{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T04:02:16Z","timestamp":1791259336105,"version":"4.1.0"},"reference-count":26,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2014,11,14]],"date-time":"2014-11-14T00:00:00Z","timestamp":1415923200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Journal of Genetic Counseling"],"published-print":{"date-parts":[[2015,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a chronic and progressive X\u2010linked lysosomal disease that mainly affects males. The National MPS Society (\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"#CR14\">2013<\/jats:ext-link>\n                    ) reports that MPS II affects 1 in 100,000 to 1 in 150,000 males worldwide. Two distinct forms of the disease are based on age of onset and clinical course: attenuated and severe. MPS II affects many organ systems including the nervous, cardiovascular, gastrointestinal and respiratory systems. Clinical manifestations can include progressive hearing loss, mental impairment, and enlarged liver and spleen. This study focuses on the health\u2010related quality of life of individuals (HRQOL) with MPS II as measured by the parent and self\u2010report versions of the Pediatric Quality of Life Inventory (PedsQL\u2122). Both parents of patients with MPS II as well as patients themselves reported lower scores on all domains of the PedsQL\u2122 (physical, emotional, social and school functioning) indicating that children with MPS II have an overall lower HRQOL when compared to a healthy sample. When compared with patients with other chronic illnesses (cancer, MSUD, galactosemia,), the MPS II sample had significantly lower scores on a number of PedsQL\u2122 scales, suggesting an overall lower HRQOL. No significant relationships were found using scores from parent or self report PedsQL\u2122 measures and length of time on ERT.\n                  <\/jats:p>","DOI":"10.1007\/s10897-014-9791-7","type":"journal-article","created":{"date-parts":[[2014,11,14]],"date-time":"2014-11-14T10:03:51Z","timestamp":1415959431000},"page":"635-644","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Health\u2010Related Quality of Life in Patients with MPS II"],"prefix":"10.1002","volume":"24","author":[{"given":"Mary","family":"Needham","sequence":"first","affiliation":[{"name":"Pacific Graduate School of Psychology at Palo Alto University University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy","family":"Packman","sequence":"additional","affiliation":[{"name":"Pacific Graduate School of Psychology at Palo Alto University University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasha","family":"Quinn","sequence":"additional","affiliation":[{"name":"Pacific Graduate School of Psychology at Palo Alto University University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxwell","family":"Rappoport","sequence":"additional","affiliation":[{"name":"Pacific Graduate School of Psychology at Palo Alto University University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christa","family":"Aoki","sequence":"additional","affiliation":[{"name":"Pacific Graduate School of Psychology at Palo Alto University University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Bostrom","sequence":"additional","affiliation":[{"name":"University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Cordova","sequence":"additional","affiliation":[{"name":"Pacific Graduate School of Psychology at Palo Alto University University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Macias","sequence":"additional","affiliation":[{"name":"Pacific Graduate School of Psychology at Palo Alto University University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia","family":"Morgan","sequence":"additional","affiliation":[{"name":"University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seymour","family":"Packman","sequence":"additional","affiliation":[{"name":"University of California San Francisco USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,11,14]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.73.1.77"},{"key":"e_1_2_10_3_1","first-page":"317","volume-title":"Handbook of neurodevelopmental and genetic disorders in children","author":"Brown M. 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RaffM.2008.Quality\u2010of\u2010life changes in MPS IIpatients receiving enzyme replace\u2010ment therapy.Poster presentation at Lysosomal Disease Network (WORLD) Symposium."},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2007-1350"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/1469-7610.00615"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-011-1606-3"},{"key":"e_1_2_10_15_1","unstructured":"National MPS Society. (2013).MPS II (Hunter syndrome).Retrieved from:http:\/\/www.mpssociety.org\/mps\/mps\u2010ii\/."},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10897-013-9665-4"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-101"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2007.00212.x"},{"key":"e_1_2_10_19_1","volume-title":"Vineland adaptive behavior scales (2nd ed.): survey forms manual","author":"Sparrow S. 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