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[波兰的血友病及其他遗传性血液凝固障碍]

[Hemophilia and other inherited blood coagulation disorders in Poland].

作者信息

Windyga Jerzy, Lopaciuk Stanisław, Stefańska Ewa, Klukowska Anna

机构信息

Samodzielna Pracownia Krzepniecia Krwi i Hemostazy Instytutu Hematologii i Transfuzjologii w Warszawie.

出版信息

Pol Arch Med Wewn. 2004 Oct;112(4):1197-202.

Abstract

The aim of the study was to analyze the data on 3224 patients with inherited blood coagulation disorders registered in Poland till December 1st, 2003. In 2269 registered hemophilia patients, 1953 were hemophilia A, and 316 were hemophilia B. Hemophilia A occurs in 1512 families, and hemophilia B in 240 families. Severe hemophilia constitutes the majority of hemophilia A and B cases (59.7% and 56.6% respectively). About 50% of the hemophiliacs have no history of bleeding diathesis in the family. The mean age of hemophilia A patients is 30.9 years, and that of hemophilia B patients--29.2 years. Prevalence of hemophilia in Poland is approximately 1:12 300 inhabitants. Hemophilia A has been diagnosed in 60.6% of all patients with inherited blood coagulation disorders registered in Poland, von Willebrand disease-- in 21.9%, hemophilia B-- in 9.8% and factor VII deficiency-- in 3.3%.

摘要

该研究的目的是分析截至2003年12月1日在波兰登记的3224例遗传性凝血障碍患者的数据。在2269例登记的血友病患者中,1953例为甲型血友病,316例为乙型血友病。甲型血友病发生在1512个家庭中,乙型血友病发生在240个家庭中。重度血友病在甲型和乙型血友病病例中占大多数(分别为59.7%和56.6%)。约50%的血友病患者家族中无出血素质病史。甲型血友病患者的平均年龄为30.9岁,乙型血友病患者的平均年龄为29.2岁。波兰血友病的患病率约为每12300名居民中有1例。在波兰登记的所有遗传性凝血障碍患者中,60.6%被诊断为甲型血友病,21.9%为血管性血友病,9.8%为乙型血友病,3.3%为因子VII缺乏症。

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