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法国3435名血友病患者群体中抑制剂的患病率。法国血友病研究小组。

Prevalence of inhibitors in a population of 3435 hemophilia patients in France. French Hemophilia Study Group.

作者信息

Sultan Y

机构信息

Centre des Hémophiles, Hôpital Cochin, Paris, France.

出版信息

Thromb Haemost. 1992 Jun 1;67(6):600-2.

PMID:1509398
Abstract

A cooperative study between the 37 centers of the French Hemophilia Study Group was undertaken to establish the prevalence of inhibitor patients in the French hemophilia population. The prevalence reported in the literature varies widely from 3.6% to 17.5%. Some of the studies are dealing with a small number of patients and inhibitor patients are reported either to the total number of hemophiliacs or to the severely affected ones. The French study provided information concerning 3,435 hemophiliacs and showed a prevalence of 6.2% for the overall population. Prevalence of inhibitors was found to be 7% in the population of hemophilia A patients and 12.8% in the population of severely affected ones. The prevalence of inhibitors in the population of hemophilia patients was 2% and 4% in the population of severely affected hemophilia B patients. The cooperative study also showed that 47.5% of inhibitors are detected before 10 years of age and that 82% of inhibitor patients are high responders. Analysis of inhibitor detection in patients under the age often showed that there was a peak in the population of 2 years old children. Although not comparable to the present study the high incidence of inhibitors with ultrapurified and recombinant FVIII reported in previously untransfused patient may be borne in mind.

摘要

法国血友病研究小组的37个中心开展了一项合作研究,以确定法国血友病患者中抑制物患者的患病率。文献报道的患病率差异很大,从3.6%到17.5%不等。一些研究涉及的患者数量较少,抑制物患者报告为血友病患者总数或重症患者数。法国的这项研究提供了有关3435名血友病患者的信息,显示总体患病率为6.2%。发现A型血友病患者中抑制物的患病率为7%,重症患者中为12.8%。血友病患者中抑制物的患病率为2%,重症B型血友病患者中为4%。这项合作研究还表明,47.5%的抑制物在10岁之前被检测到,82%的抑制物患者是高反应者。对10岁以下患者抑制物检测的分析表明,2岁儿童群体中存在一个高峰。尽管与本研究无可比性,但之前未输血患者中报告的超纯和重组FVIII抑制物的高发生率可能需要铭记。

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