1. Behaviour in Cornelia de Lange syndrome: a systematic review.
- Author
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Mulder, Paul A, Huisman, Sylvia A, Hennekam, Raoul C, Oliver, Chris, Balkom, Ingrid D C, and Piening, Sigrid
- Subjects
AUTISM ,AUTISM spectrum disorders ,AGGRESSION (Psychology) ,SOCIAL anxiety ,DATA extraction ,DE Lange's syndrome ,MENTAL illness ,QUALITY of life ,SYSTEMATIC reviews ,BIBLIOGRAPHIC databases ,DISEASE complications ,PSYCHOLOGY - Abstract
Aim: Careful study and accurate description of behaviour are important to understand developmental challenges for individuals with Cornelia de Lange syndrome (CdLS). Here we present a systematic review of current understanding of behaviour in CdLS.Method: A systematic search was performed for articles published between January 1946 and December 2015 evaluating autism, self-injury, and/or cognition in CdLS. After study-selection, 43 papers were included. The Cochrane quality criteria were adjusted to assign quality scores to the included studies.Results: Participants were mostly categorized in the severe/profound developmental level. Methodology and quality were very heterogeneous, as well as reporting occurrence of autism. Self-injurious behaviour was reported in 15 papers. Physical conditions were reported in 21 studies, mostly related to hearing and vision. Only nine studies mentioned details about medication.Interpretation: Comparison of presented results was hindered by heterogeneous assessment methods. Improving our understanding of behavioural characteristics in CdLS requires more uniform methodology. We propose a criterion standard of instruments that can ideally be used in assessment of behaviour and development. This will improve understanding of behaviour in the context of developmental level and daily functioning. [ABSTRACT FROM AUTHOR]- Published
- 2017
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