456 Hereditary Angioedema: Report of Ten Mexican Patients at West National Medical Center in Guadalajara City

BACKGROUND: Hereditary angioedema (HAE) is an autosomal dominant inherited condition characterized by swelling of the skin, subcutaneous tissue, and the walls of almost all organs, including upper respiratory and gastrointestinal systems. The aim of this paper is to show the medical community clinical characteristics of 10 Mexican patients with HAE. METHODS: We reviewed medical records of 10 patients with hereditary angioedema. RESULTS: All are women, mean age 36 years; 4 of them with C4 0 mg/dL and the rest with serum levels less than 7 mg/dL. In all patients, C1 INH was determined quantitatively with low serum levels. It was also carried out qualitative determination of C1 INH with negative results in all patients. In 1 patient it was diagnosed also systemic lupus erythematosus. CONCLUSIONS: All patients presented here have HAE type I and the diagnosis was made according to the criteria defined by Cicardi Zingale. They have been long-term treated with danazol with different response, dose range of 100 to 400 mg daily, depending of clinical response in each patient. In only 2 of them was used a selective bradykinin B2 receptor antagonist icatibant with good response.