Siegmund B, Pollmann H
Institut für Thrombophilie und Hämostaseologie, Loerstraße 19, 48143 Münster.
Hamostaseologie. 2011 Nov;31 Suppl 1:S29-33.
Desmopressin (DDAVP, Minirin® parenteral), which induces the release of von-Willebrand factor from endogenous stores, is indicated in von Willebrand disease type 1 (VWD 1). In the present study effectiveness of DDAVP was tested and side effects were recorded in patients with VWD 1, von Willebrand disease type 2 (VWD 2) or thrombocytopathy (TCP).
PATIENTS, METHODS: Subjects were analysed prior to and after Minirin parenteral infusion (0.4 μg/kg body weight (b.w.) over 60 minutes) for partial thromboplastin time (PTT, seconds), ADP/epinephrine triggered platelet-function analyzer (PFA-100) occlusion time (seconds), factor VIII activity (FVIII, %), VWF as ristocetin cofactor activity (VWF:RCo, %) and VWF antigen (VWF:Ag, %). Side effects of DDAVP during operative interventions were recorded per questionnaires by the patients.
The mean ± standard deviation dose (n = 165 patients) of Minirin parenteral administered was 0.37 ± 0.02 μg/kg b.w., most often upcoming dental operations (57%) necessitated testing. Coagulation parameters of patients with VWD 1 or TCP normalised in almost all patients, but only in approximately 50% of patients with VWD 2 respectively. Appraisal of effectiveness of Minirin parenteral as good was 96% in case of VWD 1 and 95 % in case of TCP. During minor surgeries (n = 23) in 91% of the patients no complications and in 2 patients (9%) postoperative haemorrhages without need for further interventions occurred, but 83% of the patients reported adverse reactions in the questionnaires, although Minirin parenteral was well tolerated by all patients during DDAVP efficacy tests.
Desmopressin is well tolerated and affective in patients with VWD 1 and thrombocytopathy.
去氨加压素（DDAVP，注射用弥凝）可促使内源性储存的血管性血友病因子释放，适用于1型血管性血友病（VWD 1）。在本研究中，对VWD 1、2型血管性血友病（VWD 2）或血小板病（TCP）患者测试了去氨加压素的有效性，并记录了其副作用。
患者、方法：在静脉输注注射用弥凝（0.4μg/kg体重，60分钟内输完）前后，分析受试者的部分凝血活酶时间（PTT，秒）、ADP/肾上腺素触发的血小板功能分析仪（PFA - 100）闭塞时间（秒）、凝血因子VIII活性（FVIII，%）、血管性血友病因子作为瑞斯托霉素辅因子活性（VWF:RCo，%）和血管性血友病因子抗原（VWF:Ag，%）。患者通过问卷记录手术干预期间去氨加压素的副作用。
静脉注射的注射用弥凝平均剂量±标准差（n = 165例患者）为0.37±0.02μg/kg体重，最常见的是即将进行的牙科手术（57%）需要进行测试。几乎所有VWD 1或TCP患者的凝血参数都恢复正常，但VWD 2患者分别只有约50%恢复正常。对于VWD 1，注射用弥凝有效性评价为良好的比例为96%，对于TCP为95%。在小手术（n = 23）中，91%的患者无并发症，2例患者（9%）术后出血但无需进一步干预，不过83%的患者在问卷中报告有不良反应，尽管在去氨加压素疗效测试期间所有患者对注射用弥凝耐受性良好。
去氨加压素在VWD 1和血小板病患者中耐受性良好且有效。
