目的探讨重组人凝血因子Ⅷ制剂小剂量短程预防性输注能否有效减少中重度血友病A患儿关节出血问题。方法对2008年11月-2009年4月期间就诊的13例年龄3～11岁的中重度血友病A患儿,均在为期2个月内接受重组人凝血因子Ⅷ2次/周、间隔3d、每次7.5～10.0U/kg的静脉预防性输注,记录治疗前2个月与治疗2个月时关节出血次数,以及同一关节反复发生出血的情况。结果治疗前关节出血的发生次数为(3.77±2.13)次,治疗后关节出血的发生次数为(0.46±0.87)次,治疗前后比较,差异有统计学意义(P<0.01);治疗前靶关节出血的发生率为35.7%,治疗后靶关节出血的发生率为0.0%,治疗前后比较,差异有统计学意义(P<0.01)。患儿治疗成本约510～680元/(kg.2个月)。结论重组人凝血因子Ⅷ制剂小剂量短疗程预防性输注能有效减少中重度血友病A患儿关节出血次数,同时可有效减少靶关节出血的发生率,从而在一定程度上保护关节的功能。治疗费用相对可接受。 Objective To investigate whether low-dose, short-term, prophylactic infusion of recombinant human factor Ⅷ can effectively reduce joint bleeding in children with moderate-severe hemophilia A. Methods Thirteen patients with moderate to severe hemophilia A who were treated between November 2008 and April 2009, aged 3-11 years, received recombinant human factor Ⅷ 2 times per week for 2 months at intervals of 3 days , Each 7.5 ~ 10.0U / kg intravenous prophylaxis infusion, record 2 months before treatment and treatment of 2 months when the number of joint bleeding, and repeated the same joint bleeding situation. Results The number of joint hemorrhage before treatment was (3.77 ± 2.13) times and the incidence of joint hemorrhage was (0.46 ± 0.87) times before and after treatment. The difference was statistically significant before and after treatment (P <0.01) The incidence of hemorrhage was 35.7%. The incidence of target joint bleeding after treatment was 0.0%. The difference was statistically significant before and after treatment (P <0.01). Children with treatment costs about 510 ~ 680 yuan / (kg.2 months). Conclusions The prophylactic infusion of recombinant human coagulation factor Ⅷ in small dose and short course can effectively reduce the number of joint hemorrhage in children with moderate-severe hemophilia A. It can also effectively reduce the incidence of target joint bleeding and thus protect the joint function to a certain extent . Treatment costs are relatively acceptable.