膜性及非膜性肾病综合征患者血浆血管性血友病因子及其裂解酶的变化及临床意义

目的 探讨膜性及非膜性肾病综合征（nephrotic syndrome,NS）患者治疗前后血浆中血管性血友病因子裂解酶（a disintegrin-like and metalloprotease with thrombospondin type I repeats 13,ADAMTS13）及血管性血友病因子（von Willebrand factor,vWF）的变化其临床意义.方法 将60例NS患者分为膜性肾病组（n=21）、非膜性肾病组（n=39）,43例健康体检者作为正常对照组,检测各组血、尿生化指标,ELISA法检测各组血浆AD-AMTS13、vWF水平并计算vWF/ADAMTS13比值.结果 与正常对照组相比,治疗前膜性肾病组和非膜性肾病组患者血浆ADAMTS13水平均降低（P＜0.05）,而vWF、vWF/ADAMTS13水平均升高（P＜0.05）.相关分析显示,NS患者血浆ADAMTS13与血清白蛋白（ALB）呈正相关（r=0.668,P＜0.05）,与尿蛋白定量（UTP）、D-dimer和三酰甘油（TG）呈负相关（r=-0.403,r=-0.313,r=-0.281,P＜0.05）.血浆vWF与ALB呈负相关（r=-0.376,P＜0.05）,与总胆固醇（TC）、UTP呈正相关（r=0.328,r=0.269,P＜0.05）.血浆ADAMTS13与vWF之间呈负相关（r=-0.387,P＜0.05）.非膜性肾病组ADAMTS13治疗后较治疗前升高,差异具有统计学意义（P＜0.05）,膜性肾病组ADAMTS13治疗后较治疗前升高,但差异无统计学意义（P ＞0.05）;NS两组血浆vWF及vWF/ADAMTS13比值治疗前后比较差异均无统计学意义（P＞0.05）.结论 vWF、ADAMTS13及vWF/AD-AMTS13与NS高凝状态有关,ADAMTS13可能成为判断肾病综合征病情与预后的新指标.

Clinical significance of vWF and ADAMTS13 in plasma of patients with membranous and non-membranous nephrotic syndrome

Objective To detect the levels of ADAMTS13 （ a disintegrin - like and metalloprotease with thrombospondin type I repeats 13 ） and van Willebrand factor （vWF） in plasma of patients with membranous and non- mem- branous nephrotic syndrome （NS） before and after treatment, so as to investigate its clinical significance. Methods Sixty NS patients were divided into the membranous nephropathy group （n = 21 ） and the non- membranous nephropathy group （n = 39 ）. 43 normal subjects served as normal control group. The plasma ADAMTS13 antigens and vWF antigens were detected by ELISA assay. Other biochemical indexes in plasma and urine were tested as well. Results Compared with normal control group, plasma ADAMTS13 of both membranous nephropathy group and the non - membranous nephropathy group decreased （ P 〈 0.05 ）, vWF increased （ P 〈 0.05 ）, and the ratios of vWF to ADAMTS13 increased too （P 〈 0.05 ）. Correlation analysis showed that plasma ADAMTS13 of patients with NS was positively correlated with the serum albumin （ALB） （ r = 0. 668, P 〈 0.05 ）, while negatively correlated with 24 - hour urinary total protein （ UTP）, D - dimer and triglyceride （TG） （r = -0. 403, r = -0. 313, r = -0. 281, P 〈0.05）. Plasma vWF was negatively correlated with ALB （ r = - 0. 376, P 〈 0.05） and positively correlated with total cholesterol （TC） and UTP （r = 0. 328, r =0. 269, P 〈 0.05）. There was a negative correlation between ADAMTS13 and vWF （r = -0. 387, P 〈 0.05 ）. The levels of ADAMTS13 were increased significantly after treatment in non - MN group （P 〈 0.05 ）, while the levels of AD-AMTS13 were not increased obviously in MN group （ P 〉 0.05 ） ; No statistically significant differences were found in plasma levels of vWF and the ratios of vWF to ADAMTS13 before and after treatment in both MN group and non - MN group （P 〉 0.05 ）. Conclusions vWF, ADAMTS13 and vWF/ADAMTS13 may be related with the hypercoagulative state of NS; ADAMTS13 may be