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特纳综合征短期和长期生长中心对标准生长激素剂量的分析证实了生长预测算法,并显示正常的 IGF-I 水平。

Analyses from a centre of short- and long-term growth in Turner's syndrome on standard growth hormone doses confirm growth prediction algorithms and show normal IGF-I levels.

机构信息

Paediatric Endocrinology and Diabetology, University Children's Hospital, Tuebingen, Germany.

出版信息

Horm Res Paediatr. 2012;77(4):214-21. doi: 10.1159/000336806. Epub 2012 Mar 16.

DOI:10.1159/000336806
PMID:22433161
Abstract

AIMS AND METHODS

Prediction algorithms suggest factors determining short- and long-term growth response to growth hormone (GH) in Turner's syndrome (TS). A total of 133 patients (group A; 53% with karyotype 45,X) completed 1 year of treatment and 77 patients (group B) reached adult height (AH) after >4 years on GH treatment. The patients were analysed for factors determining the outcomes, and in addition, the validity of published algorithms was tested.

RESULTS

In group A [values are given as medians (10th-90th percentiles)], starting age was 9.4 (4.8-14) years, height was -3.2 (-4.4 to -1.9) SDS (Prader references), and GH dose was 38 (23-48) μg/kg/day. Observed height velocity was 7.7 (5.2-9.8) cm/years and was equal to the predicted height velocity. In group B, projected adult height (PAH) was 147.4 (139.5-154.8) cm. Total gain in height over PAH of 6.1 (2.0-12.6) cm was negatively correlated with height at start, but positively correlated with GH duration, first year Δheight SDS, or index of responsiveness. Observed AH was 153.5 (146.6-160.1) cm and predicted AH was 155.0 (147.4-161.0) cm, which is statistically not different. On GH <5% IGF-I levels were >2 SDS.

CONCLUSIONS

The published prediction algorithms were found to be valid. If normal AH is to be reached at the lowest costs and risks, probably only TS children with a good growth potential and a high responsiveness to GH can be treated successfully with GH doses of <50 μg/kg/day.

摘要

目的和方法

预测算法提示了决定特纳综合征(TS)患者短期和长期生长激素(GH)反应的因素。共有 133 名患者(A 组;53%的核型为 45,X)完成了 1 年的治疗,77 名患者(B 组)在 GH 治疗>4 年后达到成人身高(AH)。对这些患者进行了分析,以确定影响治疗结果的因素,并对已发表的算法的有效性进行了测试。

结果

在 A 组中[数值以中位数(10-90 百分位数)表示],起始年龄为 9.4 岁(4.8-14 岁),身高为-3.2(-4.4 至-1.9)SDS(Prader 参考值),GH 剂量为 38(23-48)μg/kg/天。观察到的身高增长速度为 7.7(5.2-9.8)cm/年,与预测的身高增长速度相等。在 B 组中,预测的成人身高(PAH)为 147.4(139.5-154.8)cm。超过 PAH 的身高总增长 6.1(2.0-12.6)cm 与起始身高呈负相关,但与 GH 持续时间、第一年身高 SDS 增长值或反应指数呈正相关。观察到的 AH 为 153.5(146.6-160.1)cm,预测的 AH 为 155.0(147.4-161.0)cm,统计学上无差异。在 GH<5%IGF-I 水平时,>2 SDS。

结论

已发表的预测算法被证明是有效的。如果要以最低的成本和风险达到正常的 AH,可能只有具有良好生长潜力和对 GH 高度反应的 TS 儿童才能成功接受<50μg/kg/天的 GH 剂量治疗。

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