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

被引:65
作者
Dyer-Friedman, J
Glaser, B
Hessl, D
Johnston, C
Huffman, LC
Taylor, A
Wisbeck, J
Reiss, AL
机构
[1] Stanford Univ, Sch Med, Child Div, Dept Psychiat, Stanford, CA 94305 USA
[2] Stanford Univ, Dept Pediat, Stanford, CA 94305 USA
[3] Childrens Hlth Council, Palo Alto, CA USA
[4] Kimball Genet Inc, Denver, CO USA
[5] Stanford Univ, Dept Psychiat, Div Child & Adolescent Psychiat, Stanford, CA 94305 USA
关键词
fragile X syndrome; cognitive phenotype; home environment; special education; neurobehavioral disorders;
D O I
10.1097/00004583-200203000-00002
中图分类号
B844 [发展心理学(人类心理学)];
学科分类号
040202 ;
摘要
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 10 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.
引用
收藏
页码:237 / 244
页数:8
相关论文
共 28 条
  • [1] BAUMGARDNER TL, 1995, PEDIATRICS, V95, P744
  • [2] BENNETTO L, 1996, FRAGILE X SYNDROME D, P210
  • [3] Behavior and autonomic nervous system function assessed via heart period measures: The case of hyperarousal in boys with fragile X syndrome
    Boccia, ML
    Roberts, JE
    [J]. BEHAVIOR RESEARCH METHODS INSTRUMENTS & COMPUTERS, 2000, 32 (01): : 5 - 10
  • [4] BRADLEY RH, 1993, GENET SOC GEN PSYCH, V119, P439
  • [5] Caldwell B.M., 1984, Home observation for measurement of the environment: Administration manual
  • [6] Cohen IL, 1995, Ment Retard Dev Disabil Res Rev, V1, P286
  • [7] DEFRIES JC, 1979, BEHAV GENET, V9, P23
  • [8] DYERFRIEDMAN J, 2001, 14 ANN RES C SYST CA
  • [9] Trajectories and profiles of adaptive behavior in males with fragile X syndrome: Multicenter studies
    Dykens, E
    Ort, S
    Cohen, I
    Finucane, B
    Spiridigliozzi, G
    Lachiewicz, A
    Reiss, A
    Freund, L
    Hagerman, R
    OConnor, R
    [J]. JOURNAL OF AUTISM AND DEVELOPMENTAL DISORDERS, 1996, 26 (03) : 287 - 301
  • [10] Fisch GS, 1996, AM J MED GENET, V64, P356, DOI 10.1002/(SICI)1096-8628(19960809)64:2<356::AID-AJMG24>3.0.CO