Perfil comportamental e cognitivo de crianças com a síndrome de Prader Willi

• Autor(a) principal: Mesquita, Maria Luiza Guedes de
• Data de Publicação: 2008
• Tipo de documento: Dissertação
• Idioma: por
• Título da fonte: Biblioteca Digital de Teses e Dissertações do Mackenzie
• Texto Completo: http://dspace.mackenzie.br/handle/10899/22602
• topic: síndrome de Prader Willi; comportamento alimentar; análise experimental do comportamento; perfil cognitivo; perfil comportamental; Prader Willi syndrome; eating behavior; experimental analysis of behavior; cognitive profile; behavioral profile; CNPQ::CIENCIAS HUMANAS::PSICOLOGIA

Prader Willi syndrome is a genetic disorder caused by the absence of the paternally derived PWS region of chromosome 15. Some patognomonics symptoms of this disorder are hyperphagia and obesity, which in most cases happens before the age of six and they severely harm the quality of life of such patients. The present study has focused on the behavioral area and aimed at identifying the daily frequency of eating habits and the caloric intake of children with PWS; identifying the variables of family interaction which control the eating behavior and tracing a cognitive and behavioral profile of the PWS patients. The sample was made by eleven children with a PWS genetic diagnosis, between the ages of 7 and 16 and their parents. The data collecting instruments were: a questionnaire for the eating habits record of the children, an interview to identify controlled environment variances of the eating behavior and the Brazilian version of the Child Behavior Checklist for ages 6-18 (CBCL/6-18) by Achenbach and the WISC-III test (Wechsler Intelligence Scale for Children- 3 rd edition). The data went through an evaluation which included topographic record and functional analysis of eating behavior and others types of children´s non- adaptive behaviors and analyses and comparison of frequency distribution of these behaviors in relation to cognitive and behavioral profiles of the patients. The group was characterized as obese according to the BMI calculation. The main results showed that 82% from the total classified in the WISC-III test as mentally retarded, 100% of the sample was at clinic level and in at lest one of the syndrome scales, competences or problems of the instrument CBCL/6-18. One of the high frequency behavioral classes was the negotiations to obtain food from their parents. There is a behavioral chart configured as pathological in terms of response for tantrums, manipulation, aggressiveness, breaking of rules and oppositionist behavior. Psychiatric comorbidity has also been identified, associated to high consumption of psychotropic drugs.