Lightner E S, Penny R, Frasier S D
J Pediatr. 1975 Dec;87(6 Pt 1):922-7. doi: 10.1016/s0022-3476(75)80906-1.
A 5-5/12-year-old boy with gigantism and precocious puberty associated with the McCune-Albright syndrome is presented. Serum concentrations of growth hormone were extremely elevated (128-163 ng/ml) and were not suppressed by hyperglycemia or chlorpromazine. Serum LH (7.4 +/- 1.0 SD mIU/ml) and FSH (5.3 +/- 0.3 SD mIU/ml) concentrations were in the range observed in midpuberty. The secretion pattern of LH was episodic. The administration of estradiol suppressed the secretion of FSH and had an apparent positive feedback effect on release of LH. These findings are compatible with abnormal hypothalamic function as the mechanism for the endocrinopathies associated with the McCune-Albright syndrome.
报告了一名5又5/12岁患有与McCune-Albright综合征相关的巨人症和性早熟的男孩。血清生长激素浓度极高(128 - 163 ng/ml),且不受高血糖或氯丙嗪抑制。血清促黄体生成素(LH)(7.4 ± 1.0标准差mIU/ml)和促卵泡生成素(FSH)(5.3 ± 0.3标准差mIU/ml)浓度处于青春期中期观察到的范围内。LH的分泌模式是间歇性的。给予雌二醇可抑制FSH的分泌,并对LH的释放有明显的正反馈作用。这些发现与下丘脑功能异常作为与McCune-Albright综合征相关的内分泌病变的机制相符。