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Article

Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2022

Drummond David, Dana Jérémy, Berteloot Laureline, Schneider-Futschik Elena K, Chedevergne Frédérique, Bailly-Botuha Céline, Nguyen-Khoa Thao, Cornet Mathieu, Le Bourgeois Muriel, Debray Dominique, Girard Muriel, Sermet-Gaudelus Isabelle

Abstract excerpt

BACKGROUND: The effects of lumacaftor-ivacaftor on cystic fibrosis transmembrane conductance regulator (CFTR)-associated liver disease remain unclear. The objective of the study was to describe the effect of this treatment on features of liver involvement in a cystic fibrosis (CF) adolescent population homozygous for F508del. METHODS: Clinical characteristics, liver blood tests, abdominal ultrasonography (US),...

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Identifiers

PMID: 34454846
DOI: 10.1016/j.jcf.2021.07.018

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