Cognitive and behavioral heterogeneity in genetic syndromes
Autor(a) principal: | |
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Data de Publicação: | 2014 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Jornal de Pediatria (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572014000200155 |
Resumo: | OBJECTIVE: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. |
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Cognitive and behavioral heterogeneity in genetic syndromesCognitionBehaviorWilliams-Beuren syndromePrader-Willi syndromeFragile X syndromeOBJECTIVE: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.Sociedade Brasileira de Pediatria2014-04-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572014000200155Jornal de Pediatria v.90 n.2 2014reponame:Jornal de Pediatria (Online)instname:Sociedade Brasileira de Pediatria (SBP)instacron:SBPE10.1016/j.jped.2013.06.010info:eu-repo/semantics/openAccessPegoraro,Luiz F.L.Steiner,Carlos E.Celeri,Eloisa H.R.V.Banzato,Claudio E.M.Dalgalarrondo,Pauloeng2015-10-08T00:00:00Zoai:scielo:S0021-75572014000200155Revistahttp://www.jped.com.br/https://old.scielo.br/oai/scielo-oai.php||jped@jped.com.br1678-47820021-7557opendoar:2015-10-08T00:00Jornal de Pediatria (Online) - Sociedade Brasileira de Pediatria (SBP)false |
dc.title.none.fl_str_mv |
Cognitive and behavioral heterogeneity in genetic syndromes |
title |
Cognitive and behavioral heterogeneity in genetic syndromes |
spellingShingle |
Cognitive and behavioral heterogeneity in genetic syndromes Pegoraro,Luiz F.L. Cognition Behavior Williams-Beuren syndrome Prader-Willi syndrome Fragile X syndrome |
title_short |
Cognitive and behavioral heterogeneity in genetic syndromes |
title_full |
Cognitive and behavioral heterogeneity in genetic syndromes |
title_fullStr |
Cognitive and behavioral heterogeneity in genetic syndromes |
title_full_unstemmed |
Cognitive and behavioral heterogeneity in genetic syndromes |
title_sort |
Cognitive and behavioral heterogeneity in genetic syndromes |
author |
Pegoraro,Luiz F.L. |
author_facet |
Pegoraro,Luiz F.L. Steiner,Carlos E. Celeri,Eloisa H.R.V. Banzato,Claudio E.M. Dalgalarrondo,Paulo |
author_role |
author |
author2 |
Steiner,Carlos E. Celeri,Eloisa H.R.V. Banzato,Claudio E.M. Dalgalarrondo,Paulo |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Pegoraro,Luiz F.L. Steiner,Carlos E. Celeri,Eloisa H.R.V. Banzato,Claudio E.M. Dalgalarrondo,Paulo |
dc.subject.por.fl_str_mv |
Cognition Behavior Williams-Beuren syndrome Prader-Willi syndrome Fragile X syndrome |
topic |
Cognition Behavior Williams-Beuren syndrome Prader-Willi syndrome Fragile X syndrome |
description |
OBJECTIVE: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. |
publishDate |
2014 |
dc.date.none.fl_str_mv |
2014-04-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572014000200155 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0021-75572014000200155 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1016/j.jped.2013.06.010 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Pediatria |
publisher.none.fl_str_mv |
Sociedade Brasileira de Pediatria |
dc.source.none.fl_str_mv |
Jornal de Pediatria v.90 n.2 2014 reponame:Jornal de Pediatria (Online) instname:Sociedade Brasileira de Pediatria (SBP) instacron:SBPE |
instname_str |
Sociedade Brasileira de Pediatria (SBP) |
instacron_str |
SBPE |
institution |
SBPE |
reponame_str |
Jornal de Pediatria (Online) |
collection |
Jornal de Pediatria (Online) |
repository.name.fl_str_mv |
Jornal de Pediatria (Online) - Sociedade Brasileira de Pediatria (SBP) |
repository.mail.fl_str_mv |
||jped@jped.com.br |
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1752122319576236032 |