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先天性肾上腺皮质增生症青少年对泼尼松治疗的激素及临床反应

Hormonal and clinical responses to prednisone treatment in adolescents with congenital adrenal hyperplasia.

作者信息

Zipf W B, Bacon G E, Kelch R P

出版信息

Horm Res. 1980;12(4):206-17. doi: 10.1159/000179122.

Abstract

The effects of prednisone therapy and the withdrawal of prednisone for 3 days on hormonal relationships was investigated in 6 adolescent patients, age 10--19 years with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. Prednisone continuously suppressed adrenal release of 17 alpha-hydroxyprogesterone (17-OHP) in 4 patients and adequately controlled 17-oxosteroid (17-OS) excretion in 5. Serum dehydroepiandrosterone sulfate (DS) concentrations were not elevated during or 3 days after withdrawal of medication. Growth hormone (GH) secretion was not suppressed by prednisone. Withdrawal of treatment was associated with a decrease in the follicle-stimulating hormone (FSH) to luteinizing hormone (LH) ratio in 5 patients and a rise in serum testosterone in 4. The clinical courses of these patients emphasize the difficulty in achieving optimum control.

摘要

对6例年龄在10至19岁、因21 - 羟化酶缺乏导致先天性肾上腺皮质增生(CAH)的青少年患者,研究了泼尼松治疗及停用泼尼松3天对激素关系的影响。泼尼松持续抑制了4例患者肾上腺释放17α - 羟孕酮(17 - OHP),并充分控制了5例患者的17 - 氧类固醇(17 - OS)排泄。在用药期间及停药3天后,血清硫酸脱氢表雄酮(DS)浓度未升高。泼尼松未抑制生长激素(GH)分泌。停药与5例患者促卵泡激素(FSH)与促黄体生成素(LH)比值降低以及4例患者血清睾酮升高有关。这些患者的临床病程强调了实现最佳控制的困难。

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