Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update.
Sign Up to like & getrecommendations! Published in 2020 at "Current Opinion in Pediatrics"
DOI: 10.1097/mop.0000000000000892
Abstract: PURPOSE OF REVIEW Cystic fibrosis transmembrane conductance receptor (CFTR) modulators are a new class of drugs that treat the underlying cause of cystic fibrosis. To date, there are four approved medications, which are mutation-specific. Although… read more here.
Keywords: modulator therapy; cystic fibrosis; fibrosis; cftr modulators ... See more keywords
Large conducting airway luminal area is not different after initiating cystic fibrosis transmembrane conductance regulatory modulator therapy
Sign Up to like & getrecommendations! Published in 2024 at "Physiology"
DOI: 10.1152/physiol.2024.39.s1.455
Abstract: Background: Cystic fibrosis (CF) is a genetic disorder in which patients have dysfunctional cystic fibrosis transmembrane regulatory (CFTR) proteins in the lungs that disrupt ion regulation resulting in a thick, viscous mucus layer on the… read more here.
Keywords: modulator therapy; cftr modulator; modulator; luminal area ... See more keywords
Prenatal cystic fibrosis transmembrane conductance regulator modulator therapy: A promising way to change the impact of cystic fibrosis.
Sign Up to like & getrecommendations! Published in 2023 at "Fetal diagnosis and therapy"
DOI: 10.1159/000530261
Abstract: INTRODUCTION Cystic fibrosis (CF) is a potentially severe disease. The development of new therapies with cystic fibrosis transmembrane conductance regulator (CFTR) modulators has been a great advance in the management of this condition because they… read more here.
Keywords: fibrosis transmembrane; modulator therapy; fibrosis; cystic fibrosis ... See more keywords
Multi-center Study of Hyperpolarized Xenon MRI in Children with Cystic Fibrosis Following Initiation of CFTR Modulator Therapy (HyPOINT).
Sign Up to like & getrecommendations! Published in 2025 at "Annals of the American Thoracic Society"
DOI: 10.1513/annalsats.202501-028oc
Abstract: RATIONALE Elexacaftor/tezacaftor/ivacaftor (ETI) has significantly improved lung function in people with cystic fibrosis (CF), prompting the need for outcome measures that can detect mild disease. In this new era of CFTR modulator therapy, more sensitive… read more here.
Keywords: cystic fibrosis; modulator therapy; cftr modulator; initiation ... See more keywords