Rodriguez-Merchan E C, Blanco M, Sanjurjo M J, Magallon M
Haemophilia Centre, La Paz University Hospital, Madrid, Spain.
Haemophilia. 1999 Jan;5(1):69-72. doi: 10.1046/j.1365-2516.1999.00211.x.
A cemented Charnley total hip prosthesis was implanted in a 48-year-old man with mild haemophilia (factor VIII 4 IU dL-1) in his right spontaneously ankylosed hip. At the time of surgery he was anti-HCV positive, anti-HIV negative, and no circulating inhibitors were encountered. The indication for surgery was long-lasting intractable low back and ipsilateral knee pain. At 4-month follow-up, relief of pain was achieved as well as correction of limb-length discrepancy, with a good result according to the Mayo Clinic hip score. Doses of 50 IU kg-1 body weight of recombinant factor VIII (Recombinate; Baxter, Glendale, California, USA) was used during the 2 weeks of admittance to the hospital. The dosage was adjusted according to the recoveries of factor VIII, with an overall factor consumption of 68 000 IU. As far as we know this is the first case reported in the literature of a person with haemophilia in whom a spontaneous hip ankylosis has been satisfactorily converted in a total hip arthroplasty with a short-term follow-up. However, a much longer follow-up is still needed to ascertain the efficacy of this surgical procedure in haemophilia.
一名48岁男性，患有轻度血友病（因子VIII水平为4 IU/dL），右髋关节自发融合，接受了骨水泥型Charnley全髋关节置换术。手术时，他抗丙型肝炎病毒（HCV）呈阳性，抗人类免疫缺陷病毒（HIV）呈阴性，且未发现循环抑制物。手术指征为长期难以忍受的下背部和同侧膝关节疼痛。术后4个月随访时，疼痛缓解，肢体长度差异得到纠正，根据梅奥诊所髋关节评分结果良好。入院两周期间使用了剂量为50 IU/kg体重的重组因子VIII（瑞科强；美国加利福尼亚州格伦代尔市百特公司）。剂量根据因子VIII的恢复情况进行调整，因子VIII总消耗量为68000 IU。据我们所知，这是文献中报道的首例血友病患者，其自发融合的髋关节在全髋关节置换术中得到满意矫正且进行了短期随访。然而，仍需要更长时间的随访来确定该手术方法对血友病患者的疗效。
