Evidence for Bone and Mineral Metabolism Alterations in Children with Autosomal Dominant Polycystic Kidney Disease.

Evidence for Bone and Mineral Metabolism Alterations in Children with Autosomal Dominant Polycystic Kidney Disease.

J Clin Endocrinol Metab. 2017 Sep 19;:

Authors: De Rechter S, Bacchetta J, Godefroid N, Dubourg L, Cochat P, Maquet J, Raes A, De Schepper J, Vermeersch P, Van Dyck M, Levtchenko E, D'Haese P, Evenepoel P, Mekahli D

Abstract
Context: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common hereditary kidney disease. Hypophosphatemia was demonstrated in adult patients with preserved renal function, together with high fibroblast growth factor 23 (FGF23) and low soluble Klotho levels. The latter explained the relative FGF23 hyporesponsiveness in this cohort.
Objective: Evaluating phosphate and bone mineral metabolism in children with ADPKD, compared to what is known in adult ADPKD patients.
Design: Observational cross-sectional study.
Setting: Multicenter study, via ambulatory care in tertiary centers.
Patients & other participants: 92 children with ADPKD (52 males, mean ± SD age: 10.2 ± 5.0 years) and 22 healthy controls (HC, 10 males, mean ± SD age: 10.3 ± 4.1 years).
Predictor: Early ADPKD stage.
Main Outcome measures: Bone mineral metabolism and renal phosphate handling. Performed measurements were: serum phosphate, TmP/GFR, fibroblast growth factor 23, soluble Klotho, sclerostin and bone alkaline phosphatase.
Results: ADPKD children had significantly lower serum phosphate levels compared to HC. Low TmP/GFR was observed in 24% of patients, although not significantly different from HC. Serum FGF23 and soluble Klotho levels were comparable between patients and HC. In addition, we showed decreased bone alkaline phosphatase levels in ADPKD children, suggesting suppressed bone formation.
Conclusions: This is the first report demonstrating hypophosphatemia and suppressed bone formation in pediatric ADPKD cohort, with preserved renal function, compared to HC. Although FGF23 levels were not different from controls, they should be considered inappropriate, given the concomitant hypophosphatemia. Further studies are required to elucidate underlying pathophysiology and potential clinical consequences.

PMID: 29092060 [PubMed - as supplied by publisher]

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