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.

doi:10.1016/j.jcf.2022.12.012

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Article

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 : official journal of the European Cystic Fibrosis Society - 1 Jul 2023

Streibel Carmen, Willers Corin C, Pusterla Orso, Bauman Grzegorz, Stranzinger Enno, Brabandt Ben, Bieri Oliver, Curdy Marion, Bullo Marina, Frauchiger Bettina Sarah, Korten Insa, Krüger Linn, Casaulta Carmen, Ratjen Felix, Latzin Philipp, Kieninger Elisabeth

Abstract excerpt

BACKGROUND: With improvement in supportive therapies and the introduction of cystic fibrosis transmembrane conductance regulator (CFTR)-modulator treatment in patients with cystic fibrosis (CF), milder disease courses are expected. Therefore, sensitive parameters are needed to monitor disease course and effects of CFTR-modulators. Functional lung MRI using matrix-pencil decomposition (MP-MRI) is a promising tool...

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PMID: 36635199
DOI: 10.1016/j.jcf.2022.12.012

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