{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T17:13:27Z","timestamp":1792084407707,"version":"4.4.0"},"reference-count":0,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2014,1,1]],"date-time":"2014-01-01T00:00:00Z","timestamp":1388534400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Pediatric Rehabilitation Medicine"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:p>PURPOSE: Skeletal disease causes significant morbidity in\n\t\t\t mucopolysaccharidoses (MPS), and bone remodeling processes in MPS have not been\n\t\t\t well characterized. The objective of this study was to determine if biomarkers\n\t\t\t of bone turnover are abnormal in children with specific MPS disorders (i.e.\n\t\t\t MSP-I, MPS-II, and MPS-VI) compared to healthy children.<\/jats:p>\n                  <jats:p>METHODS: A cross-sectional study was performed of serum biomarkers\n\t\t\t of bone formation (bone-specific alkaline phosphatase [BSAP], osteocalcin) and\n\t\t\t urine biomarkers of bone resorption (pyridinoline, deoxypyridinoline) in MPS\n\t\t\t and healthy controls. Measures of physical function and pain were obtained\n\t\t\t using the Children's Health Questionnaire (CHQ).<\/jats:p>\n                  <jats:p>RESULTS: The cohort consisted of 39 children with MPS (MPS-I=26;\n\t\t\t MPS-II=11; MPS-VI=4) and 51 healthy children. Adjusting for sex and Tanner\n\t\t\t stage group, MPS individuals had statistically significant increases for\n\t\t\t osteocalcin (p&lt; 0.001), with trends toward higher BSAP (p=0.054) and urinary\n\t\t\t pyridinoline (p=0.084). These biomarkers were not significantly associated with\n\t\t\t CHQ bodily pain and physical-function scores.<\/jats:p>\n                  <jats:p>CONCLUSION: Osteocalcin was increased in children with MPS\n\t\t\t disorders, with trends for increases in BSAP and urinary pyridinoline,\n\t\t\t suggesting that bone remodeling is altered in children with MPS. Future studies\n\t\t\t to assess the ability of these biomarkers to quantify and monitor MPS skeletal\n\t\t\t disease in response to therapy are needed.<\/jats:p>","DOI":"10.3233\/prm-140285","type":"journal-article","created":{"date-parts":[[2019,12,3]],"date-time":"2019-12-03T17:13:23Z","timestamp":1575393203000},"page":"159-165","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":16,"title":["Biomarkers of bone remodeling in children with\t\t\t mucopolysaccharidosis types I, II, and VI"],"prefix":"10.1177","volume":"7","author":[{"given":"David A.","family":"Stevenson","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Medical\r\t\t\t Genetics, University of Utah, Salt Lake City, UT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyle","family":"Rudser","sequence":"additional","affiliation":[{"name":"Division of Biostatistics, University of Minnesota,\r\t\t\t Minneapolis, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia","family":"Kunin-Batson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Minnesota,\r\t\t\t Minneapolis, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen B.","family":"Fung","sequence":"additional","affiliation":[{"name":"Children's Hospital and Research Center, Oakland, CA,\r\t\t\t USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Viskochil","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical\r\t\t\t Genetics, University of Utah, Salt Lake City, UT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elsa","family":"Shapiro","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Minnesota,\r\t\t\t Minneapolis, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul J.","family":"Orchard","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Minnesota,\r\t\t\t Minneapolis, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chester B.","family":"Whitley","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Minnesota,\r\t\t\t Minneapolis, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynda E.","family":"Polgreen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Minnesota,\r\t\t\t Minneapolis, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2014,1]]},"container-title":["Journal of Pediatric Rehabilitation Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.3233\/PRM-140285","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.3233\/PRM-140285","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T04:33:54Z","timestamp":1777610034000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.3233\/PRM-140285"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,1]]},"references-count":0,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2014,5]]}},"alternative-id":["10.3233\/PRM-140285"],"URL":"https:\/\/doi.org\/10.3233\/prm-140285","relation":{},"ISSN":["1874-5393","1875-8894"],"issn-type":[{"value":"1874-5393","type":"print"},{"value":"1875-8894","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,1]]}}}