Late-onset Cholestasis with Paucity of Portal Area Secondary   

作　　者：Xuemei Zhang Kun Liu Xiaona Lu Wenlan Zheng Jia Shi Shihan Yu Hai Feng Zhuo Yu 

机构地区：[1]Department of Hepatopathy,Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine,Shanghai,China [2]Department of Pathology,Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine,Shanghai,China [3]Institute of Infectious Disease,Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine,Shanghai,China

出　　处：《Journal of Clinical and Translational Hepatology》2024年第3期327-331,共5页临床与转化肝病杂志（英文版）

基　　金：supported by National Natural Science Foun-dation of China(Nos.82222074,82074154,81774240);Siming Scholar from Shanghai Shuguang Hospital(No.SGXZ-201904);Shuguang Program of Shanghai Education Development Foundation and Shanghai Municipal Education Commission,Youth Tip-top Talent Program,Constant-emi-nent Program in Shanghai,Xinglin Youth Scholar Program from Shanghai University of Traditional Chinese Medicine.

摘　　要：Hepatocyte nuclear factor 1β(HNF1β)is essential for biliary development,while its genetic defect triggers the dysplasia of interlobular bile ducts,leading to life-threatening hepatitis and cholestasis.To date,this disorder has mainly been docu-mented in neonates.Here,we report a case of cholestasis in an adult patient caused by a de novo HNF1β mutation.A liver biopsy revealed remarkable shrinkage of the portal area accompanied by a decrease or absence of interlobular bile ducts,veins,and arteries in the portal area.Our case showed that an HNF1β defect could induce late-onset cholestasis with paucity of the portal area in adulthood.

关 键 词：HNF1β CHOLESTASIS Adult patient Paucity of the portal area Renal cysts Case report 

分 类 号：R575[医药卫生—消化系统]