Abstract
Background: Hereditary angioedema (HAE) is a rare disease characterized by recurrent, self-limiting attacks of subcutaneous and submucosal edema. Though a majority of patients will experience symptom onset before 20 years of age, there is a paucity of published literature regarding the management of HAE in children and adolescents. Methods: A comprehensive literature review regarding the management of pediatric HAE due to C1-esterase inhibitor deficiency (C1 INH) was performed. Results: A collection of case reports and case series suggest antifibrinolytics and attenuated androgens at low dose are safe and effective options for short- and long-term prophylaxis in pediatric HAE. Plasma-derived C1 INH preparations are available for both on-demand and prophylactic therapy in the United States, and post-hoc analyses of the pediatric patients enrolled in the larger clinical trials support their use in pediatric HAE. Ecallantide and icatibant are kinin-pathway modulators, but only ecallantide is currently approv...
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