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长期生长激素治疗的特纳综合征印度女孩的成年身高——印度西部三级中心的经验

Adult Height in Indian Girls with Turner Syndrome Treated with Long-Term Growth Hormone Therapy - A Western India Tertiary Centre Experience.

作者信息

Khadilkar Vaman, Mondkar Shruti, Oza Chirantap, Gondhalekar Ketan, Khadilkar Anuradha

机构信息

Department of Growth and Paediatric Endocrinology, Hirabai Cowasji Jehangir Medical Research Institute, Pune, Maharashtra, India.

Department of Health Sciences, Savitribai Phule Pune University, Pune, Maharashtra, India.

出版信息

Indian J Endocrinol Metab. 2023 May-Jun;27(3):249-254. doi: 10.4103/ijem.ijem_255_22. Epub 2023 Jun 26.

Abstract

BACKGROUND AND OBJECTIVES

Owing to paucity of data on adult height in Indian girls with Turner syndrome treated with growth hormone (GH), this study was conducted to assess improvement in height following GH therapy and adult height achieved with long-term GH therapy in Indian girls with Turner syndrome and to assess relationship between achieved and predicted height.

METHODOLOGY

Retrospective analysis was performed on 12 girls with karyotype-proven Turner syndrome, who had attained adult height following mean duration of GH therapy of 4.8 years (range: 2.7-7.6). Adult height predictions were performed using index of responsiveness (IOR) and Ranke's prediction model.

RESULTS

Mean age at starting GH was 10.2 ± 1.9 years; Pubertal induction was between 11 and 15 years. Mean height gain was 29.3 ± 9.8 cm (range: 14-39.5) from onset of treatment to adult height. Significant improvement in height Z scores (IAP 2015 and Indian Turner reference data) following GH therapy ( = 0.002 and 0.012, respectively) was noted. Using Indian Turner reference data, the height Z score improved from pre-treatment 0.8 ± 0.8 to 2.0 ± 0.9 on stopping GH and adult height Z score of 1.3 ± 0.7. Using Ranke's equation for prediction of near adult height, predicted and achieved adult height showed a strong positive correlation (Spearman correlation coefficient = 0.827, significant at 0.01 level).

CONCLUSION

At a dose in the lower range (40-50 mcg/kg/day) of recommendation and duration of 5 years, Indian girls with Turner syndrome can achieve adult height within the healthy Indian reference range. Dose individualization based on IOR would help in optimizing GH dosage and would turn out to be economically sustainable without compromising on height outcomes.

摘要

背景与目的

由于关于生长激素(GH)治疗的印度特纳综合征女童成年身高的数据匮乏,本研究旨在评估GH治疗后身高的改善情况以及长期GH治疗的印度特纳综合征女童所达到的成年身高,并评估实际身高与预测身高之间的关系。

方法

对12名经核型证实为特纳综合征且在平均4.8年(范围:2.7 - 7.6年)的GH治疗后达到成年身高的女童进行回顾性分析。使用反应指数(IOR)和兰克预测模型进行成年身高预测。

结果

开始GH治疗时的平均年龄为10.2±1.9岁;青春期诱导在11至15岁之间。从治疗开始到成年身高,平均身高增加了29.3±9.8厘米(范围:14 - 39.5厘米)。GH治疗后身高Z评分(IAP 2015和印度特纳参考数据)有显著改善(分别为 = 0.002和0.012)。使用印度特纳参考数据,停止GH治疗时身高Z评分从治疗前的0.8±0.8提高到2.0±0.9,成年身高Z评分为1.3±0.7。使用兰克方程预测接近成年身高时,预测成年身高与实际成年身高显示出强正相关(斯皮尔曼相关系数 = 0.827,在0.01水平显著)。

结论

在推荐的较低剂量范围(40 - 50微克/千克/天)和5年的治疗时间下,印度特纳综合征女童能够在健康印度参考范围内达到成年身高。基于IOR的剂量个体化将有助于优化GH剂量,并且在不影响身高结果的情况下在经济上具有可持续性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5db2/10424106/09b683a9d5d1/IJEM-27-249-g001.jpg

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