重组凝血因子VIIa（诺其）用于治疗两名III型血管性血友病患者及一种血管性血友病因子抑制剂。
Use of recombinant factor VIIa (NovoSeven) in the treatment of two patients with type III von Willebrand's disease and an inhibitor against von Willebrand factor.
作者信息
Ciavarella N, Schiavoni M, Valenzano E, Mangini F, Inchingolo F
机构信息
Servizio di Coagulazione, Centro Emofilia, Policlinico-Universita, Bari, Italy.
出版信息
Haemostasis. 1996;26 Suppl 1:150-4. doi: 10.1159/000217258.
Two siblings affected by type III von Willebrand's disease with precipitating alloantibodies against von Willebrand's factor (vWF) and not susceptible to treatment with factor VIII/vWF concentrates received recombinant activated factor VII for oral surgery. This therapy, combined with antifibrinolytic drugs and local application of fibrin glue, seems to be effective and safe. It may be considered a promising approach to the management of this rare condition.
两名患有III型血管性血友病的兄弟姐妹，体内存在针对血管性血友病因子（vWF）的沉淀同种抗体，且对VIII因子/vWF浓缩物治疗无反应，他们在接受口腔手术时使用了重组活化VII因子。这种疗法与抗纤溶药物及局部应用纤维蛋白胶相结合，似乎有效且安全。它可能被认为是治疗这种罕见病症的一种有前景的方法。
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