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Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction

BACKGROUND: Treatment of patients with Cystic Fibrosis homozygous for the Phe508del gene, with Lumacaftor /Ivacaftor (LUM/IVA) improves outcomes in patients with FEV1 > 40% predicted. We set out to observe the most sensitive clinical measure that would change with treatment in terms of exercise c...

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Detalles Bibliográficos
Autores principales: Wark, Peter A. B., Cookson, Kim, Thiruchelvam, Theeba, Brannan, John, Dorahy, Douglas J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6580585/
https://www.ncbi.nlm.nih.gov/pubmed/31208380
http://dx.doi.org/10.1186/s12890-019-0866-y