Cognitive and behavioral heterogeneity in genetic syndromes

dc.creatorPegoraro, LFL
dc.creatorSteiner, CE
dc.creatorCeleri, EHRV
dc.creatorBanzato, CEM
dc.creatorDalgalarrondo, P
dc.date2014
dc.dateMAR-APR
dc.date2014-08-01T18:26:01Z
dc.date2015-11-26T17:05:13Z
dc.date2014-08-01T18:26:01Z
dc.date2015-11-26T17:05:13Z
dc.date.accessioned2018-03-28T23:53:33Z
dc.date.available2018-03-28T23:53:33Z
dc.identifierJornal De Pediatria. Soc Brasil Pediatria, v. 90, n. 2, n. 155, n. 160, 2014.
dc.identifier0021-7557
dc.identifier1678-4782
dc.identifierWOS:000334016100008
dc.identifier10.1016/j.jped.2013.06.010
dc.identifierhttp://www.repositorio.unicamp.br/jspui/handle/REPOSIP/78862
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/78862
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1279528
dc.descriptionObjective: this study aimed to investigate the cognitive and behavioral profiles, as well as the psychiatric symptoms and disorders in children with three different genetic syndromes with similar sociocultural and socioeconomic backgrounds. Methods: thirty-four children aged 6 to 16 years, with Williams-Beuren syndrome (n = 10), Prader-Willi syndrome (n = 11), and Fragile X syndrome (n = 13) from the outpatient clinics of Child Psychiatry and Medical Genetics Department were cognitively assessed through the Wechsler Intelligence Scale for Children (WISC-III). Afterwards, a full-scale intelligence quotient (IQ), verbal IQ, performance IQ, standard subtest scores, as well as frequency of psychiatric symptoms and disorders were compared among the three syndromes. Results: significant differences were found among the syndromes concerning verbal IQ and verbal and performance subtests. Post-hoc analysis demonstrated that vocabulary and comprehension subtest scores were significantly higher in Williams-Beuren syndrome in comparison with Prader-Willi and Fragile X syndromes, and block design and object assembly scores were significantly higher in Prader-Willi syndrome compared with Williams-Beuren and Fragile X syndromes. Additionally, there were significant differences between the syndromes concerning behavioral features and psychiatric symptoms. The Prader-Willi syndrome group presented a higher frequency of hyperphagia and self-injurious behaviors. The Fragile X syndrome group showed a higher frequency of social interaction deficits; such difference nearly reached statistical significance. Conclusion: the three genetic syndromes exhibited distinctive cognitive, behavioral, and psychiatric patterns. (C) 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.
dc.description90
dc.description2
dc.description155
dc.description160
dc.languageen
dc.publisherSoc Brasil Pediatria
dc.publisherRio De Janeiro, Rj
dc.publisherBrasil
dc.relationJornal De Pediatria
dc.relationJ. Pediatr.
dc.rightsaberto
dc.sourceWeb of Science
dc.subjectCognition
dc.subjectBehavior
dc.subjectWilliams-Beuren syndrome
dc.subjectPrader-Willi syndrome
dc.subjectFragile X syndrome
dc.subjectPrader-willi-syndrome
dc.subjectFragile-x-syndrome
dc.subjectIntellectual Disability
dc.subjectMental-retardation
dc.subjectChildren
dc.subjectAdolescents
dc.subjectPhenotypes
dc.subjectProfiles
dc.subjectDisorders
dc.subjectLanguage
dc.titleCognitive and behavioral heterogeneity in genetic syndromes
dc.typeArtículos de revistas


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