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 impact the progression of structural lung disease. We sought to determine the real-world impact of LUM/IVA on lung structure and function in children aged 6-11 years.
Methods:
This real-world observational cohort study was… Show more
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