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Henry Ford Hospital Medical Journal

Abstract

Herditary angioedema (HAE) is an autosomal dominantly inherited condition in which a deficiency of the inhibitor (C1lnh) of the activated first component of complement is associated with recurrent episodes of edema of the skin, gastrointestinal tract, and larynx. The pituitary gonadotropin inhibitor, danazol, has been reported to be effective in preventing attacks and increasing C1lnh levels. Our experience with 11 patients from five kindreds corroborated those results and has revealed that most patients can be maintained symptom-free on 100-200 mg of danazol daily Side effects were minimal, although one young woman discontinued therapy because it aggravated her acne. The elevation of C1lnh levels and prevention of HAE attacks provide evidence that danazol is the present drug of choice in treating this genetic disease.

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