Department of Orthopaedic Surgery, La Paz University Hospital-IdiPaz, Madrid, Spain.
Cardiovasc Hematol Disord Drug Targets. 2021;21(3):162-166. doi: 10.2174/1871529X21666210427134232.
In people with hemophilia, hematological prophylaxis during childhood and adolescence could elude the occurrence of musculoskeletal complications (in joints and muscles) if the concentration of the defective factor is averted from decreasing under 1% of normal. Prompt management is of capital significance as the juvenile skeleton is hypersensitive to the adverse events of the disease; intense structural defects might appear rapidly. Important, articular bleeds and inveterate hypertrophy of the articular synovial membrane must be treated vigorously to preclude joint degeneration (hemophilic arthropathy). At the moment that extreme joint disease is in place with intense affliction, the goal must be to reestablish activity whilst at the same time reducing the peril to the patient. Arthroscopic articular debridement is an efficacious surgical technique to accomplish this goal, particularly around the knee or ankle, and maybe contemplated to be a backup to ankle arthrodesis or ankle or knee replacement in patients of younger age. Eventually, joint replacement can commonly reestablish both articular mobility and function in an unhealthy articulation.
在血友病患者中，如果缺陷因子的浓度避免降至正常水平的 1%以下，那么在儿童和青少年时期进行血液学预防就可以避免肌肉骨骼并发症（关节和肌肉）的发生。及时治疗具有重要意义，因为青少年骨骼对疾病的不良事件非常敏感；可能会迅速出现严重的结构缺陷。重要的是，关节出血和关节滑膜的慢性肥大必须得到积极治疗，以防止关节退化（血友病性关节炎）。一旦出现严重的关节疾病和剧烈的疼痛，目标必须是在恢复活动的同时降低患者的风险。关节镜下关节清理术是一种有效的手术技术，可以达到这一目标，尤其是在膝关节或踝关节，对于年龄较小的患者，可能可以考虑作为踝关节融合术、踝关节或膝关节置换术的后备方案。最终，关节置换术通常可以恢复不健康关节的关节活动度和功能。
