title: Bone metabolism in oxalosis: a single-center study using new imaging techniques and biomarkers.
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.
authors.affiliation: Service de Néphrologie et Rhumatologie Pédiatriques, Centre de Référence des Maladies Rénales Rares, Hôpital Femme Mère Enfant, 59 Bd Pinel, 69677 Bron, France. justine.bacchetta@chu-lyon.fr
authors.forename: Justine
authors.initials: J
authors.lastname: Bacchetta
authors.forename: Sonia
authors.initials: S
authors.lastname: Fargue
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Boutroy
authors.forename: Odile
authors.initials: O
authors.lastname: Basmaison
authors.forename: Nicolas
authors.initials: N
authors.lastname: Vilayphiou
authors.forename: Ingrid
authors.initials: I
authors.lastname: Plotton
authors.forename: Fitsum
authors.initials: F
authors.lastname: Guebre-Egziabher
authors.forename: Bruno
authors.initials: B
authors.lastname: Dohin
authors.forename: Rémi
authors.initials: R
authors.lastname: Kohler
authors.forename: Pierre
authors.initials: P
authors.lastname: Cochat
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000032:analysis*; D001842:Bone and Bones / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D000089703:Fibroblast Growth Factor-23; D005346:Fibroblast Growth Factors / Q000097:blood; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000378:metabolism* / Q000473:pathology*; D007223:Infant; D008297:Male; D014057:Tomography, X-Ray Computed / Q000379:methods*; D055815:Young Adult
chemical_list: D015415:Biomarkers; C000717427:FGF23 protein, human; D005346:Fibroblast Growth Factors; D000089703:Fibroblast Growth Factor-23