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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 plasma levels of vWF-cp (vWF-cp, also known as ADAMTS13) and von Willebrand factor (vWF) in patients with nephrotic syndrome (NS) and to investigate its clinical significance. Methods Seventy NS patients were divided into two groups: MN group and non-MN group. Plasma ADAMTS13 and vWF antigens were detected by ELISA in MN group, non-MN group and normal control group The difference of the ratio of AD-AMTS13 antigen, vWF antigen and vWF / ADAMTS13 between groups and its clinical indexes. Results (1) Compared with the normal control group, plasma ADAMTS13 antigens in both groups were significantly decreased (all P <0.05), while vWF antigens were significantly increased (all P <0.05). The ratio of vWF / ADAMTS13 was significantly different between the three groups (all P <0.01). (2) The correlation analysis showed that there was a positive correlation between plasma ADAMTS13 antigen and serum total protein (TP) and serum albumin (ALB) in patients with NS (all P <0.01), and 24 h urine protein, triglyceride (TG) (TC) (P <0.05 or 0.01). Plasma vWF antigen was negatively correlated with TP and ALB, and positively correlated with 24 h urinary protein, TG, plasma fibrinogen and D-dimer 0.01). There was no significant correlation between plasma ADAMTS13 antigen and vWF antigen (P> 0.05). Conclusion The plasma level of AD-AMTS13 antigen is low. The imbalance of ADAMTS13 and vWF antigen may be one of the important mechanisms of thrombosis in NS.