Danazol administration to females with menses-associated exacerbations of acute intermittent porphyria.
Acute intermittent porphyria (AIP) is a disorder of porphyrin metabolism in which the basic defect is a partial deficiency of uroporphyrinogen I synthase. The clinical disorder is more common in women, and some experience acute attacks before menstrual periods. Oral contraceptives have prevented menstrual-associated attacks in some cases, but exogenous estrogens and progestins are otherwise contraindicated in this disease. Danazol, a new synthetic steroid with weak androgenic activity, was thought to be of potential therapeutic benefit in AIP because of its effect of decreasing gonadotropin secretion without exposure to estrogen or progesterone. The drug was administered at a dosage of 200 mg t.i.d. to two adult females with AIP who were experiencing frequent exacerbations of their disease in association with their menstrual periods. Symptomatic and chemical evidence for exacerbation of porphyria occurred within 10 days of commencing danazol treatment in both patients.
D000328:Adult; D000622:Aminolevulinic Acid / Q000652:urine; D003613:Danazol / Q000627:therapeutic use; D005260:Female; D006427:Hemin / Q000627:therapeutic use; D006801:Humans; D008598:Menstruation; D011162:Porphobilinogen / Q000652:urine; D011164:Porphyrias / Q000188:drug therapy / Q000503:physiopathology*; D011245:Pregnadienes
D011245:Pregnadienes; D006427:Hemin; D011162:Porphobilinogen; D000622:Aminolevulinic Acid; D003613:Danazol