XYY Syndrome

Sharan Goobie, London, Ontario, Canada and Western University, London, Ontario, Canada
Chitra Prasad, London, Ontario, Canada and Western University, London, Ontario, Canada

Published online: July 2012

DOI: 10.1002/9780470015902.a0005157.pub2

XYY syndrome is a genetic condition in which males have 47 chromosomes instead of the usual 46 chromosomes. The karyotype is written as 47,XYY as these males have an extra Y chromosome. XYY occurs in approximately one in 1000 live male births and falls into the category of sex chromosome aneuploidy. Males with XYY often have tall stature but otherwise are not significantly different in physical appearance compared to other men. They are generally healthy; however, some XYY men can have learning disabilities or behaviour problems. It is possible that the information we know about XYY syndrome in the medical literature may be biased, as healthy, developmentally normal males do not often have a reason to have a karyotype performed. We may overestimate some of the problems associated with XYY such as learning disabilities and behaviour problems, as these are common reasons for children to have a karyotype.

Key Concepts:

  • Males with XYY syndrome have 47 chromosomes, which includes an extra Y chromosome.
  • Males with XYY syndrome appear physically normal, however often have tall stature.
  • Males with XYY syndrome generally have normal intelligence, however some may have learning disabilities or behaviour issues.
  • Males with XYY syndrome do not have criminal tendencies as suggested in the older literature.

Keywords: XYY; aneuploidy; sex chromosome; tall stature; learning disability; attention deficit hyperactivity disorder

Figure 1. In XYY, the karyotype (chromosome count) is 47,XYY. This means that each of the somatic cells contains 47 chromosomes, 22 normal pairs of autosomes, and three sex chromosomes, one X chromosome and two Y chromosomes. Figure Courtesy of A. J. Dawson.
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 References
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    Martin RH, McInnes B and Rademaker AW (1999) Analysis of aneuploidy for chromosomes 13, 21, X and Y by multicolour fluorescence in situ hybridization (FISH) in a 47,XYY male. Zygote 7: 131–134.
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    book Nielsen E and Wohlert E (1990) "Sex chromosomes abnormalities found among 34,910 newborn children: results from a 13 year study in Arhus, Denmark. 19". In: Evans JA, Hamerton JL and Robinson A (eds) Children and Young Adults with Sex Chromosome Aneupolidy, vol. 26, pp. 209–223. New York: Wiley Liss.
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    Ross JL, Zeger MP, Kushner H, Zinn AR and Roeltgen DP (2009) An extra X or Y chromosome: contrasting the cognitive and motor phenotypes in childhood in boys with 47,XYY syndrome or 47,XXY Klinefelter syndrome. Developmental Disabilities Research Reviews 15(4): 309–317.
    Stochholm K, Juul S and Gravholt CH (2010) Diagnosis and mortality in 47,XYY persons: a registry study. Orphanet Journal of Rare Diseases 5: 15.
    Tartaglia NR, Ayari N, Hutaff‐Lee C and Boada R (2012) Attention‐deficit hyperactivity disorder symptoms in children and adolescents with sex chromosome aneuploidy: XXY, XXX, XYY and XXYY. Journal of Developmental & Behavioral Pediatrics 33(3): 1–10.
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 Further Reading
    book Rimoin DL, Connor JM and Pyeritz RE (2006) Emery and Rimoin's Principles and Practice of Medical Genetics, 5th edn. New York: Churchill Livingstone.
 Web Links
    ePath National Organization for Rare Disorders (NORD) http://www.rarediseases.org/
    ePath Unique – Rare Chromosome Disorder Support Group http://www.rarechromo.org http://www.rarechromo.org/files/XYYStudyDayReport.pdf

Related Sub-Topics:

Neurobiology of Disease | Chromosomal Disorders | Genomic Disorders
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Article Title: XYY Syndrome
 
How to Cite close
Goobie, Sharan, and Prasad, Chitra(Jul 2012) XYY Syndrome. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0005157.pub2]