An 11-year-old premenarchial girl who demonstrated the clinical and biochemical abnormalities of the Stein-Leventhal syndrome is reported. Although the concentration of total circulating testosterone was within normal limits, levels of free testosterone and testosterone-estradiol-binding globulin (12.9 to 22.7 pg/ml and 7.9 to 11 x 10-9 moles/liter, respectively) were markedly different from those of prepubertal girls (1.1 ± 0.9 pg/ml and 102 ± 27 x 10-9 moles/liter, mean ± 1 SD) and normal women (4.3 ± 1.2 pg/ml and 82 ± 30 x 10-9 moles/liter). Luteinizing hormone but not follicle-stimulating hormone concentrations were increased. In the absence of abnormal adrenal steroid excretion or response to dexamethasone suppression and with a clear biochemical response to oral contraceptives (decreased levels of luteinizing hormone and free testosterone and increased concentrations of testosterone-estradiol-binding globulin), this patient closely resembles older individuals with the Stein-Leventhal syndrome. Therefore, the measurement of testosterone-estradiol-binding globulin and free testosterone may be useful in the laboratory evaluation of any adolescent girl in whom no other cause for hirsutism can be identified.
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机译:据报道,有一名11岁的初潮前女孩表现出Stein-Leventhal综合征的临床和生化异常。尽管总循环睾丸激素的浓度在正常范围内,但游离睾丸激素和结合睾丸激素-雌二醇的球蛋白(分别为12.9至22.7 pg / ml和7.9至11 x 10-9摩尔/升)的水平与青春期前女孩(1.1±0.9 pg / ml和102±27 x 10-9摩尔/升,平均±1 SD)和正常女性(4.3±1.2 pg / ml和82±30 x 10-9摩尔/升)。增加了促黄体生成素,但没有增加促卵泡激素的浓度。在没有异常的肾上腺类固醇排泄或对地塞米松抑制的反应以及对口服避孕药有明显的生化反应(黄体生成激素和游离睾丸激素水平降低以及睾丸激素-雌二醇结合球蛋白的浓度增加)的情况下,该患者与年龄较轻的老年人相似。 Stein-Leventhal综合征。因此,睾丸激素-雌二醇结合球蛋白和游离睾丸激素的测量可能对任何未发现其他多毛症原因的少女进行实验室评估有用。
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