DDrare: Database of Drug Development for Rare Diseases

93. 原発性胆汁性胆管炎［原発性胆汁性肝硬変 (~2017.3)］
［臨床試験数：230，薬物数：215（DrugBank：51），標的遺伝子数：34，標的パスウェイ数：107］

Searched query = "Primary biliary cholangitis", "Primary biliary cirrhosis", "PBC"
The queries were searched in Public_title, Scientific_title, and Condition. Export date: 03/15/2021. Trials are sorted by Date_enrollment from most recent to oldest in the table.

Drug = 1-20190; 11C-CSar; 13-15mg/kg/d Ursodeoxycholic; 18-22mg/kg/d Ursodeoxycholic; 3 mg [14C]-A4250 capsule; 3a,7a-dihydroxy-6a-ethyl-5betacholan-24-oic acid; 3a,7a-dihydroxy-6a-ethyl-5ßcholan-24-oic acid; 6 alpha-ethylchenodeoxycholic acid (6-EDCA), OCA, INT-747; 6 alpha-ethylchenodeoxycholic acid (6-EDCA), oCA, INT-747; 6-ECDCA; 6-ethylchenodeoxycholic acid; 6?-ethylchenodeoxycholic acid (6-ECDCA), OCA, INT-747; 6?-ethylchenodeoxycholic acid (6-ECDCA), OCA, Int-747; 6a-ethylchenodeoxycholic acid (6-ECDCA), OCA, INT-747; 6a-ethylchenodeoxycholic acid (6-ECDCA), OCA, Int-747; 6a-ethylchenodeoxycholic acid , 6-ethylchenodeoxycholic acid; 6a-ethylchenodeoxycholic acid , 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5betacholan-24-o; 6a-ethylchenodeoxycholic acid , 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5betacholan-24-o, obeticholic acid (OCA); 6a-ethylchenodeoxycholic acid , 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5ßcholan-24-o; 6a-ethylchenodeoxycholic acid , 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5ßcholan-24-o, obeticholic acid (OCA); 6a-ethylchenodeoxycholic acid, 6-ethylchenodeoxycholic acid; 6a-ethylchenodeoxycholic acid, 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5betacholan-24-o; 6a-ethylchenodeoxycholic acid, 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5betacholan-24-o , obeticholic acid (OCA); 6a-ethylchenodeoxycholic acid, 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5ßcholan-24-o; 6a-ethylchenodeoxycholic acid, 6-ethylchenodeoxycholic acid, 3a,7a-dihydroxy-6a-ethyl-5ßcholan-24-o , obeticholic acid (OCA); A11HA02; A4250; APD334; Abatacept; Acido Obeticolico; Acido ursodessicolico; Alkaline Phosphatase; Atorvastatin; BARICITINIB; BCD-085; BEZAFIBRATE; BUDESONIDE; Baricitinib; Bezafibrat; Bezafibrat Genericon retard 400 mg; Bezafibrate; Bezafibrate 200 MG; Bezafibrate 400 MG; Bezafibrate IR; Bezafibrate SR; Bezafibrate, Beza, BZF, Bezalip; Bezafibrate, Beza, BZF, Bezalip mono; Bezalip; Bezalip mono; Biological: mesenchymal stem cell; Budenofalk 3mg; Budesonide; CNTO1275; CR845 1.0 mg; CRC (A3384); Calcium; Choline; Cilofexor; Colchicine; Combination antiviral therapy; Cyclophosphamide; Cyclosporin; Cyclosporin A; Denosumab; Device: Fibroscan; E6011; EDP-305; EDP-305 Dose 1; EDP-305 Dose 2; ELAFIBRANOR; EZETIMIBE; EZT; Elafibranor; Elafibranor 120 mg; Elafibranor 80 mg; Elafibranor 80mg; Emtricitabine; Emtricitabine (FTC)/Tenofovir Disoproxil (TDF); Experimental: Seladelpar / MBX-8025 200 mg; Experimental: Seladelpar / MBX-8025 50 mg; Ezetimibe; Ezetrol; FFP104; Fenofibrate; Fenofibrate IDD-P (Insoluble Drug Delivery-Micro Particle); Fully human monoclonal antibody directed against CXCL10; Fuzhenghuayu; GFT505; GKT137831; GS-9674; GS-9674 100 mg; GS-9674 30 mg; GSK2330672; Gold; HTD1801 (BUDCA); High protein high fiber diet; ICG; INT-747; INT-747, OCA; Kaletra; LJN452; LJN452, 0,01 mg; LJN452, 0,03 mg; LJN452, 0,1 mg; LUM001; LY3009104; Linerixibat; Linerixibat (GSK2330672) 45mg; Liver biopsy; MBX 8025; MBX-8025; MBX-8025 10 mg Capsule; MBX-8025 2 mg Capsule; MBX-8025 5 mg Capsule; MBX-8025, DSIC; MBX-8025, Formulation 2; MabThera; MabThera®; Magnesium; Methionine; Methotrexate; Minodronic acid; Modafinil; Moexipril; Mycophenolate; Mycophenolate Mofetil; Mycophenolate mofetil; NGM282; NI-0801; Non-myeloablative Hematopoietic Stem Cell Transplantation; OCA (INT-747); OCA, INT-747; OP-724; Obeticholic Acid; Obeticholic Acid (OCA); Obeticholic acid; Obeticholic acid (OCA); Ocaliva; Ocaliva 10mg; Ocaliva 5mg; Olumiant; Other: Blood samples; Other: Questionnaires; PYRIDOXINE; Part 1: LJN452; Part 2: LJN452 Dose level 1; Part 2: LJN452 Dose level 2; Pemafibrate; Pentoxifylline; Phenylbutyrate; Prednisone; Probiotic; Pyridoxine; Pyridoxine 10mg; Pyridoxine 10mg BP; Questran; Raltegravir; RhuDex 100 mg (127 mg RhuDex choline salt) granules; RhuDex 25 mg (31.75 mg RhuDex choline salt) granules; RhuDex 50 mg (63.5 mg RhuDex choline salt) granules; RhuDex choline salt; Rifampicin; Risedronic acid; Rituximab; S-adenosyl-L-methionine; S-adenosyl-methionine (SAMe) capsules; SAMe; SELADELPAR; Saroglitazar; Saroglitazar magnesium 2 mg; Saroglitazar magnesium 4 mg; Seladelpar; Seladelpar 10 mg; Seladelpar 10 mg Capsule; Seladelpar 5 mg; Seladelpar 5 mg Capsule; Seladelpar 5-10 mg; Sortis; Sortis 10 mg; Sublimated mare milk; TQA3526; Tauroursodeoxycholic Acid Capsules; Tauroursodeoxycholic acid; Tenofovir; Tenofovir disoproxil; Tetrathiomolybdate; Tigecycline; Tropifexor; Truvada and Kaletra; UDCA; UDCA (Ursodeoxycholic acid); URSODEOXYCHOLIC ACID; Umbilical Cord Derived Mesenchymal Stem Cell (UC -MSC) Transplantation; Ursodeoxycholic Acid; Ursodeoxycholic Acid (URSO); Ursodeoxycholic Acid 300mg capsule; Ursodeoxycholic Acid 300mg tablet; Ursodeoxycholic Acid Capsules; Ursodeoxycholic acid; Ursodeoxycholic acid combination of immunosuppressive agents; Ursodeoxycholic acid combined with total glucosides of paeony; Ursodeoxycholic acid only; Ursodeoxycholic acid+Low Dose Glucocorticoid(prednisone); Ursodeoxycholsäure; Ursodiol; Ursofalk; Ursofalk® 500 mg Filmtabletten; Ursofalk® 500 mg film-coated tablets; Ursofalk® capsules; Ustekinumab; Ustekinumab 180 mg; Ustekinumab 45 mg; Ustekinumab 90 mg; Vitamin D; Zoledronic acid

No.	TrialID	Date_ enrollment	Date_ registration	Public_title	Scientific_title	Condition	Intervention	Primary_ sponsor	Secondary_ sponsor	Recruitment_ Status	Inclusion_ agemin	Inclusion_ agemax	Inclusion_ gender	Target_ size	Phase	Countries
1	JPRN-UMIN000015789	2012/11/30	29/11/2015	Tolerance induction by a regulartory T cell-based cell therapy in living donor liver transplantation		non-compenzated liver cirrhosis(HBV,HCV, alcoholic, etc), primary biliary cirrhosis (PBC), primary screlosing cholangitis (PSC), hepatocellular carcinoma (HCC within Milan criteria), non-alcoholic steatohepatitis (NASH), others	The patient is given cyclophosphamide (40mg/kg,iv) on postoperative days (POD) 5, and ex-vivo generated regulatory T cells on POD 13. Postoperative immunosuppression is with comventioal method. Tke immunosuppressant is reduced to 3/4 daily dose of the twice daily dose at 6 months, which is reduced to 3 times/week, 2 times/ week and 1 time/week every 3 months after confirming normal graft function and liver biopsy. the immunosuppressant is completely discontinued by 18 months after transplantation.	Traslational Research and Clinical Trial Center	NULL	Recruiting	18years-old	65years-old	Male and Female	10	Phase 1;Phase 2	Japan

No.

TrialID

Date_
enrollment

Date_
registration

Public_title

Scientific_title

Condition

Intervention

Primary_
sponsor

Secondary_
sponsor

Recruitment_
Status

Inclusion_
agemin

Inclusion_
agemax

Inclusion_
gender

Target_
size

Phase

Countries

JPRN-UMIN000015789

2012/11/30

29/11/2015

Tolerance induction by a regulartory T cell-based cell therapy in living donor liver transplantation

non-compenzated liver cirrhosis(HBV,HCV, alcoholic, etc), primary biliary cirrhosis (PBC), primary screlosing cholangitis (PSC), hepatocellular carcinoma (HCC within Milan criteria), non-alcoholic steatohepatitis (NASH), others

The patient is given cyclophosphamide (40mg/kg,iv) on postoperative days (POD) 5, and ex-vivo generated regulatory T cells on POD 13. Postoperative immunosuppression is with comventioal method. Tke immunosuppressant is reduced to 3/4 daily dose of the twice daily dose at 6 months, which is reduced to 3 times/week, 2 times/ week and 1 time/week every 3 months after confirming normal graft function and liver biopsy. the immunosuppressant is completely discontinued by 18 months after transplantation.

Traslational Research and Clinical Trial Center

NULL

Recruiting

18years-old

65years-old

Male and Female

Phase 1;Phase 2

Japan