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September 1993

Hereditary Angioedema: Danazol Therapy in a 5-Year-Old Child

Author Affiliations

Department of Pediatrics Georgetown University Medical Center 3800 Reservoir Rd NW Washington, DC 20007

Am J Dis Child. 1993;147(9):931-932. doi:10.1001/archpedi.1993.02160330021005

Sir.—Hereditary angioedema is a rare autosomal dominant disorder characterized by episodic swelling of the extremities, face, bowel wall, and upper airway. Long-term prophylaxis for symptoms of the disease in adults has been successfully achieved by the use of the attenuated androgen, danazol. There is little experience with the use of danazol in children with this disease. We present herein our experience with the successful use of danazol in reducing the frequency and severity of abdominal pain in a 5-year-old girl with hereditary angioedema.

Patient Report.—A 5-year-old white girl presented with a history of episodic erythematous rash and mild swelling of the extremities since age 18 months. At age 3½ years, she started to have severe, excruciating, colicky abdominal pain associated with nausea and vomiting and lasting 24 to 72 hours. The attacks of abdominal pain occurred every 2 weeks and consistently followed the appearance of nonpruritic generalized macular