性激素结合球蛋白作为特纳综合征激素治疗效果的标志物。

Sex hormone-binding globulin as a marker of the effect of hormonal treatment in Turner's syndrome.

作者信息

Hampl R, Snajderova M, Lebl J, Lisa L, Dvorakova M, Hill M, Sulcova J, Starka L

机构信息

Institute of Endocrinology, 119 94 Praha, Czech Republic.

出版信息

Endocr Regul. 2001 Mar;35(1):17-24.

PMID:11308992

Abstract

OBJECTIVE

In girls with Turner's syndrome a positive effect of growth hormone (GH) therapy on statutal growth has been well documented, in spite of the fact that the levels of GH and IGF-I in these patients are usually within the normal range. It is known that plasma levels of sex hormone-binding globulin (SHBG) are negatively associated with growth hormone (GH) and with [GF-I. Limited data on SHBG levels in these patients and only scarce reports on other than gonadal steroids in these patients are available so far. The major aim of this study was to find out whether SHBG does respond to GH treatment.

METHODS

Plasma levels of SHBG, estradiol, progesterone, testosterone and the adrenal steroids and their precursors, namely cortisol, 17alpha-hydroxyprogesterone, androstenedione, dehydroepiandrosterone and its sulfate were determined in 65 patients aged 2-23 years with Turner's syndrome. The patients were divided into 4 groups according to their actual treatment (untreated, sex steroids only, GH only, GH and sex steroids) and the differences between the groups were evaluated. With respect to dependence of SHBG and of steroids studied on age (with exception of cortisol), their values were expressed as per cents of medians of the physiological values.

RESULTS

As expected, low levels of sex steroids were found. As to the adrenal steroids, in general, their average levels were lower than in healthy subjects but still within the physiological limits. With one exception (androstenedione), no significant differences were found between the groups. The levels of cortisol were undistinguishable from healthy subjects. Absolute as well as relative SHBG levels in untreated group and in the group treated with estrogens were normal or even lower than those reported for normal subjects of the same chronological age. Significantly reduced absolute as well as relative SHBG levels in comparison to all other groups were found in GH treated children.

CONCLUSION

The results indicate that SHBG may serve as a sensitive biochemical marker of the response to the GH administration.

摘要

目的

在特纳综合征女孩中，生长激素（GH）治疗对身高增长具有积极作用，这一点已得到充分证实，尽管这些患者的GH和胰岛素样生长因子-I（IGF-I）水平通常在正常范围内。已知性激素结合球蛋白（SHBG）的血浆水平与生长激素（GH）以及[GF-I呈负相关。到目前为止，关于这些患者SHBG水平的数据有限，且仅有少量关于这些患者性腺类固醇以外的其他物质的报道。本研究的主要目的是确定SHBG是否对GH治疗有反应。

方法

测定了65例年龄在2至23岁的特纳综合征患者的血浆SHBG、雌二醇、孕酮、睾酮以及肾上腺类固醇及其前体的水平，即皮质醇、17α-羟孕酮、雄烯二酮、脱氢表雄酮及其硫酸盐。根据患者的实际治疗情况（未治疗、仅使用性类固醇、仅使用GH、使用GH和性类固醇）将患者分为4组，并评估组间差异。关于所研究的SHBG和类固醇与年龄的相关性（皮质醇除外），其值以生理值中位数的百分比表示。

结果

正如预期的那样，发现性类固醇水平较低。至于肾上腺类固醇，总体而言，其平均水平低于健康受试者，但仍在生理范围内。除了一个例外（雄烯二酮），组间未发现显著差异。皮质醇水平与健康受试者无法区分。未治疗组和接受雌激素治疗组的SHBG绝对水平以及相对水平均正常，甚至低于相同实际年龄正常受试者的报道水平。与所有其他组相比，接受GH治疗的儿童SHBG绝对水平以及相对水平均显著降低。