Skip to main content
Log in

Socioeconomic Status and Psychological Function in Children with Chromosome 22q11.2 Deletion Syndrome: Implications for Genetic Counseling

  • Original Research
  • Published:
Journal of Genetic Counseling

Abstract

The purpose of this study is to examine the association between parental socio-economic status (SES) and childhood neurocognition and behavior in children with chromosome 22q11.2 deletion syndrome (22q11DS). Although undoubtedly, the deletion of genes in the 22q11.2 interval is primarily responsible for the psychological manifestations, little is known about the role of the environment in either mitigating or contributing to these problems. We examined the association of parental socio-economic status (SES) with cognition and behavior in children with 22q11DS (n = 65) and matched healthy control subjects (n = 52), since SES is a component of family resources. We found that in children with 22q11DS, higher SES correlated with better overall functioning (p < .01) and social skills (p < .01), and less frequent oppositional defiant behavior (p < .001). These findings were in contrast to the control subjects in whom SES correlated with cognition and achievement, but not behavior. Our results indicate that environmental factors influence the behavioral phenotype in children with 22q11DS, providing a framework for developing appropriate interventions. As such, genetic counseling for families with 22q11DS may include consideration of family resources and inclusion of other health professionals, such as social workers, to explore with the family available social supports and resources.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Achenbach, T. M., & Ruffle, T. M. (2000). The child behavior checklist and related forms for assessing behavioral/emotional problems and competencies. Pediatrics in Review, 21(8), 265–271.

    Article  CAS  PubMed  Google Scholar 

  • Antshel, K. M., Stallone, K., AbdulSabur, N., Shprintzen, R., Roizen, N., Higgins, A. M., et al. (2007). Temperament in velocardiofacial syndrome. Journal of Intellectual Disability Research, 51(Pt 3), 218–227.

    Article  CAS  PubMed  Google Scholar 

  • Arnold, P. D., Siegel-Bartelt, J., Cytrynbaum, C., Teshima, I., & Schachar, R. (2001). Velo-cardio-facial syndrome: Implications of microdeletion 22q11 for schizophrenia and mood disorders. American Journal of Medical Genetics, 105(4), 354–362.

    Article  CAS  PubMed  Google Scholar 

  • Boss, P. (1988). Family stress management. Family Studies Text Series, 8, 27–55.

    Google Scholar 

  • Briegel, W., Schneider, M., & Schwab, K. O. (2008). 22q11.2 deletion syndrome: behaviour problems of children and adolescents and parental stress. Child, Care, Health and Development.

  • Chase-Carmichael, C. A., Ris, M. D., Weber, A. M., & Schefft, B. K. (1999). Neurologic validity of the Wisconsin Card Sorting Test with a pediatric population. The Clinical Neuropsychologist, 13(4), 405–413.

    Article  CAS  PubMed  Google Scholar 

  • Cornblatt, B. A., Risch, N. J., Faris, G., Friedman, D., & Erlenmeyer-Kimling, L. (1988). The Continuous Performance Test, identical pairs version (CPT-IP): I. New findings about sustained attention in normal families. Psychiatry Research, 26(2), 223–238.

    Article  CAS  PubMed  Google Scholar 

  • Delis, D. C., Kramer, J. H., Kaplan, E., & Ober, B. A. (1993). California Verbal Learning Test-Children’s Version (CVLT-C). San Antonio: The Psychological Corporation.

    Google Scholar 

  • Duckworth, A. L., & Seligman, M. E. (2005). Self-discipline outdoes IQ in predicting academic performance of adolescents. Psychological Science, 16(12), 939–944.

    Article  PubMed  Google Scholar 

  • Duckworth, A. L., Peterson, C., Matthews, M. D., & Kelly, D. R. (2007). Grit: perseverance and passion for long-term goals. Journal of Personality and Social Psychology, 92(6), 1087–1101.

    Article  PubMed  Google Scholar 

  • Feinstein, C., Eliez, S., Blasey, C., & Reiss, A. L. (2002). Psychiatric disorders and behavioral problems in children with velocardiofacial syndrome: usefulness as phenotypic indicators of schizophrenia risk. Biological Psychiatry, 51(4), 312–318.

    Article  PubMed  Google Scholar 

  • Fernald, L. C., Gertler, P. J., & Neufeld, L. M. (2008). Role of cash in conditional cash transfer programmes for child health, growth, and development: an analysis of Mexico’s Oportunidades. Lancet, 371(9615), 828–837.

    Article  PubMed  PubMed Central  Google Scholar 

  • Fors, S., Lennartsson, C., & Lundberg, O. (2009). Childhood living conditions, socioeconomic position in adulthood, and cognition in later life: exploring the associations. The Journals of Gerontology. Series B: Psychological Sciences and Social Sciences, 64(6), 750–757.

    Article  Google Scholar 

  • Gerdes, M., Solot, C., Wang, P. P., Moss, E., LaRossa, D., Randall, P., et al. (1999). Cognitive and behavior profile of preschool children with chromosome 22q11.2 deletion. American Journal of Medical Genetics, 85(2), 127–133.

    Article  CAS  PubMed  Google Scholar 

  • Golding-Kushner, K. J., Weller, G., & Shprintzen, R. J. (1985). Velo-cardio-facial syndrome: language and psychological profiles. Journal of Craniofacial Genetics and Developmental Biology, 5(3), 259–266.

    CAS  PubMed  Google Scholar 

  • Goodman, E., McEwen, B. S., Dolan, L. M., Schafer-Kalkhoff, T., & Adler, N. E. (2005). Social disadvantage and adolescent stress. The Journal of Adolescent Health, 37(6), 484–492.

    Article  PubMed  Google Scholar 

  • Graff, J., & Mansuy, I. M. (2008). Epigenetic codes in cognition and behaviour. Behavioural Brain Research, 192(1), 70–87.

    Article  CAS  PubMed  Google Scholar 

  • Gresham, F. M., & Elliot, S. N. (1990). Social skills rating system. Circle Pines: American Guidance Service, Inc.

    Google Scholar 

  • Guo, G., & Harris, K. M. (2000). The mechanisms mediating the effects of poverty on children’s intellectual development. Demography, 37(4), 431–447.

    Article  CAS  PubMed  Google Scholar 

  • Hackman, D. A., & Farah, M. J. (2009). Socioeconomic status and the developing brain. Trends in Cognitive Sciences, 13(2), 65–73.

    Article  PubMed  PubMed Central  Google Scholar 

  • Heineman-de Boer, J. A., Van Haelst, M. J., Cordia-de, H. M., & Beemer, F. A. (1999). Behavior problems and personality aspects of 40 children with velo-cardio-facial syndrome. Genetic Counseling, 10(1), 89–93.

    CAS  PubMed  Google Scholar 

  • Holling, H., Kurth, B. M., Rothenberger, A., Becker, A., & Schlack, R. (2008). Assessing psychopathological problems of children and adolescents from 3 to 17 years in a nationwide representative sample: results of the German health interview and examination survey for children and adolescents (KiGGS). European Child & Adolescent Psychiatry, 17(Suppl 1), 34–41.

    Article  Google Scholar 

  • Hollingshead, A. B. (1975). Four factor index of social status. New Haven: Yale University Department of Sociology.

    Google Scholar 

  • Hollingshead, A. B., & Redlich, F. C. (2007). Social class and mental illness: a community study. 1958. American Journal of Public Health, 97(10), 1756–1757.

    Article  CAS  PubMed  Google Scholar 

  • Jansen, P. W., Duijff, S. N., Beemer, F. A., Vorstman, J. A., Klaassen, P. W., Morcus, M. E., et al. (2007). Behavioral problems in relation to intelligence in children with 22q11.2 deletion syndrome: a matched control study. American Journal of Medical Genetics. Part A, 143(6), 574–580.

    Article  Google Scholar 

  • Kern, R. S., Green, M. F., Nuechterlein, K. H., & Deng, B. H. (2004). NIMH-MATRICS survey on assessment of neurocognition in schizophrenia. Schizophrenia Research, 72(1), 11–19.

    Article  PubMed  Google Scholar 

  • Kushnir, J., & Sadeh, A. (2010). Childhood fears, neurobehavioral functioning and behavior problems in school-age children. Child Psychiatry and Human Development, 41(1), 88–97.

    Article  PubMed  Google Scholar 

  • Landry, S. H., Denson, S. E., & Swank, P. R. (1997). Effects of medical risk and socioeconomic status on the rate of change in cognitive and social development for low birth weight children. Journal of Clinical and Experimental Neuropsychology, 19(2), 261–274.

    Article  CAS  PubMed  Google Scholar 

  • Lantz, P. M., House, J. S., Mero, R. P., & Williams, D. R. (2005). Stress, life events, and socioeconomic disparities in health: results from the Americans’ Changing Lives Study. Journal of Health and Social Behavior, 46(3), 274–288.

    Article  PubMed  Google Scholar 

  • Lemstra, M., Neudorf, C., D’Arcy, C., Kunst, A., Warren, L. M., & Bennett, N. R. (2008). A systematic review of depressed mood and anxiety by SES in youth aged 10–15 years. Canadian Journal of Public Health. Revue Canadienne de Sante Publique, 99(2), 125–129.

    Article  PubMed  PubMed Central  Google Scholar 

  • Lewandowski, K. E., Shashi, V., Berry, P. M., & Kwapil, T. R. (2007). Schizophrenic-like neurocognitive deficits in children and adolescents with 22q11 deletion syndrome. American Journal of Medical Genetics. Part B: Neuropsychiatric Genetics, 144(1), 27–36.

    Article  Google Scholar 

  • Luo, Y., & Waite, L. J. (2005). The impact of childhood and adult SES on physical, mental, and cognitive well-being in later life. The Journals of Gerontology. Series B: Psychological Sciences and Social Sciences, 60(2), S93–S101.

    Article  Google Scholar 

  • McConkie-Rosel A, O. D. J. (2007). Beyond the diagnosis: The process of genetic counseling. In M. M. M. Mazzocco & J. L. Ross (Eds.), Neurogenetic developmental disorders: Variation of manifestation in childhood (pp. 367–389). Cambridge: MIT.

    Google Scholar 

  • McCubbin, H. I., Thompson, A. I., & McCubbin, M. A. (1996). Resiliency in families. Family assessment: resiliency, coping and adaptation, Ist edition 1–63.

  • Moss, E. M., Batshaw, M. L., Solot, C. B., Gerdes, M., Donald-McGinn, D. M., Driscoll, D. A., et al. (1999). Psychoeducational profile of the 22q11.2 microdeletion: a complex pattern. Jornal de Pediatria, 134(2), 193–198.

    Article  CAS  Google Scholar 

  • Muennig, P., Schweinhart, L., Montie, J., & Neidell, M. (2009). Effects of a prekindergarten educational intervention on adult health: 37-year follow-up results of a randomized controlled trial. American Journal of Public Health, 99(8), 1431–1437.

    Article  PubMed  PubMed Central  Google Scholar 

  • Murphy, K. C. (2002). Schizophrenia and velo-cardio-facial syndrome. Lancet, 359(9304), 426–430.

    Article  PubMed  Google Scholar 

  • NIMH-CDISC. (2004). Computerized diagnostic interview schedule for children

  • Oakes, J. M., & Rossi, P. H. (2003). The measurement of SES in health research: current practice and steps toward a new approach. Social Science & Medicine, 56(4), 769–784.

    Article  Google Scholar 

  • Papolos, D. F., Faedda, G. L., Veit, S., Goldberg, R., Morrow, B., Kucherlapati, R., et al. (1996). Bipolar spectrum disorders in patients diagnosed with velo-cardio-facial syndrome: does a hemizygous deletion of chromosome 22q11 result in bipolar affective disorder? The American Journal of Psychiatry, 153(12), 1541–1547.

    Article  CAS  PubMed  Google Scholar 

  • Petrill, S. A., & Deater-Deckard, K. (2004). Task orientation, parental warmth and SES account for a significant proportion of the shared environmental variance in general cognitive ability in early childhood: evidence from a twin study. Developmental Science, 7(1), 25–32.

    Article  PubMed  Google Scholar 

  • Pitzer, M., Esser, G., Schmidt, M. H., & Laucht, M. (2009). Temperamental predictors of externalizing problems among boys and girls: a longitudinal study in a high-risk sample from ages 3 months to 15 years. European Archives of Psychiatry and Clinical Neuroscience, 259(8), 445–458.

    Article  PubMed  Google Scholar 

  • Prinzie, P., Swillen, A., Maes, B., Onghena, P., Vogels, A., Van Hooste, A., et al. (2004). Parenting, family contexts, and personality characteristics in youngsters with VCFS. Genetic Counseling, 15(2), 141–157.

    CAS  PubMed  Google Scholar 

  • Raizada, R. D., & Kishiyama, M. M. (2010). Effects of socioeconomic status on brain development, and how cognitive neuroscience may contribute to levelling the playing field. Frontiers in Human Neuroscience, 4, 3.

    PubMed  PubMed Central  Google Scholar 

  • Ramey, C. T., & Ramey, S. L. (1998). Prevention of intellectual disabilities: early interventions to improve cognitive development. Preventive Medicine, 27(2), 224–232.

    Article  CAS  PubMed  Google Scholar 

  • Shprintzen, R. J. (2000). Velocardiofacial syndrome. Otolaryngologic Clinics of North America, 33(6), 1217–1240. vi.

    Article  CAS  PubMed  Google Scholar 

  • Shprintzen, R. J. (2008). Velo-cardio-facial syndrome: 30 Years of study. Developmental Disabilities Research Reviews, 14(1), 3–10.

    Article  PubMed  PubMed Central  Google Scholar 

  • Shprintzen, R. J., Goldberg, R., Golding-Kushner, K. J., & Marion, R. W. (1992). Late-onset psychosis in the velo-cardio-facial syndrome. American Journal of Medical Genetics, 42(1), 141–142.

    Article  CAS  PubMed  Google Scholar 

  • Singh-Manoux, A., Richards, M., & Marmot, M. (2005). Socioeconomic position across the lifecourse: how does it relate to cognitive function in mid-life? Annals of Epidemiology, 15(8), 572–578.

    Article  PubMed  Google Scholar 

  • Swillen, A., Devriendt, K., Legius, E., Eyskens, B., Dumoulin, M., Gewillig, M., et al. (1997). Intelligence and psychosocial adjustment in velocardiofacial syndrome: a study of 37 children and adolescents with VCFS. Journal of Medical Genetics, 34(6), 453–458.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Swillen, A., Devriendt, K., Legius, E., Prinzie, P., Vogels, A., Ghesquiere, P., et al. (1999). The behavioural phenotype in velo-cardio-facial syndrome (VCFS): from infancy to adolescence. Genetic Counseling, 10(1), 79–88.

    CAS  PubMed  Google Scholar 

  • Swillen, A., Vogels, A., Devriendt, K., & Fryns, J. P. (2000). Chromosome 22q11 deletion syndrome: update and review of the clinical features, cognitive-behavioral spectrum, and psychiatric complications. American Journal of Medical Genetics, 97(2), 128–135.

    Article  CAS  PubMed  Google Scholar 

  • Turkheimer, E., Haley, A., Waldron, M., D’Onofrio, B., & Gottesman, I. I. (2003). Socioeconomic status modifies heritability of IQ in young children. Psychological Science, 14(6), 623–628.

    Article  PubMed  Google Scholar 

  • Turrell, G., Lynch, J. W., Kaplan, G. A., Everson, S. A., Helkala, E. L., Kauhanen, J., et al. (2002). Socioeconomic position across the lifecourse and cognitive function in late middle age. Journals of Gerontology. Series B, Psychological Sciences and Social Sciences, 57(1), S43–51.

    Article  Google Scholar 

  • van Oort, F. V., van der Ende, J., Wadsworth, M. E., Verhulst, F. C., & Achenbach, T. M. (2010). Cross-national comparison of the link between socioeconomic status and emotional and behavioral problems in youths. Social Psychiatry and Psychiatric Epidemiology, Epub ahead of print.

  • Wang, P. P., Woodin, M. F., Kreps-Falk, R., & Moss, E. M. (2000). Research on behavioral phenotypes: velocardiofacial syndrome (deletion 22q11.2). Developmental Medicine and Child Neurology, 42(6), 422–427.

    Article  CAS  PubMed  Google Scholar 

  • Wechsler, D. (2001). Individual Achievement Test-II (WIAT-II). San Antonio: The Psychological Corporation.

    Google Scholar 

  • Wechsler, D. (2008). Wechsler adult intelligence scale-4th Edition. San Antonio: The Psychological Corporation.

    Google Scholar 

  • Weikart, D. P. (1998). Changing early childhood development through educational intervention. Preventive Medicine, 27(2), 233–237.

    Article  CAS  PubMed  Google Scholar 

  • Werner, S., Malaspina, D., & Rabinowitz, J. (2007). Socioeconomic status at birth is associated with risk of schizophrenia: population-based multilevel study. Schizophrenia Bulletin, 33(6), 1373–1378.

    Article  PubMed  PubMed Central  Google Scholar 

  • Woodin, M., Wang, P. P., Aleman, D., Donald-McGinn, D., Zackai, E., & Moss, E. (2001). Neuropsychological profile of children and adolescents with the 22q11.2 microdeletion. Genetics in Medicine, 3(1), 34–39.

    Article  CAS  PubMed  Google Scholar 

  • Zhang, Z., Gu, D., & Hayward, M. D. (2008). Early life influences on cognitive impairment among oldest old Chinese. Journals of Gerontology. Series B, Psychological Sciences and. Social Sciences, 63(1), S25–33.

    Google Scholar 

Download references

Acknowledgements

This work was supported by R01MH078015-02 PI: SHASHI, V

Disclosures

The authors have no conflict of interest to declare. The primary author, Vandana Shashi has all the data presented in this manuscript and this data would be available to the journal, if necessary, for examination.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Vandana Shashi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Shashi, V., Keshavan, M., Kaczorowski, J. et al. Socioeconomic Status and Psychological Function in Children with Chromosome 22q11.2 Deletion Syndrome: Implications for Genetic Counseling. J Genet Counsel 19, 535–544 (2010). https://doi.org/10.1007/s10897-010-9309-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10897-010-9309-x

Keywords

Navigation