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February 10, 1989

Premenstrual Exacerbations in Hepatic Porphyria: Prevention by Intermittent Administration of an LH-RH Agonist in Combination With a Gestagen

JAMA. 1989;261(6):864. doi:10.1001/jama.1989.03420060068031

To the Editor.—  Premenstrual attacks of hepatic porphyrias, in some cases occurring with each menstrual cycle, are well-known.1 Anderson et al2 in 1984 reported the potential benefit of use of a luteinizing hormone-releasing hormone (LH-RH) agonist in preventing premenstrual attacks. This result was confirmed by further case reports.3-5 In all cases, the long-acting LH-RH agonist was administered continuously for months either subcutaneously or by nasal spray.We report one patient with cyclic exacerbations of acute intermittent porphyria and another patient with premenstrual exacerbations of variegate porphyria. In both patients, gynecologic evaluation revealed no abnormalities that could cause menstrual disorders. The attacks of neurological symptoms were prevented by the intermittent nasal administration of the long-acting LH-RH agonist buserelin in combination with the gestagen medroxyprogesterone acetate. The agonistic LH-RH analogue buserelin was given to both patients daily, with two hubs (2 × 150 μg) intranasally in the evening from