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 | NCT04561115 (ClinicalTrials.gov) | October 6, 2020 | 10/9/2020 | A Study to Evaluate the Pharmacokinetics, Safety, and Tolerability of Immune Globulin (Human) 10% (Gamunex-C) PEG Process (IVIG-PEG) Compared to Gamunex-C in Participants With Primary Humoral Immunodeficiency | A Phase 3, Multicenter, Open-label, Single-sequence, Cross-over, Bioequivalence Study to Evaluate the Pharmacokinetics, Safety, and Tolerability of IVIG-PEG Compared to Gamunex-C in Subjects With Primary Humoral Immunodeficiency | Primary Immunodeficiency | Biological: Gamunex-C;Biological: IVIG-PEG | Grifols Therapeutics LLC | NULL | Recruiting | 18 Years | 75 Years | All | 25 | Phase 3 | United States |
| 2 | EUCTR2020-001496-32-SK (EUCTR) | 01/10/2020 | 02/07/2020 | Study, in which all know the treatment assigned to the patient, conducted in more sites, to evaluate the efficaty, safety, and metabolism of Kedrion in venous Human Normal Immunoglobulin (IVIg) 10% in child with Primary Immunodeficiency Disease (PID) | A Phase III, Open-label, Prospective, Multicenter Study to Assess Efficacy, Safety, and Pharmacokinetics of Kedrion Intravenous Human Normal Immunoglobulin (IVIg) 10% in Pediatric Patients Affected by Primary Immunodeficiency Disease (PID) - PID-PED study | Pediatric Patients Affected by Primary Immunodeficiency Disease. MedDRA version: 20.0;Level: PT;Classification code 10064859;Term: Primary immunodeficiency syndrome;System Organ Class: 10010331 - Congenital, familial and genetic disorders;Therapeutic area: Diseases [C] - Immune System Diseases [C20] | Product Name: KIg10 Product Code: KIg10 INN or Proposed INN: Human Immunoglobulin | KEDRION S.P.A | NULL | Authorised-recruitment may be ongoing or finished | Female: yes Male: yes | 30 | Phase 3 | Portugal;Hungary;Slovakia;Russian Federation;Italy | ||
| 3 | EUCTR2020-001496-32-HU (EUCTR) | 09/09/2020 | 02/07/2020 | Study, in which all know the treatment assigned to the patient, conducted in more sites, to evaluate the efficaty, safety, and metabolism of Kedrion in venous Human Normal Immunoglobulin (IVIg) 10% in child with Primary Immunodeficiency Disease (PID) | A Phase III, Open-label, Prospective, Multicenter Study to Assess Efficacy, Safety, and Pharmacokinetics of Kedrion Intravenous Human Normal Immunoglobulin (IVIg) 10% in Pediatric Patients Affected by Primary Immunodeficiency Disease (PID) - PID-PED study | Pediatric Patients Affected by Primary Immunodeficiency Disease. MedDRA version: 20.0;Level: PT;Classification code 10064859;Term: Primary immunodeficiency syndrome;System Organ Class: 10010331 - Congenital, familial and genetic disorders;Therapeutic area: Diseases [C] - Immune System Diseases [C20] | Product Name: KIg10 Product Code: KIg10 INN or Proposed INN: Human Immunoglobulin | KEDRION S.P.A | NULL | Authorised-recruitment may be ongoing or finished | Female: yes Male: yes | 30 | Phase 3 | Portugal;Hungary;Slovakia;Russian Federation;Italy | ||
| 4 | NCT03961009 (ClinicalTrials.gov) | April 30, 2019 | 21/5/2019 | Clinical Assessment of phaRmacokinetics, Efficacy, and Safety of 10% IVIg in PID Patients | A Phase III, Open-label, Prospective, Multicenter Study to Assess Efficacy, Safety and Pharmacokinetics of Kedrion Intravenous Immunoglobulin (IVIg) 10% in Primary Immunodeficiency Disease (PID) Patients | Primary Immunodeficiency Disease | Biological: Kedrion IVIG 10% | Kedrion S.p.A. | NULL | Active, not recruiting | 2 Years | 70 Years | All | 47 | Phase 3 | United States;Canada |
| 5 | NCT03576469 (ClinicalTrials.gov) | June 13, 2018 | 15/6/2018 | A Study to Evaluate the Benefit of RUCONEST® in Subjects Who Experience ADRs Related to IVIG Infusions | A Single-site, Open-Label, Pilot Study to Evaluate the Benefit of RUCONEST® in Subjects Who Experience ADRs Related to IVIG Infusions | CVI - Common Variable Immunodeficiency | Biological: C1-esterase inhibitor [recombinant] (C1-INH-R) | IMMUNOe Research Centers | NULL | Recruiting | 18 Years | N/A | All | 20 | Phase 4 | United States |
| 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 | NCT01985373 (ClinicalTrials.gov) | December 2013 | 31/10/2013 | Pharmacokinetics and Safety of IVIG Nanogam 100 mg/ml | Pharmacokinetics and Safety of the Intravenous Human Immunoglobulin Product Nanogam 100 mg/ml | Primary Immunodeficiency | Drug: Intravenous immunoglobulin infusion | Sanquin | NULL | Completed | 18 Years | N/A | Both | 23 | Phase 3 | Netherlands |
| 7 | EUCTR2012-005727-32-NL (EUCTR) | 25/07/2013 | 23/05/2013 | The pharmacokinetics (the behaviour in the body) and safety of the intravenous immunoglobulin product Nanogam 100 mg/ml | Pharmacokinetics and safety of the intravenous human immunoglobulin product Nanogam 100 mg/ml - PK and safety of Nanogam 100 mg/ml | Primary a- or hypogammaglobulinemia MedDRA version: 14.1;Level: LLT;Classification code 10010112;Term: Common variable immunodeficiency;System Organ Class: 100000004870 MedDRA version: 14.1;Classification code 10010509;Term: Congenital hypogammaglobulinemia;System Organ Class: 100000004850 MedDRA version: 14.1;Classification code 10071133;Term: Congenital agammaglobulinemia;System Organ Class: 100000004850;Therapeutic area: Diseases [C] - Immune System Diseases [C20] | Product Name: Nanogam 100 mg/ml INN or Proposed INN: HUMAN NORMAL IMMUNOGLOBULIN (IV) Other descriptive name: IVIG Trade Name: Nanogam® 50 mg/ml INN or Proposed INN: HUMAN NORMAL IMMUNOGLOBULIN (IV) Other descriptive name: IVIG | Sanquin Blood Supply Foundation | NULL | Not Recruiting | Female: yes Male: yes | Netherlands | ||||
| 8 | NCT01581593 (ClinicalTrials.gov) | November 2012 | 16/4/2012 | Efficacy and Safety Study of Kedrion IVIG 10% to Treat Subjects With Primary Immunodeficiency (PID) | Multicenter, Open-label, Historically Controlled, Phase III Study to Assess the Efficacy, Tolerability, Safety and Pharmacokinetics of Kedrion IVIG 10% in Adult and Pediatric Subjects With Primary Immunodeficiency (PID). | Primary Immunodeficiency;Agammaglobulinemia;Hypogammaglobulinemia;Antibody Deficiency | Biological: Kedrion IVIG 10% | Kedrion S.p.A. | NULL | Active, not recruiting | 2 Years | 70 Years | Both | 50 | Phase 3 | United States;Canada |
| 9 | 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 |
| 10 | EUCTR2010-019249-25-HU (EUCTR) | 22/09/2010 | 06/08/2010 | Eine offene, prospektive Studie zur Untersuchung der Pharmakokinetikund Sicherheit (Teil A) des Immunglobulins vom Menschen zurintravenösen Infusion (IVIG) BT090 sowie der Verträglichkeit undSicherheit von steigenden Infusionsgeschwindigkeiten (Teil B) beiPatienten mit primären Immundefekten (PID) | An open, prospective trial investigating pharmacokinetics and safety (Part A) of the human normal immunoglobulin for intravenous infusion (IVIG) BT090 and tolerability and safety of escalating infusion rates (Part B) in patients with primary immunodeficiency disease (PID) | Primary immunodeficiency disease (PID) MedDRA version: 13.1;Level: LLT;Classification code 10010509;Term: Congenital hypogammaglobulinemia;System Organ Class: 10010331 - Congenital, familial and genetic disorders;Therapeutic area: Diseases [C] - Immune System Diseases [C20] | Product Name: Intratect Product Code: BT090 Other descriptive name: Human normal immunoglobulin for intravenous use (IVIG) | Biotest AG | NULL | Not Recruiting | Female: yes Male: yes | 30 | Hungary;Germany | |||
| 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 |
| 11 | EUCTR2010-019249-25-DE (EUCTR) | 12/08/2010 | 14/05/2010 | An open, prospective trial investigating pharmacokinetics and safety (Part A) of the human normal immunoglobulin for intravenous infusion (IVIG) BT090 and tolerability and safety of escalating infusion rates (Part B) in patients with primary immunodeficiency disease (PID) | An open, prospective trial investigating pharmacokinetics and safety (Part A) of the human normal immunoglobulin for intravenous infusion (IVIG) BT090 and tolerability and safety of escalating infusion rates (Part B) in patients with primary immunodeficiency disease (PID) | Primary immunodeficiency disease (PID) MedDRA version: 14.1;Level: LLT;Classification code 10010509;Term: Congenital hypogammaglobulinemia;System Organ Class: 10010331 - Congenital, familial and genetic disorders;Therapeutic area: Diseases [C] - Immune System Diseases [C20] | Product Name: Intratect Product Code: BT090 Other descriptive name: Human normal immunoglobulin for intravenous use (IVIG) | Biotest AG | NULL | Not Recruiting | Female: yes Male: yes | 30 | Hungary;Germany | |||
| 12 | EUCTR2004-004465-15-HU (EUCTR) | 22/07/2005 | 11/05/2005 | A multicentre, open, prospective study investigating clinical efficacy, safety, and pharmacokinetic properties of the human normal immunoglobulin for intravenous administration BT681 in patients with primary immunodeficiency disease (PID) | A multicentre, open, prospective study investigating clinical efficacy, safety, and pharmacokinetic properties of the human normal immunoglobulin for intravenous administration BT681 in patients with primary immunodeficiency disease (PID) | primary immunodeficiency syndrome as congenital agammaglobulinaemia or hypogammaglobulinaemia, common variable immunodeficiency, severe combined immunodeficiencies, Wiskott Aldrich syndrome MedDRA version: 7.0;Level: HLT;Classification code 10036700 | Trade Name: Intratect Product Name: Intratect Product Code: BT681 Other descriptive name: human normal immunoglobulin (IVIg) | Biotest AG | NULL | Not Recruiting | Female: yes Male: yes | 50 | Hungary;Germany | |||
| 13 | EUCTR2004-004465-15-DE (EUCTR) | 05/04/2005 | 04/02/2005 | A multicentre, open, prospective study investigating clinical efficacy, safety, and pharmacokinetic properties of the human normal immunoglobulin for intravenous administration BT681 in patients with primary immunodeficiency disease (PID) | A multicentre, open, prospective study investigating clinical efficacy, safety, and pharmacokinetic properties of the human normal immunoglobulin for intravenous administration BT681 in patients with primary immunodeficiency disease (PID) | primary immunodeficiency syndrome as congenital agammaglobulinaemia or hypogammaglobulinaemia, common variable immunodeficiency, severe combined immunodeficiencies, Wiskott Aldrich syndrome MedDRA version: 7.0;Level: HLT;Classification code 10036700 | Product Name: Intratect Product Code: BT681 Other descriptive name: human normal immunoglobulin (IVIg) | Biotest AG | NULL | Not Recruiting | Female: yes Male: yes | 50 | Hungary;Germany | |||
| 14 | NCT00168012 (ClinicalTrials.gov) | September 2004 | 12/9/2005 | Efficacy and Safety of Intravenous Immunoglobulin IVIG-F10 in Patients With Primary Immunodeficiencies (PID) | An Open Study to Evaluate the Safety and Efficacy of IVIG-F10 in Patients With Primary Immunodeficiency Diseases (PID) | Agammaglobulinemia;IgG Deficiency;Common Variable Immunodeficiency | Drug: Immunoglobulins Intravenous (Human) | CSL Behring | NULL | Completed | 3 Years | 70 Years | Both | 42 | Phase 3 | NULL |