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神经分泌功能障碍中的生长激素治疗

Growth Hormone Therapy in Neurosecretory Dysfunction.

作者信息

Çetiner Ebru Barsal, Donbaloğlu Zeynep, Singin Berna, Çetin Kürşat, Bahar Yasemin Funda, Tuhan Hale, Parlak Mesut

机构信息

Department of Pediatric Endocrinology, Akdeniz University Hospital, Antalya, Türkiye.

出版信息

Turk Arch Pediatr. 2025 May 2;60(3):294-300. doi: 10.5152/TurkArchPediatr.2025.24319.

Abstract

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.

摘要

目的

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。

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