成人迟发性特发性低促性腺激素性性腺功能减退——一种可治疗的男性不育症形式。

Adult-onset idiopathic hypogonadotropic hypogonadism--a treatable form of male infertility.

作者信息

Nachtigall L B, Boepple P A, Pralong F P, Crowley W F

机构信息

Reproductive Endocrine Unit, Massachusetts General Hospital, Boston, MA 02114, USA.

出版信息

N Engl J Med. 1997 Feb 6;336(6):410-5. doi: 10.1056/NEJM199702063360604.

DOI:10.1056/NEJM199702063360604

PMID:9010147

Abstract

BACKGROUND

Men with isolated gonadotropin-releasing hormone (GnRH) deficiency typically present with an absence of pubertal development. We describe an adult-onset form of idiopathic hypogonadotropic hypogonadism that develops after puberty.

METHODS

We studied 10 men (age, 27 to 57 years) with normal sexual maturation, idiopathic infertility, sexual dysfunction, low serum testosterone concentrations, and apulsatile secretion of luteinizing hormone on frequent blood sampling. All the men had otherwise normal anterior pituitary hormone secretion and sellar anatomy. We compared the results of semen analyses and measurements of testicular volume, serum testosterone, inhibin B, and gonadotropins in these men with the results in 24 men with classic GnRH deficiency before and during GnRH-replacement therapy and in 29 normal men of similar age.

RESULTS

Serum gonadotropin concentrations in the men with adult-onset GnRH deficiency were similar before and during pulsatile GnRH administration to those in the men with classic GnRH deficiency. However, as compared with men with classic GnRH deficiency, men with adult-onset hypogonadotropic hypogonadism had larger mean (+/-SD) testicular volumes (18+/-5 vs. 3+/-2 ml, P<0.001), serum testosterone concentrations (78+/-34 vs. 49+/-20 ng per deciliter [2.7+/-1.2 vs. 1.7+/-0.7 nmol per liter], P=0.004), and serum inhibin B concentrations (119+/-52 vs. 60+/-21 pg per milliliter, P<0.001). Treatment with GnRH reversed the hypogonadism and restored fertility in each of the five men who received long-term therapy.

CONCLUSIONS

The recognition of adult-onset hypogonadotropic hypogonadism in men as a distinct disorder expands the spectrum of GnRH deficiency and identifies a treatable form of male infertility.

摘要

背景

单纯性促性腺激素释放激素（GnRH）缺乏的男性通常表现为青春期发育缺失。我们描述了一种青春期后发生的成人起病型特发性低促性腺激素性性腺功能减退。

方法

我们研究了10名男性（年龄27至57岁），他们性成熟正常，患有特发性不育症、性功能障碍、血清睾酮浓度低，且经频繁采血发现促黄体生成素呈无脉冲分泌。所有这些男性的垂体前叶激素分泌及蝶鞍解剖结构在其他方面均正常。我们将这些男性的精液分析结果以及睾丸体积、血清睾酮、抑制素B和促性腺激素的测量结果，与24名经典GnRH缺乏男性在GnRH替代治疗前和治疗期间的结果，以及29名年龄相仿的正常男性的结果进行了比较。

结果

成人起病型GnRH缺乏男性在脉冲式GnRH给药前和给药期间的血清促性腺激素浓度，与经典GnRH缺乏男性的相似。然而，与经典GnRH缺乏男性相比，成人起病型低促性腺激素性性腺功能减退男性的平均（±标准差）睾丸体积更大（18±5比3±2 ml，P<0.001），血清睾酮浓度更高（78±34比49±20 ng/分升[2.7±1.2比1.7±0.7 nmol/升]，P=0.004），血清抑制素B浓度更高（119±52比60±21 pg/毫升，P<0.001）。接受长期治疗的5名男性中，GnRH治疗逆转了性腺功能减退并恢复了生育能力。