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Bone metabolism in oxalosis: a single-center study using new imaging techniques and biomarkers.
1081 - 1089.
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.
|Title:||Bone metabolism in oxalosis: a single-center study using new imaging techniques and biomarkers|
|Keywords:||Bone imaging, Children, FGF23, Primary hyperoxaluria, Skeletal age, PRIMARY HYPEROXALURIA TYPE-1, PREEMPTIVE LIVER-TRANSPLANTATION, CHRONIC-RENAL-FAILURE, OXALATE, CHILDREN, DISEASE, TOMOGRAPHY|
|UCL classification:||UCL > School of Life and Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Life Sciences
UCL > School of Life and Medical Sciences > Faculty of Life Sciences > Biosciences (Division of)
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