Severe Abdominal HAE Attacks: An Analysis of 7 Cases

Abstract

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.

Abdominal and pelvic imaging in the diagnosis of acute abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency

“…In addition, evaluation of imaging findings as well as medical history data allowed us to identify several characteristic features of free peritoneal fluid. These included variable fluid volume over subsequent attacks, which correlated with pain symptoms, as well as its spontaneous resolution or resolution after ex juvantibus treatment [3,5,10,22,23]. According to Agostoni et al [6] and the results of our findings, the appearance of fluid on ultrasound or CT imaging during an abdominal attack in patients with C1INH-HAE depends on its volume.…”

“…According to the results of our research, attacks occur in more than 90% of patients with C1INH-HAE and constitute a considerable diagnostic challenge for emergency clinicians, surgeons, gastroenterologists, and gynaecologists, requiring a differential diagnosis with numerous other possible conditions presenting with acute abdomen [5,10,14,17,19,20,[23][24][25][26].…”

“…Therefore, patients are commonly misdiagnosed and receive inappropriate treatment including unnecessary exploratory laparotomy [3,5,10,12,[16][17][18][19][20]23]. Finally, abdominal attacks cause long delays in diagnosis, even up to several years, especially when they are the only presenting symptom of HAE [11,13,19,20,[27][28][29][30][31].…”

“…Finally, abdominal attacks cause long delays in diagnosis, even up to several years, especially when they are the only presenting symptom of HAE [11,13,19,20,[27][28][29][30][31]. Therefore, numerous investigators have emphasized the clinical value of abdominal and pelvic imaging and the necessity to introduce these modalities into regular diagnostic workup of patients with abdominal attack in the course of C1INH-HAE [3,5,6,23,26,[32][33][34].…”

“…Exclusion of mesenteric venous thrombosis in patients with HAE and an abdominal attack is a common problem associated with the presence of elevated Ddimer levels, especially in attacks related to abnormalities of the kinin, coagulation and fibrinolysis systems in patients with bradykinin-mediated angioedema typical for patients with C1INH-HAE [10,23,40,41].…”