Rizza C R, Spooner R J
Br Med J (Clin Res Ed). 1983 Mar 19;286(6369):929-33. doi: 10.1136/bmj.286.6369.929.
A five year survey of the treatment of patients in the United Kingdom suffering from haemophilia and related disorders was carried out on behalf of the directors of haemophilia centres. The survey showed an increase in the number of patients receiving treatment from the centres, a substantial increase in the total amount of therapeutic materials used, and an increase in the average amount of factor VIII or factor IX used yearly per patient. Home treatment became established for severely affected patients and accounted for roughly half of the total amount of material used. Study of the acquisition of factor VIII or factor IX antibodies (inhibitors) in patients with haemophilia A or haemophilia B showed no increase in antibodies during the survey period, despite the increased use of factor VIII and factor IX concentrates. The occurrence of acute hepatitis in treated patients was also studied and no increased incidence was observed. A near normal median expectation of life in patients with severe haemophilia A was found.
代表血友病治疗中心的负责人,对英国血友病及相关疾病患者的治疗情况进行了为期五年的调查。调查显示,接受治疗中心治疗的患者数量有所增加,治疗材料的使用总量大幅增加,每位患者每年使用的凝血因子 VIII 或凝血因子 IX 的平均量也有所增加。对于重症患者,家庭治疗得以确立,且家庭治疗所用材料约占使用总量的一半。对甲型血友病或乙型血友病患者中凝血因子 VIII 或凝血因子 IX 抗体(抑制剂)的产生情况进行研究后发现,尽管凝血因子 VIII 和凝血因子 IX 浓缩剂的使用量增加,但在调查期间抗体并未增加。同时还对接受治疗患者中急性肝炎的发生情况进行了研究,未观察到发病率上升。研究发现,重度甲型血友病患者的预期寿命中位数接近正常水平。