Document Detail

Usefulness of abdominal ultrasonography in the follow-up of patients with hereditary C1-inhibitor deficiency.
MedLine Citation:
PMID:  19558006     Owner:  NLM     Status:  MEDLINE    
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. CONCLUSION: 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.
Maria Pedrosa; Teresa Caballero; Carmen Gómez-Traseira; Antonio Olveira; Concepción López-Serrano
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Publication Detail:
Type:  Evaluation Studies; Journal Article    
Journal Detail:
Title:  Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology     Volume:  102     ISSN:  1081-1206     ISO Abbreviation:  Ann. Allergy Asthma Immunol.     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-06-29     Completed Date:  2009-07-30     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  9503580     Medline TA:  Ann Allergy Asthma Immunol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  483-6     Citation Subset:  IM    
Allergy Department, University Hospital La Paz, Madrid, Spain.
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MeSH Terms
Abdominal Pain / etiology,  ultrasonography*
Acute Disease
Androgens / adverse effects,  therapeutic use
Angioedemas, Hereditary / complications,  drug therapy,  ultrasonography*
Diagnosis, Differential
Retrospective Studies
Reg. No./Substance:

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