摘要血友病是一种遗传性出血性疾病,包括血友病A及血友病B,多于儿童时期起病.长期反复出血易导致关节畸形,而按需治疗并不能逆转关节损伤,因此血友病的预防治疗逐渐受到重视.预防治疗旨在预防关节出血及关节病的发生,提高生存质量.目前已有多项临床试验证实了预防治疗的有效性.然而,预防治疗在最佳方案的选择、抑制物的产生、高昂的治疗费用等方面也面临着一些问题.

abstractsHaemophilia,including haemophilia A and haemophilia B,is a hemorrhagic hereditary disease in children.Long-time bleeding may lead to arthropathy,while on-demand therapy couldn't reverse the damage of the affected joint.Therefore,prophylaxis has attracted much attention.The main purpose of prophylaxis is to prevent the hemarthroses and arthropathy and to improve the life quality as well.Recently,several studies confirm the efficacy of prophylaxis.Failure of prophylaxis may relate to several barriers,such as the correct regimen,the development of inhibitor and the exorbitant cost.