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. 2019 Apr 14;40(4):312-316.
doi: 10.3760/cma.j.issn.0253-2727.2019.04.009.

[Clinical observation of desmopressin in the treatment of 15 patients with DDAVP]

[Article in Chinese]
M J Hou  1 , Z Q Yu, Z N Ma, W Zhang, X Bai, L J Cao, J Su, P P Sha, Z Y Wang, C G Ruan
Affiliations

[Clinical observation of desmopressin in the treatment of 15 patients with DDAVP]

[Article in Chinese]
M J Hou et al. Zhonghua Xue Ye Xue Za Zhi. .

Abstract
in English, Chinese

Objective: To assess the significance of DDAVP use in the diagnosis and treatment of VWD. Methods: An analysis of 15 VWD cases who referred to Hematology Division of First affiliated Hospital of Soochow University and treated with DDAVP from March 2016 to August 2018 was conducted. Efficacy and treatment response of DDAVP were monitored by observations of changes in factor Ⅷ procoagulant (FⅧ∶C) and von Willebrand Factor (VWF) related indicators before and 2 h after DDAVP injection. Results: Of 15 cases with VWD, 7 males and 8 females with a median age of 23 (6-46) years, 7 of 9 type I VWD patients achieved complete response (CR) , 1 type 2A VWD case CR, 5 type 3 VWD ones no response (NR) . The VWF multimer analysis in 5 patients combined with other plasma VWF values were in accordance with the known diagnosis. Conclusions: DDAVP was effective in most type 1 patients, and ineffective in some type 2 and almost all type 3 cases. It was helpful for diagnosis and subsequent treatment planning.

目的： 探讨去氨加压素（DDAVP）在血管性血友病（VWD）诊断和治疗中的应用价值。 方法： 对2016年3月至2018年8月期间15例接受DDAVP治疗的VWD患者进行分析，观察治疗反应及患者用药前及用药后2 h凝血因子Ⅷ活性及血管性血友病因子（VWF）相关指标变化。 结果： 15例VWD患者中，男7例，女8例，中位年龄23（6~46）岁。9例1型VWD患者中，7例达完全反应；1例2A型VWD患者达完全反应，5例3型VWD患者无反应。对5例患者进行VWF多聚体分析，结合其他VWF相关检测指标，DDAVP治疗效果与患者疾病分型相符合。 结论： DDAVP对1型VWD患者可达到较好疗效，对部分2型VWD及3型VWD患者反应不佳；VWD患者对DDAVP的治疗反应对治疗选择具有重要意义。.

Keywords: Desmopressin; Treatment outcome; von Willebrand diseases; von Willebrand factor.

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