Acute Hereditary Angioedema With Airway Compromise: Avoiding Airway Intervention With C1 Inhibitor Concentrate. (11th July 2018)

• Record Type: Journal Article
• Authors: Fu, WeiZhong Ernest; Tan, Sze-Chin; Ho, Eu Chin
• Is Part Of: Immunology and immunogenetics insights. Volume 10(2018)
• Publication Date: 2018-07-11
• Subjects: Hereditary angioedema -- C1 esterase inhibitor -- laryngeal edema -- airway compromise -- tracheostomy; Immunology -- Periodicals; Immunogenetics -- Periodicals; Immunogenetics; Immunology; Immunity -- Periodicals; Electronic journals; Fulltext; Internet Resources; Periodicals; Periodicals; 571.9605
• DOI: 10.1177/1178634518786254
• Languages: English
• ISSNs: 1178-6345

Abstract

Introduction: Hereditary angioedema (HAE) is an autosomal dominant disease caused by deficiency of the plasma protein C1 inhibitor (C1-INH). Patients classically present with recurrent localized subcutaneous or submucosal edema lasting for 2 to 5 days, severe abdominal pain, or acute airway obstruction which can be fatal. Case presentations: We highlight 2 patients with acute airway compromise secondary to HAE who were successfully treated with plasma-derived C1-INH concentrates. Conclusions: The timely administration of plasma-derived C1-INH concentrates for the acute treatment of HAE has been proven to be effective in both patients in aborting an airway complication. A high index of suspicion is required for the early diagnosis and treatment of this potentially fatal condition.