期刊
HEPATIC MEDICINE-EVIDENCE AND RESEARCH
卷 10, 期 -, 页码 95-104出版社
DOVE MEDICAL PRESS LTD
DOI: 10.2147/HMER.S137209
关键词
intrahepatic cholestasis; jaundice; biliary diversion
Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessive cholestatic liver diseases which are subgrouped according to the genetic defect, clinical presentation, laboratory findings and liver histology. Progressive liver fibrosis, cirrhosis, and end stage liver disease (ESLD) may eventually develop. PFIC was first described in Amish descendants of Jacob Byler, therefore it was originally called Byler disease. But it can be seen anywhere on the globe. This review summarizes the main features of the subtypes of the disease and discusses the current available diagnosis, conservative and surgical therapeutic options.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据