Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis

Among a myriad of clinical alterations, most patients with cystic fibrosis (CF) have low plasma cholesterol due to intestinal malabsorption, despite the pancreatic enzyme supplementation [1,2]. In particular, we previously found that CF patients with pancreatic insufficiency (PI) have reduced plasma phytosterols levels, markers of intestinal absorption of cholesterol, that were positively correlated with vitamin E [2]. The serum level of vitamin E represents an indirect pancreatic function test that allows to evaluate the intestinal digestion/absorption of lipids [3].

Source: Journal of Cystic Fibrosis - June 21, 2020 Category: Respiratory Medicine Authors: Monica Gelzo, Paola Iacotucci, Mafalda Caputo, Gustavo Cernera, Marika Comegna, Vincenzo Carnovale, Gaetano Corso, Giuseppe Castaldo Source Type: research