CFTR modulator therapy: transforming the landscape of clinical care in cystic fibrosis
JL Taylor-Cousar, PD Robinson, M Shteinberg… - The Lancet, 2023 - thelancet.com
Following discovery of the cystic fibrosis transmembrane conductance regulator (CFTR)
gene in 1989 and subsequent elucidation of the varied CFTR protein abnormalities that …
gene in 1989 and subsequent elucidation of the varied CFTR protein abnormalities that …
Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy
Cystic fibrosis is a multiorgan disease caused by impaired function of the cystic fibrosis
transmembrane conductance regulator (CFTR). Since the introduction of the CFTR …
transmembrane conductance regulator (CFTR). Since the introduction of the CFTR …
Sex differences in outcomes of people with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor
A Wang, MJ Lee, A Keller, S Jian, K Lowe… - Journal of Cystic …, 2024 - Elsevier
Background There is a well described sex-disparity in outcomes of individuals with cystic
fibrosis (CF), with females faring worse than males. Given the dramatic improvement in …
fibrosis (CF), with females faring worse than males. Given the dramatic improvement in …
Real‐world data confirm elexacftor/tezacaftor/ivacaftor modulators halves sweat chloride concentration in eligible people with cystic fibrosis
Sweat chloride concentration, a diagnostic feature in cystic fibrosis (CF), reflects CF
transmembrane conductance regulator (CFTR) activity. CFTR modulator therapies …
transmembrane conductance regulator (CFTR) activity. CFTR modulator therapies …
Elexacaftor/tezacaftor/ivacaftor, a game-changer in cystic fibrosis: The Portuguese experience
Background Phase 3 trials of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) combination
treatment in people with cystic fibrosis (CF) with≥ 1 F508del-CFTR allele showed profound …
treatment in people with cystic fibrosis (CF) with≥ 1 F508del-CFTR allele showed profound …
Heterogeneity of CFTR modulator-induced sweat chloride concentrations in people with cystic fibrosis
ET Zemanick, I Emerman, M McCreary… - Journal of Cystic …, 2024 - Elsevier
Background Sweat chloride (SC) concentrations in people with cystic fibrosis (PwCF) reflect
relative CF transmembrane conductance regulator (CFTR) protein function, the primary CF …
relative CF transmembrane conductance regulator (CFTR) protein function, the primary CF …
SCREENING FOR LOWER RESPIRATORY TRACT INFECTIONS IN CHILDREN WITH CYSTIC FIBROSIS
OHR Ali, EM Fouda, DH Abdelhamid… - Ain Shams Medical …, 2023 - journals.ekb.eg
Background: Cystic fibrosis (CF) is a chronic autosomal recessive disease caused by CF
transmembrane conductance regulator (CFTR) gene mutations, which encodes the CFTR …
transmembrane conductance regulator (CFTR) gene mutations, which encodes the CFTR …