Çetiner Ebru Barsal, Donbaloğlu Zeynep, Singin Berna, Çetin Kürşat, Bahar Yasemin Funda, Tuhan Hale, Parlak Mesut
Department of Pediatric Endocrinology, Akdeniz University Hospital, Antalya, Türkiye.
Turk Arch Pediatr. 2025 May 2;60(3):294-300. doi: 10.5152/TurkArchPediatr.2025.24319.
Objective: Growth hormone neurosecretory dysfunction (GH-NSD) is a rare cause of short stat- ure. Diagnosis is established by evaluating nocturnal growth hormone secretion in patients with normal growth hormone stimulation tests. The aim of this study was to evaluate the first- and second-year treatment responses and final height in patients diagnosed with GH-NSD and treated with recombinant human growth hormone (rhGH). Materials and Methods: This retrospective study examined 500 patients treated with rhGH for short stature at a pediatric endocrinology clinic at Akdeniz University. Among them, 18 patients diagnosed with GH-NSD were included in the study. At the time of treatment initiation, param- eters such as insulin-like growth factor 1 (IGF-1), growth hormone (GH) stimulation test results, mean GH level during sleep, height standard deviation score (SDS), mid-parental height (MPH), and predicted adult height (PAH) were assessed. Treatment responses during the first and second years, as well as final height data, were analyzed. Results: GH therapy improved height velocity (HV) and overall height. The mean baseline height SDS of the patients was -3.13 ± 0.36. In the first year, Δheight SDS was 0.72 ± 0.44, and in the second year, Δheight SDS was 1.00 ± 0.70. Patients who reached their final height had a mean height SDS of -1.72 ± 0.83 and Δheight SDS of 1.46 ± 0.62. A correlation was found between the baseline height SDS, PAH SDS, MPH SDS, and first-year HV. Conclusion: Patients with GH-NSD treated with rhGH respond well to treatment, achieving genetic height potential.
生长激素神经分泌功能障碍(GH-NSD)是身材矮小的罕见原因。通过评估生长激素刺激试验正常的患者夜间生长激素分泌来确诊。本研究的目的是评估诊断为GH-NSD并接受重组人生长激素(rhGH)治疗的患者第一年和第二年的治疗反应及最终身高。材料与方法:这项回顾性研究检查了在阿克德尼兹大学儿科内分泌诊所接受rhGH治疗身材矮小的500例患者。其中,18例诊断为GH-NSD的患者被纳入研究。在开始治疗时,评估胰岛素样生长因子1(IGF-1)、生长激素(GH)刺激试验结果、睡眠期间平均GH水平、身高标准差评分(SDS)、父母平均身高(MPH)和预测成人身高(PAH)等参数。分析第一年和第二年的治疗反应以及最终身高数据。结果:GH治疗改善了身高增长速度(HV)和总体身高。患者的平均基线身高SDS为-3.13±0.36。第一年,身高SDS变化值为0.72±0.44,第二年,身高SDS变化值为1.00±0.70。达到最终身高的患者平均身高SDS为-1.72±0.83,身高SDS变化值为1.46±0.62。发现基线身高SDS、PAH SDS、MPH SDS与第一年的HV之间存在相关性。结论:接受rhGH治疗的GH-NSD患者对治疗反应良好,达到了遗传身高潜力。