Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR

BACKGROUND. The ability to restore cystic fibrosis transmembrane regulator (CFTR) function with effective small molecule modulators in patients with cystic fibrosis provides an opportunity to study relationships between CFTR ion channel function, organ level physiology, and clinical outcomes. METHOD...

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Detalhes bibliográficos
Publicado no:JCI Insight
Main Authors: Donaldson, Scott H., Laube, Beth L., Corcoran, Timothy E., Bhambhvani, Pradeep, Zeman, Kirby, Ceppe, Agathe, Zeitlin, Pamela L., Mogayzel, Peter J., Boyle, Michael, Locke, Landon W., Myerburg, Michael M., Pilewski, Joseph M., Flanagan, Brian, Rowe, Steven M., Bennett, William D.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 2018
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6338313/
https://ncbi.nlm.nih.gov/pubmed/30568035
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/jci.insight.122695
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