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血管性血友病因子(VWF)与凝血因子VIII(FVIII)在治疗血管性血友病(VWD)中的相互作用。

Interaction between VWF and FVIII in treating VWD.

作者信息

Miesbach Wolfgang, Berntorp Erik

机构信息

Medical Clinic III, Institute of Transfusion Medicine, Goethe University, Frankfurt, Germany.

Centre for Thrombosis and Haemostasis, Skane University Hospital, Lund University, Malmö, Sweden.

出版信息

Eur J Haematol. 2015 Nov;95(5):449-54. doi: 10.1111/ejh.12514. Epub 2015 Feb 19.

Abstract

In patients with von Willebrand disease (VWD), the absence of von Willebrand factor (VWF) antigen leads to the premature loss of endogenous circulating secreted factor VIII (FVIII), thereby resulting in the dual defect in haemostasis. Consequently, correcting the VWF deficiency also acts to correct the associated defect in FVIII activity because exogenous VWF forms complexes with and protects endogenous FVIII. The purpose of this study was to summarise relevant aspects of the interaction between VWF and FVIII and to analyse their effects on VWD treatment. Differences in the VWF/FVIII ratios in coagulation factor concentrates should be considered when treating VWD.

摘要

在血管性血友病(VWD)患者中,血管性血友病因子(VWF)抗原的缺乏会导致内源性循环分泌的凝血因子VIII(FVIII)过早丧失,从而导致止血功能出现双重缺陷。因此,纠正VWF缺乏也有助于纠正FVIII活性的相关缺陷,因为外源性VWF会与内源性FVIII形成复合物并对其起到保护作用。本研究的目的是总结VWF与FVIII相互作用的相关方面,并分析它们对VWD治疗的影响。在治疗VWD时,应考虑凝血因子浓缩物中VWF/FVIII比例的差异。

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