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[去氨加压素在甲型血友病和血管性血友病中的应用]

[Desmopressin in hemophilia A and Von Willebrand's disease].

作者信息

Castro Cabezas M, de Graaf J C, Kraayenhagen R J, Freen M, Poortman J, Hart H C

机构信息

Afd. Inwendige Geneeskunde, Ziekenhuis De Lichtenberg, Amersfoort.

出版信息

Ned Tijdschr Geneeskd. 1991 Mar 2;135(9):367-71.

PMID:1901629
Abstract

The treatment with desmopressin prior to surgery of patients with mild haemophilia A (HA) and Von Willebrand's disease (VWD) was retrospectively evaluated in a general hospital, from 1978 until 1987. From a group of 87 treated patients, 40 patients are reported (21 VWD, 19 HA) of which plasma factor VIII (FVIII) and Von Willebrand factor (VWF) concentrations were determined before, and twice after desmopressin treatment. Desmopressin was administered intravenously at a dose of 0.4 micrograms/kg body weight. Tranexamic acid was used only when surgery in the mouth cavity was performed, at a dose of 1 gram three times a day. Side effects were seen only in 5 patients (3 VWD, 2 HA). No significant difference between both groups was seen in bleeding tendency, transfusion necessity and side effects (chi 2 test). In both groups, FVIII and VWF concentrations increased significantly after 20 and 60 minutes following DDAVP administration (paired t-test). After 360 minutes, the FVIII concentration increased significantly in both groups, however, only in the VWD patients did VWF increase significantly. In neither group did initial FVIII concentrations correlate with the increase in FVIII (linear regression analysis). One female patient reacted differently to DDAVP, with a decrease in FVIII and VWF values. Desmopressin is a safe and effective agent in the management and prophylaxis of bleeding tendency in patients with mild HA and mild VWD.

摘要

1978年至1987年期间,在一家综合医院对轻度甲型血友病(HA)和血管性血友病(VWD)患者术前使用去氨加压素的治疗情况进行了回顾性评估。在87例接受治疗的患者中,报告了40例患者(21例VWD,19例HA),测定了他们在去氨加压素治疗前以及治疗后两次的血浆因子VIII(FVIII)和血管性血友病因子(VWF)浓度。去氨加压素以0.4微克/千克体重的剂量静脉给药。仅在进行口腔手术时使用氨甲环酸,剂量为每日3次,每次1克。仅5例患者(3例VWD,2例HA)出现了副作用。两组在出血倾向、输血必要性和副作用方面未见显著差异(卡方检验)。两组中,在给予去氨加压素后20分钟和60分钟时,FVIII和VWF浓度均显著升高(配对t检验)。360分钟后,两组的FVIII浓度均显著升高,然而,仅VWD患者的VWF显著升高。两组中初始FVIII浓度均与FVIII的升高无相关性(线性回归分析)。一名女性患者对去氨加压素反应不同,FVIII和VWF值降低。去氨加压素在治疗和预防轻度HA和轻度VWD患者的出血倾向方面是一种安全有效的药物。

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