Publication details
Léčebné možnosti u hereditárního angioedému ( HAE)
| Title in English | Treatment possibilities in hereditary angioedema |
|---|---|
| Authors | |
| Year of publication | 2010 |
| Type | Article in Periodical |
| Magazine / Source | Alergie |
| MU Faculty or unit | |
| Citation | |
| Field | Immunology |
| Keywords | Complement system; hereditary angioedema; C1-inhibitor; antifibrinolytics; attenuated androgens; bradykinin receptor |
| Description | C1 esterase inhibitor (C1-INH) deficiency leads to hereditary or acquired angioedema, which are relatively infrequent, but life-threatening diseases. A non-regulated activation of not only complement, but also hemocoagulation, fibrinolytic, and kallikrein-kinin systems leads to vasoactive substances formation, causing mucosal and skin tissue oedema. Three basic approaches in the treatment of C1-INH deficiency we must consider - treatment of acute oedema, long-term prophylaxis and short-term prophylaxis. In the long-term prophylaxis, attenuated androgens and antifibrinolytic agents are used. In a short-time prophylaxis, for instance before dental treatment, C1-INH concentrate is the drug of the fist choice, but also the increase of the dose of prophylactically used drugs can be used. C1-INH concentrate is used for the treatment of acute attacks, but inhibitors of the kallikrein-kinin pathway will probably be used in the future as well. |