Lustig R H, Conte F A, Kogan B A, Grumbach M M
J Urol. 1987 Sep;138(3):587-91. doi: 10.1016/s0022-5347(17)43266-6.
The hormonal characteristics of anorchism are elevated basal levels of gonadotropins, especially follicle-stimulating hormone and a low concentration of plasma testosterone that fails to increase after the administration of human chorionic gonadotropin. However, little is known about the dynamics of plasma gonadotropin secretion in infants and children with anorchism. We analyzed plasma gonadotropin concentrations and their responses to luteinizing hormone releasing factor, along with plasma testosterone responses to human chorionic gonadotropin, in 9 children with surgically proved bilateral anorchism. Basal concentrations of gonadotropins, especially plasma follicle-stimulating hormone, are elevated above normal during the first 3 to 4 years of life, decrease gradually to normal prepubertal levels and then increase again after age 9 years. This age-dependent diphasic pattern of gonadotropin secretion is comparable to that described previously in patients with the syndrome of gonadal dysgenesis and it is consistent with steroid-independent central nervous system inhibition of pulsatile luteinizing hormone releasing factor secretion during mid childhood. Moreover, a sex difference in follicle-stimulating hormone and luteinizing hormone values was observed; the mean follicle-stimulating hormone and luteinizing hormone concentrations in anorchid boys less than 3 years old were lower than in patients with Turner's syndrome. We suggest that this sex dichotomy is a consequence, at least in part, of the actions of testosterone derived from the fetal testis on the fetal hypothalamus. Luteinizing hormone releasing factor administration to anorchid male subjects resulted in an age-dependent augmented release of gonadotropin, particularly follicle-stimulating hormone. This study emphasizes that the age of the patient must be considered when interpreting basal gonadotropin levels. We also propose that in mid childhood the luteinizing hormone releasing factor test in conjunction with the human chorionic gonadotropin stimulation test is a more accurate hormonal indicator of patients with congenital anorchism than either the human chorionic gonadotropin test or basal gonadotropin concentrations.
无睾症的激素特征是促性腺激素基础水平升高，尤其是促卵泡激素，且血浆睾酮浓度低，给予人绒毛膜促性腺激素后也不会升高。然而，对于患有无睾症的婴幼儿和儿童，血浆促性腺激素分泌的动态变化知之甚少。我们分析了9例经手术证实为双侧无睾症患儿的血浆促性腺激素浓度及其对促黄体生成素释放因子的反应，以及血浆睾酮对人绒毛膜促性腺激素的反应。促性腺激素的基础浓度，尤其是血浆促卵泡激素，在生命的前3至4年高于正常水平，逐渐降至青春期前的正常水平，然后在9岁后再次升高。这种促性腺激素分泌的年龄依赖性双相模式与先前在性腺发育不全综合征患者中描述的模式相似，并且与儿童中期类固醇非依赖性中枢神经系统对促黄体生成素释放因子脉冲分泌的抑制作用一致。此外，观察到促卵泡激素和促黄体生成素值存在性别差异；3岁以下无睾男孩的促卵泡激素和促黄体生成素平均浓度低于特纳综合征患者。我们认为，这种性别差异至少部分是由于胎儿睾丸产生的睾酮对胎儿下丘脑的作用。对无睾男性受试者给予促黄体生成素释放因子会导致促性腺激素，尤其是促卵泡激素的释放随年龄增加。这项研究强调，在解释基础促性腺激素水平时必须考虑患者的年龄。我们还提出，在儿童中期，促黄体生成素释放因子试验与人绒毛膜促性腺激素刺激试验相结合，比单独的人绒毛膜促性腺激素试验或基础促性腺激素浓度更能准确地反映先天性无睾症患者的激素状况。
