Medication adherence to CFTR modulators in patients with cystic fibrosis:a systematic review [PDF]
BACKGROUND: In the last decade, a fundamental shift in the treatment of cystic fibrosis (CF) took place due to the introduction of CF transmembrane conductance regulator (CFTR) modulators.
Breukelman, Anna J +5 more
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A case of severe pulmonary exacerbation in a young patient with cystic fibrosis in the era of CFTR modulators [PDF]
The introduction of CFTR modulator drugs like Elexacaftor-Tezacaftor-Ivacaftor (ETI) has transformed the management of Cystic Fibrosis (CF), significantly improving symptoms, lung function, and quality of life, while reducing reliance on intravenous ...
Alicandro, Gianfranco +8 more
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Respiratory ResearchBackground Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) channel. For people with CF (pwCF) affected by the most common pathogenic variant F508del, a tritherapy, named ...
Manuella Lévêque +7 more
doaj +1 more source
Rethinking hyperbilirubinemia: Gilbert syndrome in children with cystic fibrosis, a case report
Respiratory Medicine Case ReportsCystic Fibrosis leads to liver complications, including cystic fibrosis liver disease but hyperbilirubinemia in CF patients on CFTR modulators is less understood.
Yara Salameh, John Lyles, Shatha Yousef
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EPS6.04 Elexacaftor/tezacaftor/ivacaftor (ETI) reduces sputum pathogen density and lung inflammation, but infection and inflammation persist [PDF]
, 2023 Pradeep K. Singh +14 more
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Is Triple Combination CFTR Modulator Therapy Effective in Improving the Quality of Life of Patients with Cystic Fibrosis Who Have One Phe508del Allele? [PDF]
, 2020 Objective: The objective of this selective EBM review is to determine whether or not “Is triple combination CFTR modulator therapy effective in improving the quality of life of patients with cystic fibrosis who have one Phe508del allele?” Study Design: A
Avallone, Gia
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Optimism with Caution: Elexacaftor–Tezacaftor–Ivacaftor in Patients with Advanced Pulmonary Disease
, 2021 Stephanie Kuek +4 more
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EPS5.07 Effect of elexacaftor/tezacaftor/ivacaftor on nutritional status in UK children with cystic fibrosis aged 6–11 years: a single centre service evaluation [PDF]
, 2023 E. Owen, Rossa Brugha
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Vanzacaftor–tezacaftor–deutivacaftor for children aged 6–11 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial [PDF]
BACKGROUND: In phase 2 trials in people with cystic fibrosis aged 18 years and older, vanzacaftor–tezacaftor–deutivacaftor has been shown to be a safe and effective, once-daily cystic fibrosis transmembrane conductance regulator (CFTR) modulator ...
Chen, YC +17 more
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