Microbiology Spectrum, 2022 The introduction of mutation-specific combination therapy with the cystic fibrosis transmembrane conductance regulator (CFTR) modulators elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has substantially improved lung function and quality of life of people
Sophia T. Pallenberg +6 more
doaj +1 more source
Pediatric Diabetes, Volume 2026, Issue 1, 2026.Cystic fibrosis‐related diabetes (CFRD) is the most prevalent nonrespiratory complication of cystic fibrosis (CF), with its prominence growing as survival rates improve due to advances in CFTR modulator therapies. Its prevalence increases with age, affecting nearly 50% of patients with CF (PwCF) over 30 years old.
Dogus Vuralli, Andrea Scaramuzza
wiley +1 more source
EPS5.04 Liver test abnormalities in adults with cystic fibrosis taking elexacaftor/tezacaftor/ivacaftor – one size doesn’t fit all? [PDF]
, 2023 D. Tewkesbury +4 more
openalex +1 more source
Pharmacokinetics of Ivacaftor, Tezacaftor, Elexacaftor, and Lumacaftor in Special Cystic Fibrosis Populations:A Systematic Review [PDF]
BACKGROUND AND OBJECTIVE: Following the development of cystic fibrosis transmembrane conductance regulator (CFTR) modulators (ivacaftor, tezacaftor, elexacaftor, and lumacaftor), the prognosis for people diagnosed with cystic fibrosis (pwCF) has improved.
Akkerman, Onno W +7 more
core +1 more source
British Journal of Pharmacology, Volume 182, Issue 24, Page 6063-6080, December 2025.Background and Purpose Cystic fibrosis (CF) is due to loss‐of‐function variants of the CF transmembrane conductance regulator (CFTR) channel. The most effective treatment for people with CF carrying the F508del mutation is the triple combination of elexacaftor–tezacaftor–ivacaftor (ETI).
Emanuela Pesce +25 more
wiley +1 more source
EPS6.03 Pulmonary pathogen prevalence 12 months after elexacaftor/tezacaftor/ivacaftor introduction: results from the Danish National Cystic Fibrosis Cohort [PDF]
, 2023 M. Jeppesen +9 more
openalex +1 more source
Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor–tezacaftor–ivacaftor combination: a 1-year observational study [PDF]
Background: To limit the progression of disease, people with cystic fibrosis (pwCF) perform daily respiratory physiotherapy, which is perceived as the most burdensome routine in managing their condition.
Blardone, Chiara +12 more
core +1 more source
The Concise Guide to PHARMACOLOGY 2025/26: Ion channels
British Journal of Pharmacology, Volume 182, Issue S1, Page S152-S241, December 2025.The Concise Guide to Pharmacology 2025/26 marks the seventh edition in this series of biennial publications in the British Journal of Pharmacology. Presented in landscape format, the guide provides a comparative overview of the pharmacology of drug target families. The concise nature of the Concise Guide refers to the style of presentation, being clear,
Stephen P. H. Alexander +86 more
wiley +1 more source
P329 Audit of change in weight and BMI centile in children on elexacaftor/tezacaftor/ivacaftor in the Liverpool paediatric cystic fibrosis population [PDF]
, 2023 Clare Woodland, C.A. Berry
openalex +1 more source
Respiratory infections after elexacaftor/tezacaftor/ivacaftor treatment in people with cystic fibrosis: analysis of the European Cystic Fibrosis Society Patient Registry [PDF]
Background: Elexacaftor/tezacaftor/ivacaftor (ETI) has improved outcomes for people with cystic fibrosis (pwCF). This study evaluated changes in airway microbiological infection status after initiating ETI.
De Rose, Virginia +7 more
core +1 more source