Bone metabolism in oxalosis: a single-center study using new imaging techniques and biomarkers

Bone metabolism in oxalosis: a single-center study using new imaging techniques and biomarkers
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DOI:
10.1007/s00467-010-1453-x
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发表时间:
2010-06-01
影响因子:
3
通讯作者:
Cochat, Pierre
Cochat, Pierre
中科院分区:
医学3区
文献类型:
--
作者:
Bacchetta, Justine;Fargue, Sonia;Cochat, Pierre

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草酸钙晶体在肾脏和骨骼中的沉积是原发性高尿酸血症1型(PH 1)的标志。我们在此报告了一项基于骨生物标志物[甲状旁腺激素、维生素D、成纤维细胞生长因子23(FGF 23)]和放射学评估(骨龄、三维高分辨率外周定量计算机断层扫描,HR-pQCT)的12例PH 1儿童骨状态评估,这些评估在一项横断面单中心研究的框架内进行。对照组包括健康儿童和已参加当地骨和矿物质代谢研究的慢性肾病儿童。患者的平均年龄(+/-标准差)为99(+/- 63)个月。6名儿童骨折。5例患者骨成熟加速,其中4例< 5岁。新的成像技术和生物标志物的结合突出了PH 1的新的和无法解释的特征:年轻PH 1患者的骨龄提前,FGF 23水平升高,总体积骨密度降低,骨微结构改变。
The deposition of calcium oxalate crystals in the kidney and bone is a hallmark of primary hyperoxaluria type 1 (PH1). We report here an evaluation of the bone status of 12 PH1 children based on bone biomarkers [parathyroid hormone, vitamin D, fibroblast growth factor 23 (FGF23)] and radiological assessments (skeletal age, three-dimensional high-resolution peripheral quantitative computed tomography, HR-pQCT) carried out within the framework of a cross-sectional single-center study. The controls consisted of healthy and children with chronic kidney disease already enrolled in local bone and mineral metabolism studies. The mean age (+/- standard deviation) age of the patients was 99 (+/- 63) months. Six children suffered from fracture. Bone maturation was accelerated in five patients, four of whom were < 5 years. The combination of new imaging techniques and biomarkers highlighted new and unexplained features of PH1: advanced skeletal age in young PH1 patients, increased FGF23 levels and decreased total volumetric bone mineral density with bone microarchitecture alteration.