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血管性血友病中的抗血友病因子浓缩物治疗。出血时间因子与瑞斯托霉素辅因子活性的分离。

Antihemophilic factor concentrate therapy in von Willebrand disease. Dissociation of bleeding-time factor and ristocetin-cofactor activities.

作者信息

Blatt P M, Brinkhous K M, Culp H R, Krauss J S, Roberts H R

出版信息

JAMA. 1976 Dec 13;236(24):2770-2.

PMID:1086910
Abstract

Treatment of von Willebrand disease with two plasma antihemophilic factor (AHF) concentrates, cryoprecipitate and glycine-precipitated AHF, was compared. Both concentrates were equally effective in immediately raising the plasma levels of factor VIII, the factor VIII-related antigen, and the ristocetin-related von Willebrand factor (vWF) and in stimulating a secondary rise in plasma factor VIII. Given either concentrate, the vWF activity, the antigen, and factor VIII levels were normalized in a patient with von Willebrand disease. However, correction of the prolonged bleeding time and control of bleeding occurred only with the cryoprecipitate. The bleeding-time corrective factor and the ristocetin-related vWF or platelet-aggregating factor are dissociable, distinct activites.

摘要

对两种血浆抗血友病因子(AHF)浓缩物(冷沉淀和甘氨酸沉淀的AHF)治疗血管性血友病的效果进行了比较。两种浓缩物在即刻提高血浆VIII因子、VIII因子相关抗原以及瑞斯托菌素相关血管性血友病因子(vWF)水平,以及刺激血浆VIII因子继发性升高方面同样有效。给予任何一种浓缩物后,血管性血友病患者的vWF活性、抗原及VIII因子水平均恢复正常。然而,只有冷沉淀能纠正延长的出血时间并控制出血。出血时间纠正因子与瑞斯托菌素相关vWF或血小板聚集因子是可分离的不同活性物质。

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