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血友病是最常见的先天性出血性疾病,血友病A及B与性别有关,分别因缺乏因子Ⅷ及Ⅸ而引起。von Willebrand′s病无性别差异,是因缺乏因子Ⅷ及von Willebrand因子所致,后者与血小板粘着性有关,故对毛细血管止血具有重要意义。直到70年代,血友病人的拔牙还是一个危险手术,常需要多次输血、住院、复杂的局部止血措施,但自采用了凝血因子浓缩剂及抗纤维蛋白溶解因子后,情况大为改观。
Hemophilia is the most common congenital hemorrhagic disease. Hemophilia A and B are related to gender and are caused by the lack of factors VIII and IX, respectively. Von Willebrand's disease has no sex difference due to the lack of factor Ⅷ and von Willebrand factor, which is related to platelet adhesion, so it is important for capillary hemostasis. It was not until the 1970s that the extraction of blood from a hemophiliac was a dangerous operation, often requiring multiple blood transfusions, hospitalization, and complicated local hemostasis, but the situation had improved significantly since the introduction of clotting factor concentrates and anti-fibrinolytic factors.