Evensen S A, Thaule R, Grøan K
Acta Med Scand. 1979;205(5):395-9.
During the years 1975--77, 35 patients with haemophilia A or B (factor level of 1% or less) were instructed to administer concentrates of the deficient factor (mean dose 14 U/kg) i.v. in case of episodic, uncomplicated bleedings without prior consultation with a physician. At Dec. 1977, 33 of these patients (29 with haemophilia A, 4 with B) were taking part in the program. They represent 35% of all Norwegian patients above 7 years of age with severe haemophilia in whom high-titered antibodies have not been demonstrated. For 13 patients, records from 2 years on self-therapy have been compared with records from the last year before self-therapy. During the first year on self-therapy the number of days lost from work dropped by 77%, while the number of transfusions increased by 22%. During the second year, days lost from work were still low (68% decrease compared to the year preceding self-therapy) and the transfusion frequency remained unchanged. Significant side-effects of antihaemophilic concentrates were not observed and vein damage was not a problem. Joint motion studies did not indicate progress of haemophilic arthropathy during self-therapy. Properly supervised self-therapy is practical and safe and improves considerably to quality of life.
在1975年至1977年期间，35例甲型或乙型血友病患者（凝血因子水平为1%或更低）被指导在发生偶发性、无并发症出血时，无需事先咨询医生，静脉注射缺乏的凝血因子浓缩物（平均剂量14 U/kg）。1977年12月，其中33例患者（29例甲型血友病，4例乙型血友病）参与了该项目。他们占挪威所有7岁以上重度血友病患者的35%，这些患者尚未检测出高滴度抗体。对于13例患者，将自我治疗两年的记录与自我治疗前最后一年的记录进行了比较。在自我治疗的第一年，误工天数减少了77%，而输血次数增加了22%。在第二年，误工天数仍然很低（与自我治疗前一年相比减少了68%），输血频率保持不变。未观察到抗血友病浓缩物的明显副作用，静脉损伤也不是问题。关节活动研究未表明自我治疗期间血友病性关节病有进展。适当监督的自我治疗切实可行且安全，可显著改善生活质量。
