Pediatr Emerg Care 2021 Apr;37(4):218-223
Professor of Pediatrics, Department of Pediatrics, Division of Emergency Medicine, Cincinnati Children's Hospital Medical Center, and University of Cincinnati Department of Pediatrics, Cincinnati, OH.
Abstract: Hereditary angioedema (HAE) is a rare, often underrecognized genetic disorder caused by either a C1 esterase inhibitor deficiency (type 1) or mutation (type 2). This leads to overproduction of bradykinin resulting in vasodilation, vascular leakage, and transient nonpitting angioedema occurring most frequently in the face, neck, upper airway, abdomen, and/or extremities. Involvement of the tongue and laryngopharynx has been associated with asphyxiation and death. Read More