Progressive familial intrahepatic cholestasis

查看全文 作　　者：Tomohide [1]Hori;Justin [2]H.Nguyen;Shinji [3]Uemoto

机构地区：[1]Department of Neuroscience Mayo Clinic in Florida;[2]Division of Transplant Surgery,Department of Transplantation Mayo Clinic in Florida;[3]Divisions of Hepato-Biliary-Pancreatic,Pediatric and Transplant Surgery,Department of Surgery,Kyoto University Hospital

出　　处：《Hepatobiliary & Pancreatic Diseases International》索引2010年第9卷第6期,共9页

摘　　要：BACKGROUND:Three types of progressive familial intrahepatic cholestasis(PFIC)have been identified,but their etiologies include unknown mechanisms. DATA SOURCES:A PubMed search on'progressive familial intrahepatic cholestasis'and'PFIC'was performed on the topic,and the relevant articles were reviewed. RESULTS:The etiologies of the three PFIC types still include unknown mechanisms.Especially in PFIC type 1,enterohepatic circulation of bile acid should be considered.Ursodeoxycholic acid,partial external biliary diversion and liver transplantation have been used for the treatment of PFIC patients according to disease course. CONCLUSIONS:Since the etiologies and disease mechanisms of PFIC are still unclear,detailed studies are urgently required. Strategies for more advanced therapies are also needed.These developments in the future are indispensable,especially for PFIC type 1 patients. 关键词：PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS Byler’s disease liver TRANSPLANTATION STEATOSIS

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Progressive familial intrahepatic cholestasis

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