Improvement of hepatic steatosis in cystic fibrosis with ivacaftor therapy

J Pediatr Gastroenterol Nutr. 2015 May;60(5):578-9. doi: 10.1097/MPG.0000000000000765.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aminophenols / therapeutic use*
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / drug therapy*
  • Fatty Liver / diagnostic imaging
  • Fatty Liver / drug therapy*
  • Fatty Liver / etiology
  • Female
  • Humans
  • Quinolones / therapeutic use*
  • Radiography


  • Aminophenols
  • Quinolones
  • ivacaftor