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特纳综合征女童的生长激素治疗:国家合作生长研究经验

Growth hormone treatment of girls with Turner syndrome: the National Cooperative Growth Study experience.

作者信息

Plotnick L, Attie K M, Blethen S L, Sy J P

机构信息

Department of Pediatrics, Johns Hopkins University, Baltimore, Maryland 21287, USA.

出版信息

Pediatrics. 1998 Aug;102(2 Pt 3):479-81.

PMID:9685447
Abstract

OBJECTIVE

To evaluate growth rate and adult height with recombinant growth hormone (GH) treatment in girls with Turner syndrome (TS) and predictors of their growth response.

METHODS

Data on girls with TS who were treated with GH in the National Cooperative Growth Study (NCGS) were evaluated. As of January 1997, there were 2798 girls with TS in the NCGS database, 2652 of whom had not previously received GH. Follow-up data on growth were available for 2475 subjects, and data on adult height were available for 622.

RESULTS

The average age of girls with TS at enrollment in the NCGS was 10.1 +/- 3.6 years. These patients had severely short stature compared with that of unaffected American girls (height, 118.5 +/- 16.5 cm; height standard deviation score [SDS], -3.1 +/- 0.9), but their heights were typical of those of American girls with TS (TS-specific height SDS, 0.01 +/- 0.9). Treatment with GH for an average duration of 3.2 +/- 2.0 years resulted in an increase in height SDS of 0.8 +/- 0.7 compared with unaffected girls and of 1.2 +/- 0.8 compared with TS standards. Growth rates increased from 4.0 +/- 2.3 cm/year before treatment to 7.5 +/- 2.0 cm/year after 1 year of treatment. Duration of treatment with GH was the strongest predictor of change in height SDS. After 6 to 7 years of treatment with GH, there was a cumulative change of 2.0 in mean height SDS. The 622 girls who reached adult height were older when they began taking GH. Their mean height gain over pre-GH projected height was 6.4 +/- 4.9 cm after 3.7 +/- 1.9 years of treatment. Their adult height was 148.3 +/- 5.6 cm.

CONCLUSIONS

Although the response to treatment with GH varied, it was associated with highly significant gains in growth and adult height in girls with TS. Duration of treatment with GH was the most important variable predicting adult height.

摘要

目的

评估重组生长激素(GH)治疗特纳综合征(TS)女孩的生长速率和成年身高,以及其生长反应的预测因素。

方法

对国家合作生长研究(NCGS)中接受GH治疗的TS女孩的数据进行评估。截至1997年1月,NCGS数据库中有2798例TS女孩,其中2652例此前未接受过GH治疗。有2475例受试者的生长随访数据,622例有成年身高数据。

结果

NCGS中TS女孩入组时的平均年龄为10.1±3.6岁。与未受影响的美国女孩相比,这些患者身材严重矮小(身高,118.5±16.5厘米;身高标准差评分[SDS],-3.1±0.9),但其身高与美国TS女孩的典型身高相符(TS特异性身高SDS,0.01±0.9)。平均持续3.2±2.0年的GH治疗导致身高SDS较未受影响女孩增加0.8±0.7,较TS标准增加1.2±0.8。生长速率从治疗前的4.0±2.3厘米/年增加到治疗1年后的7.5±2.0厘米/年。GH治疗持续时间是身高SDS变化的最强预测因素。GH治疗6至7年后,平均身高SDS累积变化为2.0。达到成年身高的622例女孩开始使用GH时年龄较大。经过3.7±1.9年的治疗,她们的平均身高较GH治疗前预测身高增加了6.4±4.9厘米。她们的成年身高为148.3±5.6厘米。

结论

虽然GH治疗反应存在差异,但它与TS女孩的生长和成年身高显著增加相关。GH治疗持续时间是预测成年身高的最重要变量。

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