Family environment and cognitive abilities in girls with fragile‐X syndrome

Kuo, Ashley; Reiss, Allan L.; Freund, Lisa S.; Huffman, Lynne C.

doi:10.1046/j.1365-2788.2002.00396.x

Cited by 13 publications

(8 citation statements)

References 29 publications

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“…Further studies indeed support the significance of environmental factors in FXS (Dyer-Friedman et al, 2002 ; Kuo et al, 2002 ; Glaser et al, 2003 ): in particular maternal warmth and responsivity were demonstrated to ameliorate maladaptive as well as autistic behaviors, whereas maternal depression and criticism were indicated to increase FXS symptoms in children (Greenberg et al, 2012 ; Robinson et al, 2016 ; Smith et al, 2016 ). Contrary to cognitive deficits, autistic behaviors display no correlation with residual FMRP expression (Glaser et al, 2003 ; Pretto et al, 2014 ).…”

Section: The Fragile X Syndrome — Of Menmentioning

confidence: 90%

Of Men and Mice: Modeling the Fragile X Syndrome

Dahlhaus

2018

Front. Mol. Neurosci.

112

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The Fragile X Syndrome (FXS) is one of the most common forms of inherited intellectual disability in all human societies. Caused by the transcriptional silencing of a single gene, the fragile x mental retardation gene FMR1, FXS is characterized by a variety of symptoms, which range from mental disabilities to autism and epilepsy. More than 20 years ago, a first animal model was described, the Fmr1 knock-out mouse. Several other models have been developed since then, including conditional knock-out mice, knock-out rats, a zebrafish and a drosophila model. Using these model systems, various targets for potential pharmaceutical treatments have been identified and many treatments have been shown to be efficient in preclinical studies. However, all attempts to turn these findings into a therapy for patients have failed thus far. In this review, I will discuss underlying difficulties and address potential alternatives for our future research.

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Section: The Fragile X Syndrome — Of Menmentioning

confidence: 90%

Of Men and Mice: Modeling the Fragile X Syndrome

Dahlhaus

2018

Front. Mol. Neurosci.

112

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“…1987; Howell et al . 2007), and other disorders such as Down's syndrome (Pueschelo & Myers 1994) and fragile-X syndrome (Kuo et al . 2002).…”

Section: Methodsmentioning

confidence: 99%

“…The FES-R is composed of 10 sub-scales: Cohesion (alpha coefficient a = 0.78), Expressiveness (a = 0.69), Conflict (a = 0.75), Independence (a = 0.61), Achievement orientation (a = 0.64), Intellectual/Cultural orientation (a = 0.78), Active-Recreational orientation (a = 0.67), Moral-Religious emphasis (a = 0.78), Organisation (a = 0.76) and Control (a = 0.67). This scale has been broadly used in clinical and research settings as an evaluative measure of a family's dynamic social environment, including research on congenital abnormalities such as cystic fibrosis (Breslau 1983), intellectual disabilities (Blacher et al 1987;Howell et al 2007), and other disorders such as Down's syndrome (Pueschelo & Myers 1994) and fragile-X syndrome (Kuo et al 2002).…”

Section: Environmental Characteristicsmentioning

confidence: 99%

Association of the family environment with behavioural and cognitive outcomes in children with chromosome 22q11.2 deletion syndrome

Allen

Hersh

Schoch

et al. 2013

J intellect Disabil Res

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Background Children with 22q11.2 deletion syndrome (22q11DS) are at risk for social-behavioural and neurocognitive sequelae throughout development. The current study examined the impact of family environmental characteristics on social-behavioural and cognitive outcomes in this pediatric population. Method Guardians of children with 22q11DS were recruited through two medical genetics clinics. Con senting guardians were asked to complete several questionnaires regarding their child's social, emotional and behavioural functioning, as well as family social environment and parenting styles. Children with 22q11DS were asked to undergo a cognitive assessment, including IQ and achievement testing, and measures of attention, executive function and memory. Results Modest associations were found between aspects of the family social environment and parenting styles with social-behavioural and cognitive/academic outcomes. Regression models indicated that physical punishment, socioeconomic status, parental control and family organisation significantly predicted social-behavioural and cognitive outcomes in children with 22q11DS. Conclusion Characteristics of the family social environment and parenting approaches appear to be associated with functional outcomes of children with 22q11DS. Understanding the impact of environmental variables on developmental outcomes can be useful in determining more effective targets for intervention. This will be important in order to improve the quality of life of individuals affected by 22q11DS.

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“…Although family types such as cohesive, control-oriented, and child-oriented were replicated by these investigators, other research has not adopted this model or extended it to determine long-term effects. Others have used the FES or one of its subscales to study differences between families with DS and other disabilities (Seltzer, Krauss & Tsunematsu, 1993); the prediction of unmet service need (Smith, 1997); cognitive ability in girls with fragile-X (Kuo, Reiss, Freund & Huffman, 2002); and how conflict and cohesion relates to depression in Latina mothers rearing children with or without DD .…”

Section: The Fesmentioning

confidence: 99%