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Psychomotor, cognitive, and socio-emotional developmental profiles of children with Rubinstein-Taybi Syndrome and a severe intellectual disability
| dc.rights.license | open | en_US |
| hal.structure.identifier | Institut de Sciences Criminelles et de la Justice [ISCJ] | |
| hal.structure.identifier | Service de génétique médicale | |
| dc.contributor.author | TAUPIAC, Emmanuelle | |
| hal.structure.identifier | Service de génétique médicale | |
| hal.structure.identifier | Université de Bordeaux [UB] | |
| dc.contributor.author | LACOMBE, Didier | |
| hal.structure.identifier | Laboratoire lorrain de psychologie et neurosciences de la dynamique des comportements [2LPN] | |
| dc.contributor.author | THIÉBAUT, Éric | |
| hal.structure.identifier | Service de génétique médicale | |
| dc.contributor.author | VAN-GILS, Julien | |
| hal.structure.identifier | Institut de Sciences Criminelles et de la Justice [ISCJ] | |
| dc.contributor.author | MICHEL, Grégory | |
| hal.structure.identifier | Service de génétique médicale | |
| hal.structure.identifier | Université de Bordeaux [UB] | |
| dc.contributor.author | FERGELOT, Patricia | |
| hal.structure.identifier | Laboratoire de Psychopathologie et Processus de Santé [LPPS - EA 4057] | |
| dc.contributor.author | ADRIEN, Jean-Louis | |
| dc.date.accessioned | 2023-02-13T10:57:42Z | |
| dc.date.available | 2023-02-13T10:57:42Z | |
| dc.date.issued | 2021 | |
| dc.identifier.issn | 1366-8250 | en_US |
| dc.identifier.uri | https://oskar-bordeaux.fr/handle/20.500.12278/171936 | |
| dc.description.abstractEn | Background: Rubinstein-Taybi syndrome (RSTS) is a multiple congenital anomaly syndrome characterised by several typical somatic characteristics and by developmental disabilities with various degrees of severity. Focusing on children with RSTS, the aim of this study was to describe their psychomotor, cognitive, and socio-emotional developmental profiles.Method: Twenty-three children with RSTS (12 boys; 11 girls; mean chronological age: 4 years and 10 months) with severe intellectual disability (mean developmental quotient = 32.39) were recruited from an Expert Department of Medical Genetics. Developmental assessments were carried out with the Brunet-Lézine-Revised scale and the Social Cognitive Evaluation Battery.Results: The participants’ developmental profiles were characterised by heterogeneous psychomotor development, homogeneous cognitive and socio-emotional development, by more severe delays in expressive language, vocal imitation, and symbolic play skills, and by better developmental levels in socio-emotional abilities.Conclusions: Based on these atypical developmental profiles, early interventions should target the three most delayed abilities. | |
| dc.language.iso | EN | en_US |
| dc.subject.en | Severe intellectual disability | |
| dc.subject.en | Rubinstein-Taybi syndrome | |
| dc.subject.en | expressive language delay | |
| dc.subject.en | symbolic play delaya | |
| dc.subject.en | typical developmental profile | |
| dc.subject.en | intervention targets | |
| dc.title.en | Psychomotor, cognitive, and socio-emotional developmental profiles of children with Rubinstein-Taybi Syndrome and a severe intellectual disability | |
| dc.type | Article de revue | en_US |
| dc.identifier.doi | 10.3109/13668250.2020.1776455 | en_US |
| dc.subject.hal | Sciences de l'Homme et Société/Psychologie | en_US |
| bordeaux.journal | Journal of Intellectual and Developmental Disability | en_US |
| bordeaux.page | 80-89 | en_US |
| bordeaux.volume | 46 | en_US |
| bordeaux.hal.laboratories | Institut de Sciences Criminelles et de la Justice (ISCJ) - EA 4633 | en_US |
| bordeaux.issue | 1 | en_US |
| bordeaux.institution | Université de Bordeaux | en_US |
| bordeaux.peerReviewed | oui | en_US |
| bordeaux.inpress | non | en_US |
| bordeaux.import.source | hal | |
| hal.identifier | hal-02902191 | |
| hal.version | 1 | |
| hal.export | false | |
| workflow.import.source | hal | |
| dc.rights.cc | Pas de Licence CC | en_US |
| bordeaux.COinS | ctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Journal%20of%20Intellectual%20and%20Developmental%20Disability&rft.date=2021&rft.volume=46&rft.issue=1&rft.spage=80-89&rft.epage=80-89&rft.eissn=1366-8250&rft.issn=1366-8250&rft.au=TAUPIAC,%20Emmanuelle&LACOMBE,%20Didier&THI%C3%89BAUT,%20%C3%89ric&VAN-GILS,%20Julien&MICHEL,%20Gr%C3%A9gory&rft.genre=article |
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