title: Usefulness of abdominal ultrasonography in the follow-up of patients with hereditary C1-inhibitor deficiency.
abstract: BACKGROUND
  Hereditary angioedema (HAE) is caused by the deficiency of functional C1 inhibitor. Symptoms of this disease include cutaneous angioedema, abdominal pain, and even laryngeal edema.
  
  
  OBJECTIVE
  To evaluate the usefulness of abdominal ultrasonography in patients with hereditary C1-inhibitor deficiency in diagnosing acute abdominal edema attacks and possible adverse effects of long-term prophylaxis with attenuated androgens.
  
  
  METHODS
  Fifty-nine adult patients with HAE regularly observed in our department were included whether they were symptomatic or not and whether they received long-term androgen prophylaxis or not. We evaluated the ultrasonographic findings in the assessments performed routinely or in the moment of an acute abdominal attack.
  
  
  RESULTS
  Of the 59 patients, 55 ever had any symptom due to HAE (abdominal location, 78% of the symptomatic patients); 4 patients were asymptomatic. In 11 cases, ultrasonography was performed during acute attacks. Ascites and intestinal wall swelling were found in 7 of these 11 cases and, thus, diagnosis was confirmed. Of the 59 patients, 33 were or had been receiving androgen prophylaxis. Abdominal ultrasonographic assessments were performed routinely in 31 of these patients. Four cases of angiomas, 4 of steatosis, and 1 each of portal hypertension, hepatic cysts, and hepatomegaly were found. Assessments were also performed in 17 patients who did not receive androgen prophylaxis; there were no findings in any of these patients.
  
  
  CONCLUSIONS
  Abdominal ultrasonography has been proved useful as an early tool for diagnosing the adverse effects of therapy and for confirming diagnosis in the case of an acute abdominal attack.
authors: Allergy Department, University Hospital La Paz, Madrid, Spain. m.pedrosa.d@gmail.com
authors: Maria
authors: M
authors: Pedrosa
authors: Teresa
authors: T
authors: Caballero
authors: Carmen
authors: C
authors: Gómez-Traseira
authors: Antonio
authors: A
authors: Olveira
authors: Concepción
authors: C
authors: López-Serrano
mesh_terms: D015746:Abdominal Pain / Q000000981:diagnostic imaging* / Q000209:etiology; D000208:Acute Disease; D000728:Androgens / Q000009:adverse effects / Q000627:therapeutic use; D054179:Angioedemas, Hereditary / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D014463:Ultrasonography
chemical_list: D000728:Androgens