Heyworth Benton E, Su Edwin P, Figgie Mark P, Acharya Suchitra S, Sculco Thomas P
Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA.
Am J Orthop (Belle Mead NJ). 2005 Oct;34(10):479-86.
Hemophilia is an X-linked inherited bleeding disorder characterized by a deficiency of clotting factor VIII or IX in the intrinsic pathway of the coagulation cascade. This disease can have a profound, debilitating effect on the musculoskeletal system through recurrent hemarthroses and intermuscular hematomas. Although medical management with replacement factor is often effective, patients with severe hemophilia may develop chronic synovitis and arthropathy best treated with more invasive means. Radioactive synovectomy performed at select hemophilia centers and arthroscopic or open synovectomy are helpful for chronic, active synovitis. Arthroplasty helps improve function and pain relief for most patients, but complication rates, particularly for infection, are higher than those for arthroplasty performed on patients without hemophilia.
血友病是一种X连锁遗传性出血性疾病，其特征是凝血级联反应内源性途径中凝血因子VIII或IX缺乏。这种疾病可通过反复关节积血和肌肉间血肿对肌肉骨骼系统产生深远的、使人衰弱的影响。尽管用替代因子进行药物治疗通常有效，但重度血友病患者可能会发展为慢性滑膜炎和关节病，采用更具侵入性的方法治疗效果最佳。在选定的血友病中心进行的放射性滑膜切除术以及关节镜或开放性滑膜切除术对慢性活动性滑膜炎有帮助。关节成形术有助于改善大多数患者的功能并缓解疼痛，但并发症发生率，尤其是感染的发生率，高于非血友病患者进行关节成形术的并发症发生率。
