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

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

Abstract Background Data from clinical trials of lumacaftor-ivacaftor (LUM-IVA) demonstrate improvements in lung clearance index (LCI) but not in FEV1 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...

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Main Authors: Paul McNally, Barry Linnane, Michael Williamson, Basil Elnazir, Christopher Short, Clare Saunders, Laura Kirwan, Rea David, Mariette P. C. Kemner-Van de Corput, Harm A.W.M. Tiddens, Jane C Davies, Des W Cox
Format: Article
Language:English
Published: BMC 2023-08-01
Series:Respiratory Research
Subjects:
Lung clearance index
PRAGMA CF scores
Children
Real-world studies
Online Access:https://doi.org/10.1186/s12931-023-02497-0

Internet

https://doi.org/10.1186/s12931-023-02497-0

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