Attie K M, Ramirez N R, Conte F A, Kaplan S L, Grumbach M M
Department of Pediatrics, University of California, San Francisco 94143-0106.
J Clin Endocrinol Metab. 1990 Oct;71(4):975-83. doi: 10.1210/jcem-71-4-975.
The relative contributions of GH, insulin-like growth factor-I (IGF-I), estradiol, and testosterone to the pubertal growth spurt are incompletely understood. We studied 8 patients (5 girls and 3 boys) with true precocious puberty and GH deficiency due to CNS lesions to assess the role of sex steroids in pubertal growth independent of an increase in circulating GH. Included is 1 patient with an unusual hypothalamic lesion due to head trauma. A control group of 17 GH-sufficient patients with true precocious puberty (13 girls and 4 boys) was matched for chronological age. The GH-deficient girls grew at a mean velocity of 9.2 cm/yr (range, 7.2-14.4), and the boy's mean height velocity was 7.9 cm/yr (6.1-9.9). Mean bone age was advanced in the GH-deficient group (girls, +2.7 SD; boys, +2.6 SD), but not as much as the GH-sufficient controls (girls, +5.4 SD; boys, +4.3 SD). The mean concentration of plasma IGF-I was lower in the GH-deficient group than in the control group, but was greater than the mean concentration in age-matched prepubertal GH-deficient patients. Four GH-deficient patients were treated with a potent agonist of LRF. This caused suppression of gonadal sex steroid concentrations and a fall in mean height velocity from 9.1 to 4.3 cm/yr after 1 yr of therapy; however, circulating GH and IGF-I values were not uniformly altered. We conclude that a substantial pubertal growth spurt can occur in patients with true precocious puberty and GH deficiency that is dependent on gonadal sex steroids yet unaccompanied by normal pubertal levels of circulating GH or IGF-I. Reversal of this growth acceleration is possible with sex steroid suppression. The results, in light of previous in vivo and in vitro studies, suggest that the normal pubertal growth spurt is mediated in part by direct effects of sex steroids at the growth plate.
生长激素（GH）、胰岛素样生长因子-I（IGF-I）、雌二醇和睾酮对青春期生长突增的相对作用尚未完全明确。我们研究了8例因中枢神经系统病变导致真性性早熟且生长激素缺乏的患者（5名女孩和3名男孩），以评估性类固醇在不依赖循环生长激素增加情况下对青春期生长的作用。其中包括1例因头部外伤导致下丘脑病变异常的患者。选取17例生长激素充足的真性性早熟患者（13名女孩和4名男孩）作为对照组，按实际年龄进行匹配。生长激素缺乏的女孩平均生长速度为9.2厘米/年（范围为7.2 - 14.4），男孩的平均身高增长速度为7.9厘米/年（6.1 - 9.9）。生长激素缺乏组的平均骨龄提前（女孩，+2.7标准差；男孩，+2.6标准差），但不如生长激素充足的对照组提前得多（女孩，+5.4标准差；男孩，+4.3标准差）。生长激素缺乏组血浆IGF-I的平均浓度低于对照组，但高于年龄匹配的青春期前生长激素缺乏患者的平均浓度。4例生长激素缺乏患者接受了促性腺激素释放激素（LRF）强效激动剂治疗。这导致性腺性类固醇浓度受到抑制，治疗1年后平均身高增长速度从9.1厘米/年降至4.3厘米/年；然而，循环生长激素和IGF-I值并非一致改变。我们得出结论，真性性早熟且生长激素缺乏的患者可出现显著的青春期生长突增，这依赖于性腺性类固醇，但不伴有循环生长激素或IGF-I的正常青春期水平。通过抑制性类固醇可逆转这种生长加速。结合此前的体内和体外研究结果表明，正常的青春期生长突增部分是由性类固醇对生长板的直接作用介导的。
