Zarubina N A, Golubeva I V, Bobrovskaia T A, Dobracheva A D, Konnova E V
Probl Endokrinol (Mosk). 1978 Jul-Aug;24(4):41-6.
Functional condition of the hypophysis-gonad system with the determination of the LH and FSH level in the course of one month, of estrogens and testosterones in the blood, of estrogens in the urine, sex chromatine, kariotype, roentgenomorphology and histology of the gonads, as well as the changes in gonadotropines against the background of estrogen treatment were studied in 30 patients (in 18 with Shereshevsky-Turner's syndrome, and in 12 with pure gonad agenesis). There were differences in the state of gonadotropic activity in patients of these groups, rather similar by condition and time of the gonad affection. In Shereshevsky-Turner's syndrome gonadotropic activity was characterized by a sharp elevation of the LH and FSH level with frequent chaotic desynchronized fluctuations; in pure gonad agenesis the FSH level was elevated and subject to chaotic fluctuations; as to LH level--it was stable and within the normal limits. Shereshevsky-Turner's syndrome with pure agenesis displayed a retention of the feedback in the hypophysis-gonad system, this being indicated by reduction of the LH level against the background of estrogen therapy. The FSH content was a more informative index for the diagnosis of the gonad agenesis than the LH content. There was revealed no correlation between the kariotypes and the gonadotropin level in the patients. Estrogen therapy in the gonad agenesis and dysgenesis is required not only for feminization of the patients, but also for suppression of increased hypothalamic activity characteristic of these cases.
对30例患者(18例患有舍雷舍夫斯基-特纳综合征,12例患有单纯性腺发育不全)研究了垂体-性腺系统的功能状况,测定了1个月内的促黄体生成素(LH)和促卵泡生成素(FSH)水平、血液中的雌激素和睾酮、尿液中的雌激素、性染色质、核型、性腺的X线形态学和组织学,以及雌激素治疗背景下促性腺激素的变化。这些组的患者在促性腺激素活性状态方面存在差异,性腺病变的情况和时间相当相似。在舍雷舍夫斯基-特纳综合征中,促性腺激素活性的特征是LH和FSH水平急剧升高,伴有频繁的混乱不同步波动;在单纯性腺发育不全中,FSH水平升高且有混乱波动;至于LH水平——它稳定且在正常范围内。舍雷舍夫斯基-特纳综合征合并单纯性腺发育不全在垂体-性腺系统中显示出反馈保留,这在雌激素治疗背景下LH水平降低中得到体现。FSH含量比LH含量是诊断性腺发育不全更具信息性的指标。患者的核型与促性腺激素水平之间未发现相关性。性腺发育不全和发育异常的雌激素治疗不仅是为了使患者女性化,也是为了抑制这些病例中下丘脑活动增加的情况。