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However, real-world data on the effect of ETI on these sensitive outcomes of lung structure and function in school-age children with CF have not been reported. The aim of this study was therefore to examine the effect of ETI on the LCI and the lung MRI score in children aged 6\u201311\u2005years with CF and one or two<jats:italic>F508del<\/jats:italic>alleles.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This prospective, observational, multicentre, post-approval study assessed the longitudinal LCI up to 12\u2005months and the lung MRI score before and 3\u2005months after initiation of ETI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 107 children with CF including 40 heterozygous for<jats:italic>F508del<\/jats:italic>and a minimal function mutation (F\/MF) and 67 homozygous for<jats:italic>F508del<\/jats:italic>(F\/F) were enrolled in this study. Treatment with ETI improved the median (interquartile range (IQR)) LCI in F\/MF (\u22121.0 (\u22122.0\u2013\u2009\u22120.1); p&lt;0.01) and F\/F children (\u22120.8 (\u22121.9\u2013\u2009\u22120.2); p&lt;0.001) from 3\u2005months onwards. Further, ETI improved the median (IQR) MRI global score in F\/MF (\u22124.0 (\u22129.0\u20130.0); p&lt;0.01) and F\/F children (\u22123.5 (\u22127.3\u2013\u2009\u22120.8); p&lt;0.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>ETI improves early abnormalities in lung ventilation and morphology in school-age children with CF and at least one<jats:italic>F508del<\/jats:italic>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.<\/jats:p><\/jats:sec>","DOI":"10.1183\/13993003.00004-2024","type":"journal-article","created":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T14:55:15Z","timestamp":1718895315000},"page":"2400004","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":46,"title":["Impact of elexacaftor\/tezacaftor\/ivacaftor therapy on lung clearance index and magnetic resonance imaging in children with cystic fibrosis and one or 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