Improvement of Hepatic Steatosis in Cystic Fibrosis With Ivacaftor Therapy

被引：40

作者：

Hayes, Don, Jr. ^{[1
]}

Warren, Patrick S. ^{[2
]}

McCoy, Karen S. ^{[1
]}

Sheikh, Shahid I. ^{[1
]}

机构：

[1] Ohio State Univ, Coll Med, Dept Pediat, Columbus, OH 43205 USA

[2] Ohio State Univ, Coll Med, Dept Radiol, Columbus, OH 43205 USA

来源：

JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION | 2015年 / 60卷 / 05期

关键词：

cystic fibrosis; G551D; hepatic steatosis; ivacaftor; LIVER-DISEASE;

D O I：

10.1097/MPG.0000000000000765

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

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.

引用

页码：578 / 579

页数：2

共 5 条

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