| Literature DB >> 20213134 |
Justine Bacchetta1, Sonia Fargue, Stéphanie Boutroy, Odile Basmaison, Nicolas Vilayphiou, Ingrid Plotton, Fitsum Guebre-Egziabher, Bruno Dohin, Rémi Kohler, Pierre Cochat.
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.Entities:
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Year: 2010 PMID: 20213134 DOI: 10.1007/s00467-010-1453-x
Source DB: PubMed Journal: Pediatr Nephrol ISSN: 0931-041X Impact factor: 3.714