Grinspon Romina P, Loreti Nazareth, Braslavsky Débora, Bedecarrás Patricia, Ambao Verónica, Gottlieb Silvia, Bergadá Ignacio, Campo Stella M, Rey Rodolfo A
Centro de Investigaciones Endocrinológicas (CEDIE, CONICET), Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina.
J Pediatr Endocrinol Metab. 2012;25(1-2):3-11. doi: 10.1515/jpem-2011-0453.
During childhood, the pituitary-testicular axis is partially dormant: testosterone secretion decreases following a drop in luteinising hormone levels; follicle-stimulating hormone (FSH) levels also go down. Conversely, Sertoli cells are most active, as revealed by the circulating levels of anti-Müllerian hormone (AMH) and inhibin B. Therefore, hypogonadism can best be evidenced, without stimulation tests, if Sertoli cell function is assessed. Serum AMH is high from fetal life until mid-puberty. Testicular AMH production increases in response to FSH and is potently inhibited by androgens. Inhibin B is high in the first years of life, then decreases partially while remaining clearly higher than in females, and increases again at puberty. Serum AMH and inhibin B are undetectable in anorchid patients. In primary or central hypogonadism affecting the whole gonad established in fetal life or childhood, all testicular markers are low. Conversely, when hypogonadism only affects Leydig cells, serum AMH and inhibin B are normal. In males of pubertal age with central hypogonadism, AMH and inhibin B are low. Treatment with FSH provokes an increase in serum levels of both Sertoli cell markers, whereas human chorionic gonadotrophin (hCG) administration increases testosterone levels. In conclusion, measurement of serum AMH and inhibin B is helpful in assessing testicular function, without need for stimulation tests, and orientates the aetiological diagnosis of paediatric male hypogonadism.
在儿童期，垂体 - 睾丸轴部分处于休眠状态：随着黄体生成素水平下降，睾酮分泌减少；促卵泡激素（FSH）水平也降低。相反，抗苗勒管激素（AMH）和抑制素B的循环水平表明，支持细胞最为活跃。因此，如果评估支持细胞功能，无需刺激试验就能最好地证明性腺功能减退。从胎儿期到青春期中期，血清AMH水平都很高。睾丸AMH的产生对FSH有反应，并受到雄激素的强烈抑制。抑制素B在生命的最初几年含量很高，然后部分下降，但仍明显高于女性，在青春期再次升高。在无睾症患者中无法检测到血清AMH和抑制素B。在胎儿期或儿童期就已确立的影响整个性腺的原发性或中枢性性腺功能减退中，所有睾丸标志物都很低。相反，当性腺功能减退仅影响睾丸间质细胞时，血清AMH和抑制素B正常。在青春期中枢性性腺功能减退的男性中，AMH和抑制素B较低。使用FSH治疗会使两种支持细胞标志物的血清水平升高，而注射人绒毛膜促性腺激素（hCG）会使睾酮水平升高。总之，测量血清AMH和抑制素B有助于评估睾丸功能，无需刺激试验，并可为小儿男性性腺功能减退的病因诊断提供方向。
