Kurzfassung

In a subgroup of hereditary angioedema (HAE) patients with normal C1-esterase inhibitor (C1-INH) levels, HAE is caused by a Thr309Lys mutation in the coagulation factor XII (FXII) gene. Aim of the study is to examine elements of the kallikrein-kinin system ("contact system") and of the downstream linked coagulation, complement, and fibrinolytic systems in the plasma of patients with HAE caused by the Thr309Lys mutation and healthy probands.