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挪威血友病的自我治疗。对输血频率和误工天数的影响。

Self-therapy for haemophilia in Norway. Effect on transfusion frequency and days lost from work.

作者信息

Evensen S A, Thaule R, Grøan K

出版信息

Acta Med Scand. 1979;205(5):395-9.

PMID:443078
Abstract

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.

摘要

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。

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