中文摘要：

目的：探讨血友病合并骨折患者的特点及围手术期治疗方法。方法：回顾性分析2005年至2013年在北京大学人民医院创伤骨科住院治疗的8例血友病合并骨折患者，甲型血友病6例，乙型血友病2例；其中轻型2例，中型3例，重型3例；按照骨折部位不同分为股骨颈骨折4例，股骨粗隆间骨折1例，双侧股骨远端陈旧性骨折1例，胫腓骨骨折1例，肱骨髁间骨折1例；术前、术中、术后进行凝血因子替代治疗；所有患者均行闭合／切开复位内固定或者关节置换术。分析围手术期并发症并随访骨折愈合情况。结果：患者平均年龄为33．5岁（14～47岁）；6例骨折发生于股骨；股骨颈骨折行闭合复位空心钉内固定，股骨粗隆间骨折、股骨远端骨折、胫腓骨骨折行切开复位钢板内固定，肱骨髁间骨折行肘关节置换术，术中出血50～600 mL，平均262 mL；围手术期FⅧ／凝血酶原复合物平均使用量为358 U／kg（125～554 U／kg）；术后2例患者出现伤口愈合不良，经对症处理后好转；行内固定治疗的患者，术后均出现骨性愈合，平均愈合时间为14周，术后无内固定松动及断裂等并发症发生。结论：血友病合并骨折主要发生于青年，骨折部位以股骨为主；完善术前准备，在替代治疗的基础上，可以安全地进行手术治疗，术后骨折愈合良好；术后出现伤口愈合不良的风险大。

英文摘要：

Objective：To investigate the characteristics and perioperative management of hemophilia patients with fracture.Methods：Retrospectively, we analyzed 8 patients with hemophilia combined with fracture, who were admittted to our department from 2005 to 2013.Six patients were with hemophilia A and two with hemophilia B;Based on the severity of hemophilia, 2 cases were light, 3 moderate and 3 severe;Based on the location of fracture, 4 cases were femoral neck fractures, 1 femoral intertrochanteric fracture, 1 bilateral distal femur fractures, 1 tibiofibula fracture, and 1 humerus intercondylar fracture. Blood coagulation factor replacement therapy was conducted preoperatively, intraoperatively and postoper-atively, All the patients underwent closed or open reduction and internal fixation or joint replacement. Also, we analyzed the perioperative complications and observed whether the fracture healed.Results：The average age was 33.5 years （14 to 47 years）; In 6 cases, fractures occurred at femur, accounting for 75%of all the fractures; Femoral neck fracture was treated by closed reduction and hollow screws fixation;Femoral intertrochanteric fracture, distal femur fracture, and tibiofibula fracture were treated by open reduction and internal fixation with plate;Humerus intercondylar fracture was treated by elbow joint replacement.Intraoperative bleeding was from 50 to 600 mL, an average of 262 mL;Perioperatively, the average use of FⅧ/activated prothrombin complex concentrates （ APCC） was 358 U/kg （125 to 554 U/kg） .Postoperatively, poor wound healing was observed in 2 patients, and the condition improved after symptomatic treatment; In patients with internal fixation, all the fractures united, and the average hea ling time was 14 weeks.No complications such as fixation loosening or rupture occurred after internal fixation.Conclusion：Hemophilia combined with fracture mainly occurred in the young, and the site of fracture was given priority to femur.With perfect preoperative preparation, on the basis