Anderson K E, Spitz I M, Bardin C W, Kappas A
Rockefeller University Hospital, New York, NY.
Arch Intern Med. 1990 Jul;150(7):1469-74.
Acute intermittent porphyria is a genetic disease in which endogenous hormones affect clinical expression. Premenstrual exacerbations can occur, sometimes often, in women with this disease. Gonadotropin releasing hormone analogues can prevent ovulation by reducing secretion of luteinizing hormone and follicle-stimulating hormone. In six patients with well-documented acute intermittent porphyria and frequent cyclical exacerbations, daily administration of an agonistic gonadotropin releasing hormone analogue, ([ImBzl]-D-His6,Pro9-NET)gonadotropin releasing hormone, intranasally or subcutaneously for as long as 26 months reduced or eliminated premenstrual attacks and caused only minor side effects. Adjustments in dosage or route of administration were sometimes needed. We conclude that endocrine manipulation by treatment with a gonadotropin releasing hormone agonist will prevent neurovisceral attacks of acute intermittent porphyria due to cyclical changes in endogenous hormones and is a safe alternative to exogenous steroids, which may induce attacks of this disease.
急性间歇性卟啉症是一种遗传性疾病，内源性激素会影响其临床表现。患有这种疾病的女性可能会出现经前病情加重，有时发作频繁。促性腺激素释放激素类似物可通过减少黄体生成素和促卵泡激素的分泌来防止排卵。在6例有充分记录的急性间歇性卟啉症且周期性病情频繁加重的患者中，每天经鼻或皮下给予一种促性腺激素释放激素激动剂（[ImBzl]-D-His6,Pro9-NET）促性腺激素释放激素，长达26个月，可减少或消除经前发作，且仅引起轻微副作用。有时需要调整剂量或给药途径。我们得出结论，用促性腺激素释放激素激动剂进行内分泌调节可预防由于内源性激素周期性变化引起的急性间歇性卟啉症的神经内脏发作，并且是外源性类固醇的一种安全替代方法，外源性类固醇可能诱发该疾病发作。
