摘要血友病(hemophilia)是一种X染色体连锁的出血性隐性遗传病,是凝血因子基因突变导致凝血因子Ⅷ缺乏(血友病A)或凝血因子Ⅸ缺乏(血友病B)所致。最常见的并发症是肌肉骨骼的出血,特别是在关节内的出血,常伴有关节结构的改变,称为“血友病性关节炎”(hemophilic arthropathy,HA)。HA常累及膝、踝、肘关节,髋关节受累较少。即使髋HA的发病率较其他关节低,但因髋关节是人体主要的承重关节,其病变正逐渐受到广大医生和患者的重视。根据Arnold-Hilgartner分级,终末期(Ⅳ级)髋HA是指关节正常间隙完全消失,关节表面不平整,骨质内出现囊性变,有反应性骨硬化表现的髋HA。1966年重组人凝血因子的问世,使得全髋关节置换术(total hip arthroplasty,THA)治疗终末期髋HA成为现实。通过查阅近年文献,本文将从血友病性髋关节炎发病机制、血友病性髋关节炎诊断、凝血因子替代治疗、全髋关节置换术的治疗进展、术后常见并发症的预防及处理、远期疗效等六个方面进行综述。
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