已知A型血友病患者抑制剂的频率。

Irem Iqbal, Javeria Abdullah
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 Introduction: Inhibitors are IgG alloantibodies which are directed against exogenous clotting factor VIII that neutralize the function of infused clotting factor concentrate used for the treatment. The prevalence of inhibitors in Hemophilia A is around 30% worldwide. Inhibitors occurs as a result of natural immune process of the body as factor concentrates used for treatment are foreign to the body and patient’s own body either produces no factor or produces structurally abnormal protein.
 Objectives: To determine the frequency of inhibitors among known hemophilia A patients.
 Methods: Cross-sectional survey done including 100 male patients of hemophilia A and on treatment for more than 1 year, from 2 to 60 years of age, were included. All the patients were undergone aPTT based screening for inhibitor, both immediately and two hours after mixing the patients plasma with the normal plasma.
 Results: Age range in this study was from 2 to 60 years with mean age of 37.21 ± 14.73 years. Frequency of inhibitors among known hemophilia A patients was seen in 18 (18.0%) of cases.
 Conclusion: This study concluded that frequency of inhibitors among known hemophilia A patients is quite high.
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 Objectives: To determine the frequency of inhibitors among known hemophilia A patients.
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引用次数: 0

摘要

摘要# x0D;抑制剂是针对外源性凝血因子VIII的IgG同种抗体,可以中和用于治疗的输注凝血因子浓缩物的功能。在全球范围内,A型血友病抑制剂的患病率约为30%。抑制剂的产生是机体自然免疫过程的结果,因为用于治疗的浓缩因子对机体是外来的,而患者自身要么不产生因子,要么产生结构异常的蛋白质。目的:了解已知A型血友病患者使用抑制剂的频率。 方法:对100例治疗1年以上的男性A型血友病患者进行横断面调查,年龄2 ~ 60岁。所有患者均在患者血浆与正常血浆混合后立即和2小时进行基于aPTT的抑制剂筛查。 结果:年龄范围2 ~ 60岁,平均年龄37.21±14.73岁。在已知的A型血友病患者中,抑制剂的使用频率为18例(18.0%)。 结论:在已知的A型血友病患者中,抑制剂的使用频率相当高。 关键词:血友病A,抑制剂,严重程度。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Frequency of Inhibitors among known Hemophilia A Patients.
ABSTRACT Introduction: Inhibitors are IgG alloantibodies which are directed against exogenous clotting factor VIII that neutralize the function of infused clotting factor concentrate used for the treatment. The prevalence of inhibitors in Hemophilia A is around 30% worldwide. Inhibitors occurs as a result of natural immune process of the body as factor concentrates used for treatment are foreign to the body and patient’s own body either produces no factor or produces structurally abnormal protein. Objectives: To determine the frequency of inhibitors among known hemophilia A patients. Methods: Cross-sectional survey done including 100 male patients of hemophilia A and on treatment for more than 1 year, from 2 to 60 years of age, were included. All the patients were undergone aPTT based screening for inhibitor, both immediately and two hours after mixing the patients plasma with the normal plasma. Results: Age range in this study was from 2 to 60 years with mean age of 37.21 ± 14.73 years. Frequency of inhibitors among known hemophilia A patients was seen in 18 (18.0%) of cases. Conclusion: This study concluded that frequency of inhibitors among known hemophilia A patients is quite high. Keywords: Hemophilia A, inhibitors, severity.
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