在生长激素治疗期间与健康受试者之间的普拉德-威利综合征儿童之间的骨代谢差异:试点研究
Joanna Gajewska1, Magdalena Chełchowska1, Katarzyna Szamotulska2
1Department of Screening Tests and Metabolic Diagnostics, Institute of Mother and Child, Kasprzaka 17a, 01-211 Warsaw, Poland.
International journal of molecular sciences
|September 14, 2024
概括
接受生长激素 (GH) 治疗的普拉德-威利综合征 (PWS) 儿童表现出改变的骨循环标记物,包括维生素K依赖蛋白 (VKDPs). 这些变化表明PWS患者的骨质形成受损,并可能改变了VKDP碳素化.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 骨的新陈代谢 骨的新陈代谢
- 遗传学和罕见疾病
背景情况:
- 患有普拉德-威利综合征 (PWS) 的儿童尽管接受生长激素 (GH) 治疗,但经常经历低骨矿物质密度和骨科问题.
- 了解骨循环标记物,特别是维生素K依赖蛋白 (VKDPs),对于管理PWS骨健康至关重要.
- 在PWS中,GH治疗,饮食干预和骨代谢之间的相互作用需要进一步研究.
研究的目的:
- 对接受GH治疗和饮食干预的PWS正常体重儿童的骨标志物进行分析,重点关注VKDPs.
- 为了比较患有PWS的儿童与健康对照之间的骨标记者概况.
- 探索胰岛素样生长因子-I (IGF-I) 和骨代谢标志物在PWS中的关系.
主要方法:
- 包括24名患有PWS的儿童和30名健康对照.
- 测量了骨性酸酶 (BALP),骨素 (OC),碳氧化-OC (Gla-OC),低碳氧化-OC (Glu-OC),质素,骨质素,骨质保护素 (OPG),硬质素,I型原蛋白的C端端端粒 (CTX-I) 和IGF-I的血清度.
- 使用免疫酶方法来确定血清标志物.
主要成果:
- 与对照组相比,患有PWS的儿童的骨质素 (OC) 水平和OC/CTX-I比率较低 (p=0.011,p=0.006).
- 在PWS患者中观察到较低的Glu-OC (p=0.002),但较高的Gla-OC和环素度 (p=0.005,p<0.001).
- 在年龄调整后,在PWS儿童中,IGF-I与OC,Gla-OC和OC/CTX-I比率之间发现了显著的关系 (p=0.013,p=0.042,p=0.017).
结论:
- 在PWS中骨形成障碍可能源于OC度和OC/CTX-I比率的降低.
- 改变的OC形式和升高的环素表明PWS中加剧了VKDP氧化过程.
- 需要进一步的研究来阐明GH/IGF-I轴与骨代谢标记物,特别是PWS中的VKDPs之间的复杂相互作用.
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