Ehata T, Karasawa F, Satoh T
Department of Anesthesiology, National Defense Medical College, Tokorozawa.
Masui. 1998 Aug;47(8):992-4.
An emergency appendectomy was performed in a patient with von Willebrand disease (vWD) and asthma. vWD is a hemostatic disorder in which the patient lacks von Willebrand factor, playing a critical role in the mediation of platelet adhesion. A 12-year-old boy with vWD underwent an appendectomy under general anesthesia. The patient had not previously received any medication for the treatment of vWD. His bleeding time was over 18 minutes. Confact F injection improved it to 6 minutes. The patient was given Confact F during the perioperative period and no abnormal hemorrhage occurred. Heat-treated factor VIII concentrates can compensate for the low concentration of von Willebrand factor and they have been designed by several different companies and individual researchers with slight variations in composition among the various products. This indicates that some of these products can not be effective in compensating for vWD. In this particular case, Confact F was suitable for this patient with vWD (II A type). In addition, the patient was suffering from asthma. To avoid introducing any superfluous irritants that might induce an asthma attack, we removed the endotracheal tube under deep anesthesia and maintained the airway with a mask until the patient became conscious.
一名患有血管性血友病(vWD)和哮喘的患者接受了急诊阑尾切除术。vWD是一种止血障碍性疾病,患者缺乏血管性血友病因子,该因子在介导血小板黏附中起关键作用。一名12岁患vWD的男孩在全身麻醉下接受了阑尾切除术。该患者此前未接受过任何治疗vWD的药物。他的出血时间超过18分钟。注射康比特F后出血时间改善至6分钟。患者在围手术期给予了康比特F,未发生异常出血。经热处理的凝血因子VIII浓缩物可弥补血管性血友病因子浓度的降低,几家不同的公司和个别研究人员都设计了此类产品,不同产品的成分略有差异。这表明其中一些产品可能无法有效弥补vWD。在这个特殊病例中,康比特F适用于这名vWD(II A型)患者。此外,该患者患有哮喘。为避免引入任何可能诱发哮喘发作的多余刺激物,我们在深度麻醉下拔除气管导管,并用面罩维持气道,直至患者苏醒。