Abstract
The most common triggers of angioedema include mechanical trauma, mental stress, and airway infections
Hereditary angioedema (HAE) is caused by deficiency or dysfunction of the SERPING1 gene and is inherited in an autosomal dominant pattern
Three types of HAE have been described: type 1, characterized by low production of functionally active C1 esterase inhibitor (C1-INH), type 2 characterized by normal or elevated levels of C1-INH but with functional impairment of the protein, and type 3 present with similar clinical manifestations, but no abnormalities in C1-INH level or function
Symptoms often worsen during puberty, introduction of estrogen-containing medications for acne or birth control, in females
Plasma-derived C1-INH, like Berinert and Cinryze can be used for the treatment of acute attacks, for short-term or long-term prophylaxis of HEA by taking into account local approvals
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Farkas H, Martinez-Saguer I, Bork K, Bowen T, Craig T, Frank M, Germenis AE, Grumach AS, Luczay A, Varga L, Zanichelli A, HAWK. International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency. Allergy. 2017;72(2):300–13.
Agostoni A, Cicardi M. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine. 1992;71(4):206–15.
Bork K, Meng G, Staubach P, Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119(3):267–74.
Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017;129(11):1420–7.
Speletas M, Szilagyi A, Psarros F, Moldovan D, Magerl M, Kompoti M, Gramoustianou E, Bors A, Mihaly E, Tordai A, Avramouli A, Varga L, Maurer M, Farkas H, Germenis AE. Hereditary angioedema: molecular and clinical differences among European populations. J Allergy Clin Immunol. 2015;135(2):570–3.
Zuraw BL, Herschbach J. Detection of C1 inhibitor mutations in patients with hereditary angioedema. J Allergy Clin Immunol. 2000;105(3):541–6.
Pappalardo E, Cicardi M, Duponchel C, Carugati A, Choquet S, Agostoni A, Tosi M. Frequent de novo mutations and exon deletions in the C1inhibitor gene of patients with angioedema. J Allergy Clin Immunol. 2000;106(6):1147–54.
Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med. 2008;359(10):1027–36.
Morgan BP. Hereditary angioedema–therapies old and new. N Engl J Med. 2010;363(6):581–3.
Zotter Z, Csuka D, Szabo E, Czaller I, Nebenfuhrer Z, Temesszentandrasi G, Fust G, Varga L, Farkas H. The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency. Orphanet J Rare Dis. 2014;9:44.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Jandus, P. (2019). Recurrent Edema. In: Rezaei, N. (eds) Pediatric Immunology. Springer, Cham. https://doi.org/10.1007/978-3-030-21262-9_117
Download citation
DOI: https://doi.org/10.1007/978-3-030-21262-9_117
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-21261-2
Online ISBN: 978-3-030-21262-9
eBook Packages: MedicineMedicine (R0)