Universität zu Köln

Ewert, Annika, Leifheit-Nestler, Maren ORCID: 0000-0002-9203-7622, Hohenfellner, Katharina, Buescher, Anja, Kemper, Markus J., Oh, Jun, Billing, Heiko, Thumfart, Julia, Stangl, Gabriele, Baur, Anja C., Foeller, Michael, Feger, Martina, Weber, Lutz T., Acham-Roschitz, Birgit, Arbeiter, Klaus, Toenshoff, Burkhard, Zivicnjak, Miroslav and Haffner, Dieter (2020). Bone and Mineral Metabolism in Children with Nephropathic Cystinosis Compared with other CKD Entities. J. Clin. Endocrinol. Metab., 105 (8). S. E2738 - 15. WASHINGTON: ENDOCRINE SOC. ISSN 1945-7197

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Abstract

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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Ewert, Annika UNSPECIFIED UNSPECIFIED UNSPECIFIED Leifheit-Nestler, Maren UNSPECIFIED orcid.org/0000-0002-9203-7622 UNSPECIFIED Hohenfellner, Katharina UNSPECIFIED UNSPECIFIED UNSPECIFIED Buescher, Anja UNSPECIFIED UNSPECIFIED UNSPECIFIED Kemper, Markus J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Oh, Jun UNSPECIFIED UNSPECIFIED UNSPECIFIED Billing, Heiko UNSPECIFIED UNSPECIFIED UNSPECIFIED Thumfart, Julia UNSPECIFIED UNSPECIFIED UNSPECIFIED Stangl, Gabriele UNSPECIFIED UNSPECIFIED UNSPECIFIED Baur, Anja C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Foeller, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Feger, Martina UNSPECIFIED UNSPECIFIED UNSPECIFIED Weber, Lutz T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Acham-Roschitz, Birgit UNSPECIFIED UNSPECIFIED UNSPECIFIED Arbeiter, Klaus UNSPECIFIED UNSPECIFIED UNSPECIFIED Toenshoff, Burkhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Zivicnjak, Miroslav UNSPECIFIED UNSPECIFIED UNSPECIFIED Haffner, Dieter UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-324509
DOI:	10.1210/clinem/dgaa267
Journal or Publication Title:	J. Clin. Endocrinol. Metab.
Volume:	105
Number:	8
Page Range:	S. E2738 - 15
Date:	2020
Publisher:	ENDOCRINE SOC
Place of Publication:	WASHINGTON
ISSN:	1945-7197
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CHRONIC KIDNEY-DISEASE; PEDIATRIC REFERENCE INTERVALS; ALKALINE-PHOSPHATASE; CYSTEAMINE THERAPY; YOUNG-ADULTS; GROWTH; OSTEOPROTEGERIN; PROGRESSION; SCLEROSTIN; PROTEIN Multiple languages Endocrinology & Metabolism Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/32450