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胶原病患者的血浆凝血和纤维蛋白溶解参数,以及血管性血友病因子(vWF)多聚体结构分析。

Plasma coagulation and fibrinolysis parameters in patients with collagen diseases, and analysis of the multimeric structure of von Willebrand factor (vWF).

作者信息

Kagawa H, Okubo S, Yasunaga K

机构信息

First Department of Internal Medicine, Kansai Medical University, Osaka, Japan.

出版信息

Int J Hematol. 1991 Oct;54(5):429-39.

PMID:1756253
Abstract

We studied the relationship between vascular complications and coagulation and fibrinolysis parameters in 75 subjects with collagen diseases. Thirty normal healthy persons served as controls. We found that patients with collagen diseases were in a state of a hypercoagulation and hyperfibrinolysis. In SLE (systemic lupus erythematosus) in particular, coagulation and fibrinolysis parameters appeared to be indices of vascular complications. Increases in the levels of thrombin-antithrombin III complex (TAT) and alpha 2-plasmin inhibitor-plasmin (PIP) were particularly associated with proteinuria, while increases in fibrinopeptide A (FPA) levels were associated with Raynaud's phenomenon. Administration of glucocorticoid seemed to improve the hypercoagulation and hyperfibrinolytic states of patients with collagen diseases. Analysis of the multimeric structure of von Willebrand factor (vWF) revealed a tendency for large and intermediate multimers (LIM) of plasma vWF to increase in SLE patients with accompanying vascular complications, whereas such increases were not observed in SLE patients without any vascular complications. Therefore, analysis of the multimeric structure of vWF appeared to be a useful indicator of vascular complications in collagen diseases.

摘要

我们研究了75例胶原病患者血管并发症与凝血及纤溶参数之间的关系。30名正常健康人作为对照。我们发现胶原病患者处于高凝和高纤溶状态。特别是在系统性红斑狼疮(SLE)中,凝血和纤溶参数似乎是血管并发症的指标。凝血酶 - 抗凝血酶III复合物(TAT)和α2 - 纤溶酶抑制剂 - 纤溶酶(PIP)水平的升高尤其与蛋白尿相关,而纤维蛋白肽A(FPA)水平的升高与雷诺现象相关。糖皮质激素的使用似乎改善了胶原病患者的高凝和高纤溶状态。对血管性血友病因子(vWF)多聚体结构的分析显示,伴有血管并发症的SLE患者血浆vWF的大、中多聚体(LIM)有增加的趋势,而在无任何血管并发症的SLE患者中未观察到这种增加。因此,vWF多聚体结构的分析似乎是胶原病血管并发症的一个有用指标。

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