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dc.rights.licenseopenen_US
hal.structure.identifierInstitut de Sciences Criminelles et de la Justice [ISCJ]
hal.structure.identifierService de génétique médicale
dc.contributor.authorTAUPIAC, Emmanuelle
hal.structure.identifierService de génétique médicale
hal.structure.identifierUniversité de Bordeaux [UB]
dc.contributor.authorLACOMBE, Didier
hal.structure.identifierLaboratoire lorrain de psychologie et neurosciences de la dynamique des comportements [2LPN]
dc.contributor.authorTHIÉBAUT, Éric
hal.structure.identifierService de génétique médicale
dc.contributor.authorVAN-GILS, Julien
hal.structure.identifierInstitut de Sciences Criminelles et de la Justice [ISCJ]
dc.contributor.authorMICHEL, Grégory
hal.structure.identifierService de génétique médicale
hal.structure.identifierUniversité de Bordeaux [UB]
dc.contributor.authorFERGELOT, Patricia
hal.structure.identifierLaboratoire de Psychopathologie et Processus de Santé [LPPS - EA 4057]
dc.contributor.authorADRIEN, Jean-Louis
dc.date.accessioned2023-02-13T10:57:42Z
dc.date.available2023-02-13T10:57:42Z
dc.date.issued2021
dc.identifier.issn1366-8250en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/171936
dc.description.abstractEnBackground: 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.isoENen_US
dc.subject.enSevere intellectual disability
dc.subject.enRubinstein-Taybi syndrome
dc.subject.enexpressive language delay
dc.subject.ensymbolic play delaya
dc.subject.entypical developmental profile
dc.subject.enintervention targets
dc.title.enPsychomotor, cognitive, and socio-emotional developmental profiles of children with Rubinstein-Taybi Syndrome and a severe intellectual disability
dc.typeArticle de revueen_US
dc.identifier.doi10.3109/13668250.2020.1776455en_US
dc.subject.halSciences de l'Homme et Société/Psychologieen_US
bordeaux.journalJournal of Intellectual and Developmental Disabilityen_US
bordeaux.page80-89en_US
bordeaux.volume46en_US
bordeaux.hal.laboratoriesInstitut de Sciences Criminelles et de la Justice (ISCJ) - EA 4633en_US
bordeaux.issue1en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
bordeaux.import.sourcehal
hal.identifierhal-02902191
hal.version1
hal.exportfalse
workflow.import.sourcehal
dc.rights.ccPas de Licence CCen_US
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