Left behind: the potential impact of CFTR modulators on racial and ethnic disparities in cystic fibrosis

ME McGarry, ER Gibb, GR Oates… - Paediatric respiratory …, 2022 - Elsevier
The advent of CFTR modulators, a genomic specific medication, revolutionized the treatment
of CF for many patients. However, given that these therapeutics were only developed for …

Current state of CFTR modulators for treatment of Cystic Fibrosis

KA Despotes, SH Donaldson - Current Opinion in Pharmacology, 2022 - Elsevier
Small molecular modulators of the cystic fibrosis transmembrane conductance regulator
protein are transforming the care of people with cystic fibrosis. Highly effective modulators …

[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 …

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

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 …

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 …

[HTML][HTML] Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis

KW Southern, C Castellani, E Lammertyn… - Journal of Cystic …, 2023 - Elsevier
Cystic fibrosis (CF) has entered the era of variant-specific therapy, tailored to the genetic
variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR …

Impact of cystic fibrosis transmembrane conductance regulator therapy on chronic rhinosinusitis and health status: deep learning CT analysis and patient-reported …

DM Beswick, SM Humphries… - Annals of the …, 2022 - atsjournals.org
Rationale: Elexacaftor, tezacaftor, and ivacaftor (ETI) in triple combination improves
pulmonary health for people with cystic fibrosis (PwCF). However, its impact on objective …

Effects of elexacaftor/tezacaftor/ivacaftor therapy on mental health of patients with cystic fibrosis

L Piehler, R Thalemann, C Lehmann, S Thee… - Frontiers in …, 2023 - frontiersin.org
Introduction: The CFTR modulator drug elexacaftor/tezacaftor/ivacaftor (ETI) was shown to
improve CFTR function and clinical symptoms in patients with cystic fibrosis (CF) with at …

Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients

KJ Ramos, JS Guimbellot, M Valapour, LE Bartlett… - Journal of Cystic …, 2022 - Elsevier
Background Cystic fibrosis (CF) lung transplant (LT) recipients may warrant treatment with
elexacaftor/tezacaftor/ivacaftor (ETI) to improve extrapulmonary manifestations of CF. Our …

Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations

AJ Kimple, BA Senior, ET Naureckas… - International forum of …, 2022 - Wiley Online Library
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 …