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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...
Autores principales: | , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BMJ Publishing Group
2018
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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 |