Efficacy and safety of elexacaftor-tezacaftor-ivacaftor in the treatment of cystic fibrosis: a systematic review
Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) is a new CFTR (Cystic Fibrosis
Transmembrane Conductance Regulator) modulator treatment, used over the last few years …
Transmembrane Conductance Regulator) modulator treatment, used over the last few years …
[PDF][PDF] The impact of highly effective modulator therapy on cystic fibrosis microbiology and inflammation
LJ Caverly, SA Riquelme, KB Hisert - Clinics in chest medicine, 2022 - Elsevier
Highly-effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …
Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment
CA Shaughnessy, PL Zeitlin, PE Bratcher - Scientific reports, 2021 - nature.com
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR), which lead to early death due to progressive lung disease. The …
regulator (CFTR), which lead to early death due to progressive lung disease. The …
[HTML][HTML] Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation
JS Guimbellot, A Baines, A Paynter, SL Heltshe… - Journal of Cystic …, 2021 - Elsevier
Background The cystic fibrosis transmembrane conductance regulator (CFTR) potentiator,
ivacaftor, was first approved for people with CF and the G551D CFTR mutation. This study …
ivacaftor, was first approved for people with CF and the G551D CFTR mutation. This study …
Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations
Background Cystic fibrosis (CF) is a multisystem disease that often requires otolaryngology
care. Individuals with CF commonly have chronic rhinosinusitis but also present with hearing …
care. Individuals with CF commonly have chronic rhinosinusitis but also present with hearing …
[HTML][HTML] Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor
I Fajac, C Daines, I Durieu, JL Goralski… - Journal of Cystic …, 2023 - Elsevier
Abstract Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to be safe and efficacious
in people with cystic fibrosis (CF) heterozygous for F508del and a minimal function mutation …
in people with cystic fibrosis (CF) heterozygous for F508del and a minimal function mutation …
Evaluating the effect of kaftrio on perspectives of health and wellbeing in individuals with cystic fibrosis
SA Aspinall, KA Mackintosh, DM Hill, B Cope… - International Journal of …, 2022 - mdpi.com
Background: Modulator therapy represents a significant step forward in CF care and is
expected to have a significant impact on the health and mortality of many individuals with …
expected to have a significant impact on the health and mortality of many individuals with …
Predictors of sinonasal improvement after highly effective modulator therapy in adults with cystic fibrosis
DM Beswick, CM Liu, JB Overdevest… - The …, 2024 - Wiley Online Library
Objectives The 22‐question SinoNasal Outcome Test (SNOT‐22) assesses chronic
rhinosinusitis (CRS) severity. We aimed to identify predictors of SNOT‐22 score …
rhinosinusitis (CRS) severity. We aimed to identify predictors of SNOT‐22 score …
The management of cystic fibrosis chronic rhinosinusitis: an evidenced‐based review with recommendations
DB Spielman, DM Beswick, AJ Kimple… - International forum of …, 2022 - Wiley Online Library
Background Cystic fibrosis (CF) chronic rhinosinusitis (CRS) has emerged as a distinct
diagnostic entity, unique from other endotypes of CRS in its presentation, pathophysiology …
diagnostic entity, unique from other endotypes of CRS in its presentation, pathophysiology …
[PDF][PDF] Dual and triple modulator therapy for chronic rhinosinusitis in cystic fibrosis patients
S Uyttebroek, C Claeyssens, M Jorissen, L Dupont… - …, 2024 - researchgate.net
Background: The introduction of CFTR modulators has changed the landscape in the
treatment of cystic fibrosis (CF) and early case series have shown improvements in …
treatment of cystic fibrosis (CF) and early case series have shown improvements in …