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Tytuł:
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Bone and Mineral Metabolism in Children with Nephropathic Cystinosis Compared with other CKD Entities.
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Autorzy:
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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.
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Źródło:
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The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2020 Aug 01; Vol. 105 (8).
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Typ publikacji:
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Journal Article; Multicenter Study; Observational Study; Research Support, Non-U.S. Gov't
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Język:
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English
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Imprint Name(s):
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Publication: 2017- : New York : Oxford University Press
Original Publication: Springfield, Ill. : Charles C. Thomas
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MeSH Terms:
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Bone Resorption/*diagnosis
Chronic Kidney Disease-Mineral and Bone Disorder/*diagnosis
Cystinosis/*complications
Fanconi Syndrome/*etiology
Renal Insufficiency, Chronic/*etiology
Adolescent ; Bone Resorption/etiology ; Bone Resorption/physiopathology ; Calcification, Physiologic/physiology ; Child ; Chronic Kidney Disease-Mineral and Bone Disorder/etiology ; Chronic Kidney Disease-Mineral and Bone Disorder/physiopathology ; Cross-Sectional Studies ; Cystinosis/physiopathology ; Cystinosis/surgery ; Fanconi Syndrome/physiopathology ; Fanconi Syndrome/surgery ; Female ; Fibroblast Growth Factor-23 ; Humans ; Kidney Transplantation ; Male ; Prospective Studies ; Renal Insufficiency, Chronic/diagnosis ; Renal Insufficiency, Chronic/physiopathology ; Renal Insufficiency, Chronic/surgery ; Severity of Illness Index
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Contributed Indexing:
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Keywords: FGF23; Klotho; OPG; TRAP5b; children; chronic kidney disease; cystinosis; rickets; sclerostin
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Entry Date(s):
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Date Created: 20200516 Date Completed: 20210208 Latest Revision: 20211204
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Update Code:
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20240105
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DOI:
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10.1210/clinem/dgaa267
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PMID:
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32413117
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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.)
