Department of Molecular Medicine, Aarhus University Hospital, 8200 Aarhus, Denmark.
Department of Endocrinology and Internal Medicine, Aarhus University Hospital, 8200 Aarhus, Denmark.
J Clin Endocrinol Metab. 2022 Jun 16;107(7):1983-1993. doi: 10.1210/clinem/dgac167.
Women with Turner syndrome (TS) suffer from hypergonadotropic hypogonadism, causing a deficit in gonadal hormone secretion. As a consequence, these women are treated with estrogen from the age of 12 years, and later in combination with progesterone. However, androgens have been given less attention.
To assess sex hormone levels in women with TS, both those treated and those nontreated with hormone replacement therapy (HRT), and investigate the impact of HRT on sex hormone levels.
At Aarhus University Hospital, 99 women with TS were followed 3 times from August 2003 to February 2010. Seventeen were lost during follow-up. Control group 1 consisted of 68 healthy age-matched control women seen once during this period. Control group 2 consisted of 28 young, eumenorrheic women sampled 9 times throughout the same menstrual cycle. Serum concentrations of follicle-stimulating hormone (FSH), luteinizing hormone (LH), 17β-estradiol, estrone sulfate, DHEAS, testosterone, free androgen index, androstenedione, 17-OH progesterone, and sex hormone-binding globulin (SHBG) were analyzed.
All androgens, 17-OH progesterone, and sex hormone-binding globulin (SHBG) were 30% to 50% lower in TS compared with controls (P < 0.01). FSH, LH, and estrone sulfate were more than doubled in women with TS compared with controls (P < 0.02). Using principal component analysis, we describe a positive correlation between women with TS receiving HRT, elevated levels of SHBG, and decreased levels of androgens.
The sex hormone profile in TS reveals a picture of androgen deficiency, aggravated further by HRT. Conventional HRT does not normalize estradiol levels in TS.
特纳综合征（TS）患者患有促性腺激素性性腺功能减退症，导致性腺激素分泌不足。因此，这些患者从 12 岁起就接受雌激素治疗，之后则与孕激素联合治疗。然而，雄激素的关注度相对较低。
评估特纳综合征患者（包括接受和未接受激素替代治疗（HRT）的患者）的性激素水平，并研究 HRT 对性激素水平的影响。
在奥胡斯大学医院，对 99 名特纳综合征患者进行了 3 次随访，随访时间为 2003 年 8 月至 2010 年 2 月。在随访过程中有 17 名患者脱落。对照组 1 由在此期间仅接受过 1 次检查的 68 名年龄匹配的健康对照组女性组成。对照组 2 由 28 名月经正常的年轻女性组成，这些女性在同一月经周期内接受了 9 次采样。分析了血清卵泡刺激素（FSH）、黄体生成素（LH）、17β-雌二醇、雌酮硫酸酯、DHEAS、睾酮、游离雄激素指数、雄烯二酮、17-羟孕酮和性激素结合球蛋白（SHBG）的浓度。
与对照组相比，特纳综合征患者的所有雄激素、17-羟孕酮和性激素结合球蛋白（SHBG）水平均降低了 30%至 50%（P < 0.01）。与对照组相比，特纳综合征患者的 FSH、LH 和雌酮硫酸酯水平增加了一倍以上（P < 0.02）。使用主成分分析，我们描述了接受 HRT 的特纳综合征患者、SHBG 水平升高以及雄激素水平降低之间的正相关关系。
特纳综合征患者的性激素谱显示出雄激素缺乏的特征，而 HRT 进一步加重了这种情况。传统的 HRT 并不能使特纳综合征患者的雌二醇水平正常化。
