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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

2023-02-27

Abstract excerpt

<h4>Background: </h4> Data from clinical trials of lumacaftor-ivacaftor (LUM-IVA) demonstrate improvements in lung clearance index (LCI) but not in FEV 1 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...

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Literature Corpus work
86271a63-a7be-514c-90eb-b680fb7e07da
DOI
10.21203/rs.3.rs-2582978/v1
Open publication

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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 settingDOI 10.21203/rs.3.rs-2582978/v1
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