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一项针对血友病参考中心的五年研究。

A five-year study of a haemophilia reference centre.

作者信息

Ramsay D M, Khoo K K

出版信息

J Clin Pathol. 1975 Sep;28(9):696-700. doi: 10.1136/jcp.28.9.696.

Abstract

Attendances at the Regional Haemophilia Reference Centre in the Edinburgh Royal Infirmary have been analysed over a period of five years from 1969 to 1973. Of 77 patients registered in 1969, 64 had haemophilia A (factor VIII deficiency) and 13 had haemophilia B (factor IX deficiency). In 1973 the numbers were 68 and 14 respectively. An increased attendance at the Centre from 123 in 1969 to 624 in 1973 was noted. This was due to patients reporting earlier and more frequently for outpatient treatment of haemarthroses which occurred with the greater availability of supplies of factor VIII. The number of admissions for inpatient treatment did not change significantly over the period. Haemarthrosis of the knee was the commonest dominant lesion. The amount of replacement therapy in the form of fresh frozen plasma, cryoprecipitate, antihaemophilic fraction, and factors II, IX, and X concentrate used in the Centre (ie, excluding that used for elective procedures) increased from 2704 donor units in 1969 to 8778 donor units in 1973, the main increase being in cryoprecipitate. The number of factor VIII units used per patient attending per year, for both elective and emergency treatment in haemophiliacs, is recorded and discussed as are its effects on the treatment patterns of patients and the implications for the future.

摘要

对1969年至1973年这五年间爱丁堡皇家医院地区血友病参考中心的就诊情况进行了分析。1969年登记的77名患者中,64人患有甲型血友病(因子VIII缺乏症),13人患有乙型血友病(因子IX缺乏症)。1973年,这两个数字分别为68人和14人。该中心的就诊人数有所增加,从1969年的123人增加到1973年的624人。这是由于随着因子VIII供应的增加,血友病性关节积血患者更早且更频繁地前来接受门诊治疗。在此期间,住院治疗的入院人数没有显著变化。膝关节血友病性关节积血是最常见的主要病变。该中心使用的新鲜冷冻血浆、冷沉淀、抗血友病因子以及因子II、IX和X浓缩物形式的替代疗法用量(即不包括用于择期手术的用量)从1969年的2704单位增加到1973年的8778单位,主要增加的是冷沉淀。记录并讨论了每年每名血友病患者接受择期和急诊治疗所使用的因子VIII单位数量,以及其对患者治疗模式的影响和对未来的意义。

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引用本文的文献

本文引用的文献

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The management of haemophilia.血友病的管理
Practitioner. 1970 Jun;204(224):763-72.
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Hemophilia. Current concepts in management.血友病。当前的治疗理念。
Med Clin North Am. 1972 Jan;56(1):105-17. doi: 10.1016/s0025-7125(16)32426-9.

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