Musculoskeletal Care of the Hemophiliac Patient
Musculoskeletal Care of the Hemophiliac Patient
复制标题
血友病患者的肌肉骨骼护理
DOI：
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发表时间：
2012
影响因子：
3.2
通讯作者：
F. Farley
中科院分区：
文献类型：
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作者：
Kelly L. Vanderhave;M. Caird;M. Hake;R. Hensinger;A. Urquhart;Selina R Silva;F. Farley
摘要血友病是由凝血因子VIII或IX缺乏引起的，由性连锁隐性遗传。血管性血友病是一种常见的中度出血性疾病，由血管性血友病因子定量或定性蛋白缺乏引起，常染色体显性遗传或隐性遗传。血友病患者治疗的最重要的临床策略是通过持续的静脉血液学预防来避免复发的关节。在关节检查正常之前，应积极进行抽吸和凝血因子浓缩治疗早期关节出血。在婴儿期开始预防性因子替代治疗可以预防慢性滑膜炎和关节病。疾病控制不良的自然病史是多关节血友病关节病；功能预后较差。慢性滑膜炎的患者可以通过放射滑膜切除术得到有效的治疗；那些出现关节表面侵蚀的患者可能需要重新排列截骨术、关节成形术和假瘤治疗。血友病关节病的重建手术，特别是使用因子抑制剂的患者，需要由经验丰富的多学科团队进行仔细的血液学处理。
Abstract Hemophilia is caused by a deficiency of clotting factor VIII or IX and is inherited by a sex‐linked recessive pattern. von Willebrand disease, a common, moderate bleeding disorder, is caused by a quantitative or qualitative protein deficiency of von Willebrand factor and is inherited in an autosomal dominant or recessive manner. The most important clinical strategy for the management of patients with hemophilia is the avoidance of recurrent hemarthrosis by continuous, intravenous hematologic prophylaxis. Early hemarthrosis should be aggressively managed with aspiration and clotting factor concentrate until the joint examination is normal. Starting prophylactic factor replacement in infancy may prevent chronic synovitis and arthropathy. The natural history of poorly controlled disease is polyarticular hemophilic arthropathy; functional prognosis is poor. Patients with chronic synovitis may be treated effectively with radiosynovectomy; those who develop joint surface erosions may require realignment osteotomies, joint arthroplasty, and treatment of pseudotumors. Reconstructive surgery for hemophilic arthropathy, especially in patients with factor inhibitor, requires careful hematologic management by an experienced, multidisciplinary team.