Bone and Mineral Metabolism in Children with Nephropathic Cystinosis Compared with other CKD Entities.

Autor:	Ewert A; Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany., Leifheit-Nestler M; Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany., Hohenfellner K; Division of Pediatric Nephrology, Children's Hospital, Rosenheim, Germany., Büscher A; Department of Pediatrics II, University Hospital Essen, Essen, Germany., Kemper MJ; Asklepius Hospital, Hamburg, Germany., Oh J; Division of Pediatric Nephrology, University Children's Hospital Hamburg, Hamburg, Germany., Billing H; Division of Pediatric Nephrology, University Children's Hospital Tübingen, Tübingen, Germany., Thumfart J; Department of Pediatric Gastroenterology, Nephrology and Metabolism, Charite Hospital, Berlin, Germany., Stangl G; Institute of Agricultural and Nutritional Sciences, Martin Luther University Halle-Wittenberg, Halle, Germany., Baur AC; Institute of Agricultural and Nutritional Sciences, Martin Luther University Halle-Wittenberg, Halle, Germany., Föller M; Institute of Physiology, University of Hohenheim, Stuttgart, Germany., Feger M; Institute of Physiology, University of Hohenheim, Stuttgart, Germany., Weber LT; Division of Pediatric Nephrology, Children´s and Adolescents´ Hospital, University of Cologne, Faculty of Medicine and University Hospital, Cologne, Germany., Acham-Roschitz B; Department of Pediatrics, Medical University Graz, Graz, Austria., Arbeiter K; Division of Pediatric Nephrology and Gastroenterology, Medical University Vienna, Austria., Tönshoff B; Department of Pediatrics I, University Children's Hospital Heidelberg, Heidelberg, Germany., Zivicnjak M; Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany., Haffner D; Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany.
Jazyk:	angličtina
Zdroj:	The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2020 Aug 01; Vol. 105 (8).
DOI:	10.1210/clinem/dgaa267
Abstrakt:	Context: Children with nephropathic cystinosis (NC) show persistent hypophosphatemia, due to Fanconi syndrome, as well as mineral and bone disorders related to chronic kidney disease (CKD); however, systematic analyses are lacking. Objective: To compare biochemical parameters of bone and mineral metabolism between children with NC and controls across all stages of CKD. Design: Cross-sectional multicenter study. Setting: Hospital clinics. Patients: Forty-nine children with NC, 80 CKD controls of the same age and CKD stage. Main Outcome Measures: Fibroblast growth factor 23 (FGF23), soluble Klotho, bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase 5b (TRAP5b), sclerostin, osteoprotegerin (OPG), biochemical parameters related to mineral metabolism, and skeletal comorbidity. Results: Despite Fanconi syndrome medication, NC patients showed an 11-fold increased risk of short stature, bone deformities, and/or requirement for skeletal surgery compared with CKD controls. This was associated with a higher frequency of risk factors such as hypophosphatemia, hypocalcemia, low parathyroid hormone (PTH), metabolic acidosis, and a specific CKD stage-dependent pattern of bone marker alterations. Pretransplant NC patients in mild to moderate CKD showed a delayed increase or lacked an increase in FGF23 and sclerostin, and increased BAP, TRAP5b, and OPG concentrations compared with CKD controls. Post-transplant, BAP and OPG returned to normal, TRAP5b further increased, whereas FGF23 and PTH were less elevated compared with CKD controls and associated with higher serum phosphate. Conclusions: Patients with NC show more severe skeletal comorbidity associated with distinct CKD stage-dependent alterations of bone metabolism than CKD controls, suggesting impaired mineralization and increased bone resorption, which is only partially normalized after renal transplantation. (© Endocrine Society 2020. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
Databáze:	MEDLINE
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