A GONADOTROPIN-RELEASING HORMONE ANALOG PREVENTS CYCLICAL ATTACKS OF PORPHYRIA
A GONADOTROPIN-RELEASING HORMONE ANALOG PREVENTS CYCLICAL ATTACKS OF PORPHYRIA
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DOI：
10.1001/archinte.150.7.1469
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发表时间：
1990-07-01
影响因子：
--
通讯作者：
KAPPAS, A
中科院分区：
文献类型：
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作者：
ANDERSON, KE;SPITZ, IM;KAPPAS, A
急性间歇性卟啉症是一种内源性激素影响临床表现的遗传性疾病。经前恶化可发生，有时经常，在妇女与这种疾病。促性腺激素释放激素类似物可通过减少促黄体激素和促卵泡激素的分泌来阻止排卵。在6例有明确记录的急性间歇性卟啉症和频繁的周期性加重的患者中，每日鼻内或皮下给予促性腺激素释放激素类似物（[ImBzl]-D-His 6，Pro 9-NET）促性腺激素释放激素，长达26个月，减少或消除了经前发作，仅引起轻微的副作用。有时需要调整剂量或给药途径。我们的结论是，内分泌操作治疗促性腺激素释放激素激动剂将防止神经内脏急性间歇性卟啉症的攻击，由于内源性激素的周期性变化，是一个安全的替代外源性类固醇，这可能会引起这种疾病的攻击。
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 sometime 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.