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Abstract

Hereditary angio-oedema (HAE) is characterized by recurrent, localized, non-pitting, non-pruritic, non-urticarial oedema. Nearly all patients experience skin swelling as a feature of HAE. There may be painful abdominal attacks, accompanied by nausea and vomiting. The disease is life-threatening should laryngeal oedema occur. HAE results from a deficiency or dysfunction of C1 inhibitor, a plasma protein with an important role in regulating the contact, complement and fibrinolytic systems. Effective management of HAE should include a plan for treatment of attacks, as well as routine and preprocedure prevention. Acute and prophylactic therapy with C1 inhibitor therapy for correcting the underlying deficiency in HAE is a valuable option.