Demydiuk P F, Perekhrestenko P M, Aver'ianov Ie V, Iushchenko P V, Assa O V, Demydiuk O P, Burnaieva S V, Tomilin V V
Klin Khir. 2011 May(5):59-62.
Hemophilia A and Villebrand's disease owe similar clinical features, for their treatment the preparations, containing factor VIII and Villebrand's factor are used. Preoperatively it is necessary, first of all, to establish the disease diagnosis and its form definitely and then an optimal scheme of hemostatic therapy must be selected with its intraoperative and postoperative correction under control of laboratory monitoring. It is important to reveal timely a hemostasis disorders and a hemophilia of inhibitory form occurrence. Modern rational methods of prophylaxis and treatment of hemorrhage, using desmopresin (for mild forms of hemophilia A and Villebrand's disease type III) as well as preparations of restoration therapy, containing factor VIII and Villebrand's factor, are recommended. Preparations of auxiliary therapy are applied according to certain indications present for elimination of vascular-thrombocytic disorders, fibrinolysis activation, hemophilia of inhibitory form and hormonal disorders occurrence. It is also stressed, that, while these diseases are present, it is necessary to follow direct indications for the operations performance and guaranteeing a reserve of sufficient quantity of corresponding hemostatic preparations.
甲型血友病和血管性血友病有相似的临床特征,在治疗时使用含有凝血因子 VIII 和血管性血友病因子的制剂。术前首先必须明确疾病诊断及其类型,然后选择最佳的止血治疗方案,并在实验室监测的控制下进行术中及术后的调整。及时发现止血障碍和抑制性血友病的发生很重要。推荐采用现代合理的出血预防和治疗方法,如使用去氨加压素(用于轻度甲型血友病和 III 型血管性血友病)以及含有凝血因子 VIII 和血管性血友病因子的替代治疗制剂。根据出现的特定指征应用辅助治疗制剂,以消除血管 - 血小板紊乱、纤维蛋白溶解激活、抑制性血友病和激素紊乱的发生。还强调,在患有这些疾病时,必须遵循手术操作的直接指征,并确保储备足够数量的相应止血制剂。