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PEDIATRICS Vol. 108 No. 5 November 2001, p. e88

ELECTRONIC ARTICLE:
The Influence of Environmental and Genetic Factors on Behavior Problems and Autistic Symptoms in Boys and Girls With Fragile X Syndrome

Received Mar 9, 2001; accepted Jun 18, 2001.

David Hessl*, Jennifer Dyer-Friedman*, Bronwyn Glaser*, Jacob WisbeckDagger , R. Gabriela Barajas*, Annette Taylor§, and Allan L. Reiss*

From the * Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Stanford, California; Dagger  Temple University School of Medicine, Philadelphia, Pennsylvania; and § Kimball Genetics, Inc, Denver, Colorado.

Objective.  Fragile X syndrome, caused by mutations in a single gene of the X chromosome (FMR1), is associated with neurobehavioral characteristics including social deficits with peers, social withdrawal, gaze aversion, inattention, hyperactivity, anxiety, depression, and autistic behavior. However, there is considerable variability in the behavioral and psychiatric problems among children with this condition. The purpose of this study was to measure genetic and environmental factors influencing behavior problems and autistic symptoms in children with fragile X syndrome.

Design.  We conducted an in-home evaluation of 120 children (80 boys and 40 girls) with the fragile X full mutation and their unaffected siblings, including measurements of the FMR1 protein (FMRP), quality of the home environment, maternal and paternal psychopathology, effectiveness of educational and therapeutic services, and child behavior problems.

Results.  Results of multiple regression analyses showed that for boys with fragile X, effectiveness of educational and therapeutic services and parental psychological problems predicted internalizing and externalizing types of problems, while the quality of the home environment predicted autistic behavior. For girls with fragile X, the results emphasized significant effects of FMRP on behavior, in particular social withdrawal and anxious/depressed behavior.

Conclusions.  These findings are among the first to link FMRP expression to behavior. They also emphasize the significance of home- and school-based environmental variables in the neurobehavioral phenotype and help to lay the foundation for studies designed to identify specific interventions for reducing problem behavior in children with fragile X syndrome.  Key words:  fragile X syndrome, FMR1 protein, home environment, special education, autistic behavior.


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