Danazol-Langzeittherapie bei hereditärem Angioödem

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Abstract
Three patients with hereditary angio-oedema, two men of 19 and 35 years and a woman of 69 years, have been treated for 10 years with danazol, an androgen preparation with diminished androgenic effects. The 19-year-old man started taking it at the age of nine years and continued throughout puberty. Maintenance therapy with 200 mg twice a week, together with occasional booster doses of 200 mg daily, started at the age of 16 years and given when required cut the frequency of attacks from one a week to roughly six a year and greatly reduced their severity. Sexual maturation was entirely normal. The 35-year-old man, who had previously had three severe attacks a week, became symptom-free on a dose of 200 mg four or five times a week. During treatment he fathered a healthy child. The 69-year-old woman had had attacks every four weeks after the menopause, but obtained complete relief from a dose of 200 mg six times weekly. C1-Esterase inhibitor--originally depressed to around 30% of normal in all these patients--rose to about 50% during treatment. Endocrine, metabolic and toxic side-effects were minimal.

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