65. 原発性免疫不全症候群
［臨床試験数：413，薬物数：581（DrugBank：97），標的遺伝子数：68，標的パスウェイ数：202］
Searched query = "Primary immunodeficiency", "X-SCID", "Reticular dysgenesis", "Adenosine deaminase deficiency", "Omenn syndrome", "Purine nucleoside phosphorylase deficiency", "CD8 deficiency", "ZAP-70 deficiency", "MHC class I deficiency", "MHC class II deficiency", "Combined immunodeficiency", "Wiskott-Aldrich syndrome", "Telangiectasia ataxia", "Nijmegen breakage syndrome", "Bloom syndrome", "Immunodeficiency, centromere region instability, facial anomalies syndrome", "ICF syndrome", "PMS2 deficiency", "Radiosensitivity, immunodeficiency, dysmorphic features, and learning difficulties syndrome", "RIDDLE syndrome", "Schimke syndrome", "Netherton syndrome", "Thymic hypoplasia", "DiGeorge syndrome", "22q11.2 deletion syndrome", "Hyper-IgE syndrome", "Hepatic venoocclusive immunodeficiency", "Immunodeficiency with central hepatic vein atresia", "Dyskeratosis congenita", "X-linked agammaglobulinaemia", "Common variable immunodeficiency", "Hyper-IgM syndrome", "Isolated IgG subclass deficiency", "Selective IgA deficiency", "Specific antibody production deficiency", "Infant transient hypogammaglobulinemia", "Chédiak-Higashi syndrome", "Chediak-Higashi syndrome", "X-linked lymphoproliferative syndrome", "SAP deficiency", "SH2D1A/SLAM-associated protein deficiency", "XIAP deficiency", "X-linked inhibitor of apoptosis deficiency", "Autoimmune lymphoproliferative syndrome", "ALPS", "Familial hemophagocytic syndrome", "Perforin deficiency", "Munc13-4 deficiency", "Syntaxin 11 deficiency", "Munc18-2 deficiency", "Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy", "APECED", "Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome", "IPEX syndrome", "CD25 deficiency", "ITCH deficiency", "Primary phagocytic dysfunction", "Severe congenital neutropenia", "Cyclic neutropenia", "Hermanskyi-Pudlak syndrome type 2", "Hermanskyi-Pudlak syndrome 2", "Griscelli syndrome type 2", "Griscelli syndrome 2", "p14 deficiency", "Warts, hypogammaglobulinemia, infections, myelokathexis syndrome", "WHIM syndrome", "Glycogen storage disease type Ib", "Leukocyte adhesion deficiency", "Shwachman-Diamond syndrome", "Chronic granulomatous disease", "Myeloperoxidase deficiency", "Mendelian susceptibility to mycobacterial disease", "MSMD", "Anhidrotic ectodermal dysplasia with immunodeficiency", "EDA-ID", "Interleukin-1 receptor-associated kinase-4 deficiency", "IRAK4 deficiency", "IMyD88 deficiency", "Chronic mucocutaneous candidiasis", "Epidermodysplasia verruciformis", "Herpes simplex encephalitis", "Caspase recruitment domain family member 9 deficiency", "CARD9 deficiency", "Trypanosomiasis", "Congenital complement deficiency", "C1q deficiency", "CC1r deficiency", "CC1s deficiency", "CC2 deficiency", "CC3 deficiency", "CC4 deficiency", "CC5 deficiency", "CC6 deficiency", "CC7 deficiency", "CC8 deficiency", "CC9 deficiency", "Factor D deficiency", "Properdin deficiency", "Factor I deficiency", "Factor H deficiency", "MASP1 deficiency", "3MC syndrome", "Mannose-binding protein-associated serine protease 2 deficiency", "MASP2 deficiency", "FCN3", "Hereditary angioedema type 1", "Hereditary angioedema type I", "C1 inhibitor deficiency type 1", "C1 inhibitor deficiency type I", "Hereditary angioedema type 2", "Hereditary angioedema type II", "C1 inhibitor deficiency type 2", "C1 inhibitor deficiency type II", "Hereditary angioedema type 3", "Hereditary angioedema type III", "C1 inhibitor deficiency type 3", "C1 inhibitor deficiency type III"
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.
| 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 | NCT02269163 (ClinicalTrials.gov) | January 26, 2016 | 16/10/2014 | Study of ProMetic BioTherapeutics Immune Globulin Intravenous (Human) 10% | A Phase 3, Multicenter, Open-Label Study of the Safety, Tolerability, Efficacy, and PK of ProMetic BioTherapeutics IGIV (Human) 10% in Adults and Children With Primary Immunodeficiency Diseases | Primary Immunodeficiency | Biological: Immune Globulin Intravenous;Biological: Prometic's Immune Globulin Intravenous 10% | Prometic Biotherapeutics, Inc. | Atlantic Research Group | Completed | 2 Years | 80 Years | All | 82 | Phase 3 | United States |
| 2 | NCT01218438 (ClinicalTrials.gov) | January 2013 | 8/10/2010 | Phase 2/3 Study of IGSC, 20% in PIDD | A Clinical Study of Immune Globulin Subcutaneous (Human), 20% Solution (IGSC, 20%) for the Evaluation of Efficacy, Safety, Tolerability and Pharmacokinetics in Subjects With Primary Immunodeficiency Diseases (PIDD) | Primary Immunodeficiency Diseases (PID) | Biological: Immune Globulin Intravenous (Human), 10% Solution;Drug: Immune Globulin Subcutaneous (Human), 20% Solution | Baxalta now part of Shire | NULL | Completed | 2 Years | N/A | All | 86 | Phase 2;Phase 3 | United States;Canada |
| 3 | NCT01406470 (ClinicalTrials.gov) | September 2011 | 26/7/2011 | Phase 3 Study of Immune Globulin Intravenous (Human)IVIG-SN™ in Subjects With Primary Immunodeficiency | An Open-Label, Single-Arm, Historically Controlled, Prospective, Multicenter Phase III Study to Evaluate the Safety, Efficacy and Pharmacokinetics of Immune Globulin Intravenous (Human) IVIG-SN™ in Subjects With Primary Immunodeficiency | Immunologic Deficiency Syndrome | Drug: Immune Globulin Intravenous (Human) 5% Liquid, IVIG-SN™ | Green Cross Corporation | Atlantic Research Group | Completed | 2 Years | 70 Years | Both | 45 | Phase 3 | United States;Canada |
| 4 | NCT01412385 (ClinicalTrials.gov) | June 20, 2011 | 8/8/2011 | Immune Globulin Subcutaenous (Human), 20% | A Clinical Study of Immune Globulin Subcutaneous (Human) (IGSC), 20% for the Evaluation of Efficacy, Safety, and Pharmacokinetics in Subjects With Primary Immunodeficiency Diseases | Primary Immunodeficiency Diseases (PID) | Biological: Immune Globulin Subcutaneous (Human), 20%;Biological: Immune Globulin Intravenous (Human), 10%;Biological: Human Normal Immunoglobulin (Subcutaneous - Intramuscular Immunoglobulin) | Baxalta now part of Shire | NULL | Completed | 2 Years | N/A | All | 55 | Phase 2;Phase 3 | Austria;Germany;Hungary;Sweden;United Kingdom |
| 5 | NCT00814320 (ClinicalTrials.gov) | December 18, 2008 | 23/12/2008 | Gammagard Liquid and rHuPH20 in PID | Efficacy, Tolerability and Pharmacokinetic Comparison of Immune Globulin Intravenous (Human), 10% (GAMMAGARD LIQUID/KIOVIG) Administered Intravenously or Subcutaneously Following Administration of Recombinant Human Hyaluronidase (rHuPH20) in Subjects With Primary Immunodeficiency Diseases | Primary Immunodeficiency Diseases (PID) | Biological: Recombinant human hyaluronidase (rHuPH20)+ immune globulin intravenous (IGIV) | Baxalta now part of Shire | NULL | Completed | 2 Years | N/A | All | 89 | Phase 3 | United States;Canada |
| 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 |
| 6 | NCT00546871 (ClinicalTrials.gov) | October 3, 2007 | 18/10/2007 | Comparison of Intravenous and Subcutaneous Administration of IGIV, 10% in Primary Immunodeficiency (PID) Subjects | Tolerability and Pharmacokinetic Comparison of Immune Globulin Intravenous (Human) 10% (IGIV, 10%) Administered Intravenously or Subcutaneously in Subjects With Primary Immunodeficiency Diseases | Primary Immunodeficiency Diseases (PID) | Drug: Immune Globulin Intravenous (Human), 10% | Baxalta now part of Shire | NULL | Completed | 24 Months | N/A | All | 49 | Phase 2;Phase 3 | United States |
| 7 | NCT00389324 (ClinicalTrials.gov) | November 2006 | 17/10/2006 | A Trial of the Pharmacokinetics, Safety, and Tolerability of Subcutaneous Gamunex® in Primary Immunodeficiency | An Open-Label Single-Sequence, Crossover Trial to Evaluate the Pharmacokinetics and Safety of Subcutaneous Gamunex® 10% (Immune Globulin Intravenous [Human], 10% Caprylate/Chromatography Purified) in Subjects With Primary Immunodeficiency | Immunologic Deficiency Syndrome | Biological: Immune Globulin Intravenous (Human) | Grifols Therapeutics Inc. | NULL | Completed | 13 Years | 75 Years | All | 35 | Phase 2 | United States;Canada |
| 8 | NCT00220766 (ClinicalTrials.gov) | August 2002 | 13/9/2005 | Rapid Infusion of Immune Globulin Intravenous (Human) In Primary Immunodeficiency Patients | IGIV-C 10% Rapid Infusion Trial in Primary Immune Deficient Patients | Immunologic Deficiency Syndrome;Agammaglobulinemia;Severe Combined Immunodeficiency;Wiskott-Aldrich Syndrome;Common Variable Immunodeficiency | Drug: Immune Globulin Intravenous [Human], 10% Caprylate/Chromatography Purified;Drug: Dextrose, 5% in Water | Grifols Therapeutics Inc. | NULL | Completed | 18 Years | 75 Years | Both | 100 | Phase 3 | United States;Canada |
| 9 | NCT00157079 (ClinicalTrials.gov) | June 25, 2002 | 8/9/2005 | Safety and Efficacy Study of a 10% Intravenous Immune Globulin Solution in Subjects With Primary Immunodeficiency Disorders | A Clinical Investigation to Assess the Safety and Efficacy of Immune Globulin Intravenous (Human), 10% in Subjects With Primary Immunodeficiency Disorders | Primary Immunodeficiency Diseases (PID);Immune Thrombocytopenic Purpura (ITP);Kawasaki Syndrome | Biological: Immune Globulin Intravenous (Human), 10% | Baxalta now part of Shire | NULL | Completed | 24 Months | N/A | All | Phase 3 | United States | |
| 10 | NCT00161993 (ClinicalTrials.gov) | June 13, 2002 | 8/9/2005 | Safety, Pharmacokinetic and Efficacy Study of a 10% Triple Virally Reduced Intravenous Immune Globulin Solution in Patients With Primary Immunodeficiency (Hypo- or Agammaglobulinemia) | Prospective Open-Label Study of Pharmacokinetics, Efficacy and Safety of Immune Globulin Intravenous (Human), 10% TVR Solution in Patients With Hypo- or Agammaglobulinemia | Primary Immunodeficiency Diseases (PID);Agammaglobulinemia;Hypogammaglobulinemia | Drug: Immune Globulin Intravenous (Human), 10% TVR (Triple Virally Reduced) Solution;Drug: Gammagard S/D (Solvent/Detergent) | Baxalta now part of Shire | NULL | Completed | 18 Years | N/A | All | Phase 2 | Finland;Sweden |