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- W3157453885 abstract "New treatments targeting the underlying defect in cystic fibrosis (CF) have dramatically improved outcomes for select cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations. Highly effective modulators are available for ivacaftor-responsive variants (10-15% of CF patients) [ [1] Ramsey BW Davies J McElvaney NG Tullis E Bell SC Drevinek P et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011; 365: 1663-1672 Crossref PubMed Scopus (1650) Google Scholar ] and/or a copy of the most common CFTR variant, F508del (up to 85%) [ [2] Davies JC Moskowitz SM Brown C Horsley A Mall MA McKone EF et al. VX-659-Tezacaftor-Ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles. N Engl J Med. 2018; 379: 1599-1611 Crossref PubMed Scopus (224) Google Scholar , [3] Keating D Marigowda G Burr L Daines C Mall MA McKone EF et al. VX-445-Tezacaftor-Ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles. N Engl J Med. 2018; 379: 1612-1620 Crossref PubMed Scopus (394) Google Scholar ]. In-vitro CFTR function quantification and modulation using human nasal epithelial (HNE) cells indicate clear and direct relationships between in-vitro and short-term in-vivo responses (e.g.: CFTR-dependent chloride transport, mucociliary clearance, lung function, sweat chloride concentration) [ 4 Pranke IM Hatton A Simonin J Jais JP Le Pimpec-Barthes F Carsin A et al. Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators. Sci Rep. 2017; 7: 7375 Crossref PubMed Scopus (97) Google Scholar , 5 Solomon GM Liu Z. Baines A. Heltshe S. Guimbellot J.S. Joseloff E. Tearney G.J. Sagel S. Clancy J.P. Rowe S.M. In vitro responses to ivacaftor in G551D and R117H human nasal epithelial cells correlate to clinical improvement with ivacaftor treatment. Peds Pulm. 2017; 52: S47 Google Scholar , 6 Brewington JJ Filbrandt ET LaRosa 3rd, FJ Moncivaiz JD Ostmann AJ Strecker LM et al. Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies. JCI Insight. 2018; 3 Crossref Scopus (55) Google Scholar ]. However, early treatment responses do not consistently predict long-term modulator benefits [ [7] Heltshe SL Rowe SM Skalland M Baines A Jain M GIotCFFTD Network Ivacaftor-treated patients with cystic fibrosis derive long-term benefit despite no short-term clinical improvement. Am J Respir Crit Care Med. 2018; 197: 1483-1486 Crossref PubMed Scopus (17) Google Scholar ]." @default.
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- W3157453885 date "2021-09-01" @default.
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- W3157453885 title "Rapid cystic fibrosis lung-function decline and in-vitro CFTR modulation" @default.
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- W3157453885 doi "https://doi.org/10.1016/j.jcf.2021.04.001" @default.
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