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Evidence for bone and mineral metabolism alterations in children with autosomal dominant polycystic kidney disease

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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 with what is known in adult ADPKD patients. Design: Observational cross-sectional study. Setting: Multicenter study via ambulatory care in tertiary centers. Participants: Ninety-two children with ADPKD (52 males; mean 6 standard deviation age, 10.2 +/- 5.0 years) and 22 healthy controls (HCs, 10 males; mean 6 standard deviation age, 10.3 +/- 4.1 years). Main Outcome Measures: The predictor was early ADPKD stage. Bone mineral metabolism and renal phosphate handling were the main outcome measures. Performed measurements were serum phosphate, tubular maximum phosphorus reabsorption per glomerular filtration rate, FGF23, soluble Klotho, sclerostin, and bone alkaline phosphatase. Results: ADPKD children had significantly lower serum phosphate levels compared with HC. Low tubular maximum phosphorus reabsorption per glomerular filtration rate 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 report demonstrates hypophosphatemia and suppressed bone formation in a pediatric ADPKD cohort, with preserved renal function, compared with 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.
Keywords
PHOSPHATE, SKELETOGENESIS, ABNORMALITIES, REABSORPTION, EXPRESSION, DIAGNOSIS, SKELETAL, SPECTRUM

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Chicago
De Rechter, Stéphanie, Justine Bacchetta, Nathalie Godefroid, Laurence Dubourg, Pierre Cochat, Julie Maquet, Ann Raes, et al. 2017. “Evidence for Bone and Mineral Metabolism Alterations in Children with Autosomal Dominant Polycystic Kidney Disease.” Journal of Clinical Endocrinology & Metabolism 102 (11): 4210–4217.
APA
De Rechter, Stéphanie, Bacchetta, J., Godefroid, N., Dubourg, L., Cochat, P., Maquet, J., Raes, A., et al. (2017). Evidence for bone and mineral metabolism alterations in children with autosomal dominant polycystic kidney disease. JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 102(11), 4210–4217.
Vancouver
1.
De Rechter S, Bacchetta J, Godefroid N, Dubourg L, Cochat P, Maquet J, et al. Evidence for bone and mineral metabolism alterations in children with autosomal dominant polycystic kidney disease. JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM. 2017;102(11):4210–7.
MLA
De Rechter, Stéphanie et al. “Evidence for Bone and Mineral Metabolism Alterations in Children with Autosomal Dominant Polycystic Kidney Disease.” JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM 102.11 (2017): 4210–4217. Print.
@article{8539127,
  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 with what is known in adult ADPKD patients. 
Design: Observational cross-sectional study. 
Setting: Multicenter study via ambulatory care in tertiary centers. 
Participants: Ninety-two children with ADPKD (52 males; mean 6 standard deviation age, 10.2 +/- 5.0 years) and 22 healthy controls (HCs, 10 males; mean 6 standard deviation age, 10.3 +/- 4.1 years). 
Main Outcome Measures: The predictor was early ADPKD stage. Bone mineral metabolism and renal phosphate handling were the main outcome measures. Performed measurements were serum phosphate, tubular maximum phosphorus reabsorption per glomerular filtration rate, FGF23, soluble Klotho, sclerostin, and bone alkaline phosphatase. 
Results: ADPKD children had significantly lower serum phosphate levels compared with HC. Low tubular maximum phosphorus reabsorption per glomerular filtration rate 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 report demonstrates hypophosphatemia and suppressed bone formation in a pediatric ADPKD cohort, with preserved renal function, compared with 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.},
  author       = {De Rechter, Stéphanie and Bacchetta, Justine and Godefroid, Nathalie and Dubourg, Laurence and Cochat, Pierre and Maquet, Julie and Raes, Ann and De Schepper, Jean and Vermeersch, Pieter and Van Dyck, Maria and Levtchenko, Elena and D’Haese, Patrick and Evenepoel, Pieter and Mekahli, Djalila},
  issn         = {0021-972X},
  journal      = {JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM},
  keywords     = {PHOSPHATE,SKELETOGENESIS,ABNORMALITIES,REABSORPTION,EXPRESSION,DIAGNOSIS,SKELETAL,SPECTRUM},
  language     = {eng},
  number       = {11},
  pages        = {4210--4217},
  title        = {Evidence for bone and mineral metabolism alterations in children with autosomal dominant polycystic kidney disease},
  url          = {http://dx.doi.org/10.1210/jc.2017-01157},
  volume       = {102},
  year         = {2017},
}

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