Abstract

The hereditary angioedema due to C1 inhibitor deficiency (C1 esterase inhibitor), is classified in two types and is considered a disease, included in the group of rare diseases, which usually occurs during childhood and adolescence with intermittent attacks of angioedema that can endanger the patient's life. The diagnosis should be possible as early as we should avoid establishing ineffective treatments, and always seek the best treatment for inflammation. There have been several expert meetings on the subject, the most important being sponsored by the WAO in 2012 and subsequently publishing the International Working Group, although the responsible medical for treating a patient with HAE can use other usage guidelines. Of all the treatments available in Pediatrics one can conclude that the concentrate of C1-inhibitor derived from plasma is considered the best treatment option for this patient group. The case presented in the discussion section is a patient in whom it begins to use Berinert but finally, due to the intensity of the attack in addition to possible drug resistance, opted for a combination treatment Berinert-Cinryze that was the most adequate, and there is currently no publication on their combined use.

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