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患有特纳综合征的青春期前女孩体内,内源性生长激素的代谢清除率降低,且生长激素分泌的脉冲模式出现特定改变。基因泰克协作组。

Decreased metabolic clearance of endogenous growth hormone and specific alterations in the pulsatile mode of growth hormone secretion occur in prepubertal girls with Turner's syndrome. Genentech Collaborative Group.

作者信息

Veldhuis J D, Sotos J F, Sherman B M

机构信息

Department of Internal Medicine, University of Virginia Health Sciences Center, Charlottesville 22908.

出版信息

J Clin Endocrinol Metab. 1991 Nov;73(5):1073-80. doi: 10.1210/jcem-73-5-1073.

Abstract

We have used deconvolution analysis to test the hypothesis that specific facets of GH secretion and clearance differ in young patients with Turner's syndrome and normal prepubertal girls. To this end, we sampled blood at 20-min intervals for 12 h overnight in 50 girls, 37 of whom had Turner's syndrome and 13 of whom were healthy Tanner stage I controls. Deconvolution analysis revealed that the half-life of endogenous GH in Turner's syndrome was significantly prolonged at 14 +/- 0.93 vs. 11 +/- 0.44 min in normal girls (P = 0.029). The number of significant GH secretory bursts was reduced in Turner's patients to 4.7 +/- 0.27 vs. 6.8 +/- 0.60 events/12 h in healthy girls (P less than 0.01). GH secretory burst half-duration was significantly prolonged in Turner's syndrome, viz. 23 +/- 1.3 vs. 15 +/- 0.87 min (controls; P less than 0.001). The changes in GH secretory burst frequency, duration, and half-life were specific, since neither the mass of GH secreted per burst nor the maximal rate of GH secretion attained per burst (amplitude of the secretion pulse) was significantly different in the 2 study groups. Thus, although 12-h GH secretion rates corrected for body weight were similar (3.9 +/- 0.76 in Turner's patients and 3.3 +/- 0.76 micrograms/L.kg/12 h in the control girls), equivalent GH production rates were achieved by different mechanisms in the 2 groups. We conclude that specific alterations in GH secretory burst frequency and duration and endogenous GH half-life can be documented in young girls with Turner's syndrome.

摘要

我们采用去卷积分析来检验这样一个假设

患有特纳综合征的年轻患者与正常青春期前女孩在生长激素(GH)分泌和清除的特定方面存在差异。为此,我们在50名女孩中进行了为期12小时的夜间采血,每隔20分钟采样一次。其中37名女孩患有特纳综合征,13名健康的坦纳I期女孩作为对照。去卷积分析显示,特纳综合征患者内源性GH的半衰期显著延长,为14±0.93分钟,而正常女孩为11±0.44分钟(P = 0.029)。特纳综合征患者显著的GH分泌脉冲数量减少至4.7±0.27次/12小时,而健康女孩为6.8±0.60次/12小时(P<0.01)。特纳综合征患者GH分泌脉冲的半持续时间显著延长,即23±1.3分钟,而对照组为15±0.87分钟(P<0.001)。GH分泌脉冲频率、持续时间和半衰期的变化是特定的,因为在两个研究组中,每次脉冲分泌的GH量以及每次脉冲达到的GH最大分泌速率(分泌脉冲幅度)均无显著差异。因此,尽管校正体重后的12小时GH分泌率相似(特纳综合征患者为3.9±0.76,对照女孩为3.3±0.76微克/升·千克/12小时),但两组通过不同机制实现了相当的GH产生率。我们得出结论,在患有特纳综合征的年轻女孩中,可以记录到GH分泌脉冲频率、持续时间和内源性GH半衰期的特定改变。

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