Berg A A, Kjessler B
Acta Endocrinol Suppl (Copenh). 1976;207:49-59.
Certain defects in the intratesticular androgen biogenetic system may lead to a significant impairment of gonadal function in total in the human male. In the present investigation, four males with impaired reproductive performance were analysed before and after long term treatment with gonadotrophic hormones with regard to their in vitro metabolism of 3H-progesterone in testicular incubates; one of whom representing the first instance of an XYY-male studied in some detail with regard to intratesticular steroidogenesis. A steroid metabolisc pattern of an "immature" type, i.e., possibly indicating a relative understimulation of the gonads by gonadotrophic hormones, in vivo, was found in one of the four patients, i.e., the XYY-male, inspite of normal levels of gonadotrophic hormones in the peripheral circulation. Gametic output was found to increase significantly during the course of gonadotrophin substitution therapy, and the individual steroid metabolic pattern also changed drastically towards a more mature type subsequent to therapy. In a second patients, who originally presented with completely immotile sperm, a certain shift in the steroid metabolic pattern was observed after the gonadotrophin therapy in favour of increasing relative amounts of 17a-hydroxyprogesterone, concurrent with a decreasing proportion of dead sperm, and a certain, though minimal, improvement in sperm kinetics. The last two patients, with originally mature types of in vitro steroid metabolism in testicular incubates, displayed no changes in steroid metabolic patterns or spermiogram qualities subsequent to the gonadotrophin therapy. However, one of them might have been azoospermic because of anatomical reasons. The present demonstration that an individual steroid metabolic pattern in testicular incubates under certain conditions may be deliberately modified as expected, by long term gonadotrophin therapy, further supports our previous suggestion that the relative proportions of newly synthesized and recovered deltal4-3-oxo-C21-steroids in testicular incubates, especially 20a-dihydroprogesterone and 17a-hydroxyprogesterone, may well reflect the actual gonadotrophic stimulation in a particular individual at the time of testicular biopsy. Investigations of individual steroid metabolic patterns in testicular incubates may therefore contribute useful information for the adequate selection of patients with disturbed gonadal function, who may benefit from gonadotrophin substitution therapy.
睾丸内雄激素生物合成系统的某些缺陷可能导致男性性腺功能严重受损。在本研究中,对四名生殖功能受损的男性在长期接受促性腺激素治疗前后,进行了睾丸孵育物中3H-孕酮体外代谢分析;其中一人是首例就睾丸内类固醇生成进行详细研究的XYY男性。在四名患者中的一名,即XYY男性中,尽管外周循环中促性腺激素水平正常,但在睾丸孵育物中发现了一种“不成熟”类型的类固醇代谢模式,即可能表明体内促性腺激素对性腺的刺激相对不足。在促性腺激素替代治疗过程中,配子产量显著增加,并且治疗后个体类固醇代谢模式也急剧转变为更成熟的类型。在第二名最初表现为精子完全不动的患者中,促性腺激素治疗后观察到类固醇代谢模式有一定转变,有利于17α-羟孕酮相对含量增加,同时死精比例降低,精子活力有一定程度(尽管很微小)的改善。最后两名患者,其睾丸孵育物中最初具有成熟类型的体外类固醇代谢,促性腺激素治疗后类固醇代谢模式或精液图质量未显示变化。然而,其中一人可能由于解剖学原因无精子症。目前的研究表明,在某些条件下,通过长期促性腺激素治疗,睾丸孵育物中的个体类固醇代谢模式可按预期进行刻意改变,这进一步支持了我们之前的观点,即睾丸孵育物中新合成和回收的δ14-3-氧代-C21-类固醇的相对比例,尤其是20α-二氢孕酮和17α-羟孕酮,很可能反映了睾丸活检时特定个体实际的促性腺激素刺激情况。因此,对睾丸孵育物中个体类固醇代谢模式的研究可能为适当选择性腺功能紊乱且可能从促性腺激素替代治疗中获益的患者提供有用信息。