生长抑素类似物治疗1型神经纤维瘤病和生长激素过量的效果

IF 2 4区医学 Q4 CELL BIOLOGY

Growth Hormone & Igf Research Pub Date : 2025-10-01 Epub Date: 2025-10-21 DOI:10.1016/j.ghir.2025.101667

Sofie Skov Koch , Jonathan Frederik Carlsen , Cecilie Ejerskov , Ulla Feldt-Rasmussen , Katharina M. Main , Astrid Sehested , Rene Mathiasen , Sarah Linea von Holstein , Stense Farholt , Rikke Beck Jensen

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引用次数: 0

摘要

儿童生长激素（GH）过量是罕见的，但可能在1型神经纤维瘤病（NF1）儿童中看到，然后通常与视神经胶质瘤（OPG）相关。目的本研究的目的是确定NF1和GH过量患者的人体测量学和治疗效果。此外，研究生长激素过量和OPG之间的关系。设计：对9例NF1和GH过量患者进行描述性、回顾性研究，这些患者被转介到儿科内分泌学三级中心。设置：单三级中心。儿童内分泌学家对9例生长激素过量和NF1患者进行常规随访，进行临床评估、人体测量学和激素分析。所有患者均接受脑磁共振成像（MRI）检查，并重新评估所有扫描结果以确定OPGs的分类。此外，眼科医生对患者进行了常规随访。主要结果测量：人体测量和IGF-I水平以标准差评分（SDS）表示。结果患者身高速度（HV）、身高（SDS）、IGF-I （SDS）均高于正常参考范围。9名患者中有8名患有opg，其中7名与下丘脑有关。给予生长抑素类似物（SSas）治疗。在治疗的第一年，所有患者的IGF-I （SDS）水平迅速下降，并在2至3年半内恢复正常。结论NF1患儿的快速生长需要进一步评估，特别是OPG患儿，因为它可能是由生长激素过量引起的。用SSas治疗生长激素过量是有效的。

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The effect of treatment with somatostatin analogs in children with neurofibromatosis type 1 and growth hormone excess

Context

Growth hormone (GH) excess in children is rare but may be seen in children with neurofibromatosis type 1 (NF1) and is then often associated with optic pathway glioma (OPG).

Objective

The aim of this study was to determine anthropometrics and the efficacy of treatment in patients with NF1 and GH excess. Additionally, to examine the association between GH excess and OPG.

Design

A descriptive, retrospective study on nine patients with NF1 and GH excess referred to a tertiary center of pediatric endocrinology.

Setting: Single tertiary center.

Patients

Nine patients with GH excess and NF1 were routinely followed by pediatric endocrinologists for clinical evaluation, anthropometrics, and hormone analysis. All patients underwent brain Magnetic Resonance Imaging (MRI) and all the scans were reevaluated for classification of the OPGs. Furthermore, the patients were routinely followed by ophthalmologists.

Main outcome measures

Anthropometrics and IGF-I levels expressed as standard deviation scores (SDS).

Results

The patients presented with height velocity (HV) (SDS), height (SDS) and IGF-I (SDS) above the normal reference range. Eight out of nine patients had OPGs, seven of which with hypothalamic involvement. They were treated with somatostatin analogs (SSas). Within the first year of treatment, IGF-I (SDS) levels decreased rapidly and normalized in all patients within two to three and a half years.

Conclusion

Rapid growth in children with NF1 requires further evaluation especially in those with OPG since it could be a caused by GH excess. Treatment of GH excess with SSas was effective.

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来源期刊

Growth Hormone & Igf Research 医学-内分泌学与代谢

CiteScore

3.30

自引率

0.00%

发文量

审稿时长

57 days

期刊介绍： Growth Hormone & IGF Research is a forum for research on the regulation of growth and metabolism in humans, animals, tissues and cells. It publishes articles on all aspects of growth-promoting and growth-inhibiting hormones and factors, with particular emphasis on insulin-like growth factors (IGFs) and growth hormone. This reflects the increasing importance of growth hormone and IGFs in clinical medicine and in the treatment of diseases.