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血友病A患者群体中抑制物抗体的评估。

Evaluation of inhibitor antibody in hemophiliaA population.

作者信息

Mahmoodi Nesheli Hassan, Hadizadeh Amereh, Bijani Ali

机构信息

Non- Communicable Pediatrics Diseases Research Center, Babol University of Medical Sciences, Babol, Iran.

出版信息

Caspian J Intern Med. 2013 Summer;4(3):727-30.

Abstract

BACKGROUND

Inhibitor antibody to exogenous Factor VIII (FVIII) is a major complication of hemophilia treatment. This study was conducted to determine the prevalence of inhibitor antibody directed against FVIII.

METHODS

From May 2010 to May 2011, 52 patients with severe hemophilia A admitted in Amirkola Children's Hospital were evaluated. Those who had abnormal mixing study, antibody against FVIII were measured. Data were collected and analyzed.

RESULTS

The age range of the patients was 4-60 years. The inhibitor antibody was seen in 9 (17.3%) patients. The mean age of patients with inhibitor at the time of diagnosis was 10.22 years (ranged 4-31 years). Old patients had more hemarthrosis than young patients. The mean level of inhibitor antibody was 8.47 Bethesda unit (ranged 2.3-29). Six patients had inhibitor antibody level ≥5 Bethesda unit and three patients had inhibitor antibody level <5 Bethesda unit.

CONCLUSION

This study showed that the prevalence of inhibitor antibodies in young patients is more than the old patients.

摘要

背景

外源性凝血因子VIII(FVIII)抑制性抗体是血友病治疗的主要并发症。本研究旨在确定针对FVIII的抑制性抗体的患病率。

方法

2010年5月至2011年5月,对阿米尔科拉儿童医院收治的52例重度甲型血友病患者进行评估。对混合试验异常的患者检测抗FVIII抗体。收集并分析数据。

结果

患者年龄范围为4至60岁。9例(17.3%)患者出现抑制性抗体。诊断时出现抑制性抗体的患者平均年龄为10.22岁(范围4至31岁)。老年患者的关节积血比年轻患者更多。抑制性抗体的平均水平为8.47贝塞斯达单位(范围2.3至29)。6例患者的抑制性抗体水平≥5贝塞斯达单位,3例患者的抑制性抗体水平<5贝塞斯达单位。

结论

本研究表明,年轻患者中抑制性抗体的患病率高于老年患者。

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