Article
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.
Respiratory research - 11 Aug 2023
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
Abstract excerpt
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...
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