Behavioral and social phenotypes in boys with 47,XYY syndrome or 47,XXY klinefelter syndrome

Judith L. Ross, David P. Roeltgen, Harvey Kushner, Andrew R. Zinn, Allan Reiss, Martha Zeger Bardsley, Elizabeth McCauley, Nicole Tartaglia

Research output: Contribution to journalArticle

76 Citations (Scopus)

Abstract

OBJECTIVE: To contrast the behavioral and social phenotypes including a screen for autistic behaviors in boys with 47,XYY syndrome (XYY) or 47,XXY Klinefelter syndrome (KS) and controls and investigate the effect of prenatal diagnosis on the phenotype. METHODS: Patients included 26 boys with 47,XYY, 82 boys with KS, and 50 control boys (ages 4-15 years). Participants and parents completed a physical examination, behavioral questionnaires, and intellectual assessments. RESULTS: Most boys with XYY or KS had Child Behavior Checklist parental ratings within the normal range. On the Child Behavior Checklist, mean problem behaviors t scores were higher in the XYY versus KS groups for the Problem Behavior, Externalizing, Withdrawn, Thought Problems, and Attention Problems subscales. On the Conners' Parent Rating Scale-Revised, the XYY versus KS group had increased frequency of hyperactive/impulsive symptoms (P < .006). In addition, 50% and 12% of the XYY and KS groups, respectively, had scores >15 for autism screening from the Social Communication Questionnaire. For the boys with KS, prenatal diagnosis was associated with fewer problem behaviors. CONCLUSIONS: A subset of the XYY and KS groups had behavioral difficulties that were more severe in the XYY group. These findings could guide clinical practice and inform patients and parents. Boys diagnosed with XYY or KS should receive a comprehensive psychoeducational evaluation and be screened for learning disabilities, attention-deficit/hyperactivity disorder, and autism spectrum disorders.

Original languageEnglish (US)
Pages (from-to)769-778
Number of pages10
JournalPediatrics
Volume129
Issue number4
DOIs
StatePublished - Apr 2012

Fingerprint

Klinefelter Syndrome
Phenotype
Child Behavior
Checklist
Prenatal Diagnosis
Parents
XYY syndrome 47
Syndrome
Boys
Learning Disorders
Attention Deficit Disorder with Hyperactivity
Autistic Disorder
Physical Examination
Reference Values
Communication

Keywords

  • ADHD
  • Autism
  • Klinefelter syndrome
  • XXY
  • XYY

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Arts and Humanities (miscellaneous)

Cite this

Ross, J. L., Roeltgen, D. P., Kushner, H., Zinn, A. R., Reiss, A., Bardsley, M. Z., ... Tartaglia, N. (2012). Behavioral and social phenotypes in boys with 47,XYY syndrome or 47,XXY klinefelter syndrome. Pediatrics, 129(4), 769-778. https://doi.org/10.1542/peds.2011-0719

Behavioral and social phenotypes in boys with 47,XYY syndrome or 47,XXY klinefelter syndrome. / Ross, Judith L.; Roeltgen, David P.; Kushner, Harvey; Zinn, Andrew R.; Reiss, Allan; Bardsley, Martha Zeger; McCauley, Elizabeth; Tartaglia, Nicole.

In: Pediatrics, Vol. 129, No. 4, 04.2012, p. 769-778.

Research output: Contribution to journalArticle

Ross, JL, Roeltgen, DP, Kushner, H, Zinn, AR, Reiss, A, Bardsley, MZ, McCauley, E & Tartaglia, N 2012, 'Behavioral and social phenotypes in boys with 47,XYY syndrome or 47,XXY klinefelter syndrome', Pediatrics, vol. 129, no. 4, pp. 769-778. https://doi.org/10.1542/peds.2011-0719
Ross, Judith L. ; Roeltgen, David P. ; Kushner, Harvey ; Zinn, Andrew R. ; Reiss, Allan ; Bardsley, Martha Zeger ; McCauley, Elizabeth ; Tartaglia, Nicole. / Behavioral and social phenotypes in boys with 47,XYY syndrome or 47,XXY klinefelter syndrome. In: Pediatrics. 2012 ; Vol. 129, No. 4. pp. 769-778.
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