Pharmacokinetic Profile of Subcutaneous C1-Esterase Inhibitor (C1-INH [SC]) in Adolescent and Adult Patients with Hereditary Angioedema (HAE)

Background: Hereditary angioedema (HAE) types I and II are genetic disorders due to low levels or improper function of a protein called C1-esterase inhibitor (C1-INH) and are characterized by recurrent, disfiguring, and potentially life-threatening attacks of subcutaneous and submucosal edema. HAE typically manifests in childhood or adolescence, with onset of symptoms occurring at a mean age of 10 to 11 years. Symptoms and attacks may become more severe at puberty due to hormonal factors. Subcutaneous C1-INH (C1-INH [SC], HAEGARDA®, CSL Behring, Marburg, Germany) at 60 IU/kg twice weekly was …