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先天性和获得性出血性疾病患者使用去氨加压素(DDAVP)的经验。

Experience of desmopressin (DDAVP) administration in patients with congenital and acquired bleeding disorders.

作者信息

Tsay W, Shen M C

机构信息

Department of Internal Medicine, National Taiwan University, College of Medicine, Taipei, R.O.C.

出版信息

J Formos Med Assoc. 1992 Oct;91(10):962-9.

PMID:1362675
Abstract

Desmopressin (DDAVP) 0.3 micrograms/kg was administered intravenously to three normal volunteers and 12 patients with von Willebrand's disease (vWD), congenital or acquired platelet function defect, or uremic bleeding to assess its effects and side effects. DDAVP significantly shortened the bleeding time as compared with basal values. The mean peak post-DDAVP level of factor VIII coagulant activity increased 5.9 +/- 0.5 (mean +/- SEM) fold, von Willebrand factor antigen increased 3.7 +/- 0.3 fold, von Willebrand factor ristocetin cofactor activity increased 4.6 +/- 0.6 fold and the tissue-type plasminogen activator antigen increased 3.4 +/- 0.6 fold. Analysis of the multimeric structure of the von Willebrand factor revealed that type I vWD had complete correction after DDAVP infusion transiently. Except for a mild drop in both systolic and diastolic blood pressures, few side effects were noted. By concomitant intravenous infusion of DDAVP and oral administration of tranexamic acid, we successfully treated two cases of type I vWD undergoing tooth extraction, and one case of acquired bleeding disorder undergoing a biopsy of a mandibular mass, and a uremic patient complicated by intractable traumatic hematuria. Our experiences confirmed that most patients with vWD and some patients with congenital or acquired bleeding disorders can be treated effectively by DDAVP infusion without the need for plasma product replacement. In this study we found that a patient with a variant form of type I vWD had prolongation of the bleeding time, thrombocytopenia and platelet aggregation after DDAVP infusion.

摘要

对3名正常志愿者以及12名患有血管性血友病(vWD)、先天性或获得性血小板功能缺陷或尿毒症出血的患者静脉注射去氨加压素(DDAVP),剂量为0.3微克/千克,以评估其效果和副作用。与基础值相比,DDAVP显著缩短了出血时间。DDAVP注射后,因子VIII凝血活性的平均峰值水平增加了5.9±0.5(平均值±标准误)倍,血管性血友病因子抗原增加了3.7±0.3倍,血管性血友病因子瑞斯托霉素辅因子活性增加了4.6±0.6倍,组织型纤溶酶原激活物抗原增加了3.4±0.6倍。对血管性血友病因子多聚体结构的分析显示,I型vWD患者在输注DDAVP后短暂完全纠正。除了收缩压和舒张压略有下降外,几乎未观察到副作用。通过同时静脉输注DDAVP和口服氨甲环酸,我们成功治疗了2例接受拔牙的I型vWD患者、1例接受下颌肿块活检的获得性出血性疾病患者以及1例并发顽固性创伤性血尿的尿毒症患者。我们的经验证实,大多数vWD患者以及一些先天性或获得性出血性疾病患者可以通过输注DDAVP得到有效治疗,而无需血浆制品替代。在本研究中,我们发现1例I型vWD变异型患者在输注DDAVP后出血时间延长、血小板减少且血小板聚集。

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