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Desmopressin

神经

关键词神经 治疗药物 尿崩症

词汇介绍

拓展阅读

解析

desmopressin

释    义   n. 去氨加压素

例    句   All intranasal formulations of desmopressin are now contraindicated for the treatment of PNE. 从现在开始去氨加压素所有鼻腔用剂型都禁忌用于PNE。

概述

去氨加压素系人工合成的环状九肽,巯基丙酰-L-酪氨酰-L-苯丙氨酰-L-谷氨酰氨酰-L-天冬酰氨酰-L-半胱氨酰-L-脯氨酰-D-精氨酰-L-甘氨酰氨,类似于抗利尿激素(ADH)。去氨加压素是治疗夜尿症以及中枢性尿崩症的一线替代治疗药物。适应证 ①在介人性治疗或诊断性手术前,用于先天性或药物诱发的血小板功能障碍、尿毒症、肝硬化及不明原因引起的出血时间延长,使延长的出血时间缩短或恢复正常。②对本品试验剂量呈阳性反应的轻度甲型血友病及血管性血友病的患者,可用于控制及预防小型手术时的出血,甚至对中

Mechanisms of thrombocytopenia in platelet-type von Willebrand disease复制标题

血小板型血管性血友病血小板减少的机制

发表时间:2019-07-10

影响因子:7.6

作者: Loredana Bury

期刊:Haematologica

Platelet-type von Willebrand disease is an inherited platelet disorder characterized by thrombocytopenia with large platelets caused by gain-of-function variants in GP1BA leading to enhanced GPIbα-von Willebrand factor (vWF) interaction. GPIbα and vWF play a role in megakaryocytopoiesis, thus we aimed to investigate megakaryocyte differentiation and proplatelet-formation in platelet-type von Willebrand disease using megakaryocytes from a patient carrying the Met239Val variant and from mice carrying the Gly233Val variant. Platelet-type von Willebrand disease megakaryocytes bound vWF at an early differentiation stage and generated proplatelets with a decreased number of enlarged tips compared to control megakaryocytes. Moreover, they formed proplatelets upon contact with collagen, differently from normal megakaryocytes. Similarly, collagen triggered megakaryocytes showed defective activation of the RhoA-MLC2 axis, which prevents proplatelet formation, and increased phosphorylation of Lyn, which acts as a negative regulator of GPVI signaling, thus preventing ectopic proplatelet-formation on collagen. Consistently, human and murine bone marrow contained an increased number of extravascular platelets compared to controls. In addition, platelet survival of mutant mice was shortened compared to control mice, and the administration of desmopressin, raising circulating vWF, caused a marked drop in platelet count.

译文

血小板型血管性血友病是一种以血小板减少症为特征的遗传性血小板疾病,其特征是gp1ba中的功能变异体增加导致gpibα-血管性血友病因子(vwf)相互作用增强而导致血小板大量减少。gpibα和vwf在巨核细胞生成中起作用,因此我们旨在研究血小板型血管性血友病(von willebrand disease)患者和携带gly233val变异小鼠的巨核细胞分化和血小板前体形成。血小板型血管性血友病(von willebrand disease)巨核细胞在早期分化阶段与vwf结合,产生的前血小板与对照巨核细胞相比,末端增大数量减少。此外,它们在与胶原接触时形成了不同于正常巨核细胞的前体细胞。类似地,胶原触发巨核细胞显示RoA MLC2轴有缺陷的激活,这阻止了血小板的形成,并且增加了LYN的磷酸化,其作为GPVI信号的负调控因子,从而防止胶原上异位的血小板形成。与对照组相比,人类和小鼠骨髓中的血管外血小板数量一直在增加。此外,与对照组相比,突变体小鼠的血小板存活时间缩短,使用去氨加压素,提高循环vwf,导致血小板计数显著下降。