Impact of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in children with cystic fibrosis and one or two<i>F508del</i>alleles

Abstract

ETI improves early abnormalities in lung ventilation and morphology in school-age children with CF and at least one <i>F508del</i> allele in a real-world setting. Our results support early initiation of ETI to reduce or even prevent lung disease progression in school-age children with CF.