Goti F, Melcher G A, Späth P, Wüthrich B
Chirurgische Klinik, Spital Uster.
Dtsch Med Wochenschr. 1998 Oct 2;123(40):1166-71. doi: 10.1055/s-2007-1024139.
Since the age of 16 years a now 25-year-old woman had been known to have C1-inhibitor (C1-INH) deficiency. She presented herself at the emergency department because of acute severe lower abdominal cramps. A urinary infection had been treated with antibiotics for the previous 4 days. There was marked pain on pressure over the lower abdomen, but there were no signs of peritonitis and bowel sound were normal. There had been no nausea or vomiting and the stools had been normal.
There was a leukocytosis of 10,200/microliter, moderately elevated C-reactive protein (44.8 mg/l), haemoglobin concentration of 17 g/dl and haematocrit of 51%. Radiology revealed oedema of the duodenum and sonography showed free fluid in the abdomen.
After excluding an acute abdomen and in view of the C1-INH deficiency treatment was symptomatic. All symptoms completely disappeared after 2 days.
Exclusively gastrointestinal symptoms and ascites are rare in patients with hereditary angioedema. But knowledge of this manifestation of the disease is important because patients are sometimes operated under the false diagnosis of acute abdomen. In severe cases symptomatic treatment may have to be supplemented by C1-INH administration. Prevention with attenuated androgens should be started or modified, respectively.
一名现年25岁的女性自16岁起就被诊断为C1抑制物（C1-INH）缺乏。她因急性严重下腹部绞痛前往急诊科就诊。前4天因泌尿系统感染接受了抗生素治疗。下腹部按压时有明显疼痛，但无腹膜炎体征，肠鸣音正常。无恶心或呕吐，大便正常。
白细胞计数为10,200/微升，C反应蛋白中度升高（44.8毫克/升），血红蛋白浓度为17克/分升，血细胞比容为51%。放射学检查显示十二指肠水肿，超声检查显示腹腔内有游离液体。
排除急腹症后，鉴于C1-INH缺乏，进行了对症治疗。2天后所有症状完全消失。
在遗传性血管性水肿患者中，仅出现胃肠道症状和腹水的情况较为罕见。但了解该疾病的这种表现很重要，因为患者有时会在急性腹痛的错误诊断下接受手术。在严重病例中，可能需要在对症治疗的基础上补充C1-INH。应分别开始或调整使用减毒雄激素进行预防。
