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Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor

BACKGROUND: Ivacaftor is the first cystic fibrosis transmembrane conductance regulator (CFTR) modulator demonstrating clinical benefit in patients with cystic fibrosis (CF). As ivacaftor is intended for chronic, lifelong use, understanding long-term effects is important for patients and healthcare p...

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Detalles Bibliográficos
Autores principales: Bessonova, Leona, Volkova, Nataliya, Higgins, Mark, Bengtsson, Leif, Tian, Simon, Simard, Christopher, Konstan, Michael W, Sawicki, Gregory S, Sewall, Ase, Nyangoma, Stephen, Elbert, Alexander, Marshall, Bruce C, Bilton, Diana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204955/
https://www.ncbi.nlm.nih.gov/pubmed/29748252
http://dx.doi.org/10.1136/thoraxjnl-2017-210394