Hereditary angioedema and its current management
Keywords:
angioedema, complement C1 inhibitor protein, laryngeal edema, abdomen, acuteAbstract
Hereditary angioedema is a primary immune deficiency of the complement system, of genetic origin, caused by alteration of the gene that encodes the inhibitor protein C1 esterase activated. It is characterized by recurrent episodes of angioedema of the skin, mucous and submucosal tissues and can affect any part of the body; typically it involves the extremities, face, airway and digestive tract. Abdominal pain associated with angioedema can be confused with an acute abdomen when there is no diagnosis of the disease and lead to unnecessary surgery. The laryngeal edema represents a risk to the life of the patient because it can cause death by suffocation. In women, the hormonal changes seem to have a strong impact on the disease, it is important that doctors know what to do when a patient is pregnant. Some professionals have ignorance of the disease and no access to treatment. Recently the therapeutic approach is directed to prevent the production of bradykinin or inhibit its function to block the function of its B2 receptor. At present, the drugs approved internationally for the effective treatment of this affection are Berinert P and Icatiban.Downloads
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