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1-去氨基-8-D-精氨酸加压素治疗获得性因子VIII抑制剂的非血友病患者

1-Deamino-8-D-arginine vasopressin in the treatment of non-haemophilic patients with acquired factor VIII inhibitor.

作者信息

Muhm M, Grois N, Kier P, Stümpflen A, Kyrle P, Pabinger I, Bettelheim P, Hinterberger W, Lechner K

机构信息

First Department of Medicine, University of Vienna, Austria.

出版信息

Haemostasis. 1990;20(1):15-20. doi: 10.1159/000216100.

Abstract

1-Deamino-8-D-arginine vasopressin (DDAVP) was administered to 3 patients with spontaneous factor VIII inhibitors. In 2 patients with baseline factor VIII levels above 1%, a marked increase of factor VIII activity after DDAVP infusion could be observed. No rise of factor VIII activity after DDAVP was seen in the 3rd patient with a baseline factor VIII level of less than 1%. Daily infusion of DDAVP resulted in a reduction of the efficacy, but the full effect could be retained when DDAVP was given at 48-hour intervals. The effect of DDAVP infusion on factor VIII levels in the 2 responding patients was superior to the treatment with 30 U/kg factor VIII concentrate and approximately equivalent to infusion of 45 U/kg factor VIII concentrate. DDAVP may be a useful and less expensive treatment for patients with acquired factor VIII inhibitors and a baseline factor VIII level of more than 1%.

摘要

对3例自发性因子VIII抑制物患者给予1-去氨基-8-D-精氨酸加压素(DDAVP)。在2例基线因子VIII水平高于1%的患者中,DDAVP输注后可观察到因子VIII活性显著增加。第3例基线因子VIII水平低于1%的患者,DDAVP输注后因子VIII活性未见升高。每日输注DDAVP会导致疗效降低,但每48小时给予DDAVP可保持全部效果。DDAVP输注对2例有反应患者的因子VIII水平的影响优于30 U/kg因子VIII浓缩物治疗,且大致相当于输注45 U/kg因子VIII浓缩物。对于获得性因子VIII抑制物且基线因子VIII水平高于1%的患者,DDAVP可能是一种有用且成本较低的治疗方法。

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