Delayed Diagnosis of Hereditary Angioedema with C1-inhibitor Deficiency in Iranian Children and Adolescents.

Hereditary angioedema is an autosomal dominant deficiency of C1-inhibitor (C1-INH-HAE). This rare but potentially fatal disease gives rise to episodes of bradykinin-mediated edema in face, trunk, extremities, genitalia, gastrointestinal tract and upper airway.(1) This leads to severe outcomes including asphyxiation, unnecessary emergency abdominal surgeries, and a considerably diminished quality of life in addition to high economic burden for the health care system. Rare nature, misleading symptoms, and a lack of awareness among health care providers have resulted in a worldwide delayed diagnosis of C1-INH-HAE. (2) This article is protected by copyright. All rights reserved.