Complement deficiencies II: hereditary angioedema
Authors:
R. Hakl; P. Kuklínek; P. Chovancová; J. Litzman
Authors‘ workplace:
Ústav klinické imunologie a alergologie, Fakultní nemocnice u sv. Anny v Brně, Lékařská fakulta Masarykovy univerzity, Brno
Published in:
Čes-slov Pediat 2021; 76 (4): 218-221.
Category:
Overview
Hereditary angioedema (HAE) is a rare, hereditary disorder classified to HAE with C1 inhibitor deficiency (HAE-C1-INH) and HAE with normal C1 inhibitor level and function (HAE nC1-INH). HAE is characterized by recurrent attacks of subcutaneous or submucosal oedema. These results in a very wide clinical manifestation and a patient with HAE may visit an outpatient clinic of various specialties. The cause of oedema is uncontrolled complement and kinin system activation.
The diagnosis of HAE is based on clinical manifestation, anamnestic data and laboratory diagnostics. Treatment strategies include short-term and long-term prophylaxis and on demand treatment.
Keywords:
hereditary angioedema – Complement – C1 inhibitor
Sources
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Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2021 Issue 4
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