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