血管性血友病因子裂解酶在肾病综合征患者血浆中的变化及其临床意义

Clinical Significance of ADAMTS13 in Patients with Nephrotic Syndrome

目的检测血管性血友病因子裂解酶(vWF-cp,亦称ADAMTS13)与血管性血友病因子(vWF)在肾病综合征(NS)患者血浆中的抗原水平并探讨其临床意义。方法将70例NS患者分为膜性肾病(MN)组和非膜性肾病(non-MN)组,用ELISA法检测MN组、non-MN组和正常对照组血浆ADAMTS13和vWF抗原,比较各组间AD-AMTS13抗原、vWF抗原及vWF/ADAMTS13比值的差异及其与临床指标的关系。结果 (1)与正常对照组相比,两组患者血浆ADAMTS13抗原均明显降低(均P<0.05),而vWF抗原均明显升高(均P<0.05)。vWF/ADAMTS13比值在3组间两两比较,差异均有高度统计学意义(均P<0.01)。(2)相关分析显示,NS患者血浆ADAMTS13抗原与血清总蛋白(TP)、血清白蛋白(ALB)成正相关(均P<0.01),与24 h尿蛋白、甘油三酯(TG)、总胆固醇(TC)成负相关(P<0.05或0.01);血浆vWF抗原与TP、ALB成负相关,与24 h尿蛋白、TG、血浆纤维蛋白原、D-二聚体成正相关(P<0.05或0.01);血浆ADAMTS13抗原与vWF抗原之间无明显相关性(P>0.05)。结论血浆AD-AMTS13抗原水平低下、ADAMTS13和vWF抗原比例失衡可能是NS形成血栓的重要机制之一。

Objective To detect the antigen levels of von Willebrand factor cleaving protease（vWF-cp,also known as ADAMTS13） and von Willebrand factor（vWF） in plasma of patients with nephritic syndrome（NS） and investigate its clinical significance.Methods A total of 70 NS patients were enrolled in the study.They were divided into the membranous nephropathy（MN） group and non-membranous nephropathy（non-MN） group.In MN group and non-MN group as well as the control group,the plasma ADANTS13 antigens and vWF antigens in both patients and normal controls were assayed by ELISA assay.The differences of plasma ADAMTS13 antigen,vWF antigen and ratio of vWF to ADAMTS13 were compared in the three groups and the correlation relation was analyzed among ADAMTS13 antigen,vWF antigen and other clinical indexes.Results（1） Compared with normal control group,plasma ADAMTS13 antigens of both MN group and non-MN group decreased（all P0.05）,vWF antigens increased（all P0.05）,and the ratios of VWF antigen to ADAMTS13 antigen increased too（all P0.01）.（2） Correlation analysis showed that plasma ADAMTS13 antigen of patients with NS was positively correlated with the serum total protein（TP） and albumin（ALB）,while negatively correlated with the quantitative measurement of 24 hours urinary protein,total cholesterol（TC） and triglyceride（TG）;plasma vWF antigen was negatively correlated with TP and ALB,and positively correlated with fibrinogen and D-Dimer;there was no significant correlation among plasma ADAMTS13 antigen and vWF antigen.Conclusion The deficiency of plasma ADAMTS13 antigens,and the disbalance of ADAMTS13 and vWF may be one of the mechanism of the thrombosis which is a common complication of nephritic syndrome.