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[先天性因子X缺乏症,即普劳斯-斯图尔特缺乏症]

[Congenital factor X deficiency, the Prower-Stuart deficiency].

作者信息

Martinić P

出版信息

Bilt Hematol Transfuz. 1981;9(1-3):69-75.

PMID:7349760
Abstract

The congenital deficit of factor-X is extremely rare and usually goes with mild bleeding tendency. The variations of the factor-X activities are described individually and depend on the tests used, that is: activating factor-X by tissue or blood thromboplastin, Russel's Viper Venom, by neutralisation of the antibodies or immunoprecipitation. The test depends on the possibility of the specific congenital deficient plasma or on the use of the filtered bovine plasma. That's why it is rather difficult to classify the cases and order the minimum of the hemostatic level. Besides a short description of some physical, chemical and biochemical characteristics of factor-X, present the case with congenital deficit of Prower-Stuart factor discovered in our laboratory, together with the findings and procedures in identifying and confirming it.

摘要

因子X先天性缺乏极为罕见,通常伴有轻度出血倾向。因子X活性的变化分别进行描述,且取决于所使用的检测方法,即:通过组织或血液凝血活酶、罗素蝰蛇毒激活因子X,通过抗体中和或免疫沉淀法。该检测取决于特定先天性缺陷血浆的可用性或过滤牛血浆的使用。因此,对病例进行分类并确定止血水平的最小值相当困难。除了对因子X的一些物理、化学和生化特性进行简要描述外,还介绍了在我们实验室发现的先天性普劳尔-斯图尔特因子缺乏病例,以及识别和确认该病例的结果与程序。

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