Results 51 to 60 of about 6,532 (197)
G970R‐CFTR Mutation (c.2908G>C) Results Predominantly in a Splicing Defect
Clinical and Translational Science, 2021 In previous work, participants with a G970R mutation in cystic fibrosis transmembrane conductance regulator (CFTR) (c.2908G>C) had numerically lower sweat chloride responses during ivacaftor treatment than participants with other CFTR gating mutations ...
Meredith C. Fidler +11 more
doaj +1 more source
British Journal of Clinical Pharmacology, Volume 92, Issue 6, Page 1934-1939, June 2026.Pharmacogenomic (PGx) information has the potential to support the safe and effective use of medicines, yet there is uncertainty about how this information can be best communicated to patients. Summaries of product characteristics (SmPCs) and patient information leaflets (PILs) for all UK‐approved medicines with strong evidence supporting a PGx ...
Parth Narlawar +5 more
wiley +1 more source
Ivacaftor potentiation of multiple CFTR channels with gating mutations
, 2012 BackgroundThe investigational CFTR potentiator ivacaftor (VX-770) increased CFTR channel activity and improved lung function in subjects with CF who have the G551D CFTR gating mutation.
Johnson, James P. +11 more
core +1 more source
No drug-drug interaction between tezacaftor-ivacaftor and clofazimine: A case report.
, 2022 In this case report the potential drug-drug interaction between cytochrome P450 (CYP) 3A4 substrates tezacaftor-ivacaftor and CYP3A4/5 inhibitor clofazimine is investigated in a patient with cystic fibrosis.
Terheggen-Lagro, S. W. J. +4 more
core +1 more source
Health Technology Assessment, 2014 Background: Ivacaftor (Kalydeco®, Vertex Pharmaceuticals) is the first of a new class of drugs that target the underlying protein defect in cystic fibrosis (CF).
Penny Whiting +9 more
doaj +1 more source
Structure of ABCB1/P-Glycoprotein in the Presence of the CFTR Potentiator Ivacaftor
Membranes, 2021 ABCB1/P-glycoprotein is an ATP binding cassette transporter that is involved in the clearance of xenobiotics, and it affects the disposition of many drugs in the body.
Alessandro Barbieri +5 more
doaj +1 more source
Journal of Magnetic Resonance Imaging, Volume 63, Issue 6, Page 1652-1661, June 2026.ABSTRACT Background Elexacaftor/tezacaftor/ivacaftor (ETI) is a current standard therapy for pediatric cystic fibrosis (CF). Multiple‐breath washout 129Xe MRI (MBW Xe‐MRI) is improved following 1 month of treatment. However, the utility of MBW Xe‐MRI over extended ETI treatment and its comparison to single‐breath Xe‐MRI and pulmonary function tests ...
Faiyza S. Alam +6 more
wiley +1 more source
Real-world effectiveness of ivacaftor in children with cystic fibrosis and the G551D mutation
Anales de Pediatría (English Edition), 2019 Introduction: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that has been shown to improve the nutritional status and lung function of cystic fibrosis patients with the G551D mutation in clinical trials.
David Gomez-Pastrana +5 more
doaj +1 more source
Cystic fibrosis modulator therapy can reverse cystic bronchiectasis
Respirology Case Reports, 2023 Bronchiectasis is often considered progressive and irreversible, so cases of regression or reversal are an important step in understanding the underlying pathophysiological mechanisms.
Peter G. Middleton, Nicholas J. Simmonds
doaj +1 more source
Clinical Use of Home Spirometry in Children With Cystic Fibrosis
Pediatric Pulmonology, Volume 61, Issue 6, June 2026.ABSTRACT Background The use of home spirometry (HSPIR) has increased in pediatric cystic fibrosis (CF) care, but how it has been used clinically and its impact on clinical care have not been described. The purpose of this study was to address this knowledge gap through a secondary analysis of data from a HSPIR quality improvement project to ...
Lucy Tan +8 more
wiley +1 more source