Jungmann E, Trautermann C
Schwerpunkt Diabetes-Endokrinologie, St.-Vinzenz-Hospital Wiedenbrück, Rheda-Wiedenbrück.
Med Klin (Munich). 1994 Oct 15;89(10):529-33.
In patients with delayed puberty with a bone age less than 11 years in girls or 12 years in boys, the clinical and endocrinological examination allows the differentiation of patients with the various forms of hypergonadotropic hypogonadism, but not of patients with hypogonadotropic hypogonadism from more prevalent constitutional delay in puberty. Therefore, watchful waiting is generally recommended for differential diagnosis in patients with delayed puberty. On the other hand, the late onset of sexual hormone replacement in patients with hypogonadism will worsen their outcome.
Therefore, we decided to carry out a retrospective study in 105 adolescents who were examined because of short stature or delayed puberty, who were aged 14 to 22 years at first visit and in whom the differential diagnosis of delayed puberty was documented after an at least one-year follow-up in order to find out which endocrinological parameters could have effectively predicted the final diagnosis already at the first visit.
Patients with hypogonadotropic hypogonadism differed from patients with constitutional delay in puberty by lower responses of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels to gonadotropin-releasing hormone stimulation (GnRH, 100 micrograms iv) (p < 0.01) as well as by smaller testicular volume (p < 0.05) and by lower testosterone levels (p < 0.01). Stimulated LH < 10 mU/ml differentiated patients with hypogonadotropic hypogonadism from constitutional delay in puberty with a sensitivity of 82% and a specificity of 98%.
In patients with delayed puberty aged 14 years and older bone age usually exceeds 11 years in girls or 12 years in boys. It thus is in the range, in which normal adult responses of LH to GnRH can be expected. In contrast to patients aged less than 14 years, therefore, measuring GnRH-stimulated LH levels in these patients allows the rapid and effective differential diagnosis of delayed puberty.
对于青春期延迟且女孩骨龄小于11岁或男孩骨龄小于12岁的患者，临床和内分泌检查可区分各种形式的高促性腺激素性性腺功能减退患者，但无法将低促性腺激素性性腺功能减退患者与更常见的青春期体质性延迟患者区分开来。因此，对于青春期延迟患者，一般建议进行观察等待以进行鉴别诊断。另一方面，性腺功能减退患者性激素替代治疗开始较晚将使预后恶化。
因此，我们决定对105名因身材矮小或青春期延迟而接受检查的青少年进行一项回顾性研究，这些青少年首次就诊时年龄为14至22岁，且经过至少一年的随访后记录了青春期延迟的鉴别诊断情况，以便找出哪些内分泌参数在首次就诊时就能有效预测最终诊断。
低促性腺激素性性腺功能减退患者与青春期体质性延迟患者的区别在于，促黄体生成素（LH）和促卵泡生成素（FSH）水平对促性腺激素释放激素刺激（静脉注射100微克GnRH）的反应较低（p<0.01），睾丸体积较小（p<0.05）以及睾酮水平较低（p<0.01）。刺激后LH<10 mU/ml可将低促性腺激素性性腺功能减退患者与青春期体质性延迟患者区分开来，敏感性为82%，特异性为98%。
对于14岁及以上青春期延迟的患者，女孩骨龄通常超过11岁，男孩骨龄通常超过12岁。因此处于可预期LH对GnRH有正常成人反应的范围内。因此，与年龄小于14岁的患者相比，测量这些患者GnRH刺激后的LH水平可实现对青春期延迟的快速有效鉴别诊断。
