{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T01:23:17Z","timestamp":1774401797211,"version":"3.50.1"},"reference-count":25,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,12,11]],"date-time":"2015-12-11T00:00:00Z","timestamp":1449792000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["5R01HD043100\u201005"],"award-info":[{"award-number":["5R01HD043100\u201005"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["#303189"],"award-info":[{"award-number":["#303189"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["#1004384"],"award-info":[{"award-number":["#1004384"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["#572742"],"award-info":[{"award-number":["#572742"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2016,6]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Scoliosis is a common comorbidity in Rett syndrome and spinal fusion may be recommended if severe. We investigated the impact of spinal fusion on survival and risk of severe lower respiratory tract infection in Rett syndrome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>Data were ascertained from hospital medical records, the Australian Rett Syndrome Database, a longitudinal and population\u2010based registry, and from the Australian Institute of Health and Welfare National Death Index database. Cox regression and generalized estimating equation\u00a0models were used to estimate the effects of spinal surgery on survival and severe respiratory infection respectively in 140 females who developed severe scoliosis (Cobb angle \u226545\u00b0) before adulthood.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group (hazard ratio [<jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>] 0.30, 95% confidence interval [<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>] 0.12\u20130.74; <jats:italic>p<\/jats:italic>=0.009) compared to those without surgery. Rate of death was particularly reduced for those with early onset scoliosis (<jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content> 0.17, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 0.06\u20130.52; <jats:italic>p<\/jats:italic>=0.002). There was some evidence to suggest that spinal fusion was associated with a reduction in risk of severe respiratory infection among those with early onset scoliosis (risk ratio 0.41, 95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 0.16\u20131.03; <jats:italic>p<\/jats:italic>=0.06).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.12984","type":"journal-article","created":{"date-parts":[[2015,12,11]],"date-time":"2015-12-11T06:02:56Z","timestamp":1449813776000},"page":"632-638","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study"],"prefix":"10.1111","volume":"58","author":[{"given":"Jenny","family":"Downs","sequence":"first","affiliation":[{"name":"Telethon Kids Institute The University of Western Australia Perth WA Australia"},{"name":"School of Physiotherapy and Exercise Science Curtin University Perth WA Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian","family":"Torode","sequence":"additional","affiliation":[{"name":"Department of Orthopaedics Royal Children's Hospital Melbourne Vic. Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kingsley","family":"Wong","sequence":"additional","affiliation":[{"name":"Telethon Kids Institute The University of Western Australia Perth WA Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolyn","family":"Ellaway","sequence":"additional","affiliation":[{"name":"Discipline of Genetic Medicine The University of Sydney Sydney NSW Australia"},{"name":"Discipline of Paediatrics and Child Health The Children's Hospital at Westmead The University of Sydney Sydney NSW Australia"},{"name":"The Sydney Children's Hospitals Network (Westmead) Sydney NSW Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth J","family":"Elliott","sequence":"additional","affiliation":[{"name":"Discipline of Paediatrics and Child Health The Children's Hospital at Westmead The University of Sydney Sydney NSW Australia"},{"name":"The Sydney Children's Hospitals Network (Westmead) Sydney NSW Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maree T","family":"Izatt","sequence":"additional","affiliation":[{"name":"Paediatric Spine Research Group Lady Cilento Children's Hospital Queensland University of Technology Brisbane Qld Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Geoffrey N","family":"Askin","sequence":"additional","affiliation":[{"name":"Paediatric Spine Research Group Lady Cilento Children's Hospital Queensland University of Technology Brisbane Qld Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruce I","family":"Mcphee","sequence":"additional","affiliation":[{"name":"Department of Surgery University of Queensland Brisbane Qld Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Cundy","sequence":"additional","affiliation":[{"name":"Discipline of Orthopaedics and Trauma University of Adelaide Adelaide SA Australia"},{"name":"Department of Orthopaedic Surgery Women's and Children's Hospital Adelaide SA Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helen","family":"Leonard","sequence":"additional","affiliation":[{"name":"Telethon Kids Institute The University of Western Australia Perth WA Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"the Rett syndrome spinal fusion group","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,12,11]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22124"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/08830738060210091501"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/BRS.0b013e3181a95ca4"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.12093"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1177\/08830738060210100101"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/BRS.0b013e3182404698"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00586-008-0876-6"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/cch.12243"},{"key":"e_1_2_9_11_1","first-page":"1","article-title":"Family satisfaction following spinal fusion in Rett syndrome","volume":"24","author":"Downs J","year":"2015","journal-title":"Dev Neurorehabil"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2007-1641"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/jsr.12240"},{"key":"e_1_2_9_14_1","volume-title":"Outline for the Study of Scoliosis","author":"Cobb JR","year":"1948"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1467-842X.2000.tb00143.x"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.2106\/JBJS.F.00754"},{"key":"e_1_2_9_17_1","volume-title":"Australian Standard Geographical Classification (ASGC)","author":"Australian Bureau of Statistics","year":"2011"},{"key":"e_1_2_9_18_1","first-page":"168","volume-title":"Measuring interactions. Epidemiology: An Introduction","author":"Rothman KJ","year":"2002"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-9-87"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000304752.50773.ec"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/BRS.0b013e3181453073"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/BRS.0000000000000365"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1097\/BRS.0b013e3181f3a326"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00586-012-2542-2"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200006223422507"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1093\/ije\/dyr218"}],"container-title":["Developmental Medicine &amp; Child Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fdmcn.12984","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/dmcn.12984","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T03:39:19Z","timestamp":1696304359000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/dmcn.12984"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,12,11]]},"references-count":25,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2016,6]]}},"alternative-id":["10.1111\/dmcn.12984"],"URL":"https:\/\/doi.org\/10.1111\/dmcn.12984","archive":["Portico"],"relation":{},"ISSN":["0012-1622","1469-8749"],"issn-type":[{"value":"0012-1622","type":"print"},{"value":"1469-8749","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,12,11]]},"assertion":[{"value":"2015-10-05","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2015-12-11","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}