Haemophilia prophylaxis in young patients - a long-term follow-up
Haemophilia prophylaxis in young patients - a long-term follow-up
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DOI：
10.1046/j.1365-2796.1997.130135000.x
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发表时间：
1997-05-01
影响因子：
11.1
通讯作者：
Pettersson, H
中科院分区：
文献类型：
--
作者：
Lofqvist, T;Nilsson, IM;Pettersson, H
目标.回顾对年轻的重度A型和B型血友病患者进行长期预防性因子治疗，重点关注骨科和放射学结局。我们收到了34例重度血友病A（n = 29）和B（n = 5），年龄7-22岁。治疗开始时的年龄为1-4.5岁。因子浓缩物（分别为F VIII和F IX）的剂量为25-40 IU/kg体重，对于血友病A每周3次，对于血友病B每周2次。在5年期间（1990- 1995年），每年对患者进行检查。根据世界血友病联合会的建议，对矫形和放射学关节评分进行评价。所有结果均在瑞典马尔莫的马尔莫大学医院的隆德大学凝血障碍科获得。在随访期间，几乎所有患者的骨科和放射学关节评分保持不变，79%（n = 27）的患者仍为零（即没有未受影响的关节）。有一个日益增长的国际共识血友病性关节病可以预防管理早期高剂量预防。目前的调查结果有力地支持了这一观点。
Objectives. To review long-term prophylactic factor treatment in young patients with severe haemophilia A and B, focusing on the orthopaedic and radiological outcome.Design. We received 34 patients with severe haemophilia A (n = 29) and B (n = 5), aged 7-22 years. Age at start of treatment was 1-4.5 years. Dosages of factor concentrate (F VIII and F IX, respectively) were 25-40 IU/kg body weight, three times a week for haemophilia A and twice a week for haemophilia B. The patients had been checked annually over a 5-year period (1990-95). Orthopaedic and radiological joint scores were evaluated according to recommendations by the World Federation of Haemophilia.Setting. All results were obtained at the Department for Coagulation Disorders, University of Lund, Malmo University Hospital, Malmo, Sweden.Results. Orthopaedic and radiological joint scores were found to have remained unchanged during follow-up in almost all patients and to be still zero (i.e. no unaffected joints) in 79% (n = 27) of the patients.Conclusion. There is a growing international consensus haemophilic arthropathy can be prevented by administering early high-dose prophylaxis. The results of the present investigation strongly support this opinion.