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Title: Abdominal attacks in patients with hereditary angioedema due to deficiency of C1 inhibitor (rare disease) : diagnostic possibilities of imaging tests
Abstract:
Hereditary angioedema (HAE) due to Cl-inhibitor (Cl-INH) deficiency is a rare hereditary disease characterized by recurrent subcutaneous or submucosal angioedema due to uncontrolled bradykinin production caused by Cl-INH dysfunction. Submucosal gastrointestinal swellings provoking abdominal attacks are common and mimie acute abdomen, thus constituting a diagnostic challenge. We aimed to investigate the difficulties in diagnosing abdominal attacks in patients with Cl-INH-HAE and to assess the diagnostic value of medical history, the course of the attack, abdominal imaging, and treatment efficacy. The retrospective analysis of diagnostic problems and treatment complications of abdominal attacks in 274 patients with Cl-INH-HAE were performed. The value of history, laboratory findings, prodromal symptoms and course of attacks and imaging were assessed. Abdominal attacks were confirmed in 274 of the 322 patients (85%; 190 women and 84 men; age, 4-70 years). In 49% of cases, the abdominal attack was the first and the only symptom for years. The simultaneous presence of marginal 'e rythema (45% of cases), subcutaneous edema (30%), and pharyngo-laryngeal edema (10%) facilitated the diagnosis of an abdominal attack due to Cl-INHlHAE. Abdominal attacks manifested with recurrent acute abdominal symptoms lasting 2 to 5 days. The disease course was characterized by the p ; hase of progressive prodromal symptoms followed by peak symptoms and spontaneous symptom resolution. Abdominal imaging often revealed abundant ascites and limited bowel edema. In 60 cases (22% ), the diagnostic difficulties resulted in exploratory laparotomy, which was inconclusive in 48 patients (80%). The attacks usually subsided within 2 hours from the administration of recommended drugs (plasma-derived Cl-INH , recombinant Cl-INH or icatibant). We conclude that recurrent abdominal attacks lasting a few days and resolving spontaneously were common symptoms of ClINH- HAE. Abdominal imaging revealed transitional fluid or bowel edema. The effectiveness of recommended drugs as plasma-derived Cl-INH, recombinant Cl-INH or icatibant confirmed the diagnosis.
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Rada Dyscypliny Nauki medyczne
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Last modified:
Feb 20, 2024
In our library since:
Feb 20, 2024
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20
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22
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http://dl.cm-uj.krakow.pl:8080/publication/5043
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| Edition name | Date |
|---|---|
| ZB-138620 | Feb 20, 2024 |
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