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Psychological profile of children with Noonan syndrome

Published online by Cambridge University Press:  12 January 2005

DA Lee
Affiliation:
Sub Department of Clinical Health Psychology, University College London, UK.
S Portnoy
Affiliation:
Department of Child Psychology, Chelsea & Westminster Hospital, UK.
P Hill
Affiliation:
Department of Psychological Medicine, Hospital for Sick Children, UK.
C Gillberg
Affiliation:
Departments of Child Psychiatry and Medical Genetics, St George's Hospital Medical School, London, UK.
MA Patton
Affiliation:
Departments of Child Psychiatry and Medical Genetics, St George's Hospital Medical School, London, UK.
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Abstract

A cohort of 48 children with Noonan syndrome, with a mean age of 9 years 10 months (SD 3y 7mo; 28 males, 20 females), was recruited from a national study. Children were assessed using the Wechsler Intelligence Scales and Test of Motor Impairment–Revised (TOMI-R). The Piers–Harris Self-evaluation Questionnaire was also completed in a subgroup of age-appropriate children. Children's parents and teachers completed the Rutter A and B scales to assess of the degree of behavioural and emotional problems respectively. Mean Full-scale IQ score was 84, and one-quarter of the participants had learning disabilities. Verbal IQ tended to be slightly lower than Performance IQ. About half of the group showed evidence of mild to moderate impairment, confirming the impression of ‘clumsiness/developmental coordination disorder’ on the TOMI-R. Level of self-esteem, as determined by the Piers–Harris Questionnaire, was comparable to that of a standardized population. This research has identified some characteristic psychological features in Noonan syndrome. However, a specific behavioural phenotype could not be identified.

Type
Original Articles
Copyright
© 2005 Mac Keith Press

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