Feng B, Zhu W, Gao P, Zhang B Z, Liu Y, Lin J, Qian W W, Wang S J, Zhu T N, Qiu G X, Zhao Y Q, Weng X S
Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2020 Nov 14;41(11):903-907. doi: 10.3760/cma.j.issn.0253-2727.2020.11.004.
To study the orthopedic treatment strategy for hemophilia complicated with musculoskeletal disorders as well as the peri-operative consumption of clotting factor. Total 338 orthopedic surgeries were performed for 261 patients, average age of 30.6 y (6-65 y) , with hemophilia between January 1996 and December 2019 at our institute. Two hundred and twenty-six patients presented with bleeds within the joints. Sixty-one patients presented with intramuscular bleeds, 45 presented with hemophilic pseudotumors, and six presented with miscellaneous complaints. Strategy of clotting factor replacement therapy was designed as per differences in the level of the operation procedure. Information regarding clinical manifestation, operative strategy, clotting factor consumption, and re-operation for complications was retrospectively recorded. The costs for multiple joint procedure and single joint procedure were studied. We found that 270 of the 338 surgical procedures were major surgical procedures (79.9%) . There were 203 procedures of joint arthroplasty (60%) . Fourteen patients underwent reoperations for local recurrence (4.2%) . The average factor Ⅷ consumption before the surgery was 44.4 ± 8.1 IU/kg. The average FⅧ consumption within postoperative 2 weeks was 40 962 IU (647±177 IU/kg) . Seven type A hemophilic patients developed F Ⅷ inhibitor following the surgical procedure, with an average level of 13.7±11.2 BU/mL. Sixty-eight patients underwent multiple joint procedures under one anesthesia session (26%) . There was no significant difference in the factor consumption between the multiple joint procedure and single joint procedure. Surgical treatment was found to be effective for hemophilic arthropathy and lesion of the musculoskeletal apparatus, with the clotting factor replacement therapy. Multiple joint procedures under one anesthesia were more cost effective for patients with hemophilia, with less factor consumption than staged single joint procedure.
研究血友病合并肌肉骨骼疾病的骨科治疗策略以及围手术期凝血因子的消耗量。1996年1月至2019年12月期间，我院对261例平均年龄30.6岁（6 - 65岁）的血友病患者进行了总计338例骨科手术。226例患者出现关节内出血。61例患者出现肌肉内出血，45例出现血友病性假肿瘤，6例有其他杂项主诉。根据手术操作水平的差异设计凝血因子替代治疗策略。回顾性记录临床表现、手术策略、凝血因子消耗量及并发症再次手术情况。研究了多关节手术和单关节手术的费用。我们发现338例手术中有270例为大手术（79.9%）。关节置换术有203例（60%）。14例患者因局部复发接受再次手术（4.2%）。术前平均Ⅷ因子消耗量为44.4±8.1 IU/kg。术后2周内平均FⅧ消耗量为40962 IU（647±177 IU/kg）。7例甲型血友病患者术后出现FⅧ抑制物，平均水平为13.7±11.2 BU/mL。68例患者在一次麻醉下进行了多关节手术（26%）。多关节手术和单关节手术之间的因子消耗量无显著差异。发现手术治疗联合凝血因子替代疗法对血友病性关节病和肌肉骨骼系统病变有效。对于血友病患者，一次麻醉下的多关节手术更具成本效益，因子消耗量比分期单关节手术少。
