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生长激素治疗的特纳综合征(TS)患者身高发育的主要决定因素:对国际生长数据库(KIGS)中987例患者的分析

Major determinants of height development in Turner syndrome (TS) patients treated with GH: analysis of 987 patients from KIGS.

作者信息

Ranke Michael B, Lindberg Anders, Ferrández Longás Angel, Darendeliler Feyza, Albertsson-Wikland Kerstin, Dunger David, Cutfield Wayne S, Tauber Maïthé, Wilton Patrick, Wollmann Hartmut A, Reiter Edward O

机构信息

Paediatric Endocrinology Section, Children's Hospital, University of Tuebingen, D-72076 Tuebingen, Germany.

出版信息

Pediatr Res. 2007 Jan;61(1):105-10. doi: 10.1203/01.pdr.0000250039.42000.c9.

Abstract

Little is known about factors determining height outcome during GH treatment in Turner syndrome (TS). We investigated 987 TS children within the Kabi International Growth Study (KIGS) who had reached near adult height (NAH) after >4 y GH treatment (including >1 y before puberty). Through multiple regression analysis we developed a model for NAH and total gain. Our results were as follows (median): 1) At start, age 9.7 yrs, height (HT) 118.0 cm (0.0 TS SDS), projected adult height 146.1 cm, GH dose 0.27 mg/kg wk; 2) NAH HT 151.0 cm (1.5 TS SDS); 3) Prepubertal gain 21.2 cm (1.6 TS SDS); 4) Pubertal gain 9.4 cm (0.0 TS SDS). NAH correlated (r = 0.67) with (ranked) HT at GH start (+), 1 year responsiveness to GH (+), MPH (+), age at puberty onset (+), age at GH start (-), and dose (+). The same factors explained (R = 0.90) the total HT gain. However, HT at GH start correlated negatively. Karyotype had no influence on outcome. Evidently, height at GH start (the taller, the better), age at GH start (the younger, the better), the responsiveness to GH (the higher, the better) and age at puberty (the later, the better) determine NAH.

摘要

关于特纳综合征(TS)患者生长激素(GH)治疗期间决定身高结局的因素,目前所知甚少。我们在卡比国际生长研究(KIGS)中调查了987例接受了超过4年GH治疗(包括青春期前1年以上)后达到接近成人身高(NAH)的TS患儿。通过多元回归分析,我们建立了一个关于NAH和总身高增长的模型。我们的结果如下(中位数):1)开始治疗时,年龄9.7岁,身高(HT)118.0厘米(TS标准差评分为0.0),预测成人身高146.1厘米,GH剂量0.27毫克/千克/周;2)NAH时HT为151.0厘米(TS标准差评分为1.5);3)青春期前身高增长21.2厘米(TS标准差评分为1.6);4)青春期身高增长9.4厘米(TS标准差评分为0.0)。NAH与GH开始治疗时的(排序后的)HT(正相关)、对GH的1年反应性(正相关)、甲泼尼龙(MPH)(正相关)、青春期开始年龄(正相关)、GH开始治疗年龄(负相关)以及剂量(正相关)相关(r = 0.67)。相同的因素解释了总身高增长情况(R = 0.90)。然而,GH开始治疗时的HT呈负相关。核型对结局无影响。显然,GH开始治疗时的身高(越高越好)、GH开始治疗的年龄(越小越好)、对GH的反应性(越高越好)以及青春期年龄(越晚越好)决定了NAH。

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