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轻度和中度甲型血友病及血管性血友病患者对去氨加压素治疗的反应。

Response of patients with mild and moderate hemophilia A and von Willebrand's disease to treatment with desmopressin.

作者信息

de la Fuente B, Kasper C K, Rickles F R, Hoyer L W

出版信息

Ann Intern Med. 1985 Jul;103(1):6-14. doi: 10.7326/0003-4819-103-1-6.

Abstract

Desmopressin was administered intravenously to 68 patients with hemophilia and von Willebrand's disease of mild or moderate severity to assess the safety, reproducibility, and range of response to this new therapeutic alternative. A rise in factor VIII-von Willebrand factor levels was seen in 64 patients, and the magnitude was sufficient to provide normal hemostasis in 55 to 68 spontaneous or traumatic bleeding episodes, dental procedures, or operations. Thus, our experience shows that most patients with mild or moderate hemophilia and von Willebrand's disease can be treated effectively without plasma derivatives. Patients who had two or more infusions of desmopressin at different times had similar responses each time, and members of the same family also had similar responses after desmopressin infusions. Because this drug can be administered without significant side effects, it should have an important role in the management of patients with mild or moderate hemophilia and von Willebrand's disease.

摘要

对68例轻度或中度严重程度的血友病和血管性血友病患者静脉注射去氨加压素,以评估这种新治疗方法的安全性、可重复性和反应范围。64例患者的VIII因子-血管性血友病因子水平升高,其升高幅度足以在55至68次自发性或外伤性出血发作、牙科手术或手术中提供正常止血。因此,我们的经验表明,大多数轻度或中度血友病和血管性血友病患者无需血浆衍生物即可得到有效治疗。在不同时间接受两次或更多次去氨加压素输注的患者每次反应相似,同一家庭的成员在接受去氨加压素输注后反应也相似。由于这种药物给药时无明显副作用,它在轻度或中度血友病和血管性血友病患者的治疗中应发挥重要作用。

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