DYNAMICS OF 24-HOUR PULSATILE CORTISOL, 17-HYDROXYPROGESTERONE, AND ANDROSTENEDIONE RELEASE IN PREPUBERTAL PATIENTS WITH NONCLASSIC 21-HYDROXYLASE DEFICIENCY AND NORMAL PREPUBERTAL CHILDREN
DYNAMICS OF 24-HOUR PULSATILE CORTISOL, 17-HYDROXYPROGESTERONE, AND ANDROSTENEDIONE RELEASE IN PREPUBERTAL PATIENTS WITH NONCLASSIC 21-HYDROXYLASE DEFICIENCY AND NORMAL PREPUBERTAL CHILDREN
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DOI：
10.1016/0026-0495(94)90107-4
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发表时间：
1994-03-01
影响因子：
9.8
通讯作者：
GIOVANNELLI, G
中科院分区：
文献类型：
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作者：
GHIZZONI, L;BERNASCONI, S;GIOVANNELLI, G
为探讨非经典型先天性肾上腺增生症(NCCAH)患者皮质醇、17-羟孕酮(17-OHP)和雄烯二酮(D4A)分泌的定量和定性特征是否与正常儿童不同，测定了5例青春期前NCCAH患者和5例正常青春期前儿童血清中这些类固醇的浓度。用Pulsar程序分析30分钟采样获得的肾上腺类固醇分布。与正常儿童相比，NCCAH患者24小时分泌17-OHP和D4A的定量参数显著增加，而血清皮质醇浓度无明显变化。当分别分析白天和夜间的激素释放时，只有正常人的皮质醇面积在夜间显著高于零水平曲线(AUCo)和12小时平均值和17-OHP AUCo，AUC高于基线，平均峰值高度、幅度、面积和12小时平均值。相反，NCCAH患者表现出夜间17-OHP分泌峰的频率和数量增加，峰间间隔缩短。无论是正常人还是患者，D4A浓度的昼夜差异均不显著。总之，本研究结果表明，NCCAH患者具有明显的肾上腺类固醇分泌模式，其特征是17-OHP的高频释放伴随相对夜间皮质醇缺乏。
To assess whether the quantitative and qualitative aspects of cortisol, 17-hydroxyprogesterone (17-OHP), and androstenedi-one (D4A) secretion in patients with nonclassic congenital adrenal hyperplasia (NCCAH) differ from those in normal children, 24-hour serum concentrations of these steroids were measured in five prepubertal patients with NCCAH and five normal prepubertal children. Adrenal steroid profiles obtained by 30-minute sampling were analyzed by the Pulsar program. In comparison to normal children, the 24-hour quantitative parameters of 17-OHP and D4A secretion were significantly greater in NCCAH patients, but serum cortisol concentrations were similar in the two groups. When daytime and nighttime hormone releases were separately analyzed, a significant nocturnal elevation of the cortisol area under the curve above zero level (AUCo) and 12-hour mean and 17-OHP AUCo, AUC above baseline, mean peak height, amplitude, area, and 12-hour mean was detected in normal subjects only. Conversely, NCCAH patients exhibited an increased frequency and number of 17-OHP secretory peaks at night together with a reduction of the interpeak interval. No significant day/night differences in D4A concentrations were detected either in normals or in the patients. In conclusion, the results of the present study indicate that patients with NCCAH have a distinct pattern of adrenal steroid secretion characterized by a high-frequency 17-OHP release accompanied by a relative nocturnal cortisol deficiency.