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血友病中凝血因子IX浓缩物治疗的研究。

Studies of factor IX concentrate therapy in hemophilia.

作者信息

Hultin M B

出版信息

Blood. 1983 Sep;62(3):677-84.

PMID:6603880
Abstract

The effects of factor IX concentrate therapy on hemostasis in hemophilia patients were studied by means of the radiometric factor IXa assay, the coupled amidolytic assay for factor VIIa, and coagulant assays for factors II, IX, and X, and antithrombin III. Both activated and unactivated concentrates contained factors VIIa and IXa, with the highest levels in the activated concentrates. Factors VIIa and IXa were detected in patient plasma after infusion of unactivated concentrates. Increases of 3-5--fold in factors II and X were also found. Major decreases in antithrombin III activity, but not antigen, were found after unactivated concentrate therapy. This functional decrease may be due to the presence of inactive antithrombin III complexes, since a decreased mobility of antithrombin III antigen by crossed immunoelectrophoresis was found. These studies support the possible importance of factors IXa and VIIa as therapeutic agents and suggest that a transient functional deficiency in antithrombin III may be involved in the thrombotic potential of the concentrates.

摘要

通过放射性因子IXa测定法、因子VIIa的偶联酰胺水解测定法以及因子II、IX、X和抗凝血酶III的凝血测定法,研究了因子IX浓缩物疗法对血友病患者止血的影响。活化和未活化的浓缩物均含有因子VIIa和IXa,活化浓缩物中的含量最高。输注未活化浓缩物后,在患者血浆中检测到因子VIIa和IXa。还发现因子II和X增加了3至5倍。未活化浓缩物治疗后,抗凝血酶III活性显著降低,但抗原未降低。这种功能下降可能是由于存在无活性的抗凝血酶III复合物,因为通过交叉免疫电泳发现抗凝血酶III抗原的迁移率降低。这些研究支持了因子IXa和VIIa作为治疗剂的潜在重要性,并表明抗凝血酶III的短暂功能缺陷可能与浓缩物的血栓形成潜力有关。

相似文献

1
Studies of factor IX concentrate therapy in hemophilia.血友病中凝血因子IX浓缩物治疗的研究。
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2
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3
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Factors IXa and Xa play distinct roles in tissue factor-dependent initiation of coagulation.凝血因子IXa和Xa在组织因子依赖性凝血起始过程中发挥不同作用。
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Factor IXa-factor VIIIa-cell surface complex does not contribute to the basal activation of the coagulation mechanism in vivo.凝血因子IXa-凝血因子VIIIa-细胞表面复合物对体内凝血机制的基础激活没有作用。
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10
Thrombin generation is not increased in the blood of hemophilia B patients after the infusion of a purified factor IX concentrate.输注纯化的凝血因子IX浓缩物后,B型血友病患者血液中的凝血酶生成并未增加。
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