Comparação dos fenótipos comportamentais de crianças e adolescentes com síndrome de Prader-Willi, síndrome de Williams-Beuren e síndrome de Down

AUTOR(ES)

Yara Garzuzi

DATA DE PUBLICAÇÃO

2009

RESUMO

There are few studies in Brazil that comprise the theme of behavioral phenotypes in people with genetic syndromes. The knowledge of behavioral patterns associated with such syndromes contributes to the planning of standardized therapeutic assessment, intervention and handling strategies, and for an improvement in assistance practices. This study presents three genetic syndromes, which have as their common behavioral phenotype the presence of mental retardation that is associated with neurobiological, clinical, behavioral, social and psychiatric patterns specific to each of them. To describe and compare the major behavior patterns of children and adolescents with Prader-Willi Syndrome (PWS), Williams-Beuren Syndrome (WBS) and Down Syndrome (DS). The sample consisted of 68 children and adolescents with diagnosis for the syndromes. From these subjects, 11 presented cytogenetic-molecular diagnosis for PWS, 10 presented clinical and/or cytogeneticmolecular diagnosis for WBS, and 47 presented clinical and/or cytogenetic-molecular diagnosis for DS. The Child Behavior Checklist for ages 15 (CBCL/15) and the Child Behavior Checklist for ages 6-18 (CBCL/618) were used for data collection. The major results of the comparison between groups showed that, with respect to behavior changes, the PWS group scored higher, followed by the WBS group and the DS group, respectively. The following main patterns were found: in PWS, Externalizing Problems, Social Problems, Thought Problems and Aggressive Behavior; in WBS, Social and Attention Problems; and in DS, Total Problems. Statistically significant differences were also observed between some of this response patterns when groups paired by sex and age were compared (PWS-DS and WBS-DS).The changes found mainly in groups with PWS and WBS interfere considerably with the social adjustment of these children and adolescents. If these changes are not treated, they may result in the development of risk factors for several psychiatric comorbidities tending to chronicity. Therefore, it is necessary to implement public health services for the care of behavioral changes and to help families to deal with these groups of children and adolescents.

ASSUNTO(S)

prader-willi syndrome, williams-beuren syndrome behavioral phenotype down syndrome adolescent adolescente síndrome de williams-beuren criança psicologia síndrome de prader-willi síndrome de down child fenótipo comportamental

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