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优化特纳综合征的雌激素替代治疗。

Optimizing estrogen replacement treatment in Turner syndrome.

作者信息

Rosenfield R L, Perovic N, Devine N, Mauras N, Moshang T, Root A W, Sy J P

机构信息

Department of Pediatrics, University of Chicago, Chicago, Illinois 60637-1470, USA.

出版信息

Pediatrics. 1998 Aug;102(2 Pt 3):486-8.

PMID:9685449
Abstract

Estrogen has a biphasic effect on growth, stimulatory at low doses but inhibitory at higher doses. Therefore, designing optimal sex hormone replacement treatment in girls with Turner syndrome (TS) who are being treated with growth hormone (GH) involves considering the dose and form of the estrogen as well as the route and timing of its administration. We report here a preliminary analysis of a study to test the concept that an optimal estrogen replacement regimen should consist of estradiol administered in a low dose by a systemic route. The study population consisted of 9 girls with TS who had been treated with GH for 6 or more months. When the girls were 12 to 15 years old, we added depot estradiol at a monthly intramuscular dose of 0.2 mg and increased the dose at 6-month intervals to 0.4, 0.6, and, in 7 of the girls, 0.8 mg. We compared the results in these subjects with those in a matched group of 37 patients with TS in whom routine estrogen treatment had been started at similar ages and who were treated with a similar course of GH therapy. The gain in height at 2 years was 2.6 cm greater in those who were treated with depot estradiol than in those who were treated with routine estrogen. The bone age in the patients who were treated with depot estradiol increased in proportion to their chronologic age, suggesting that this difference indicates an increase in their predicted adult height. We conclude that using very low doses of systemic estradiol to induce puberty before the age of 15 years in girls with TS who are treated with GH, instead of using routine estrogen therapy, can result in increased final heights.

摘要

雌激素对生长具有双相作用,低剂量时具有刺激作用,但高剂量时具有抑制作用。因此,在接受生长激素(GH)治疗的特纳综合征(TS)女孩中设计最佳性激素替代治疗方案,需要考虑雌激素的剂量和剂型以及给药途径和时间。我们在此报告一项研究的初步分析,以验证最佳雌激素替代方案应由通过全身途径给予低剂量雌二醇组成这一概念。研究人群包括9名接受GH治疗6个月或更长时间的TS女孩。当女孩们12至15岁时,我们添加了每月肌肉注射剂量为0.2 mg的长效雌二醇,并每6个月将剂量增加至0.4、0.6 mg,其中7名女孩增加至0.8 mg。我们将这些受试者的结果与37名年龄相仿且接受相似疗程GH治疗的TS患者的匹配组结果进行了比较。接受长效雌二醇治疗的患者在2年时的身高增长比接受常规雌激素治疗的患者高2.6 cm。接受长效雌二醇治疗的患者的骨龄与实际年龄成比例增加,这表明这种差异表明她们预测的成人身高有所增加。我们得出结论,在接受GH治疗的TS女孩中,在15岁之前使用极低剂量的全身雌二醇诱导青春期,而不是使用常规雌激素治疗,可导致最终身高增加。

相似文献

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Optimizing estrogen replacement treatment in Turner syndrome.优化特纳综合征的雌激素替代治疗。
Pediatrics. 1998 Aug;102(2 Pt 3):486-8.
2
Evidence for early initiation of growth hormone and transdermal estradiol therapies in girls with Turner syndrome.关于特纳综合征女孩早期开始生长激素和经皮雌二醇治疗的证据。
Growth Horm IGF Res. 2006 Jul;16 Suppl A:S91-7. doi: 10.1016/j.ghir.2006.04.002. Epub 2006 Jun 2.
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Growth hormone treatment of girls with Turner syndrome: the National Cooperative Growth Study experience.特纳综合征女童的生长激素治疗:国家合作生长研究经验
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Salutary effects of combining early very low-dose systemic estradiol with growth hormone therapy in girls with Turner syndrome.在特纳综合征女孩中,早期联合使用极低剂量系统性雌二醇与生长激素治疗的有益效果。
J Clin Endocrinol Metab. 2005 Dec;90(12):6424-30. doi: 10.1210/jc.2005-1081. Epub 2005 Sep 27.
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[Effect of estrogen therapy on the growth of Turner syndrome in girls treated with growth hormone].雌激素疗法对接受生长激素治疗的特纳综合征女童生长情况的影响
Medicina (B Aires). 2001;61(3):271-4.
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[Optimizing estrogen treatment in Turner syndrome].[优化特纳综合征的雌激素治疗]
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The psychological consequences of Turner syndrome and review of the National Cooperative Growth Study psychological substudy.特纳综合征的心理影响及全国合作生长研究心理子研究综述
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Combination growth hormone and estrogen increase bone mineralization in girls with Turner syndrome.
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Adult height in children with growth hormone deficiency who are treated with biosynthetic growth hormone: the National Cooperative Growth Study experience.接受生物合成生长激素治疗的生长激素缺乏症儿童的成人身高:全国合作生长研究经验。
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