Hayes Don, Warren Patrick S, McCoy Karen S, Sheikh Shahid I
*Department of Pediatrics †Department of Radiology, Ohio State University College of Medicine, Columbus.
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.
囊性纤维化（CF）患者的肝病治疗，包括肝脂肪变性，方法有限。随着依伐卡托的研发，它可纠正囊性纤维化跨膜传导调节因子通道的门控缺陷，为这一亚组CF患者群体提供了一种潜在的新疗法。我们报告了一名患有CF的青少年，其肝脂肪变性在接受依伐卡托治疗后有显著改善，同时对其发生机制进行了推测。
