Hereditary Angioedema in Pediatric Age: An Overview

Introduction : Hereditary angioedema is an autosomal dominant inherited disease that leads to either dysfunction or low level of a circulating inhibitory protein called C1 esterase inhibitor. This condition is characterized by the localized swelling of parts of the body and can be life-threatening if it affects the larynx. Objectives : We aimed to review the recent literature on hereditary angioedema, along with its latest management. Methodology: PubMed database was used for articles selection, papers on were obtained and reviewed. PubMed database was used for articles selection, and the following keys terms: Hereditary angioedema, Bradykinin-mediated angioedema, C1 esterase inhibitor, and emergent therapy. Conclusion: Hereditary angioedema is a self-limited, localized swelling of the dermis, subcutaneous tissues. Or it is a submucosal tissue that is caused by fluid which leaks into the interstitial tissue. It is mediated by the vasoactive substance bradykinin and can be classified into three types depending on the pathophysiology. Patients usually present with swelling of the eyelids, lips, and tongue. However, immediate airway protection is required because life-threatening laryngeal edema may occur. If applicable, aggressive supportive care and avoidance of triggers are in the treatment process. In acute cases, hereditary angioedema is treated with C1 inhibitor (C1-INH) concentrate, bradykinin-B₂-receptor antagonists, or kallikrein inhibitors.