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甲型血友病和血管性血友病患者对去氨加压素(DDAVP)的反应。

Response of patients with hemophilia A and von Willebrand disease to desmopressin (DDAVP).

作者信息

Santiago-Borrero P J, Casanova R

机构信息

Department of Pediatrics, University of Puerto Rico Medical Sciences, School of Medicine, San Juan.

出版信息

Bol Asoc Med P R. 1990 May;82(5):207-10.

PMID:2115785
Abstract

Factor VIII (FVIII) response to desmopressin (deamino-8-D-arginine vasopressin, abbreviated DDAVP) was studied in patients with mild Hemophilia A and von Willebrand Disease (vWD) who attend our Hemophilia Clinic. Thirty eight children and 9 adults had their F VIII components assayed 60 minutes after intravenous (IV) administration of DDAVP, 0.35 microgram/kg. Among 27 hemophiliacs, F VIII coagulant activity (F VIII: C) increased from a mean of 16.8 to 59.2 u/dL; with an average 3.2-fold increase. In 20 vWD patients, the mean F VIII:C and von Willebrand Factor increased from a mean of 50.1 to 136%; and from 22.6 to 75.6%; with an average 3.0 and 5.7-fold increase, respectively. The overall F VIII:C response was good or excellent in 81.5% of the hemophiliacs, and in 89.5% of the vWD patients tested. No significant side effects were observed. This study has demonstrated that IV DDAVP can cause an increase of F VIII:C and vWF to hemostatic levels, and thus it may be useful for the control of bleeding episodes in most of the patients tested in our clinic.

摘要

在我们血友病门诊就诊的轻度甲型血友病和血管性血友病(vWD)患者中,研究了因子VIII(FVIII)对去氨加压素(去氨基-8-D-精氨酸血管加压素,缩写为DDAVP)的反应。38名儿童和9名成人在静脉注射0.35微克/千克DDAVP 60分钟后检测了FVIII成分。在27名血友病患者中,FVIII凝血活性(FVIII:C)从平均16.8单位/分升增加到59.2单位/分升;平均增加3.2倍。在20名vWD患者中,平均FVIII:C和血管性血友病因子分别从平均50.1%增加到136%;从22.6%增加到75.6%;平均分别增加3.0倍和5.7倍。在81.5%的血友病患者和89.5%的接受检测的vWD患者中,FVIII:C的总体反应良好或极佳。未观察到明显的副作用。本研究表明,静脉注射DDAVP可使FVIII:C和vWF增加至止血水平,因此对于控制我们门诊大多数检测患者的出血发作可能有用。

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