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Journal of Pediatric Psychology Advance Access first published online on December 12, 2008
This version published online on April 2, 2009

Journal of Pediatric Psychology, doi:10.1093/jpepsy/jsn129
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© The Author 2008. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oxfordjournals.org

Trajectories and Predictors of the Development of Very Young Boys with Fragile X Syndrome

Jane E. Roberts, PhD1, Jean B. Mankowski, PhD1, John Sideris, PhD1, Barbara Davis Goldman, PhD1,2, Deborah D. Hatton, PhD1, Penny L. Mirrett, PhD1, Grace T. Baranek, PhD3, J. Steven Reznick, PhD2, Anna C. J. Long, BA1 and Donald B. Bailey, Jr, PhD4

1FPG Child Development Institute, The University of North Carolina at Chapel Hill, 2Department of Psychology, The University of North Carolina at Chapel Hill, 3Division of Occupational Science, Department of Allied Health Sciences, The University of North Carolina at Chapel Hill, and 4RTI International

All correspondence concerning this article should be addressed to Jane E. Roberts, PhD, Department of Psychology, University of South Carolina, Barnwell College, Columbia, SC 29208, USA. E-mail: jane.roberts{at}sc.edu


   Abstract

Objective To describe the development of young boys with fragile X syndrome (FXS). Methods Fifty-five boys (aged 8–48 months at study entry) with the full mutation FXS received multiple developmental assessments. Results As expected, the boys’ rate of development was significantly lower than chronological age expectations. No evidence of slowing in the rate of development was found. Autistic behavior was negatively associated with development, but maternal IQ was not. Developmental delays were evident in some domains as early as 9 months; however, initial detection of delays is complicated by measures and criteria used. Developmental age scores at 31 months of age were related to scores obtained at 61 months of age only in the global composite and visual reception domain. Conclusions Developmental delays are evident in some infants with FXS as young as 9 months of age. Pediatric psychologists need to be informed about the developmental profiles in young children with FXS to accurately diagnose, treat, and support these children and their families.

Key words: cognitive development; fmr1; fragile X.

Received June 6, 2008; revision received November 11, 2008; accepted November 11, 2008


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