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Thus, historic control data on disease progression and variability are urgently needed. We conducted a 10\u2010year prospective observational study of 55 children with MPS IH (<jats:italic>N<\/jats:italic>\u00a0=\u200923), MPS IA (<jats:italic>N<\/jats:italic>\u00a0=\u200910), non\u2010neuronopathic MPS II (<jats:italic>N<\/jats:italic>\u00a0=\u200913), and MPS VI (<jats:italic>N<\/jats:italic>\u00a0=\u20099) to systematically evaluate bone and joint disease. Annual measurements included height, weight, and goniometry. Mixed effects modeling was used to evaluate changes over time. All participants had been treated with hematopoietic cell transplantation and\/or enzyme replacement therapy. Height <jats:italic>z<\/jats:italic>\u2010score decreased over time in MPS IH, MPS II, and MPS VI, but not MPS IA. Adult heights were 136\u2009\u00b1\u200910\u00a0cm in MPS IH, 161\u2009\u00b1\u200911\u2009cm in MPS IA, 161\u2009\u00b1\u200914\u2009cm in MPS II, and 128\u2009\u00b1\u200915\u2009cm in MPS VI. Adult average BMI percentiles were high: 75\u2009\u00b1\u200930%ile in MPS IH, 71\u2009\u00b1\u200937%ile in MPS IA, 71\u2009\u00b1\u200925%ile in MPS II, and 60\u2009\u00b1\u200942%ile in MPS VI. Every participant had joint contractures of the shoulders, elbows, hips, and\/or knees. Joint contractures remained stable over time. In conclusion, despite current treatments for MPS I, II, and VI, short stature and joint contractures persist. The elevation in average BMI may be related, in part, to physical inactivity due to the ongoing bone and joint disease. Data from this longitudinal historical control study may be used to expedite testing of experimental bone and joint directed therapies and to highlight the need for weight management as part of routine clinical care for patients with MPS.<\/jats:p>","DOI":"10.1002\/jimd.12598","type":"journal-article","created":{"date-parts":[[2023,2,25]],"date-time":"2023-02-25T13:53:01Z","timestamp":1677333181000},"page":"695-704","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Persistent bone and joint disease despite current treatments for mucopolysaccharidosis types I, <scp>II<\/scp>, and <scp>VI<\/scp>: Data from a 10\u2010year prospective study"],"prefix":"10.1002","volume":"46","author":[{"given":"Bradley S.","family":"Miller","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen 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