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关于血浆与甲型血友病血浆混合时因子VIII激活受影响方式的体外研究。

In vitro studies on the way in which the activation of factor VIII is affected in mixtures of plasma with hemophilia A plasma.

作者信息

Mingers A M

出版信息

Eur J Pediatr. 1976 Oct 1;123(3):199-213. doi: 10.1007/BF00452097.

DOI:10.1007/BF00452097
PMID:976284
Abstract

Factor VIII exchange test experiments with hemophilia A plasmas were performed to find out how the results were affected by submitting plasmas and plasma mixtures to different incubation periods at 37 degrees C, heat precipitation, and ether extraction. The experiments led to the following results: 1. In plasma mixtures, frequently higher factor VIII activities are found than can be expected from the single activities of the used plasmas. Activity increases are factor-specific. 2. The component to be activated is in the hemophilic plasma; the activity-increasing agent is in the normal plasma. These results lead to the hypothesis that hemophilia A patients have sufficient quantities of inactive factor VIII, but it stays inactive for lack of the necessary activator. In normal plasma the activator is in balance or surplus to the inactive factor. By adding normal plasma, thus supplying free activator, the inactive factor VIII of hemophilia A plasma can, under suitable conditions, be developed into active factor VIII.

摘要

进行了甲型血友病血浆的因子VIII交换试验,以探究将血浆及血浆混合物在37℃下置于不同孵育时间、热沉淀及乙醚萃取后,结果会受到怎样的影响。实验得出以下结果:1. 在血浆混合物中,经常会发现因子VIII活性高于根据所用血浆的单一活性所预期的值。活性增加具有因子特异性。2. 待激活的成分存在于血友病血浆中;活性增强剂存在于正常血浆中。这些结果引出一个假设,即甲型血友病患者有足够量的无活性因子VIII,但由于缺乏必要的激活剂而保持无活性状态。在正常血浆中,激活剂与无活性因子处于平衡或过剩状态。通过添加正常血浆从而提供游离激活剂,在合适条件下,甲型血友病血浆中的无活性因子VIII可转化为活性因子VIII。

相似文献

1
In vitro studies on the way in which the activation of factor VIII is affected in mixtures of plasma with hemophilia A plasma.关于血浆与甲型血友病血浆混合时因子VIII激活受影响方式的体外研究。
Eur J Pediatr. 1976 Oct 1;123(3):199-213. doi: 10.1007/BF00452097.
2
Studies on factor VIII activation potential in hemophilia A-plasma and its significance for the comprehension of hemophilia.
Eur J Pediatr. 1976 Oct 1;123(3):187-98. doi: 10.1007/BF00452096.
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引用本文的文献

1
Comparative studies on coagulation activities in normal plasmas and their mixtures.正常血浆及其混合物凝血活性的比较研究。
Eur J Pediatr. 1976 Oct 1;123(3):215-24. doi: 10.1007/BF00452098.

本文引用的文献

1
Effect of antihemophilic factor on one-stage clotting tests; a presumptive test for hemophilia and a simple one-stage antihemophilic factor assy procedure.抗血友病因子对一期凝血试验的影响;血友病的一种推定试验及一种简单的一期抗血友病因子测定方法。
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[Enhanced activity of diminished blood clotting factors in inborn blood coagulation defects].
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Immunologic differentiation of classic hemophilia (factor 8 deficiency) and von Willebrand's dissase, with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor.经典血友病(因子Ⅷ缺乏症)和血管性血友病的免疫学分型,以及关于抗血友病因子和加速素(因子Ⅴ)联合缺乏症和一种获得性抗血友病因子循环抗凝剂的观察
J Clin Invest. 1971 Jan;50(1):244-54. doi: 10.1172/JCI106480.
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[Family members with a congenital defect in the prothrombin complex].凝血酶原复合物存在先天性缺陷的家庭成员
Arch Kinderheilkd. 1970;182(1):59-74.
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[Factor 8 in a female patient with haemophilia A during pregnancy].[一名患甲型血友病的女性患者孕期的凝血因子Ⅷ情况]
Arch Gynakol. 1971 Oct 28;210(4):386-94. doi: 10.1007/BF01628218.
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Detection of the carrier state for classic hemophilia.经典血友病携带者状态的检测。
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Immunologic studies of antihemophilic factor (AHF, factor VIII). IV. Radioimmunoassay of AHF antigen.抗血友病因子(AHF,因子VIII)的免疫学研究。IV. AHF抗原的放射免疫测定。
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Studies on factor VIII activation potential in hemophilia A-plasma and its significance for the comprehension of hemophilia.
Eur J Pediatr. 1976 Oct 1;123(3):187-98. doi: 10.1007/BF00452096.