Smals A G, Kloppenborg P W, van Haelst U J, Lequin R, Benraad T J
Acta Endocrinol (Copenh). 1978 Feb;87(2):389-99. doi: 10.1530/acta.0.0870389.
The functioning of the hypothalamo-pituitary-target organs axis was assessed in 3 patients with 'fertile eunuch' syndrome (FE) and 6 patients with 'classic' hypogonadotrophic hypogonadism (HH) with or without hyposmia. Both groups of patients did not differ from each other with regard to basal serum prolactin levels, pituitary growth hormone and thyrotrophin reserve and the thyroid or adrenal gland function. Both groups differed, however, with respect to the hypothalamo-pituitary-gonadal function: 1. the pituitary LH response to exogenous LH-RH was (low)-normal in FE and blunted in HH; 2. the basal FSH levels were normal in FE and undetectable in HH; 3. the basal LH levels were normal in FE and 3/6 patients with HH and low in the remaining three; 4. the basal and HCG stimulated plasma testosterone concentrations were significantly higher in FE than HH. The data suggest that FE represents a less severe form of LH-RH deficiency, rather than a distinct disorder.
对3例“生育无能型宦官症”(FE)患者和6例伴有或不伴有嗅觉减退的“经典型”低促性腺激素性性腺功能减退(HH)患者的下丘脑 - 垂体 - 靶器官轴功能进行了评估。两组患者在基础血清催乳素水平、垂体生长激素和促甲状腺激素储备以及甲状腺或肾上腺功能方面并无差异。然而,两组在下丘脑 - 垂体 - 性腺功能方面存在差异:1. FE患者垂体促黄体生成素(LH)对外源性促性腺激素释放激素(LH - RH)的反应为(低)正常,而HH患者则迟钝;2. FE患者基础促卵泡生成素(FSH)水平正常,HH患者则检测不到;3. FE患者基础LH水平正常,HH患者中有3/6正常,其余3例较低;4. FE患者基础和人绒毛膜促性腺激素(HCG)刺激后的血浆睾酮浓度显著高于HH患者。数据表明,FE代表了一种较轻形式的LH - RH缺乏,而非一种独特的疾病。