title: Management of hereditary angioedema in pediatric patients.
abstract: Hereditary angioneurotic edema is a rare disorder caused by the congenital deficiency of C1 inhibitor. Recurring angioedematous paroxysms that most commonly involve the subcutis (eg, extremities, face, trunk, and genitals) or the submucosa (eg, intestines and larynx) are the hallmarks of hereditary angioneurotic edema. Edema formation is related to reduction or dysfunction of C1 inhibitor, and conventional therapy with antihistamines and corticosteroids is ineffective. Manifestations occur during the initial 2 decades of life, but even today there is a long delay between the onset of initial symptoms and the diagnosis of hereditary angioneurotic edema. Although a variety of reviews have been published during the last 3 decades on the general management of hereditary angioneurotic edema, little has been published regarding management of pediatric hereditary angioneurotic edema. Thus, we review our experience and published data to provide an approach to hereditary angioneurotic edema in childhood.
authors.affiliation: 3rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4, H-1125, Budapest, Hungary. farkash@kut.sote.hu
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.forename: Lilian
authors.initials: L
authors.lastname: Varga
authors.forename: Gábor
authors.initials: G
authors.lastname: Széplaki
authors.forename: Beáta
authors.initials: B
authors.lastname: Visy
authors.forename: George
authors.initials: G
authors.lastname: Harmat
authors.forename: Tom
authors.initials: T
authors.lastname: Bowen
mesh_terms: D000006:Abdomen, Acute / Q000209:etiology; D000465:Algorithms; D000799:Angioedema / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000628:therapy*; D002648:Child; D050718:Complement C1 Inhibitor Protein / Q000600:supply & distribution / Q000627:therapeutic use; D003181:Complement C4 / Q000378:metabolism; D051056:Complement Inactivating Agents / Q000600:supply & distribution / Q000627:therapeutic use; D042241:Early Diagnosis; D004487:Edema / Q000209:etiology; D006801:Humans; D007819:Laryngeal Edema / Q000209:etiology; D012646:Self Administration; D040521:Subcutaneous Tissue
chemical_list: D050718:Complement C1 Inhibitor Protein; D003181:Complement C4; D051056:Complement Inactivating Agents