Canada
838	NCT00014768	February 2001	19 February 2015	Study of Metabolic Effects of Pregnancy in Women With Cystic Fibrosis		Cystic Fibrosis	Drug: glucose;Drug: insulin;Drug: leucine	National Center for Research Resources (NCRR)	University of Utah	Not recruiting	N/A	N/A	Female	36	N/A	United States
839	NCT00016445	February 2001	19 February 2015	Phase II Study of Growth Hormone in Children With Cystic Fibrosis		Cystic Fibrosis	Drug: growth hormone	University of Utah		Not recruiting	5 Years	12 Years	Both	40	Phase 2	United States
840	NCT00043316	February 2001	19 February 2015	Interferon Gamma-1b by Inhalation for the Treatment of Patients With Cystic Fibrosis	A Phase I/II Study of Interferon Gamma-1b by Inhalation for the Treatment of Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: interferon gamma-1b	InterMune		Not recruiting	12 Years	N/A	Both	66	Phase 1/Phase 2	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
841	NCT00164021	February 2001	25 January 2016	The Prevalence and Significance of Gastro-oesophageal Reflux in Cystic Fibrosis Before and After Lung Transplantation	The Prevalence and Significance of Gastro-oesophageal Reflux in Adults With Cystic Fibrosis Before and After Lung Transplantation, Together With the Effects of Physiotherapy Airway Clearance Techniques on Gastro-oesophageal Function	Cystic Fibrosis	Procedure: Physiotherapy, exercise, percutaneous gastrostomy feeds;Drug: Anti-reflux pharmacotherapy	Bayside Health	The Alfred;Monash University	Recruiting	16 Years	70 Years	Both	180	N/A	Australia
842	NCT00205634	December 2000	20 August 2018	Macrolide Antibiotic Therapy for Patients With Cystic Fibrosis	Macrolide Antibiotic Therapy for Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Biaxin ( clarithromycin)	Wake Forest University	Abbott	Not recruiting	6 Years	50 Years	All	50	Phase 2	United States
843	NCT00271310	September 2000	19 February 2015	The Effects of Long Term Inhalation of Hypertonic Saline in Subjects With Cystic Fibrosis	The Effects of Long Term Inhalation of Hypertonic Saline in Subjects With Cystic Fibrosis	Cystic Fibrosis	Drug: hypertonic saline	Royal Prince Alfred Hospital, Sydney, Australia	Cystic Fibrosis Foundation Therapeutics;National Health and Medical Research Council, Australia;Cystic Fibrosis Trust	Not recruiting	6 Years	N/A	Both	164	Phase 3
844	NCT00005110	April 2000	19 February 2015	A Multi-Centered Study of the Long-Term Effect of Salmeterol and Albuterol in Cystic Fibrosis		Cystic Fibrosis	Drug: Salmeterol;Drug: Albuterol	National Center for Research Resources (NCRR)		Not recruiting	5 Years	45 Years	Both		N/A	United States
845	NCT00005112	April 2000	19 February 2015	Growth Hormone Use in Cystic Fibrosis - a Multicenter Study		Cystic Fibrosis	Drug: Human recombinant growth hormone	National Center for Research Resources (NCRR)	Genentech, Inc.	Not recruiting	5 Years	12 Years	Both		Phase 3	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
846	NCT00006280	February 2000	19 February 2015	A Study of the Safety and Efficacy of Tobramycin for Inhalation in Young Children With Cystic Fibrosis	A Phase II Multicenter Randomized Trial of Tobramycin for Inhalation in Young Children With Cystic Fibrosis	Cystic Fibrosis	Drug: Tobramycin for Inhalation	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	National Center for Research Resources (NCRR)	Not recruiting	6 Months	5 Years	Both	98	Phase 2	United States
847	NCT00256555	February 2000	21 January 2019	Growth Hormone Treatment Study in Children With Cystic Fibrosis	GROWTH HORMONE USE IN CYSTIC FIBROSIS - A MULTICENTER STUDY	Cystic Fibrosis	Drug: Nutropin AQ	University of Texas Southwestern Medical Center	Genentech, Inc.	Not recruiting	5 Years	12 Years	All	0	Phase 2/Phase 3
848	NCT00004533	August 1999	16 December 2017	Phase I Randomized Study of Adeno-Associated Virus-CFTR Vector in Patients With Cystic Fibrosis	Phase I Randomized Study of Adeno-Associated Virus-CFTR Vector in Patients With Cystic Fibrosis	Cystic Fibrosis	Drug: Adeno-associated virus-CFTR vector	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)	University of Florida	Not recruiting	15 Years	N/A	All	19	Phase 1
849	NCT00006063	July 1999	19 February 2015	Randomized Study of Pancrelipase With Bicarbonate (PANCRECARB) Capsules in Reducing Steatorrhea in Patients With Cystic Fibrosis		Cystic Fibrosis	Drug: pancrelipase with bicarbonate	National Center for Research Resources (NCRR)	Indiana University School of Medicine	Not recruiting	N/A	N/A	Both	24	N/A
850	NCT00004489	October 1998	7 April 2015	Randomized Study of Alendronate in Adult Patients With Cystic Fibrosis Related Osteoporosis		Osteoporosis;Cystic Fibrosis	Drug: alendronate sodium;Drug: calcium carbonate;Drug: cholecalciferol	University of North Carolina		Not recruiting	18 Years	45 Years	Both	60	N/A	United States
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
851	NCT00004441	September 1997	7 April 2015	Study of Tauroursodeoxycholic Acid for Hepatobiliary Disease in Cystic Fibrosis		Cystic Fibrosis	Drug: tauroursodeoxycholic acid;Drug: ursodiol	Children's Hospital Medical Center, Cincinnati		Not recruiting	N/A	N/A	Both	39	N/A	United States;Italy
852	NCT00004705	September 1996	7 April 2015	Study of Uridine Triphosphate (UTP) as an Aerosol Spray for Cystic Fibrosis		Cystic Fibrosis	Drug: amiloride;Drug: uridine	FDA Office of Orphan Products Development	University of North Carolina	Not recruiting	4 Years	N/A	Both		N/A
853	NCT00004440	August 1996	7 April 2015	Study of Ibuprofen to Preserve Lung Function in Patients With Cystic Fibrosis		Cystic Fibrosis	Drug: ibuprofen	FDA Office of Orphan Products Development	Case Western Reserve University	Not recruiting	5 Years	N/A	Both	75	N/A
854	NCT00004315	November 1995	19 February 2015	Phase II Pilot Study to Compare the Bioavailability of Buffered, Enteric-Coated Ursodiol With Unmodified Ursodiol for Chronic Cholestatic Liver Disease and Cystic Fibrosis-Associated Liver Disease		Cystic Fibrosis;Gastrointestinal Diseases;Cholestasis	Drug: ursodiol	National Center for Research Resources (NCRR)	Children's Hospital Medical Center, Cincinnati	Not recruiting	4 Months	N/A	Both	20	Phase 2	United States
855	NCT00004747	July 1995	19 February 2015	Phase II Randomized, Double-Blind, Placebo-Controlled Study of Intravenous Mucoid Exopolysaccharide Pseudomonas Aeruginosa Immune Globulin for Cystic Fibrosis		Cystic Fibrosis;Bacterial Infections	Drug: mucoid exopolysaccharide P. aeruginosa immune globulin IV	National Center for Research Resources (NCRR)	Vanderbilt University	Not recruiting	6 Years	N/A	Both	170	Phase 2
No.	TrialID	Date_ enrollement	Last_Refreshed_ on	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
856	NCT00004829	June 1995	7 April 2015	Phase III Randomized Study of the Inhalation of Tobramycin in Patients With Cystic Fibrosis		Cystic Fibrosis;Bacterial Infection	Drug: tobramycin	FDA Office of Orphan Products Development		Not recruiting	6 Years	N/A	Both	200	Phase 3

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