Ito Hiroyuki, Satoh Masaaki, Seo Norimasa
Department of Anesthesiology and Critical Care Medicine, Jichi Medical University, Shimotsuke, Japan.
Masui. 2008 Feb;57(2):203-5.
von Willebrand disease is one of the inherited bleeding disorders, and caused by the deficiency or the abnormality of von Willebrand factor. A 28-year-old woman with von Willebrand disease (type II) was scheduled for cesarean section under general anesthesia. Platelet count was within normal limits. However, the prolongation of both APTT and bleeding time was confirmed. The level of von Willbrand factor was within normal ranges, but its activity was about 55%. The platelet aggregation was below 1.3%. We managed to administer factor VIII concentrate to her intravenously before and after operation. We did not perform epidural catheterization because we cannot forecast the amount of bleeding. We could successfully manage this case without any complications. Intravenous patient controlled analgesia was given for postoperative pain. The perioperative course was uneventful and the patient was discharged 7 days after the operation.
血管性血友病是一种遗传性出血性疾病，由血管性血友病因子缺乏或异常引起。一名28岁的血管性血友病（II型）女性计划在全身麻醉下进行剖宫产。血小板计数在正常范围内。然而，确认活化部分凝血活酶时间（APTT）和出血时间均延长。血管性血友病因子水平在正常范围内，但其活性约为55%。血小板聚集率低于1.3%。我们在手术前后成功地为她静脉注射了凝血因子VIII浓缩物。由于无法预测出血量，我们没有进行硬膜外导管置入。我们成功地处理了该病例，没有任何并发症。术后给予静脉自控镇痛以缓解疼痛。围手术期过程顺利，患者术后7天出院。
