Treatment of attacks with plasma-derived C1-inhibitor concentrate in pediatric hereditary angioedema patients

摘要

Hereditary angioedema due to Cl-inhibitor (HAE-C1-INH) deficiency is a disorder with autosomal-dominant inheritance, resulting from the mutation of the Cl-INH gene. It is characterized by paroxysms of edema in the subcutis and/or the submucosa of the upper airways, as well as of the gastrointestinal tract.