Literature DB >> 11886017

Genetic and environmental influences on the cognitive outcomes of children with fragile X syndrome.

Jennifer Dyer-Friedman1, Bronwyn Glaser, David Hessl, Cindy Johnston, Lynne C Huffman, Annette Taylor, Jacob Wisbeck, Allan L Reiss.   

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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Mesh:

Year:  2002        PMID: 11886017     DOI: 10.1097/00004583-200203000-00002

Source DB:  PubMed          Journal:  J Am Acad Child Adolesc Psychiatry        ISSN: 0890-8567            Impact factor:   8.829


  27 in total

1.  Change in the Behavioral Phenotype of Adolescents and Adults with FXS: Role of the Family Environment.

Authors:  Leann E Smith; Jinkuk Hong; Jan S Greenberg; Marsha R Mailick
Journal:  J Autism Dev Disord       Date:  2016-05

2.  Family environment and behavior problems in children, adolescents, and adults with fragile X syndrome.

Authors:  Jan Greenberg; Marsha Seltzer; Jason Baker; Leann Smith; Steven F Warren; Nancy Brady; Jinkuk Hong
Journal:  Am J Intellect Dev Disabil       Date:  2012-07

3.  Cognitive, environmental, and linguistic predictors of syntax in fragile X syndrome and Down syndrome.

Authors:  Bruno Estigarribia; Gary E Martin; Joanne E Roberts
Journal:  J Speech Lang Hear Res       Date:  2012-04-03       Impact factor: 2.297

Review 4.  Fragile X-associated disorders: a clinical overview.

Authors:  Anne Gallagher; Brian Hallahan
Journal:  J Neurol       Date:  2011-07-12       Impact factor: 4.849

5.  Autism spectrum disorder in children and adolescents with fragile X syndrome: within-syndrome differences and age-related changes.

Authors:  Andrea McDuffie; Leonard Abbeduto; Pamela Lewis; Sara Kover; Jee-Seon Kim; Ann Weber; W Ted Brown
Journal:  Am J Intellect Dev Disabil       Date:  2010-07

6.  Fronto-striatal dysfunction and potential compensatory mechanisms in male adolescents with fragile X syndrome.

Authors:  Fumiko Hoeft; Arvel Hernandez; Sudharshan Parthasarathy; Christa L Watson; Scott S Hall; Allan L Reiss
Journal:  Hum Brain Mapp       Date:  2007-06       Impact factor: 5.038

7.  Trajectories and predictors of the development of very young boys with fragile X syndrome.

Authors:  Jane E Roberts; Jean B Mankowski; John Sideris; Barbara Davis Goldman; Deborah D Hatton; Penny L Mirrett; Grace T Baranek; J Steven Reznick; Anna C J Long; Donald B Bailey
Journal:  J Pediatr Psychol       Date:  2008-12-12

Review 8.  Fragile X syndrome.

Authors:  Wilmar Saldarriaga; Flora Tassone; Laura Yuriko González-Teshima; Jose Vicente Forero-Forero; Sebastián Ayala-Zapata; Randi Hagerman
Journal:  Colomb Med (Cali)       Date:  2014-12-30

9.  Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics.

Authors:  Claudia Bagni; Flora Tassone; Giovanni Neri; Randi Hagerman
Journal:  J Clin Invest       Date:  2012-12-03       Impact factor: 14.808

10.  A solution to limitations of cognitive testing in children with intellectual disabilities: the case of fragile X syndrome.

Authors:  David Hessl; Danh V Nguyen; Cherie Green; Alyssa Chavez; Flora Tassone; Randi J Hagerman; Damla Senturk; Andrea Schneider; Amy Lightbody; Allan L Reiss; Scott Hall
Journal:  J Neurodev Disord       Date:  2009-03       Impact factor: 4.025

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