Barbosa Fabiano Timbó, Cunha Rafael Martins da, Barbosa Luciano Timbó
Centro de Ensino Superior de Maceió.
Rev Bras Anestesiol. 2007 Jun;57(3):315-23.
von Willebrand's disease is secondary to a mutation on chromosome 12, and is characterized by a qualitative and quantitative deficiency of the von Willebrand's factor. The diversity of the mutations is responsible for several different clinical manifestations, enabling the classification of several types and subtypes. The coagulopathy is manifested basically through a platelet dysfunction associated with a reduction in the serum levels of factor VIII. The objective of this review was to present the perioperative care of patients with von Willebrand's disease.
The physiopathology, classification, laboratorial diagnosis, and current treatment of von Willebrand's disease, as well as the perioperative management of these patients are discussed.
von Willebrand's disease is the most common hereditary coagulopathy, but it is underdiagnosed due to the complexity of the disease itself. The right classification, proper use of desmopressin, and transfusion of von Willebrand's factor are fundamental for a successful anesthesia.
血管性血友病继发于12号染色体突变，其特征为血管性血友病因子在质和量上的缺乏。突变的多样性导致了几种不同的临床表现，从而可对多种类型和亚型进行分类。凝血功能障碍主要表现为与血清VIII因子水平降低相关的血小板功能障碍。本综述的目的是介绍血管性血友病患者的围手术期护理。
讨论了血管性血友病的生理病理学、分类、实验室诊断、当前治疗方法以及这些患者的围手术期管理。
血管性血友病是最常见的遗传性凝血功能障碍，但由于疾病本身的复杂性，其诊断不足。正确的分类、去氨加压素的合理使用以及血管性血友病因子的输注对于成功实施麻醉至关重要。
