Resumo

MOTA, Érica Regina; ROMEO BERTOLA, Débora; AE KIM, Chong  e  TRIGUERO VELOZ TEIXEIRA, Maria Cristina. Behavioral disturbances in Noonan Syndrome: Brazilian preliminary data. rev.latinoam.psicol. [online]. 2010, vol.42, n.1, pp.87-95. ISSN 0120-0534.

Different from other countries of Europe and North American, studies about the behavioral profile of Noonan syndrome's patients are inexistent. The objective of this study was to report the profiles of behavioral functions of 10 participants (4 females and 6 males), with mutations in the PTPN11 gene. For this assessment it was used the Inventory of Behaviors of Children and Adolescents from 6 to 18 years (CBCL/6-18) and the Inventory of Auto-Evaluation for Adults from 18 to 59 years (ASR). The main results point that in Adaptive Functioning Scale all the participants were in the normality range. In the Syndrome Scale the adult participants were in normality range and the children were in clinical range to the sub-scales anxious/depressed, somatic complaints and aggressive behavior. In the DSM-Oriented Scale, 25% of the adult patients were in the borderline clinical range and clinical range, respectively, for Avoidant Personality Problems and Antisocial Personality Problems. About the both children in this scale were in the clinical range of Affective Problems and Anxiety Problems. This relatively homogenous sample, regarding the PTPN11 gene, shows a normal adult behavioral profile, on the average. However, the individual children and adult profiles show diverse internalizing and externalizing behavioral disturbances.

Palavras-chave : Noonan syndrome; behavioral phenotype; cognitive abilities.

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