{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T02:14:56Z","timestamp":1740104096071,"version":"3.37.3"},"reference-count":28,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,11,11]],"date-time":"2021-11-11T00:00:00Z","timestamp":1636588800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2022,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Mucopolysaccharidoses (MPSs) are a group of rare diseases caused by an intralysosomal accumulation of glycosaminoglycans, resulting in a multisystemic clinical condition characterized by variable degrees of physical\u2010functional impairment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To evaluate the functional capacity (FC) of MPS patients and compare with a healthy control group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a cross\u2010sectional study of 6\u2010 to 39\u2010year\u2010old patients followed at a medical reference center and compared with their control peers, matched by age and sex. FC was assessed using the Sit\u2010to\u2010Stand Test (SST) and Incremental Shuttle Walk Test (ISWT). Heart rate (HR) and Borg rating of perceived exertion were measured before and after ISWT. HR recovery (HRR) was defined as the HR at the end of the test minus the HR in the second minute after ISWT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nineteen (19) MPS patients, 69% with type II MPS and mean age 17\u2009\u00b1\u200911 years were evaluated. Every patient was under enzyme replacement therapy. The time to perform the SST was longer in the MPS group (10.6\u2009\u00b1\u20092.5\u2009s vs. 6.7\u2009\u00b1\u20091.2\u2009s; <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.01). The MPS group achieved lower values of distance covered on the ISWT (407.6\u2009\u00b1\u2009329.8\u2009m vs. 1131.9\u2009\u00b1\u2009183.3\u2009m; <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.01), with a significantly higher Borg (6 [5\u20138] vs. 2 [1\u20134]; <jats:italic>p<\/jats:italic>\u2009=\u2009.02). The MPS group's HRR was slower than the controls (32.9\u2009\u00b1\u200920.2 beats per minute [bpm] vs. 69.1\u2009\u00b1\u200925.9\u2009bpm; <jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>We observed a pronounced reduction in the MPS group's FC compared to their healthy peers and a worse HRR after completing the test.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.25750","type":"journal-article","created":{"date-parts":[[2021,11,6]],"date-time":"2021-11-06T13:13:52Z","timestamp":1636204432000},"page":"538-543","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Mucopolysaccharidosis patients have reduced functional capacity"],"prefix":"10.1002","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7648-0029","authenticated-orcid":false,"given":"Bianca M. 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