血友病患儿围手术期的处理

回顾和评价血友病患儿合并外科疾病围手术期处理。 [方法 ]　全部病例术前测定APTT ,因子Ⅷ和因子Ⅸ的活性。围手术期处理 :术前 ,对合并血友病A ,因子Ⅷ的活性降低及PT延长者 ,给予AHG30u/kg·天 ,静脉滴注 1～ 3天。对合并血友病B者 ,给予凝血酶原复合物 2 0u/kg·天 ,静脉滴注 1～ 3天。术后 3天分别予维持量 ,术后 4～ 6天 ,减量 1/ 3,术后 7～ 9天 ,再减量 1/ 3,术后 10天停药。治疗过程作血浆凝血因子动态监测。 [结果 ]　所有病例转归顺利 ,切口愈合佳 ,无感染及其他并发症。 [结论 ]　血友病不是手术禁忌证 ,及时明确诊断 ,围手术期合理应用针对性血浆制剂 ,是确保手术成功和术后康复的关键。

Perioperative management of children with hemophilia

Objective] To assess the clinical approach in the management of children with hemophilia during the perioperative period. [Methods] All patients were tested for KPTT,factor Ⅷ and Ⅸ The preoperative treatment included intravenous administration of AHG 30u/kg/d,1～3 days for patients with type A hemophilia,and PCC 20u/kg/d,1～3 days for those with type B.This therapeutic regimen was maintained for additional 3 days after the operation. The dose was then reduced to one-third of the initial at 4 to 6 days after operation, and was further decreased to one-third of the preceeding value at 7 to 9th days post operation. The treatment was finally ceased at the tenth day. Serial examinations of plasma concentration of coagulant factors were often performed during the treatment. [Results] There was no complication and all patients had a favorable recovery. [Conclusions] Hemophilia is not a counter-indication for surgery in children. An accurate preoperative clinical diagnosis of the disease and optimal use of coagulant factors during the perioperative phase are critical for the overall success of the surgery and post -operative convalescence.