Effects of elexacaftor/tezacaftor/ivacaftor therapy in children with cystic fibrosis – a comprehensive assessment using lung clearance index, spirometry, and functional and structural lung MRI

Journal of Cystic Fibrosis(2023)

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摘要
•Structural and functional MP-MRI, LCI and FEV1 were compared after ETI treatment.•Ventilation and perfusion impairment assessed by MP-MRI improved upon ETI therapy.•MP-MRI adds information on ETI treatment effects complementary to FEV1 and LCI.•MP-MRI is a suitable and sensitive tool to monitor CFTR-modulator treatment effects.
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关键词
Cystic fibrosis,Lung disease,Children,CFTR modulator therapy,Elexacaftor/tezacaftor/ivacaftor therapy,Lung function,Multiple breath washout,Lung clearance index,Functional lung MRI
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