Cited by 100Genetics and Neurodevelopmental DisordersDown syndrome and intellectual disability researchAutism Spectrum Disorder Research

Genetic and Environmental Influences on the Cognitive Outcomes of Children With Fragile X Syndrome

DYER-FRIEDMAN, JENNIFER, GLASER, BRONWYN, HESSL, DAVID, JOHNSTON, CINDY, HUFFMAN, LYNNE C., TAYLOR, ANNETTE, WISBECK, JACOB, REISS, ALLAN L.·2002·Journal of the American Academy of Child & Adolescent Psychiatry

Abstract

Objective To measure the genetic and environmental factors influencing the cognitive outcomes in children with fragile X, a common genetic disorder causing cognitive impairments.Method In-home evaluations were conducted on 120 children (80 boys and 40 girls) with the fragile X full mutation and their unaffected siblings.Results Multiple regression analyses show that the cognitive outcomes for girls with fragile X are most strongly predicted by the mean IQ of their parents, with a small proportion of the variance accounted for by the quality of their home environment. FMR1 protein (FMRP) was associated with girls' levels of distractibility. Mean parental IQ was associated only with boys' Performance IQs, while FMRP was associated with boys' Full Scale IQs. The quality of boys' home environments accounted for more of the variance in their cognitive outcomes than it did for affected girls.Conclusions Both biological/genetic factors and environmental factors are significant predictors of IQ in children with fragile X syndrome; however, the influence of specific factors differs between girls and boys. These findings lay the foundation for further investigation into biological and environmental interventions.

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Crossref · 10.1097/00004583-200203000-00002

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19 of 19 references verified0 citation errors · 0 not checkable

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  • Cognitive and behavioral therapies combined with educational stimuli are beneficial for developing self-care and social and adaptive behaviors in patients with FXS.

    “cognitive and behavioral therapies in patients, together with educational stimuli, have been beneficial in facilitating the development of self-care and social and adaptive behaviors”
  • These therapies enhance the quality of family life.

    “all of which greatly enhance the quality of family life within the given contexts (131-135).”

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Authors
DYER-FRIEDMAN, J., GLASER, B., HESSL, D., JOHNSTON, C., HUFFMAN, L. C., TAYLOR, A., WISBECK, J., & REISS, A. L.
Affiliations
  • Stanford Medicine (US)
  • Children's Health Council (US)
Corresponding author
DYER-FRIEDMAN, JENNIFER
Venue
Journal of the American Academy of Child & Adolescent Psychiatry
Publisher
Elsevier BV
Published
2002-03
Volume
41
Issue
3
Pages
237-244
ISSN
0890-8567 (ISSN-L)
OpenAlex
W2087858364
Cited by
100
References
19
Citations by year
2025: 2 · 2024: 2 · 2023: 1 · 2022: 4 · 2021: 3 · 2020: 3 · 2019: 6 · 2018: 9 · 2017: 4 · 2016: 5 · 2015: 4 · 2014: 9 · 2013: 3 · 2012: 6
FWCI
2.31
Type
journal article
Language
en
Open access
closed
Locations
  • Journal of the American Academy of Child & Adolescent Psychiatry (publishedVersion)
  • PubMed (publishedVersion)
Funders
NIH (grants MHO1142 and MH50047)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
  • Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
Keywords
fragile X syndrome, FMR1 full mutation, cognitive outcomes, IQ, family environment, FMRP, distractibility, sex differences, genetic and environmental factors, children
MeSH terms
Child, Cognition Disorders, Environment, Female, Fragile X Syndrome, Humans, Male, Neuropsychological Tests, Genetic Predisposition to Disease

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