Partsch C J, Hermanussen M, Sippell W G
J Clin Endocrinol Metab. 1985 Jun;60(6):1196-203. doi: 10.1210/jcem-60-6-1196.
It is not possible to differentiate reliably between male idiopathic hypothalamic hypogonadism (HH) and severe constitutional delay of puberty (CD) on the basis of a standard GnRH bolus test (GBT) or other known endocrine or clinical parameters. Therefore, we studied the response of 17 hypogonadal men, 8 with a diagnosis of HH (age, 15.5-41; bone age, 12.5-19 yr; testes, 1-4 ml) and 9 with CD (age, 14.5-20; bone age, 11-15 yr, testes, 2-10 ml) to pulsatile GnRH stimulation. Basal and peak LH and FSH levels after a single dose of GnRH greatly overlapped between the two groups. In each patient, a spontaneous nocturnal plasma profile of LH and FSH, sampled every 20 min, was followed by a pulsatile GnRH stimulation (5 micrograms iv every 90 min) via a portable minipump for 36 h. Before and after this pulsatile GnRH stimulation, a GBT (60 micrograms/m2 iv) was performed and plasma LH, FSH, testosterone, androstenedione, and dehydroepiandrosterone sulfate were measured. Pulse analysis revealed 0-5 spontaneous nocturnal LH peaks in the CD patients but only one in all of the HH patients. During the 36 h of pulsatile GnRH, mean LH and FSH levels were significantly higher (P less than 0.0001) than during the spontaneous nocturnal profile in all patients (except 1 from each group for LH). The GBT after pulsatile stimulation caused significantly higher (P less than 0.001) LH increments in CD than in HH patients, with no overlap between the two groups (range, 4.1-15.6 in CD vs. 0.8-2.4 mIU/ml in HH). Plasma testosterone rose significantly (P less than 0.01) during pulsatile GnRH from 67 to 155 ng/dl (median) in the CD men, but did not change in the HH group (21 to 22.5 ng/dl). Plasma androstenedione and dehydroepiandrosterone sulfate did not rise in either group. We conclude that, in contrast to other parameters investigated so far, the LH increment in the second GBT after 36 h of pulsatile GnRH allows clear-cut differentiation between CD and HH. These results indicate significantly lower pituitary LH reserve in patients with permanent HH after short term priming of the pituitary by pulsatile GnRH administration.
基于标准促性腺激素释放激素推注试验（GBT）或其他已知的内分泌或临床参数，无法可靠地区分男性特发性下丘脑性性腺功能减退（HH）和严重体质性青春期延迟（CD）。因此，我们研究了17例性腺功能减退男性的反应，其中8例诊断为HH（年龄15.5 - 41岁；骨龄12.5 - 19岁；睾丸体积1 - 4毫升），9例为CD（年龄14.5 - 20岁；骨龄11 - 15岁，睾丸体积2 - 10毫升）对脉冲式促性腺激素释放激素刺激的反应。两组单次注射促性腺激素释放激素后的基础及峰值促黄体生成素（LH）和促卵泡生成素（FSH）水平有很大重叠。对每位患者，先每隔20分钟采集一次夜间自发性LH和FSH血浆谱，然后通过便携式微型泵进行36小时的脉冲式促性腺激素释放激素刺激（每90分钟静脉注射5微克）。在这种脉冲式促性腺激素释放激素刺激前后，进行一次GBT（静脉注射60微克/平方米）并测量血浆LH、FSH、睾酮、雄烯二酮和硫酸脱氢表雄酮。脉冲分析显示，CD患者夜间自发性LH峰值为0 - 5个，而所有HH患者仅1个。在36小时的脉冲式促性腺激素释放激素刺激期间，所有患者（每组除1例LH外）的平均LH和FSH水平均显著高于夜间自发性血浆谱期间（P < 0.0001）。脉冲刺激后的GBT导致CD患者的LH增量显著高于HH患者（P < 0.001），两组之间无重叠（CD组范围为4.1 - 15.6，HH组为0.8 - 2.4 mIU/ml）。CD组男性在脉冲式促性腺激素释放激素刺激期间血浆睾酮从67显著升高（P < 0.01）至155 ng/dl（中位数），而HH组未变化（21至22.5 ng/dl）。两组的血浆雄烯二酮和硫酸脱氢表雄酮均未升高。我们得出结论，与迄今研究的其他参数不同，脉冲式促性腺激素释放激素刺激36小时后第二次GBT中的LH增量可明确区分CD和HH。这些结果表明，通过脉冲式促性腺激素释放激素给药对垂体进行短期激发后，永久性HH患者的垂体LH储备显著降低。
