Pathophysiology treatment of Hereditary Angioedema

Hereditary angioedema is an autosomic dominant disease that is characterized by recurrent angioedema and effected by a defective enzyme known as C1 inhibitor. There are three types of the disease, the first one related to a decrease in the production of the enzyme, the second one characterized by an altered function of the enzyme and the third one due to an altered factor xiI gene. The recurrent angioedema may localized in the periphery, in the gastrointestinal system or laryngeal, the last one being life threatening. For the treatment there are a many drugs available, such as attenuated androgens, fresh frozen plasma, tranexamic acid or the enzyme substitution by a C1 inhibitor concentrate purified from plasma, nanofiltered or recombinant. Recently, treatment has directed to the avoidance of baradykinin production or its action through its B2 receptor.

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