{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T18:06:49Z","timestamp":1788631609906,"version":"build-2803163510"},"reference-count":45,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,2,22]],"date-time":"2023-02-22T00:00:00Z","timestamp":1677024000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100012012","name":"Dravet Syndrome UK","doi-asserted-by":"publisher","award":["16GLW00"],"award-info":[{"award-number":["16GLW00"]}],"id":[{"id":"10.13039\/100012012","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Dravet syndrome (DS) is a severe developmental and epileptic encephalopathy, leading to reduced health\u2010related quality of life (HRQOL). Prospective outcome data on HRQOL are sparse, and this study investigated long\u2010term predictors of HRQOL in DS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      One hundred thirteen families of\n                      <jats:italic>SCN1A<\/jats:italic>\n                      \u2010positive patients with DS, who were recruited as part of our 2010 study were contacted at 10\u2010year follow\u2010up, of which 68 (60%) responded. The mortality was 5.8%. Detailed clinical and demographic information was available for each patient. HRQOL was evaluated with two epilepsy\u2010specific instruments, the Impact of Pediatric Epilepsy Scale (IPES) and the Epilepsy &amp; Learning Disabilities Quality of Life Questionnaire (ELDQOL); a generic HRQOL instrument, the Pediatric Quality of Life Inventory (PedsQL); and a behavioral screening tool, the Strength and Difficulties Questionnaire (SDQ).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Twenty\u2010eight patients were 10\u201315\u2009years of age (0\u20135\u2009years at baseline) and 40 were \u226516\u2009years of age (\u22656\u2009years at baseline). Patients 0\u2010 to 5\u2013years\u2010old at baseline showed a significant decline in mean scores on the PedsQL total score (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u2009.004), physical score (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0&lt;\u2009.001), cognitive score (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u2009.011), social score (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u2009.003), and eating score (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u2009.030) at follow\u2010up. On multivariate regression, lower baseline and follow\u2010up HRQOL for the whole cohort were associated with worse epilepsy severity and a high SDQ total score (\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u00a0=\u200933% and 18%, respectively). In the younger patient group, younger age at first seizure and increased severity of epilepsy were associated with a lower baseline HRQOL (\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u00a0=\u200935%). In the older age group, worse epilepsy severity (\n                      <jats:italic>F<\/jats:italic>\n                      \u00a0=\u20096.40,\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u2009.016,\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u00a0=\u200914%) and the use of sodium\u2010channel blockers were independently associated with a lower HRQOL at 10\u2010year follow\u2010up (\n                      <jats:italic>F<\/jats:italic>\n                      \u00a0=\u20094.13,\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u2009.05,\n                      <jats:italic>R<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u00a0=\u20098%).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Significance<\/jats:title>\n                    <jats:p>This 10\u2010year, prospective follow\u2010up study highlights the significant HRQOL\u2010associated cognitive, social, and physical decline particularly affecting younger patients with DS. Sodium channel blocker use appears to negatively impact long\u2010term HRQOL, highlighting the importance of early diagnosis and disease\u2010specific management in DS.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/epi.17531","type":"journal-article","created":{"date-parts":[[2023,2,5]],"date-time":"2023-02-05T22:04:20Z","timestamp":1675634660000},"page":"1012-1020","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Comorbidities and predictors of health\u2010related quality of life in Dravet syndrome: A 10\u2010year, prospective follow\u2010up study"],"prefix":"10.1111","volume":"64","author":[{"given":"Phoebe","family":"Makiello","sequence":"first","affiliation":[{"name":"The Paediatric Neurosciences Research Group Royal Hospital for Children  Glasgow UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tony","family":"Feng","sequence":"additional","affiliation":[{"name":"The Paediatric 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