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 …

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles

SY Graeber, C Vitzthum, ST Pallenberg… - American journal of …, 2022 - atsjournals.org
Rationale: The CFTR (cystic fibrosis transmembrane conductance regulator) modulator
combination elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to improve clinical …

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] A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of …

JC Davies, I Sermet-Gaudelus, L Naehrlich… - Journal of Cystic …, 2021 - Elsevier
Background The CFTR modulator tezacaftor/ivacaftor was efficacious and generally safe
and well tolerated in Phase 3 studies in participants≥ 12 years of age with cystic fibrosis …

Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for 24 Weeks or Longer in People with Cystic Fibrosis and One or More F508del Alleles: Interim Results of an …

M Griese, S Costa, RW Linnemann… - American journal of …, 2021 - atsjournals.org
Methods Participants who completed the last visit of the 24-week treatment period in the
F/MF pivotal study (NCT03525444) or of the 4-week treatment period in the F/F pivotal study …

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 …

Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function …

MA Mall, R Brugha, S Gartner, J Legg… - American journal of …, 2022 - atsjournals.org
Rationale: The triple-combination regimen elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA)
was shown to be safe and efficacious in children aged 6 through 11 years with cystic fibrosis …

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 …

[HTML][HTML] Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a …

CL Daines, E Tullis, S Costa… - European …, 2023 - Eur Respiratory Soc
Background In two pivotal phase 3 trials, up to 24 weeks of treatment with
elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious and safe in patients with cystic …

Effects of lumacaftor–ivacaftor on lung clearance index, magnetic resonance imaging, and airway microbiome in Phe508del homozygous patients with cystic fibrosis

SY Graeber, S Boutin, MO Wielpütz… - Annals of the …, 2021 - atsjournals.org
Rationale: Previous studies showed that lumacaftor–ivacaftor therapy results in partial
rescue of CFTR (cystic fibrosis [CF] transmembrane conductance regulator) activity and a …