Increase of liver stiffness and altered bile acid metabolism after triple CFTR modulator initiation in children and young adults with cystic fibrosis.

doi:10.1111/liv.15544

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Article

Increase of liver stiffness and altered bile acid metabolism after triple CFTR modulator initiation in children and young adults with cystic fibrosis.

Liver international : official journal of the International Association for the Study of the Liver - 1 Apr 2023

Schnell Alexander, Jüngert Jörg, Klett Daniel, Hober Hannah, Kaiser Natalie, Ruppel Renate, Geppert Annika, Tremel Christina, Sobel Julia, Plattner Erika, Schmitt-Grohé Sabina, Zirlik Sabine, Strobel Deike, Neurath Markus F, Knieling Ferdinand, Rauh Manfred, Woelfle Joachim, Hoerning André, Regensburger Adrian P

Abstract excerpt

BACKGROUND: Novel cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies (elexacaftor/tezacaftor/ivacaftor-ETI) promise clinically significant and sustained improvements for patients with cystic fibrosis (CF). In this study, we investigated the impact of ETI therapy on liver stiffness and bile acid metabolism in a cohort of children and young adults with CF. METHODS: A prospective...

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PMID: 36797990
DOI: 10.1111/liv.15544

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