Department of Pediatrics, Thomas Jefferson University DuPont Hospital for Children, Philadelphia, Pa., USA.
Horm Res Paediatr. 2011;76(6):392-9. doi: 10.1159/000333073. Epub 2011 Nov 29.
BACKGROUND/AIMS: To assess height standard deviation scores (HSDS) in patients with Turner syndrome (TS) by age at treatment initiation and varying durations of treatment with growth hormone (GH).
GH treatment-naïve patients with TS from the American Norditropin Studies: Web-enabled Research (ANSWER) Program® Registry were analyzed at baseline, 4 months, and annually.
Three hundred and eighty-two patients with TS had a baseline mean (±SD) HSDS of -2.6 ± 0.9. Patients received short-term (1 year), long-term (<3 years), and extended GH treatment (≥3 years, mean = 4.54 years), resulting in 40.2% (n = 99/246), 60.5% (n = 69/114), and 62.3% (n = 86/138) of the patients achieving HSDS >-2. Patients starting GH at a younger age experienced better growth response, regardless of treatment duration. Change in HSDS from baseline (ΔHSDS) at 4 months correlated positively with ΔHSDS at 1 and 3 years, and ΔHSDS at 1 year with ΔHSDS at 3 years (p values from 0.0017 to<0.0001).
Height gains in patients with TS during short-term treatment were found to be highly predictive of longer-term results. Continuation of GH treatment (≥3 years) resulted in 62.3% of the patients achieving an HSDS within the normal population range, indicating the clinical importance of early initiation and continuation of GH treatment in patients with TS.
背景/目的:评估特纳综合征(TS)患者在不同起始治疗年龄和不同生长激素(GH)治疗持续时间时的身高标准差评分(HSDS)。
对美国 Norditropin 研究:Web 支持的研究(ANSWER)计划®注册处的 GH 治疗初治 TS 患者进行分析,在基线、4 个月和每年进行分析。
382 例 TS 患者的基线平均(±SD)HSDS 为-2.6±0.9。患者接受短期(1 年)、长期(<3 年)和延长 GH 治疗(≥3 年,平均=4.54 年),结果有 40.2%(n=99/246)、60.5%(n=69/114)和 62.3%(n=86/138)的患者 HSDS>-2。无论治疗持续时间如何,年龄较小开始 GH 治疗的患者生长反应更好。4 个月时 HSDS 的变化(ΔHSDS)与 1 年和 3 年时的ΔHSDS 呈正相关,1 年时的 ΔHSDS 与 3 年时的 ΔHSDS 也呈正相关(p 值均<0.0001)。
在短期治疗期间,TS 患者的身高增长被发现与长期结果高度相关。继续 GH 治疗(≥3 年)导致 62.3%的患者 HSDS 达到正常人群范围,表明在 TS 患者中早期开始和继续 GH 治疗的临床重要性。