Curtin University Homepage
  • Library
  • Help
    • Admin

    espace - Curtin’s institutional repository

    JavaScript is disabled for your browser. Some features of this site may not work without it.
    View Item 
    • espace Home
    • espace
    • Curtin Research Publications
    • View Item
    • espace Home
    • espace
    • Curtin Research Publications
    • View Item

    Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study

    237188_237188.pdf (723.0Kb)
    Access Status
    Open access
    Authors
    Downs, Jennepher
    Torode, I.
    Wong, K.
    Ellaway, C.
    Elliott, E.
    Izatt, M.
    Askin, G.
    Mcphee, B.
    Cundy, P.
    Leonard, H.
    Date
    2016
    Type
    Journal Article
    
    Metadata
    Show full item record
    Citation
    Downs, J. and Torode, I. and Wong, K. and Ellaway, C. and Elliott, E. and Izatt, M. and Askin, G. et al. 2016. Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study. Developmental Medicine and Child Neurology. 58 (6): pp. 632-638.
    Source Title
    Developmental Medicine and Child Neurology
    DOI
    10.1111/dmcn.12984
    ISSN
    0012-1622
    School
    School of Physiotherapy and Exercise Science
    Remarks

    This is the peer reviewed version of the following article: Downs, J. and Torode, I. and Wong, K. and Ellaway, C. and Elliott, E. and Izatt, M. and Askin, G. et al. 2016. Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study. Developmental Medicine and Child Neurology. 58 (6): pp. 632-638, which has been published in final form at http://doi.org/10.1111/dmcn.12984. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving at http://olabout.wiley.com/WileyCDA/Section/id-820227.html#terms

    URI
    http://hdl.handle.net/20.500.11937/43563
    Collection
    • Curtin Research Publications
    Abstract

    Aim: 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. Method: Data were ascertained from hospital medical records, the Australian Rett Syndrome Database, a longitudinal and population-based registry, and from the Australian Institute of Health and Welfare National Death Index database. Cox regression and generalized estimating equation models 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 =45°) before adulthood. Results: After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group (hazard ratio [HR] 0.30, 95% confidence interval [CI] 0.12-0.74; p=0.009) compared to those without surgery. Rate of death was particularly reduced for those with early onset scoliosis (HR 0.17, 95% CI 0.06-0.52; p=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% CI 0.16-1.03; p=0.06). Interpretation: With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.

    Related items

    Showing items related by title, author, creator and subject.

    • Spinal fusion in girls with Rett syndrome: postoperative recovery and family experiences
      Marr, C.; Leonard, H.; Torode, I.; Downs, Jennepher (2015)
      Background: Rett syndrome is a severe neurodevelopmental disorder mainly affecting females and scoliosis is a common co-morbidity. Spinal fusion may be recommended if the scoliosis is progressive. This qualitative study ...
    • The Natural History of Scoliosis in Females with Rett Syndrome
      Downs, Jennepher; Torode, I.; Wong, K.; Ellaway, C.; Elliott, E.; Christodoulou, J.; Jacoby, P.; Thomson, M.; Izatt, M.; Askin, G.; McPhee, B.; Bridge, C.; Cundy, P.; Leonard, H. (2015)
      STUDY DESIGN.: Population-based longitudinal observational study. OBJECTIVES.: To describe the prevalence of scoliosis in Rett syndrome, structural characteristics and progression, taking into account the influences of ...
    • How can clinical ethics guide the management of comorbidities in the child with Rett syndrome?
      Downs, Jennepher; Forbes, D.; Johnson, M.; Leonard, H. (2016)
      Rett syndrome is a rare disorder caused by a mutation in the MECP2 gene. Those affected generally have severe functional impairments, and medical comorbidities such as scoliosis and poor growth are common. There is a ...
    Advanced search

    Browse

    Communities & CollectionsIssue DateAuthorTitleSubjectDocument TypeThis CollectionIssue DateAuthorTitleSubjectDocument Type

    My Account

    Admin

    Statistics

    Most Popular ItemsStatistics by CountryMost Popular Authors

    Follow Curtin

    • 
    • 
    • 
    • 
    • 

    CRICOS Provider Code: 00301JABN: 99 143 842 569TEQSA: PRV12158

    Copyright | Disclaimer | Privacy statement | Accessibility

    Curtin would like to pay respect to the Aboriginal and Torres Strait Islander members of our community by acknowledging the traditional owners of the land on which the Perth campus is located, the Whadjuk people of the Nyungar Nation; and on our Kalgoorlie campus, the Wongutha people of the North-Eastern Goldfields.