Department of Anesthesia, Otsu Red-Cross Hospital, 1-1-35, Nagara, Otsu, Shiga, 520-8511, Japan.
J Anesth. 2010 Feb;24(1):121-3. doi: 10.1007/s00540-009-0832-6. Epub 2010 Jan 6.
Hereditary angioedema (HAE) is caused by complement factor 1 inhibitor (C1-INH) deficiency, and its mode of inheritance is autosomal dominant. We present a case of an 8-year-old patient who required emergency laparotomy after a traffic accident. General anesthesia with tracheal intubation was necessary. The patient's mother and maternal grandmother had been diagnosed with HAE. HAE is associated with high mortality when airway edema is caused by tracheal intubation. It was impossible to rule out HAE preoperatively in the patient. Therefore, we presumed that he had HAE and treated him with pasteurized C1-INH concentrate. The patient underwent laparotomy uneventfully. Several days after the operation, the laboratory data revealed that the perioperative plasma complement 1 q subunit (C1q) protein level and C1-INH function were not lowered. The diagnosis of HAE was not confirmed, but it was not possible to rule out the diagnosis either. The prophylactic use of a C1-INH in this case may be justified, because the procedure was an emergency and because of the high mortality associated with tracheal intubation in patients with HAE.
遗传性血管性水肿（HAE）是由补体因子 1 抑制剂（C1-INH）缺乏引起的，其遗传方式为常染色体显性遗传。我们报告了一例 8 岁患者，该患者在交通事故后需要紧急剖腹手术。需要全身麻醉和气管插管。患者的母亲和外祖母均被诊断为 HAE。当气道水肿由气管插管引起时，HAE 会导致高死亡率。术前无法排除患者 HAE 的可能性。因此，我们推测他患有 HAE，并使用巴氏杀菌的 C1-INH 浓缩物对其进行治疗。患者顺利进行了剖腹手术。手术后几天，实验室数据显示围手术期血浆补体 1 q 亚基（C1q）蛋白水平和 C1-INH 功能没有降低。未确诊 HAE，但也不能排除该诊断。在这种情况下预防性使用 C1-INH 可能是合理的，因为手术是紧急手术，并且 HAE 患者的气管插管死亡率很高。
