Indicadores de funcionamento intelectual e adaptativo em uma amostra brasileira de crianças, adolescentes e adultos com síndrome de Willians

• Autor(a) principal: Faria, Karla Tomáz
• Data de Publicação: 2020
• Tipo de documento: Dissertação
• Idioma: por
• Título da fonte: Biblioteca Digital de Teses e Dissertações do Mackenzie
• Texto Completo: http://dspace.mackenzie.br/handle/10899/27926
• topic: funcionamento adaptativo; síndrome de Williams; pais; problemas emocionais; comportamentais; funcionamento intelectual; CNPQ::CIENCIAS BIOLOGICAS

Williams syndrome (WS) is a rare genetic disorder caused by multiple gene microdeletion of region 11.23 of the long arm of chromosome 7 (region 7q11.23). Scientific evidences indicate that the evaluation of adaptive functioning (AF) is fundamental in WS to identify support levels and development of potentialities, considering the intellectual disability (ID) associated with the syndrome. The main objective of the study was to explore how the AF socialization indicator is related to verbal and performance cognitive functioning regardless of intellectual impairment, as well as emotional and behavioral problems (EBP). The study adopted a cross-sectional design with a sample composed by 78 individuals with WS between 6 and 41 years matched with 74 healthy people of control group (CG), and 78 caregiver’s WS group. The data collection instruments were the Behavior Inventory for Children and Adolescents between 6 and 18 years old (CBCL/6-18), the Behavior Inventory for Adults between 18 and 59 years old (ABCL), the Self-Assessment Inventory for between 18 and 59 years old (ASR) and the abbreviated Wechsler Intelligence Scale (WASI). Data analysis procedures were the Kolmogorov-Smirnov test for normality testing, Mann-Whitney U test for comparison of means, Cohen test verified the effect size and Spearman correlation analysis to test the degree of association between the variables. The main results indicated that there were no statistically significant differences between the group with WS and the CG in the socialization scale. In the WS group of 6 to 18 years old it was observed that the higher the socialization score, the better their performance in tasks that evaluate indicators of performance IQ. In the socialization scale there were no statistically significant differences between the group with WS and the CG, however it was found that adults show higher levels of socialization, according to the caregivers' report, when compared to children and adolescents, with statistically significant differences. As a recommendation for future studies, in addition to multiple informant methodologies for assessment purposes, caregivers need to receive training to discriminate stimulation needs of children with associated WS and ID, as well as interventions in natural environments that can be developed by them in social and family contexts