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一名患有3型血管性血友病的12岁女孩在择期心脏手术后持续输注血管性血友病因子和凝血因子VIII。

Continuous infusion of von Willebrand factor and factor VIII after elective heart surgery in a 12-year-old girl with von Willebrand disease type 3.

作者信息

Kühne T, Imbach P, Marbet G A, Caliezi C, Devay J

机构信息

Division of Oncology/Haematology, University Children's Hospital, Basel, Switzerland.

出版信息

Pediatr Hematol Oncol. 1999 Nov-Dec;16(6):551-6. doi: 10.1080/088800199276859.

Abstract

The continuous infusion of von Willebrand factor (VWF) in a 12-year-old girl with type 3 von Willebrand disease is described. The patient had elective heart surgery with cardiopulmonary bypass for closure of her atrial septum defect. The surgical procedure lasted 3 h. A presurgical bolus followed by a postoperative continuous infusion of factor VIII/VWF concentrates was administered. During the continuous infusion of clotting factors, stable plasma levels of hemostasis and avoidance of dangerously low levels of factor concentrates were achieved. No peri- or postsurgical complications occurred. Continuous infusion of clotting factors allows constant and hemostatic factor concentrations to be maintained with the possibility of dose titration and adjustment.

摘要

本文描述了对一名患有3型血管性血友病的12岁女孩持续输注血管性血友病因子(VWF)的情况。该患者接受了择期心脏手术,采用体外循环闭合房间隔缺损。手术过程持续了3小时。术前给予一次推注,术后持续输注VIII因子/VWF浓缩物。在持续输注凝血因子期间,实现了稳定的血浆止血水平,并避免了凝血因子浓缩物出现危险的低水平。围手术期和术后均未发生并发症。持续输注凝血因子可维持恒定且具有止血作用的因子浓度,同时有可能进行剂量滴定和调整。

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