Abstract

Hereditary angioedema (HAE) is a rare, autosomal dominant disorder caused by a C1-esterase inhibitor (C1-INH) deficiency (type 1) or qualitative defect (type 2). It is characterized by recurrent subcutaneous or submucosal edema attacks in various organs with a frequency from a few attacks to over one hundred attacks per year. We report on the treatment of 141 attacks in a patient with hereditary angioedema with the bradykinin B2 receptor antagonist icatibant. During the entire observation period, efficacy of icatibant was maintained and not altered due to repeated administrations. No systemic or cardiovascular side affects were observed. In the present case, icatibant proved to be an effective and safe drug for the symptomatic treatment of acute HAE attacks. Its efficacy was not impaired by repeated administrations over a three-year period in this patient.

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