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成人主动脉瓣狭窄患者获得性血管性血友病因子缺乏症:一项系统评价

Acquired Von Willebrand Deficiency in Adults With Aortic Stenosis: A Systematic Review.

作者信息

Ramesh Prasana, Kanagalingam Suthasenthuran, Zargham Ul Haq Fnu, Victory Srinivasan Nishok, Khan Aujala Irfan, Mashat Ghadi D, Hazique Mohammad, Khan Kokab Irfan, Khan Safeera

机构信息

Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.

General Surgery, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.

出版信息

Cureus. 2022 Sep 7;14(9):e28879. doi: 10.7759/cureus.28879. eCollection 2022 Sep.

Abstract

Von Willebrand factor (VWF) deficiency is associated with bleeding complications. The congenital type of Von Willebrand disease(VWD) is a very well-known bleeding disorder and sometimes may be associated with life-threatening hemorrhage. This systematic review is aimed at gathering further knowledge regarding the pathology of an acquired VWD form within a population of patients with aortic stenosis (AS) by shortlisting quality articles on this theme, through the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) 2020 guidelines. High shear stress caused by the stenotic valve cleaves VWF multimers, causing a relative state of deficiency. The condition returns to baseline immediately following surgical replacement of the valve. Results across eight studies reviewed by a majority concluded that in an AS patient with bleeding, the most likely cause is an acquired deficiency of VWF, associated with factors influencing blood flow and caused by the in-situ valve. However, several studies suggested otherwise/were misclassifications. This review highlighted the relationship between AS and acquired VWF deficiency and should be foreseen as an adverse complication, attracting further research and future theragnostic strategies for this condition.

摘要

血管性血友病因子(VWF)缺乏与出血并发症相关。先天性血管性血友病(VWD)是一种广为人知的出血性疾病,有时可能与危及生命的出血有关。本系统评价旨在通过按照系统评价和Meta分析的首选报告项目(PRISMA)2020指南筛选关于该主题的高质量文章,收集更多关于主动脉瓣狭窄(AS)患者群体中获得性VWD形式的病理学知识。狭窄瓣膜引起的高剪切应力会切割VWF多聚体,导致相对缺乏状态。瓣膜手术置换后,病情立即恢复到基线水平。大多数人回顾的八项研究结果得出结论,在有出血症状的AS患者中,最可能的原因是获得性VWF缺乏,这与影响血流的因素有关,且由原位瓣膜引起。然而,一些研究提出了不同观点/存在错误分类。本综述强调了AS与获得性VWF缺乏之间的关系,应将其视为一种不良并发症,吸引对此病症的进一步研究和未来的诊疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/92bc/9541123/cde8b5ef8222/cureus-0014-00000028879-i01.jpg

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