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产妇 1C 型(清除型)血管性血友病的围产期管理。

Peripartum management of a parturient with type 1C (clearance) von Willebrand disease.

机构信息

Department of Anesthesia, British Columbia Women's Hospital, Vancouver, BC, Canada.

Adult Bleeding Disorders Program of British Columbia, St Paul's Hospital, Vancouver, BC, Canada.

出版信息

Int J Obstet Anesth. 2020 Nov;44:112-115. doi: 10.1016/j.ijoa.2020.08.005. Epub 2020 Aug 20.

Abstract

Peripartum replacement of factor VIII and von Willebrand factor is not usually required in type 1 von Willebrand disease, as the levels of endogenous factors tend to increase to within the normal range as a physiological change of pregnancy. However, there is wide heterogeneity of genotypes and phenotypes associated with type 1 von Willebrand disease. Here, we describe the anesthetic management of a parturient with type 1C von Willebrand disease, a subtype characterized by decreased plasma von Willebrand factor survival.

摘要

1 型血管性血友病患者通常不需要在围产期补充凝血因子 VIII 和血管性血友病因子,因为随着妊娠的生理变化,内源性因子水平会升高至正常范围内。然而,1 型血管性血友病存在广泛的基因型和表型异质性。在此,我们描述了一例 1C 型血管性血友病产妇的麻醉管理,该亚型的特点是血浆血管性血友病因子生存时间缩短。

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