{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T20:32:39Z","timestamp":1777494759011,"version":"3.51.4"},"reference-count":16,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2018,7,18]],"date-time":"2018-07-18T00:00:00Z","timestamp":1531872000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Child Health Care"],"published-print":{"date-parts":[[2019,6]]},"abstract":"<jats:p>Mucopolysaccharidosis (MPS) is a rare and neglected disorder. Only a few studies to date have focused on humanistic impacts of the disease, particularly health-related quality of life (HRQOL). The objective of our research is to (1) evaluate HRQOL in Brazilian patients with MPS and (2) assess the validity of the PedsQL 4.0 Generic Core Scales (PedsQL) in this specific disease. We performed an analytical cross-sectional study using the Brazilian Portuguese version of the PedsQL in 22 patients with MPS between the ages of 8 and 21. With regard to assessing the validity of the PedsQL for MPS, we evaluated internal consistency using Cronbach\u2019s \u03b1 coefficient and reliability using the Spearman\u2013Brown estimate of agreement. The mean HRQOL score in our sample was 63.6 points. The worst score was obtained in the Psychosocial domain (61.9) on account of interference with school (56.1), while the Physical Health domain had the highest score (67.6). The total PedsQL internal consistency was .764 points. The Physical Health domain obtained the highest internal consistency (.914), whereas the Psychosocial Health domain obtained the lowest one (.754). MPS was demonstrated to decrease HRQOL, and PedsQL seems to be a valid instrument to perform this kind of analysis.<\/jats:p>","DOI":"10.1177\/1367493518787319","type":"journal-article","created":{"date-parts":[[2018,7,18]],"date-time":"2018-07-18T21:22:47Z","timestamp":1531948967000},"page":"278-285","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":7,"title":["Quality of life evaluation in patients with mucopolysaccharidosis using PedsQL"],"prefix":"10.1177","volume":"23","author":[{"given":"Marcos Almeida","family":"Matos","sequence":"first","affiliation":[{"name":"Bahiana School of Medicine and Public Health, Salvador, Bahia, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F\u00e1bio","family":"Ferri-de-Barros","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery, University of Calgary, Calgary, Alberta, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberto","family":"Guarniero","sequence":"additional","affiliation":[{"name":"University of S\u00e3o Paulo, S\u00e3o Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2018,7,18]]},"reference":[{"key":"bibr1-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1007\/s00038-012-0416-3"},{"key":"bibr2-1367493518787319","first-page":"1","volume":"4","author":"Guarany NR","year":"2016","journal-title":"Journal of Inborn Errors of Metabolism and Screening"},{"key":"bibr3-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0259-0"},{"key":"bibr4-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1590\/S0021-75572008000400005"},{"key":"bibr5-1367493518787319","doi-asserted-by":"publisher","DOI":"10.2307\/2529310"},{"key":"bibr6-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1177\/0009922815586051"},{"key":"bibr7-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ker397"},{"key":"bibr8-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1007\/s10897-014-9791-7"},{"key":"bibr9-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-101"},{"key":"bibr10-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgmr.2016.03.005"},{"key":"bibr11-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1023\/A:1015291021312"},{"key":"bibr12-1367493518787319","first-page":"245","volume":"8","author":"Vairo F","year":"2015","journal-title":"The Application of Clinical Genetics"},{"key":"bibr13-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.10428"},{"key":"bibr14-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1007\/s11136-014-0703-y"},{"key":"bibr15-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1007\/s11136-012-0196-5"},{"key":"bibr16-1367493518787319","doi-asserted-by":"publisher","DOI":"10.1517\/17425247.2015.966682"}],"container-title":["Journal of Child Health Care"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/1367493518787319","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/1367493518787319","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/1367493518787319","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T14:58:51Z","timestamp":1777388331000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.sagepub.com\/doi\/10.1177\/1367493518787319"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,7,18]]},"references-count":16,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,6]]}},"alternative-id":["10.1177\/1367493518787319"],"URL":"https:\/\/doi.org\/10.1177\/1367493518787319","relation":{},"ISSN":["1367-4935","1741-2889"],"issn-type":[{"value":"1367-4935","type":"print"},{"value":"1741-2889","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,7,18]]}}}