血管性血友病因子裂解酶及凝血酶敏感蛋白1在狼疮性肾炎中的意义  

The Clinical Significance of the Detection of von Willebrand Factor Cleaving Protease(ADAMTS13) Activity and Thrombospondin 1 in Lupus Nephritis

作　　者：卢国元[1] 刘芳 郭小芳[1] 王兆钺 苏健 白霞 阮长耿 

机构地区：[1]苏州大学附属一院肾内科,江苏苏州215006 [2]江苏省血液研究所,江苏苏州215006

出　　处：《苏州大学学报（医学版）》2006年第2期236-239,共4页Suzhou University Journal of Medical Science

基　　金：国家自然科学基金资助项目(30470732);苏州大学"211工程"基金资助项目(R2317175);苏州市"教科兴卫"青年专项资金资助项目(SWQ14)

摘　　要：目的检测狼疮性肾炎(LN)患者血浆凝血酶敏感蛋白1(TSP1)含量及血管性血友病因子裂解酶(ADAMTS13)的活性水平,以探讨TSP1及ADAMTS13在LN中的意义。方法用TSP1检测试剂盒和残余胶原结合试验(R-CBA)对31例LN患者血浆血管性血友病因子、TSP1及ADAMTS13的活性水平进行检测,并与20例血栓性血小板减少性紫癜(TTP)患者及健康人进行比较。结果LN及TTP患者血浆vWF:Ag显著高于对照组(P<0.05),血浆ADAMTS13活性水平均显著低于正常组(P<0.01),TTP患者TSP1含量显著低于对照组,而LN患者低于对照组,但无统计学差别(P>0.05)。18例肾活检的LN患者中,Ⅳ型患者的ADAMTS13活性最低,其余三型间无显著性差别,TSP1各型间无显著性差别(P>0.05)。结论ADAMTS13活性低下参与LN的发生发展,在一定程度上反映了LN病理类型的轻重,而ADAMTS13活性低下及TSP1水平下降共同参与TTP的发病机制。Objective To evaluate the clinical significance of plasma yon Willebrand factor-cleaving protease（ADAMTS13） activity and thrombospondin 1 （TSP1）in lupus nephritis（LN）. Methods Von Willebrand Factor antigen（vWF：Ag）, TSP1 and ADAMTS13 activity were evaluated by using Residual-collagen Binding Assay（R-CBA） or ELISA in 31 patients with LN, 20 with TTP and in 20 normal subjects as the controls. Results The level of vWF：Ag in LN and TTP was significantly higher than that in the normal control group（P〈0.05）, while the ADAMTS 13 activity in LN and TTP were significantly lower than that in the controls（P 〈 0.01）. The concentration of TSP1 in TTP was significantly lower than that in the normal groups, TSP1 in LN was lower but not significantly than that in the controls. The level of the ADAMTS13 activity in type Ⅳ LN was the lowest in the four types of LN（ Ⅱ, Ⅲ, Ⅳ and Ⅴ ）. Conclusions The lower ADAMTS13 activity may influence the progress of LN, while both the lower ADAMTS13 activity and TSP1 level may be one of mechanisms of TTP progress.

关 键 词：狼疮性肾炎 vWF裂解蛋白酶活性 凝血酶敏感蛋白1 

分 类 号：R593.243[医药卫生—内科学]