Hospital for Children and Adolescents, University of Heidelberg, Heidelberg, Germany.
Horm Res Paediatr. 2013;80(5):356-62. doi: 10.1159/000356045. Epub 2013 Nov 6.
Ullrich-Turner syndrome (UTS) girls often present with short stature in adolescence to the endocrinologist when the efficacy of growth hormone (GH) to improve growth remains unknown and parameters to estimate individual GH responsiveness have yet to be determined.
Retrospective evaluation of adult height (AH) and predicted adult height at GH start (descriptive model of Ranke, Model PredAH) in early and late GH-treated German UTS patients.
SUBJECTS/METHODS: 313 patients treated with GH, early [chronological age (CA) at GH start <12 years, n = 259] or late (CA at GH start ≥12 years, n = 54) who reached AH were selected from KIGS (Pfizer International Growth Database).
AH (152.5 ± 5.9 vs. 151.1 ± 5.4 cm, p = n.s.) after GH treatment for 7.5 ± 2.12 years (GH start early) and for 5.2 ± 1.2 years (GH start late) were similar (p = n.s.) as Model PredAH (155.7 ± 4.8 vs. 154.7 ± 4.8 cm; p = n.s.) but higher (p < 0.001) than projected adult height (Ranke, ProjAH; 148.2 ± 5.5 vs. 145.2 ± 6.7 cm; p = 0.001). Total height gain over ProjAH was 4.3 ± 4.6 cm (GH start early) and 5.8 ± 4.7 cm (GH start late, p = 0.021), respectively.
GH may improve AH in UTS patients even when started late. The individual growth response could be estimated by the descriptive Model PredAH independent of age at treatment start.
乌利希-特纳综合征（UTS）女孩在青春期常常因身材矮小而就诊于内分泌科，此时生长激素（GH）治疗能否改善生长尚不清楚，个体 GH 反应性的评估参数也尚未确定。
回顾性评估早期和晚期 GH 治疗的德国 UTS 患者的成年身高（AH）和 GH 起始时预测的成年身高（Ranke 描述模型，Model PredAH）。
受试者/方法：从 KIGS（辉瑞国际生长数据库）中选择了 313 名接受 GH 治疗且达到 AH 的患者，这些患者分为早期治疗组（GH 起始年龄＜12 岁，n=259）和晚期治疗组（GH 起始年龄≥12 岁，n=54）。
接受 GH 治疗 7.5±2.12 年（GH 起始早）和 5.2±1.2 年后（GH 起始晚）的 AH（152.5±5.9 与 151.1±5.4cm，p=n.s.）相似（p=n.s.），与 Model PredAH（155.7±4.8 与 154.7±4.8cm；p=n.s.）相比更高，但低于预测成年身高（Ranke，ProjAH；148.2±5.5 与 145.2±6.7cm；p=0.001）。与 ProjAH 相比，GH 起始早组和 GH 起始晚组的身高总增长分别为 4.3±4.6cm 和 5.8±4.7cm（p=0.021）。
即使 GH 治疗开始较晚，也可能改善 UTS 患者的 AH。个体生长反应可通过独立于治疗起始年龄的描述性 Model PredAH 进行评估。
