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血管发育不良在 von Willebrand 病中的表现:临床与基础科学的认识。

Angiodysplasia in von Willebrand Disease: Understanding the Clinical and Basic Science.

机构信息

Department of Pathology and Molecular Medicine, Queen's University, Kingston, Canada.

Department of Medicine, Queen's University, Kingston, Canada.

出版信息

Semin Thromb Hemost. 2017 Sep;43(6):572-580. doi: 10.1055/s-0037-1599145. Epub 2017 May 5.

Abstract

Severe and intractable gastrointestinal bleeding caused by angiodysplasia is a debilitating problem for up to 20% of patients with von Willebrand disease (VWD). Currently, the lack of an optimal treatment for this recurrent problem presents an ongoing challenge for many physicians in their management of affected patients. Over the past few years, studies have pointed to a regulatory role for the hemostatic protein, von Willebrand factor (VWF), in angiogenesis, providing a novel target for the modulation of vessel development. This article will review the clinical implications and molecular pathology of angiodysplasia in VWD.

摘要

由血管发育不良引起的严重和难治性胃肠道出血是 von Willebrand 病(VWD)患者中高达 20%的致残问题。目前,由于缺乏对这种复发性问题的最佳治疗方法,许多医生在治疗受影响的患者时都面临着持续的挑战。在过去的几年中,研究表明止血蛋白 von Willebrand 因子(VWF)在血管生成中具有调节作用,为调节血管发育提供了新的靶点。本文将综述 VWD 中血管发育不良的临床意义和分子病理学。

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