Abstract |
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 (+ or - standard deviation) age of the patients was 99 (+ or - 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.
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Authors | Justine Bacchetta, Sonia Fargue, Stéphanie Boutroy, Odile Basmaison, Nicolas Vilayphiou, Ingrid Plotton, Fitsum Guebre-Egziabher, Bruno Dohin, Rémi Kohler, Pierre Cochat |
Journal | Pediatric nephrology (Berlin, Germany)
(Pediatr Nephrol)
Vol. 25
Issue 6
Pg. 1081-9
(Jun 2010)
ISSN: 1432-198X [Electronic] Germany |
PMID | 20213134
(Publication Type: Clinical Trial, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Biomarkers
- FGF23 protein, human
- Fibroblast Growth Factors
- Fibroblast Growth Factor-23
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Topics |
- Adolescent
- Biomarkers
(analysis)
- Bone and Bones
(metabolism)
- Child
- Child, Preschool
- Cross-Sectional Studies
- Female
- Fibroblast Growth Factor-23
- Fibroblast Growth Factors
(blood)
- Humans
- Hyperoxaluria, Primary
(metabolism, pathology)
- Infant
- Male
- Tomography, X-Ray Computed
(methods)
- Young Adult
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