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淤胆型肝损伤的临床与病理
Clinical and pathological features of cholestatic liver injury
文章发布日期:2018年09月30日  来源:  作者:刘杜先,刘兰侠,杨永峰  点击次数:293次  下载次数:99次

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【摘要】:胆汁淤积可因胆汁代谢异常、胆汁流动及回流不畅、胆管损伤及梗阻等原因引起。胆汁代谢异常往往由遗传基因异常引起,而胆汁流通不畅往往由胆管炎所致的损伤或胆道阻塞引发。胆汁代谢异常以进行性家族性胆汁淤积(PFIC)最为常见,而胆管损伤性疾病以自身免疫性胆管炎为主要原因。先天性/自身免疫性胆管损伤以及继发性/获得性胆管反应的病理组织学改变,均具有相对特异性,疾病的最终确诊常需要结合临床、病理学、影像学、免疫学、遗传学、基因检测等综合分析。重点阐述了自身免疫性胆管损伤和PFIC病理形态学方面的研究进展,引申介绍了其他类似病变,以期对胆管损伤性、胆汁淤积性肝病的临床诊断提供参考。
【Abstract】:Major causes of cholestasis include abnormal bile metabolism, obstructed bile flow and regurgitation, and bile duct injury and obstruction. Abnormal bile metabolism is mainly caused by gene abnormality, while obstructed bile flow is often caused by bile duct injury or occlusion due to cholangitis. Abnormal bile metabolism is the most common cause of progressive familial intrahepatic cholestasis (PFIC), while autoimmune cholangitis is the major cause of bile duct injury. Both congenital/autoimmune bile duct injury and secondary/acquired ductular reaction had relatively specific histopathological changes, and a confirmed diagnosis needs a comprehensive analysis based on clinical, pathological, imaging, immunological, and genetic examinations. This article elaborates on the research advances in the pathomorphology of autoimmune bile duct injury and PFIC and introduces the similar lesions, in order to provide a reference for the clinical diagnosis of bile duct injury and cholestatic liver diseases.
【关键字】:肝疾病; 胆汁淤积; 疾病特征; 病理学
【Key words】:liver diseases; cholestasis; disease attributes; pathology
【引证本文】:LIU DX, LIU LX, YANG YF. Clinical and pathological features of cholestatic liver injury[J]. J Clin Hepatol, 2018, 34(11): 2283-2288. (in Chinese) 刘杜先, 刘兰侠, 杨永峰. 淤胆型肝损伤的临床与病理[J]. 临床肝胆病杂志, 2018, 34(11): 2283-2288.

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