Biomarkers of bone remodeling in children with mucopolysaccharidosis types I, II, and VI

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  • Ellen B. Fung
    Children's Hospital and Research Center, Oakland, CA, USA
  • David Viskochil
    Department of Pediatrics, Division of Medical Genetics, University of Utah, Salt Lake City, UT, USA
  • Alicia Kunin-Batson
    Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
  • Elsa Shapiro
    Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
  • Paul J. Orchard
    Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
  • Chester B. Whitley
    Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
  • Lynda E. Polgreen
    Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
  • David A. Stevenson
    Department of Pediatrics, Division of Medical Genetics, University of Utah, Salt Lake City, UT, USA
  • Kyle Rudser
    Division of Biostatistics, University of Minnesota, Minneapolis, MN, USA

書誌事項

公開日
2014-01
権利情報
  • https://journals.sagepub.com/page/policies/text-and-data-mining-license
DOI
  • 10.3233/prm-140285
公開者
SAGE Publications

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<jats:p>PURPOSE: Skeletal disease causes significant morbidity in mucopolysaccharidoses (MPS), and bone remodeling processes in MPS have not been well characterized. The objective of this study was to determine if biomarkers of bone turnover are abnormal in children with specific MPS disorders (i.e. MSP-I, MPS-II, and MPS-VI) compared to healthy children.</jats:p> <jats:p>METHODS: A cross-sectional study was performed of serum biomarkers of bone formation (bone-specific alkaline phosphatase [BSAP], osteocalcin) and urine biomarkers of bone resorption (pyridinoline, deoxypyridinoline) in MPS and healthy controls. Measures of physical function and pain were obtained using the Children's Health Questionnaire (CHQ).</jats:p> <jats:p>RESULTS: The cohort consisted of 39 children with MPS (MPS-I=26; MPS-II=11; MPS-VI=4) and 51 healthy children. Adjusting for sex and Tanner stage group, MPS individuals had statistically significant increases for osteocalcin (p< 0.001), with trends toward higher BSAP (p=0.054) and urinary pyridinoline (p=0.084). These biomarkers were not significantly associated with CHQ bodily pain and physical-function scores.</jats:p> <jats:p>CONCLUSION: Osteocalcin was increased in children with MPS disorders, with trends for increases in BSAP and urinary pyridinoline, suggesting that bone remodeling is altered in children with MPS. Future studies to assess the ability of these biomarkers to quantify and monitor MPS skeletal disease in response to therapy are needed.</jats:p>

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