title: Association of the family environment with behavioural and cognitive outcomes in children with chromosome 22q11.2 deletion syndrome.
abstract: 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 paediatric population.
  
  
  METHODS
  Guardians of children with 22q11DS were recruited through two medical genetics clinics. Consenting 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Psychology & Neuroscience, Duke University, Durham, NC, USA.
authors.forename: T M
authors.initials: TM
authors.lastname: Allen
authors.forename: J
authors.initials: J
authors.lastname: Hersh
authors.forename: K
authors.initials: K
authors.lastname: Schoch
authors.forename: K
authors.initials: K
authors.lastname: Curtiss
authors.forename: S R
authors.initials: SR
authors.lastname: Hooper
authors.forename: V
authors.initials: V
authors.lastname: Shashi
mesh_terms: D000293:Adolescent; D002648:Child; D002652:Child Behavior; D003071:Cognition / Q000502:physiology*; D004062:DiGeorge Syndrome / Q000523:psychology* / Q000534:rehabilitation; D005190:Family / Q000523:psychology*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000523:psychology / Q000534:rehabilitation; D008297:Male; D016487:Parenting / Q000523:psychology; D010290:Parents / Q000523:psychology; D011237:Predictive Value of Tests; D012044:Regression Analysis; D012919:Social Behavior
keywords: DiGeorge syndrome; chromosome 22q11.2 deletion syndrome; family environment; parenting; social-behavioural functioning; velocardiofacial syndrome