Published online August 27, 2007
PEDIATRICS Vol. 120 No. 3 September 2007, pp. e713-e722 (doi:10.1542/peds.2006-3303)
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REVIEW ARTICLE

Management of Hereditary Angioedema in Pediatric Patients

Henriette Farkas, MD, PhDa, Lilian Varga, PhDa, Gábor Széplaki, MDa, Beáta Visy, MDb, George Harmat, MD, PhDb and Tom Bowen, MD, FRCPCc

a 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary
b Heim Pál Children's Hospital, Budapest, Hungary
c Departments of Medicine and Pediatrics, University of Calgary, Calgary, Alberta, Canada

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.

Key Words: hereditary angioedema • pediatrics • C1 inhibitor • danazol • tranexamic acid

Abbreviations: HAE—hereditary angioneurotic edema • TA—tranexamic acid

Accepted Mar 23, 2007.




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