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青少年促性腺激素缺乏症的诊断:标准促性腺激素释放激素试验在肥胖男孩中的应用价值有限。

Diagnosis of gonadotropin deficiency in adolescents: limited usefulness of a standard gonadotropin-releasing hormone test in obese boys.

作者信息

Kelch R P, Hopwood N J, Marshall J C

出版信息

J Pediatr. 1980 Nov;97(5):820-4. doi: 10.1016/s0022-3476(80)80279-4.

Abstract

The pubertal maturation of five boys (Group A) who were initially thought to be gonadotropin deficient was studied over 10 to 58 months (mean 36 months) by serial physical examinatons and standard GnRH tests. Four were seen because of obesity, delayed sexual maturation, depression, and poor school performance. The other boy had acquired hypothalamic hypopituitarism at 13 years of age. Gonadotropin responses during the initial GnRH test were either absent or abnormally low as related to the degree of skeletal maturation. Subsequent responses showed progressive maturation into the normal range for adult males. These boys had normal olfaction and moderate-to-marked obesity, but initial assessment of testicular size, basal gonadotropins, and testosterone or gonadotropin responses to GnRH did not distinguish these boys from seven patients with isolated gonadotropin deficiency (Group B). Contrary to previous reports and expectations, these studies indicate that an absent or markedly blunted response to synthetic GnRH is not diagnostic of gonadotropin deficiency, even when skeletal age is 12 years or greater. Furthermore, unless a patient is hyposomic or anosmic, or has an associated anomaly such as cleft palate, isolated gonadotropin deficiency cannot be diagnosed reliably until late adolescence or early adulthood.

摘要

对最初被认为促性腺激素缺乏的5名男孩(A组)的青春期成熟情况进行了为期10至58个月(平均36个月)的研究,采用了系列体格检查和标准促性腺激素释放激素(GnRH)测试。其中4名男孩因肥胖、性成熟延迟、抑郁和学业成绩差前来就诊。另一名男孩在13岁时患上了获得性下丘脑垂体功能减退症。在最初的GnRH测试中,促性腺激素反应与骨骼成熟程度相关,要么缺失,要么异常低。随后的反应显示逐渐成熟至成年男性的正常范围。这些男孩嗅觉正常,有中度至重度肥胖,但最初对睾丸大小、基础促性腺激素以及睾酮或促性腺激素对GnRH的反应评估,并未将这些男孩与7名孤立性促性腺激素缺乏患者(B组)区分开来。与之前的报道和预期相反,这些研究表明,即使骨骼年龄在12岁及以上,对合成GnRH的反应缺失或明显减弱也不能诊断为促性腺激素缺乏。此外,除非患者身材矮小或嗅觉缺失,或有腭裂等相关异常情况,否则直到青春期后期或成年早期,孤立性促性腺激素缺乏都无法可靠诊断。

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