Publication details

Současné možnosti léčby hereditárního angioedému

Title in English Current treatment options for hereditary angioedema
Authors

HAKL Roman

Year of publication 2016
Type Article in Periodical
Magazine / Source Vnitřní lékařství
MU Faculty or unit

Faculty of Medicine

Citation
Field Epidemiology, infectious diseases and clinical immunology
Keywords bradykinin receptor; C1 inhibitor; complement system; hereditary angioedema
Description Hereditary angioedema (HAE) caused by Cl-inhibitor deficiency is a rare autosomal dominant disease. Clinically, HAE is manifested by repeated episodes of localized subcutaneous or submucosal edema. The disease is disabling and can be lethal. There is an extreme variability in frequency and severity of symptoms. This article concerns on the organization of care and current treatment options of the disease. Treatment strategy includes short-term and long-term prophylaxis and treatment of attacks. Currently, treatment options include attenuated androgens, antifibrinolytics, recombinant (rhCI-INH) and plasma-derived (pdCI-INH)CI-INH, bradykinin receptor antagonist and inhibitor of kallikrein. Since 2011, the care of HAE patients in the Czech Republic is concentrated into 4 diagnostic and therapeutic centers.
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