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The clinical impact of Lumacaftor-Ivacaftor on structural lung disease and lung function in children aged 6–11 with cystic fibrosis in a real-world setting

BACKGROUND: Data from clinical trials of lumacaftor-ivacaftor (LUM-IVA) demonstrate improvements in lung clearance index (LCI) but not in FEV(1) in children with Cystic Fibrosis (CF) aged 6–11 years and homozygous for the Phe508del mutation. It is not known whether LUM/IVA use in children can impact...

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Detalles Bibliográficos
Autores principales:	McNally, Paul, Linnane, Barry, Williamson, Michael, Elnazir, Basil, Short, Christopher, Saunders, Clare, Kirwan, Laura, David, Rea, Kemner-Van de Corput, Mariette P. C., Tiddens, Harm A.W.M., Davies, Jane C, Cox, Des W
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	BioMed Central 2023
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10416528/ https://www.ncbi.nlm.nih.gov/pubmed/37568199 http://dx.doi.org/10.1186/s12931-023-02497-0

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