Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults …

L Wucherpfennig, SMF Triphan, S Wege… - Journal of Cystic …, 2022 - Elsevier
Abstract Background Therapy with Elexacaftor/Tezacaftor/Ivacaftor (ETI) was recently
approved for adult cystic fibrosis (CF) patients with at least one F508del mutation. However …

Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: Early report

R Bec, M Reynaud-Gaubert, F Arnaud, R Naud… - European Journal of …, 2022 - Elsevier
Abstract Rationale and Objectives Cystic fibrosis transmembrane conductance regulator
(CFTR) modulators have revolutionised the treatment of cystic fibrosis (CF). Chest computed …

Effects of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in patients with cystic fibrosis and one or two F508del …

SY Graeber, DM Renz, M Stahl… - American journal of …, 2022 - atsjournals.org
Rationale: We recently demonstrated that triple-combination CFTR (cystic fibrosis
transmembrane conductance regulator) modulator therapy with elexacaftor/tezacaftor …

Clinical effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: a clinical trial

DP Nichols, AC Paynter, SL Heltshe… - American journal of …, 2022 - atsjournals.org
Rationale: The cystic fibrosis (CF) modulator drug, elexacaftor/tezacaftor/ivacaftor (ETI),
proved highly effective in controlled clinical trials for individuals with at least one F508del …

[HTML][HTML] Elexacaftor-Tezacaftor-Ivacaftor improves sinonasal outcomes in cystic fibrosis

AL Stapleton, AJ Kimple, JL Goralski, SM Nouraie… - Journal of Cystic …, 2022 - Elsevier
Background Many individuals with cystic fibrosis (CF) have chronic rhinosinusitis resulting in
nasal obstruction, sinus infections, and repeated surgeries. Elexacaftor-tezacaftor-ivacaftor …

Improvement in lung clearance index and chest computed tomography scores with elexacaftor/tezacaftor/ivacaftor treatment in people with cystic fibrosis aged 12 …

P McNally, K Lester, G Stone, B Elnazir… - American Journal of …, 2023 - atsjournals.org
Rationale: Clinical trials have shown that use of elexacaftor/tezacaftor/ivacaftor (ETI) is
associated with improvements in sweat chloride, pulmonary function, nutrition, and quality of …

[HTML][HTML] Computed tomography correlates with improvement with ivacaftor in cystic fibrosis patients with G551D mutation

SI Sheikh, FR Long, KS McCoy, T Johnson… - Journal of Cystic …, 2015 - Elsevier
Background Ivacaftor corrects the cystic fibrosis transmembrane conductance regulator
(CFTR) gating defect associated with G551D mutation and is quickly becoming an important …

[HTML][HTML] Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis

E DiMango, J Overdevest, C Keating, SF Francis… - Journal of Cystic …, 2021 - Elsevier
Background Elexacaftor-tezacaftor-ivacaftor is a highly effective modulator for cystic fibrosis
(CF) patients homozygous or heterozygous for F508del. Effects of the drug on sinonasal …

The French compassionate programme of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant

PR Burgel, I Sermet-Gaudelus, I Durieu… - European …, 2023 - Eur Respiratory Soc
Background The European Medicines Agency has approved the cystic fibrosis
transmembrane conductance regulator (CFTR) modulator combination …

Effects of elexacaftor/tezacaftor/ivacaftor therapy in children with cystic fibrosis–a comprehensive assessment using lung clearance index, spirometry, and functional …

C Streibel, CC Willers, O Pusterla, G Bauman… - Journal of cystic …, 2023 - Elsevier
Background With improvement in supportive therapies and the introduction of cystic fibrosis
transmembrane conductance regulator (CFTR)-modulator treatment in patients with cystic …