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特纳综合征:寻求更好的治疗效果。

Turner syndrome: searching for better outcomes.

作者信息

Ramos Adauto Versiani, Silva Ivani Novato, Goulart Eugênio Marcos Andrade

机构信息

Department of Endocrinology, Hospital Felício Rocho, Belo Horizonte, MG, Brazil.

出版信息

Clinics (Sao Paulo). 2008 Apr;63(2):173-8. doi: 10.1590/s1807-59322008000200004.

Abstract

OBJECTIVES

To assess the results of growth hormone on the growth of girls with Turner Syndrome and identify relevant parameters to improve outcomes.

METHODS

Growth velocity and final height were studied in a historical cohort of 41 girls, regularly followed up for hormone distribution at three referral centers. The influence of oxandrolone and of estrogens on the final height was analyzed. The girls (initial chronological age=8.9+/-3.4years; initial bone age=7.0+/-3.1years) used 0.19 mg/kg/week of growth hormone for 4.0 +/- 2.0 years.

RESULTS

In the first year, growth velocity increased by 71.5% in 41 girls and 103.4% in those who reached final height (11 girls). The whole group had a gain in the height SDS of 0.8 +/- 0.7 (p<0.01) and for those who reached a final height of 1.0 +/- 0.8 (p<0.01). Final height (143.6 +/-6.3 cm) was 3.9 +/- 5.3 cm higher than the predicted height, and the height gain occurred before estrogen therapy. Oxandrolone had no significant influence on height gain. The significant variables contributing to the final height were the duration of growth hormone used and its use prior to starting estrogens, the initial height SDS, and the growth velocity during the first year of treatment.

CONCLUSIONS

We concluded that the use of growth hormone significantly increased the final height, which remained lower than the target. Results point to a need for starting growth hormone use as early as possible and to maximize treatment before estrogen replacement. It has been observed that even moderate doses of growth hormone may significantly increase early growth velocity.

摘要

目的

评估生长激素对特纳综合征女孩生长的影响,并确定相关参数以改善治疗效果。

方法

对41名女孩的历史队列进行生长速度和最终身高研究,这41名女孩在三个转诊中心接受定期随访以进行激素治疗。分析了氧雄龙和雌激素对最终身高的影响。这些女孩(初始实际年龄=8.9±3.4岁;初始骨龄=7.0±3.1岁)使用0.19mg/kg/周的生长激素治疗4.0±2.0年。

结果

在第一年,41名女孩的生长速度提高了71.5%,达到最终身高的女孩(11名)生长速度提高了103.4%。整个组身高标准差得分增加了0.8±0.7(p<0.01),达到最终身高的女孩身高标准差得分增加了1.0±0.8(p<0.01)。最终身高(143.6±6.3cm)比预测身高高3.9±5.3cm,身高增加发生在雌激素治疗之前。氧雄龙对身高增加没有显著影响。影响最终身高的显著变量包括生长激素的使用时长、开始使用雌激素之前使用生长激素的情况、初始身高标准差得分以及治疗第一年的生长速度。

结论

我们得出结论,生长激素的使用显著提高了最终身高,但仍低于目标值。结果表明需要尽早开始使用生长激素,并在雌激素替代治疗之前最大限度地进行治疗。据观察,即使是中等剂量的生长激素也可能显著提高早期生长速度。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0355/2664211/43b551c9dc89/cln63_2p0173f1.jpg

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