Abdominal angioedema attacks are a frequent and typical symptom of hereditary angioedema (HAE) but very often generate diagnostic problems. The study presents laboratory and clinical findings of 7 patients with HAE 1/2 hospitalized due to severe attacks. In all cases, at admittance severe abdominal pain, flatulence, strong weakness, different grade of nausea/vomiting or diarrhoea and abundant free fluid in peritoneal cavity were present. In the history of all patients, recurrent 2 to 3 day long abdominal attack with ascites, were announced. Laboratory data done before the treatment showed elevated leukocytosis, hematocrit, serum glucose, high D-dimers and decreased value of APTT. All patients had an abdominal ultrasound examination, in 5 patients additional abdominal angio-CT was performed to exclude thromboembolic episode. The infusion of human C1 inhibitor concentrate was administered as causative treatment. Completely withdrawal of symptoms was noted up to72 hrs after infusion. In addition all laboratory parameters normalized as well as the free fluid in abdominal cavity disappeared, however, Ddimers serum level despite a decreasing tendency reached the normal range just after 2 weeks.

Introduction: Hereditary angioedema (HAE) is a rare inherited autosomal dominant disease caused by deficiency or dysfunction of C1 inhibitor (C1INH). Clinical symptoms include recurrent subcutaneous and submucosal angioedema of the internal organs. Abdominal attacks affect more than 90% of patients, are often misdiagnosed and result in unnecessary surgical procedures. Aim: To analyse the utility of imaging studies (USG, CT) in patients with C1INH-HAE during an abdominal attack and remission. Material and methods: We enrolled 40 patients with type I and II HAE (30 women, 10 men; mean age 39 years). The diagnosis of C1INH-HAE was based on patient and family history, significantly reduced values of C1INH serum level and activity. Abdominal and pelvic ultrasound were performed in patients within the first 6 hours of the abdominal attack and repeated during remission. Moreover, 23 cases underwent abdominal or pelvic computed tomography during acute abdominal symptoms. The most common ultrasound and CT findings showed the transient presence of a significant amount of fluid in the free abdominal cavity and intestinal oedema during the symptom progression and spontaneously disappearing during in 90% and 50% of patients, respectively. CT revealed also an enlargement of the mesenteric lymph nodes as well as a fat stranding along the bowel wall thickening. Conclusions: Ultrasound or CT imaging facilitates the diagnosis of the patient suspected of having an abdominal attack due to C1INH-HAE. They allow to identify transitional presence of an abundant fluid in the free abdominal cavity and intestinal swelling which spontaneously disappear with a symptoms attack.