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血友病患者踝关节的骨科问题。

Orthopaedic problems about the ankle in hemophilia.

作者信息

Rodriguez-Merchan E Carlos

机构信息

La Paz University Hospital, Madrid, Spain.

出版信息

J Foot Ankle Surg. 2012 Nov-Dec;51(6):772-6. doi: 10.1053/j.jfas.2012.06.005. Epub 2012 Jul 10.

Abstract

Hemophilia is an inherited recessive, sex-linked bleeding disorder. The lack of sufficient coagulation factor VIII produces hemophilia A, and the lack of factor IX causes hemophilia B. The prevention and treatment of the disease requires intravenous infusion of the deficient factor. Hemophilic patients present with multiarticular joint degeneration (hemophilic arthropathy), secondary to recurrent hemarthroses. With the availability of deficient factors, hemophilic patients requiring elective ankle surgery can undergo such surgery with a high expectation of success. A thorough analysis of each case by a multidisciplinary team will increase the likelihood of successful surgical intervention in the hemophilic patient. Radiosynovectomy decreases both the frequency and the intensity of recurrent ankle bleeding episodes related to ankle synovitis. The general recommendation is that when 3 early consecutive radiosynovectomies (repeated every 6 months) fail to halt synovitis, arthroscopic synovectomy should be considered. For advanced hemophilic arthropathy of the ankle, the first alternative for treatment, in our opinion, is arthroscopic ankle debridement. In the most severe cases, we recommend either ankle arthrodesis or total ankle replacement. In every other case, we feel that the best therapy is prophylaxis and radiosynovectomy in order to avoid hemophilic synovitis and ankle arthropathy.

摘要

血友病是一种遗传性隐性X连锁出血性疾病。缺乏足够的凝血因子VIII会导致甲型血友病,而缺乏因子IX会导致乙型血友病。该疾病的预防和治疗需要静脉输注缺乏的因子。血友病患者会出现多关节退变(血友病性关节病),继发于反复的关节积血。有了缺乏的因子后,需要择期踝关节手术的血友病患者进行此类手术有望取得很高的成功率。多学科团队对每个病例进行全面分析将增加血友病患者手术干预成功的可能性。放射性滑膜切除术可降低与踝关节滑膜炎相关的反复踝关节出血发作的频率和强度。一般建议是,当连续3次早期放射性滑膜切除术(每6个月重复一次)未能阻止滑膜炎时,应考虑关节镜下滑膜切除术。对于晚期踝关节血友病性关节病,我们认为首选的治疗方法是关节镜下踝关节清创术。在最严重的情况下,我们建议进行踝关节融合术或全踝关节置换术。在其他所有情况下,我们认为最佳治疗方法是预防和放射性滑膜切除术,以避免血友病性滑膜炎和踝关节病。

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