title: Improvement of hepatic steatosis in cystic fibrosis with ivacaftor therapy.
abstract: Treatment of liver disease, including hepatic steatosis, in patients with cystic fibrosis (CF) is limited. With the development of ivacaftor, which corrects the gating defect of the CF transmembrane regulator channel, there is a potential new therapy available for this subgroup of the CF patient population. We present an adolescent with CF who had significant improvement in hepatic steatosis with ivacaftor treatment while hypothesizing on a mechanism of why it occurred.
authors: *Department of Pediatrics †Department of Radiology, Ohio State University College of Medicine, Columbus.
authors: Don
authors: D
authors: Hayes
authors: Patrick S
authors: PS
authors: Warren
authors: Karen S
authors: KS
authors: McCoy
authors: Shahid I
authors: SI
authors: Sheikh
mesh_terms: D000293:Adolescent; D000627:Aminophenols / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000150:complications / Q000188:drug therapy*; D005234:Fatty Liver / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D005260:Female; D006801:Humans; D015363:Quinolones / Q000627:therapeutic use*; D011859:Radiography
chemical_list: D000627:Aminophenols; D015363:Quinolones; C545203:ivacaftor