Article
Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jan 2021
Gelzo Monica, Iacotucci Paola, Caputo Mafalda, Cernera Gustavo, Comegna Marika, Carnovale Vincenzo, Corso Gaetano, Castaldo Giuseppe
Abstract excerpt
BACKGROUND: Cystic fibrosis (CF) patients have reduced intestinal absorption of sterols and, despite enhanced endogenous synthesis, low plasma cholesterol. Lumacaftor/ivacaftor CFTR protein modulator therapy is used to improve the clinical outcome of CF patients homozygous for F508del mutation (homo-deltaF508). Aim of the study is to evaluate the cholesterol metabolism and hepatobiliary injury/function in adult...
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