Journal article

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

Jenny Downs, Ian Torode, Kingsley Wong, Carolyn Ellaway, Elizabeth J Elliott, Maree T Izatt, Geoffrey N Askin, Bruce I Mcphee, Peter Cundy, Helen Leonard

DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY | WILEY-BLACKWELL | Published : 2016

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°) befo..

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Grants

Awarded by National Institutes of Health


Awarded by National Health and Medical Research Council (NHMRC)


Awarded by EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH &HUMAN DEVELOPMENT


Funding Acknowledgements

We express our special appreciation to all the families and carers of females with Rett syndrome who have contributed to the Australian Rett Syndrome Database. We thank the Australian Paediatric Surveillance Unit (APSU) for collaboration in case ascertainment, and the paediatricians and health professionals who were specifically involved. We also thank Bill Callaghan and the Rett Syndrome Association of Australia for their important contribution to case ascertainment over the years. The Australian Rett syndrome research programme has previously been funded by the National Institutes of Health (5R01HD043100-05) and the National Health and Medical Research Council (NHMRC) project grants #303189, and #1004384 and an NHMRC programme grant #572742. Professor Helen Leonard's funding (2009-2014) was from an NHMRC Senior Research Fellowship #572568. Professor Elizabeth J Elliott is supported by an NHMRC Practitioner Fellowship #457084. The funding bodies for this study have not been involved in study design, data collection, data analysis, manuscript preparation, and/or publication decisions. The authors have stated that they had no interests that might be perceived as posing a conflict or bias.