血浆血管性血友病因子裂解酶在肝脏疾病发病机制和诊断中的作用  

Role of ADAMTS13 in diagnosis and pathogenesis of liver diseases

作　　者：许姗姗[1] 陈亚利[1] 张晶[1] 

机构地区：[1]首都医科大学附属北京佑安医院,北京100069

出　　处：《临床肝胆病杂志》2016年第2期390-392,共3页Journal of Clinical Hepatology

基　　金：北京市教育委员会科技计划面上项目(KM201310025008);北京市属高等学校高层次人才引进与培养计划项目(CIT&TCD201304178)

摘　　要：近期研究表明机体凝血功能和肝脏微循环障碍在肝脏疾病的发生、发展过程中起着重要作用。ADAMTS13又称血管性血友病因子裂解酶,是由肝星状细胞产生的金属蛋白酶,主要功能是裂解血管内皮细胞产生的血管性血友病因子多聚体,从而调节血小板粘附能力,进而影响机体的凝血功能和微循环。回顾ADAMTS13在多种重症肝病如肝硬化、重症酒精性肝炎、干细胞移植后肝静脉阻塞病和移植肝功能障碍等病理生理过程中发挥的重要作用,阐述了其与肝脏疾病严重程度的关系,认为血浆ADAMTS13参与了多种肝脏疾病的发生、发展过程,通过检测其水平和活性有助于肝脏疾病的诊断及鉴别诊断。Recent studies have demonstrated that coagulation function and liver microcirculation disturbance are important in the develop- ment and progression of liver diseases. ADAMTS13, also known as yon Willebrand factor- cleaving protease, is a metalloproteinase pro- duced by hepatic stellate cells, with major functions to cleave the yon Willebrand factor muhimers produced by vascular endothelial cells, regulate the adhesive capacity of platelets, and influence the body＇s coagulation function and microcirculation. The important role of AD- AMTS13 in the pathophysiological processes of various severe liver diseases such as liver cirrhosis, severe alcoholic hepatitis, hepatic veno - occlusive disease after stem cell transplantation, and liver graft dysfunction is reviewed in this paper, and its association with the severity of liver diseases is clarified. Plasma ADAMTS13 is involved in the development and progression of various liver diseases, and measurement of its level and activity can help the diagnosis and differential diagnosis of liver diseases.

关 键 词：肝疾病 von WILLEBRAND因子 金属蛋白酶类 综述 

分 类 号：R575[医药卫生—消化系统]