Complement deficiencies II: hereditary angioedema

Česká verzia

Authors: R. Hakl; P. Kuklínek; P. Chovancová; J. Litzman
Authors place of work: Ústav klinické imunologie a alergologie, Fakultní nemocnice u sv. Anny v Brně, Lékařská fakulta Masarykovy univerzity, Brno
Published in the journal: Čes-slov Pediat 2021; 76 (4): 218-221.
Category:

Summary

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

Zdroje

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