Gradually increasing ethinyl estradiol for Turner syndrome may produce good final height but not ideal BMD
Gradually increasing ethinyl estradiol for Turner syndrome may produce good final height but not ideal BMD
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DOI：
10.1507/endocrj.ej16-0170
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发表时间：
2017-01-01
影响因子：
2
通讯作者：
Goto, Masahiro
中科院分区：
文献类型：
--
作者：
Hasegawa, Yukihiro;Ariyasu, Daisuke;Goto, Masahiro
Turner综合征的雌激素替代疗法理论上应该模仿健康女孩的生理机能。本研究的目的是描述17例特纳综合征患者（E组）的最终身高和骨密度（BMD），这些患者开始使用超低剂量（1-5 ng/kg/天）乙炔雌二醇治疗9.8-13.7年。E组自平均年龄7.4岁起给予GH 0.35 mg/kg/周治疗。L组是历史组之一，30名受试者给予相当剂量的生长激素，并在12.2-18.7岁时开始结合雌激素0.3125 mg/周，与0.3125 mg/天相似。S组为另一历史组，为21例乳房发育和月经初潮自发的患者。E组最终身高（身高增加< 2 cm/年）为152.4 +/- 3.4 cm， Turner综合征的标准差（SD）为2.02 +/- 0.62。L组的最终身高为148.5 +/- 3.0 cm， SD为1.30 +/- 0.55，与E组差异显著。S组的体积骨密度（0.290 +/- 0.026 g/cm(3)）与L组和E组（平均值分别为0.262和0.262 g/cm3）差异显著。这是第一个对特纳综合征患者的研究，最初以超低剂量施用雌激素，然后逐渐增加。我们的雌激素治疗在E组产生了良好的最终身高，但不理想的骨密度。
Estrogen replacement therapy in Turner syndrome should theoretically mimic the physiology of healthy girls. The objective of this study was to describe final height and bone mineral density (BMD) in a group of 17 Turner syndrome patients (group E) who started their ethinyl estradiol therapy with an ultra-low dosage (1-5 ng/kg/day) from 9.8-13.7 years. The subjects in group E had been treated with GH 0.35 mg/kg/week since the average age of 7.4 years. The 30 subjects in group L, one of the historical groups, were given comparable doses of GH, and conjugated estrogen 0.3125 mg/week similar to 0.3125 mg/day was initiated at 12.2-18.7 years. The subjects in group S, the other historical group, were 21 patients who experienced breast development and menarche spontaneously. Final height (height gain < 2 cm/year) in group E was 152.4 +/- 3.4 cm and the standard deviation (SD) was 2.02 +/- 0.62 for Turner syndrome. The final height in group L was 148.5 +/- 3.0 cm with a SD of 1.30 +/- 0.55, which was significantly different from the values for group E. The volumetric BMD of group S (0.290 +/- 0.026 g/cm(3)) was significantly different from that of group L or E (0.262 or 0.262 g/cm3 as a mean, respectively). This is the first study of patients with Turner syndrome where estrogen was administered initially in an ultra-low dose and then increased gradually. Our estrogen therapy in group E produced good final height but not ideal BMD.