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血管性血友病与血管发育异常之间的关联

[Association between von Willebrand disease and angiodysplasia].

作者信息

Salinas Laval José, Triantafilo Nicolás, Zúñiga Pamela

机构信息

Facultad de Medicina, Universidad Austral de Chile, Valdivia, Chile.

Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

出版信息

Rev Med Chil. 2020 Oct;148(10):1475-1480. doi: 10.4067/S0034-98872020001001475.

Abstract

Von Willebrand factor (vWf) is a fundamental multimeric plasma glycoprotein in the coagulation process. Its function is to mediate platelet adhesion and to stabilize circulating factor VIII. A functional or quantitative alteration of vWf gives rise to von Willebrand disease (vWD). The association between vWD and angiodysplasia was described in 1967, but it was only until 2011 that Starke et al demonstrated the in vitro and in vivo role of vWf in angiogenesis. Congenital or acquired vWf deficiency, especially of high molecular weight multimeters, not only favors bleeding, but also contributes to increased angiogenesis in these patients. The treatment should be focused both on the control of the acute episode of gastrointestinal bleeding, with vWf replacement therapy and local endoscopic treatment, as well as on the prevention of the progression of angiodysplasia and future bleeding. There are different published therapeutic approaches using vWf replacement that are not effective in all patients. Recently, angiogenesis inhibitor medications have been used.

摘要

血管性血友病因子(vWf)是凝血过程中一种重要的多聚体血浆糖蛋白。其功能是介导血小板黏附并稳定循环中的凝血因子VIII。vWf的功能或定量改变会引发血管性血友病(vWD)。1967年首次描述了vWD与血管发育异常之间的关联,但直到2011年,斯塔克等人才证明了vWf在血管生成中的体内和体外作用。先天性或获得性vWf缺乏,尤其是高分子量多聚体的缺乏,不仅会导致出血倾向增加,还会促使这些患者的血管生成增加。治疗应侧重于通过vWf替代疗法和局部内镜治疗来控制胃肠道出血的急性发作,以及预防血管发育异常的进展和未来出血。目前已发表了多种使用vWf替代疗法的治疗方法,但并非对所有患者都有效。最近,血管生成抑制剂药物也已被应用。

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