Vonwildebrand病围生期处理1例报告

El Hadloussi Abdelaziz, El Hamdouchi Hajar, A. Mohammed, N. Youness, A. Abidi, Jebli Salma, A. Tazi
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引用次数: 0

摘要

了解凝血障碍是至关重要的,特别是对产科医生和重症监护提供者管理的产妇,因为这些疾病可能会对围产期的母亲和孩子产生重大影响。血管性血友病或血管性血友病是最常见的先天性出血性疾病。它是受常染色体遗传与可变外显率和表达。本病的病因在于vonWillebrand因子(vWF)的定量(1型、3型)或定性(2型)异常。vWF在凝血中有两个重要功能。一方面,它作为VIII因子的载体蛋白在血浆中循环,具有保护作用;另一方面,它负责血小板粘附到受损血管的内皮下层和血小板聚集。出血的风险因损伤的严重程度而异。治疗管理策略取决于疾病的类型。在本文中,我们报告了两例阴道分娩的血管性血友病患者的观察。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Peripartum Management of Vonwildebrand Disease: A Case Report
Understanding coagulation disorders is essential, especially for obstetricians and intensive care providers managing parturients, as these disorders can have significant consequences for the mother and child during the peripartum period. Von Willebrand-Jürgens syndrome or von Willebrand disease is the most common congenital bleeding disorder. It is subject to autosomal inheritance with variable penetrance and expressivity. The cause of the disease lies in quantitative (type 1, type 3) or qualitative (type 2) abnormalities of vonWillebrand factor (vWF). vWF has two important functions in coagulation. On the one hand, it circulates in plasma as a carrier protein for factor VIII and thus has a protective function; on the other hand, it is responsible for platelet adhesion to the subendothelium of injured vessels and platelet aggregation . The risk of bleeding varies according to the severity of the injury. The therapeutic management strategy depends on the type of disease. In this paper, we report the observation of two patients with von Willebrand disease who had vaginal deliveries.
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