Hereditary angio-oedema: its pathogenesis and management.

Hereditary angio-oedema is a genetically-determined disease. Usually the disease is due to a deficiency of C1-inhibitor or less commonly to the production of a functionally inactive molecule. The pathogenesis and clinical features of the disease are reviewed, and discussed in relation to the homeostatic role of C1 inhibitor. Finally the therapeutic approach to the disease is described and the scientific bases for the newer therapeutic discussed.