Quality of Life Measures in Asthma and Allergic Diseases: 549 Management of Twenty-Five Pediatric Patients with Hereditary Angioedema (Hae) Undergoing Home Treatment—A Clinical Surveillance Program

摘要

BackgroundHereditary angioedema (HAE) is a rare disorder characterized by C1 esterase inhibitor (C1-INH) deficiency. Clinically, HAE is characterized by relapsing episodes of edema at various body sites followed by disease-free intervals of variable duration. Episodes of upper airway obstruction (usually laryngeal edema) are potentially life-threatening and many patients died by asphyxiation in the families. In literature Longhurst et al, Buygun et al and Levi et al showed improvement of quality of life in patients with hereditary angioedema due to home treatment with C1-inhibitor-concentrate.