CIRCADIAN PATTERNS OF PLASMA-CORTISOL, 17-HYDROXYPROGESTERONE, AND TESTOSTERONE IN CONGENITAL ADRENAL-HYPERPLASIA

CIRCADIAN PATTERNS OF PLASMA-CORTISOL, 17-HYDROXYPROGESTERONE, AND TESTOSTERONE IN CONGENITAL ADRENAL-HYPERPLASIA
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DOI:
10.1136/adc.56.3.208
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发表时间:
1981-01-01
影响因子:
5.2
通讯作者:
SWOBODA, W
SWOBODA, W
中科院分区:
医学2区
文献类型:
--
作者:
FRISCH, H;PARTH, K;SWOBODA, W

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在11名2至17岁的儿童(非失盐性)先天性肾上腺皮质增生症(21-羟化酶缺乏症),在24小时内,以90分钟的时间间隔抽血,17-羟孕酮,睾酮和皮质醇的水平进行了测量。在24小时尿样中测定17-酮类固醇和孕烷三醇的水平。在不同的治疗方案下和治疗中断后进行这些测量。皮质醇水平在给予皮质类固醇后迅速上升和下降,并达到非生理性的高水平。睾酮水平显示出明显的变化,但即使在未经治疗的患者中,大多数时间也保持在正常范围内;因此睾酮提供了一个较差的控制参数。未治疗患者的17-羟孕酮水平波动剧烈,峰值高峰非常高;每日2或3次皮质类固醇[泼尼松、氢化可的松]标准治疗不能阻止午夜后17-羟孕酮水平的显著升高。在第一次早晨剂量的氢化可的松后,观察到非常陡峭的下降。24小时孕三醇排泄相关性以及相应的总集成17-羟孕酮面积。单一的17-羟孕酮值不太可能提供有关治疗质量的充分信息。
In 11 children aged between 2 and 17 yr with (nonsalt-losing) congenital adrenal hyperplasia (21-hydroxylase deficiency) blood was drawn at 90-minute intervals during a 24-h period and levels of 17-hydroxyprogesterone, testosterone and cortisol were measured. Levels of 17-ketosteroids and pregnanetriol were measured in 24-h urine samples. These measurements were taken under different regimens of treatment and after interruption of treatment. Cortisol levels rose and fell rapidly after administered corticosteroid, and reached unphysiologically high levels. Testosterone levels showed pronounced variations but stayed in the normal range for most of the time even in untreated patients; thus testosterone provides a poor control parameter. Levels of 17-hydroxyprogesterone showed extreme fluctuations and very high peak levels in untreated patients; standard treatment with 2 or 3 daily doses of corticosteroids [prednisone, hydrocortisone] did not prevent a pronounced rise in its level after midnight. After the 1st morning dose of hydrocortisone a very steep fall was observed. The 24-h pregnanetriol excretion correlated well with the corresponding total integrated 17-hydroxyprogesterone area. Single 17-hydroxyprogesterone values are unlikely to give adequate information about the quality of treatment.