Fickert Peter, Pollheimer Marion J, Beuers Ulrich, Lackner Carolin, Hirschfield Gideon, Housset Chantal, Keitel Verena, Schramm Christoph, Marschall Hanns-Ulrich, Karlsen Tom H, Melum Espen, Kaser Arthur, Eksteen Bertus, Strazzabosco Mario, Manns Michael, Trauner Michael
Research Unit for Experimental and Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Austria; Institute of Pathology, Medical University of Graz, Austria.
Research Unit for Experimental and Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Austria; Institute of Pathology, Medical University of Graz, Austria.
J Hepatol. 2014 Jun;60(6):1290-303. doi: 10.1016/j.jhep.2014.02.006. Epub 2014 Feb 19.
Primary sclerosing cholangitis (PSC) is a chronic cholangiopathy characterized by biliary fibrosis, development of cholestasis and end stage liver disease, high risk of malignancy, and frequent need for liver transplantation. The poor understanding of its pathogenesis is also reflected in the lack of effective medical treatment. Well-characterized animal models are utterly needed to develop novel pathogenetic concepts and study new treatment strategies. Currently there is no consensus on how to evaluate and characterize potential PSC models, which makes direct comparison of experimental results and effective exchange of study material between research groups difficult. The International Primary Sclerosing Cholangitis Study Group (IPSCSG) has therefore summarized these key issues in a position paper proposing standard requirements for the study of animal models of PSC.
原发性硬化性胆管炎（PSC）是一种慢性胆管病，其特征为胆管纤维化、胆汁淤积和终末期肝病的发展、恶性肿瘤风险高以及频繁需要肝移植。对其发病机制的了解不足也体现在缺乏有效的药物治疗上。迫切需要特征明确的动物模型来发展新的发病机制概念并研究新的治疗策略。目前，关于如何评估和表征潜在的PSC模型尚无共识，这使得研究小组之间直接比较实验结果和有效交换研究材料变得困难。因此，国际原发性硬化性胆管炎研究小组（IPSCSG）在一份立场文件中总结了这些关键问题，提出了PSC动物模型研究的标准要求。
