Prevention of Cyclical Attacks of Acute Intermittent Porphyria with a Long-Acting Agonist of Luteinizing Hormone–Releasing Hormone

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Abstract
THE genetic disease acute intermittent porphyria may be exacerbated by a number of factors that are distinct from the inherited deficiency of the heme pathway enzyme, porphobilinogen deaminase (also known as uroporphyrinogen I synthase). These factors include endogenous steroid hormones and their metabolites, drugs, and nutrition.1 2 3 4 5 In some women with acute intermittent porphyria, exacerbations of symptoms occur in relation to the menstrual cycle, and in a minority, disabling premenstrual attacks occur regularly with every cycle.1 , 2 , 5 6 7 8 9 We report on a patient in whom premenstrual exacerbations of acute intermittent porphyria, which had previously occurred every month for at least two years, were . . .