Journal
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
Volume 60, Issue 5, Pages 578-579Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPG.0000000000000765
Keywords
cystic fibrosis; G551D; hepatic steatosis; ivacaftor
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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.
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