25 YEARS EXPERIENCE OF PROPHYLACTIC TREATMENT IN SEVERE HEMOPHILIA-A AND HEMOPHILIA-B
25 YEARS EXPERIENCE OF PROPHYLACTIC TREATMENT IN SEVERE HEMOPHILIA-A AND HEMOPHILIA-B
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DOI：
10.1111/j.1365-2796.1992.tb00546.x
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发表时间：
1992-07-01
影响因子：
11.1
通讯作者：
PETTERSSON, H
中科院分区：
文献类型：
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作者：
NILSSON, IM;BERNTORP, E;PETTERSSON, H
在瑞典，自1958年以来一直对患有严重血友病的男孩进行预防性治疗，试图将这种疾病从严重转为较轻的形式。本研究人群包括60例重度血友病患者（52例A，8例B），年龄3-32岁。当男孩为1-2岁时开始治疗，所用方案为24-40 IU F VIII kg-1，每周三次，用于A型血友病病例（即> 2000 IU kg-1，每年），25-40 IU F IX kg-1，每周两次，用于B型血友病病例。按照世界血友病联合会的建议评价骨科和放射学关节评分（最高评分分别为90和78）。在3-17岁的受试者中，35人中有29人的关节评分为零。年龄最大的一组只有轻微的关节缺陷。VIII：C和IX：C浓度通常不会低于正常值的1%。所有60名患者都能过上正常的生活。总之，似乎可以通过从早期开始进行有效的持续预防，并防止VIII：C或IX：C浓度降至正常值的1%以下，来预防血友病性关节病。
In Sweden, prophylactic treatment of boys with severe haemophilia has been practised since 1958 in an attempt to convert the disease from a severe to a milder form. The present study population consisted of 60 severe haemophiliacs (52 A, 8 B), aged 3-32 years. Treatment is started when the boys are 1-2 years of age, the regimens used being 24-40 IU F VIII kg-1 three times weekly in haemophilia-A cases (i.e. > 2000 IU kg-1 annually) and 25-40 IU F IX kg-1 twice weekly in haemophilia-B cases. The orthopaedic and radiological joint scores (maximum scores of 90 and 78, respectively) are evaluated as recommended by the World Federation of Haemophilia. Of those subjects aged 3-17-years, 29 out of 3 5 individuals had joint scores of zero. The oldest group had only minor joint defects. The VIII: C and IX: C concentrations had usually not fallen below 1 % of normal. All 60 patients are able to lead normal lives. In conclusion, it appears to be possible to prevent haemophilic arthropathy by giving effective continuous prophylaxis from an early age, and preventing the VIII:C or IX:C concentration from falling below 1 % of normal.