Cardiff University | Prifysgol Caerdydd ORCA
Online Research @ Cardiff 
WelshClear Cookie - decide language by browser settings

Mental health and behavioural problems in children with XXYY: a comparison with intellectual disabilities

Srinivasan, Ramya, Wolstencroft, J., Erwood, M., Raymond, F. L., Van Den Bree, M. ORCID:, Hall, J. ORCID: and Skuse, D. 2019. Mental health and behavioural problems in children with XXYY: a comparison with intellectual disabilities. Journal of Intellectual Disability Research 63 (5) , pp. 477-488. 10.1111/jir.12607

[thumbnail of Mental health and behavioural problems in children with XXYY.pdf]
PDF - Accepted Post-Print Version
Download (348kB) | Preview


Background The phenotype of children with XXYY has predominantly been defined by comparison to other sex chromosome aneuploidies trisomies affecting male children; however, the intellectual ability of children with XXYY is lower than children with other sex chromosome aneuploidies trisomies. It is not known to what extent the phenotype identified to date is specific to XXYY, rather than a reflection of lower IQ. This study evaluates the mental health and behaviour of children with XXYY, in comparison to children with intellectual disabilities of heterogeneous genetic origin. Methods Fifteen children with XXYY and 30 controls matched for age (4–14 years), sex and intellectual ability were ascertained from the IMAGINE ID study. IMAGINE ID participants have intellectual disabilities due to genetic anomalies confirmed by National Health Service Regional Genetic Centre laboratories. The mental health and behaviour of participants was examined with the Development and Well‐being Assessment and the Strengths and Difficulties Questionnaire. Results Children with XXYY experienced significantly more frequent and intense temper outbursts than the control group. Conclusion Our results suggest that temper outbursts may be specifically associated with the XXYY phenotype. These problems have a significant impact on the daily lives of boys with XXYY and their families. It is crucial to ensure that families are well supported to manage these difficulties.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Neuroscience and Mental Health Research Institute (NMHRI)
Publisher: Wiley
ISSN: 0964-2633
Date of First Compliant Deposit: 18 April 2019
Date of Acceptance: 14 January 2019
Last Modified: 06 Nov 2023 21:08

Citation Data

Cited 4 times in Scopus. View in Scopus. Powered By Scopus® Data

Actions (repository staff only)

Edit Item Edit Item


Downloads per month over past year

View more statistics