Acute Hereditary Angioedema With Airway Compromise: Avoiding Airway Intervention With C1 Inhibitor Concentrate:

Immunology and Immunogenetics Insights

WeiZhong ErnestFu¹ 

关键词: Hereditary angioedema;    C1 esterase inhibitor;    laryngeal edema;    airway compromise;    tracheostomy;   

DOI  :  10.1177/1178634518786254

学科分类：生物科学（综合）

来源: Sage Journals

【 摘 要 】

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.

【 授权许可】

CC BY