Cystic fibrosis transmembrane conductance regulator ( DrugBank: - )


1 disease
告示番号疾患名(ページ内リンク)臨床試験数
299嚢胞性線維症11

299. 嚢胞性線維症


臨床試験数 : 1,695 薬物数 : 1,527 - (DrugBank : 268) / 標的遺伝子数 : 111 - 標的パスウェイ数 : 174
No.TrialIDDate_
enrollment
Date_
registration
Public_titleScientific_titleConditionInterventionPrimary_
sponsor
Secondary_
sponsor
Recruitment_
Status
Inclusion_
agemin
Inclusion_
agemax
Inclusion_
gender
Target_
size
PhaseCountries
1NCT05740618
(ClinicalTrials.gov)
March 1, 20233/2/2023Inhaled Mannitol on Mucociliary Clearance in Moderate to Severe Cystic FibrosisEffect of Bronchitol on Mucociliary Clearance in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Modulator Treated Patients With Cystic Fibrosis With Moderate to Severe Lung DiseaseCystic FibrosisDrug: Mannitol Inhalant ProductUniversity of North Carolina, Chapel HillChiesi USA, Inc.Not yet recruiting18 YearsN/AAll25Phase 4United States
2NCT05663255
(ClinicalTrials.gov)
March 1, 202315/12/2022Effectiveness of CFTR Modulators According to Co-therapyIs Effectiveness of CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Modulators Impacted by Co-therapy? A Population-based Comparative Effectiveness Study Using Data From French Cystic Fibrosis Registry Linked to French National Healthcare Insurance DatabaseCystic FibrosisDrug: Discontinuation of respiratory co-therapy (azithromycin, RhDNase, or inhaled antibiotics)Hospices Civils de LyonNULLNot yet recruiting6 YearsN/AAll1990NULL
3NCT05095246
(ClinicalTrials.gov)
March 8, 202227/8/2021A Study of Inhaled KB407 for the Treatment of Cystic FibrosisA Phase I Study of Inhaled KB407, a Replication-Incompetent, Non-Integrating Vector Expressing Human Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), for the Treatment of Cystic FibrosisCystic FibrosisBiological: KB407 (Nebulization)Krystal Biotech, Inc.Novotech (Australia) Pty LimitedRecruiting18 YearsN/AAll13Phase 1Australia
4EUCTR2018-002579-16-SE
(EUCTR)
26/08/201816/07/2018Measurement 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®Karolinska University Hospital, Stockholm CF centerNULLAuthorised-recruitment may be ongoing or finishedFemale: yes
Male: yes
12Phase 2Sweden
5NCT02653027
(ClinicalTrials.gov)
January 1, 20187/1/2016Effect of Lumacaftor-ivacaftor on Glucose Handling and Tolerance in Cystic Fibrosis Phe508delA 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 FibrosisDrug: Lumacaftor-ivacaftor;Other: OGTTMassachusetts General HospitalNULLWithdrawn18 Years65 YearsAll0N/ANULL
6NCT02392234
(ClinicalTrials.gov)
March 201512/3/2015A 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-cystic Fibrosis Transmembrane Conductance Regulator (CFTR) MutationA 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 FunctionCystic FibrosisDrug: VX-661/Ivacaftor;Drug: Ivacaftor;Drug: Placebo matched to VX-661/ ivacaftor;Drug: Placebo matched to IvacaftorVertex Pharmaceuticals IncorporatedNULLCompleted12 YearsN/AAll248Phase 3United States;Australia;Belgium;Canada;France;Germany;Israel;Italy;Netherlands;Switzerland;United Kingdom
7EUCTR2013-003774-27-GB
(EUCTR)
20/01/201427/11/2013A 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)
INN or Proposed INN: OligoG
Product Name: Radiolabelled OligoG CF-5/20 6% Solution for Nebulisation
INN or Proposed INN: OligoG
AlgiPharma ASNULLNot RecruitingFemale: yes
Male: yes
12Phase 2United Kingdom
8NCT01531673
(ClinicalTrials.gov)
February 20121/2/2012Study of VX-661 Alone and in Combination With Ivacaftor in Subjects Homozygous or Heterozygous to the F508del-Cystic Fibrosis Transmembrane Conductance Regulator(CFTR) MutationA 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 MutationCystic FibrosisDrug: VX-661;Drug: Ivacaftor;Drug: Placebo matched to VX-661;Drug: Placebo matched to ivacaftorVertex Pharmaceuticals IncorporatedNULLCompleted12 YearsN/AAll194Phase 2United States;Canada;Germany;United Kingdom
9NCT00219882
(ClinicalTrials.gov)
April 200516/9/2005Safety Study of Orally Administered Curcuminoids in Adult Subjects With Cystic FibrosisA 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) MutationCystic FibrosisDrug: standardized turmeric root extractRamsey, Bonnie, MDSeer Pharmaceuticals;CF Therapeutics Development Network Coordinating Center;Cystic Fibrosis Foundation TherapeuticsCompleted18 Years40 YearsBoth11Phase 1United States
10NCT00004806
(ClinicalTrials.gov)
June 199524/2/2000Phase I Study of Liposome-Mediated Gene Transfer in Patients With Cystic FibrosisCystic FibrosisGenetic: Cystic fibrosis transmembrane conductance regulatorUniversity of Alabama at BirminghamNational Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)Completed18 YearsN/ABoth9Phase 1NULL
11NCT00004779
(ClinicalTrials.gov)
January 199324/2/2000Phase I Pilot Study of Ad5-CB-CFTR, an Adenovirus Vector Containing the Cystic Fibrosis Transmembrane Conductance Regulator Gene, in Patients With Cystic FibrosisCystic FibrosisGenetic: Ad5-CB-CFTRNational Center for Research Resources (NCRR)University of North CarolinaCompletedN/AN/ABoth12Phase 1NULL