Behaviour in Cornelia de Lange syndrome: a systematic review

Title

Behaviour in Cornelia de Lange syndrome: a systematic review

Creator

Mulder P A; Huisman S A; Hennekam R C; Oliver C; Van Balkom I D C; Piening S

Identifier

Publisher

Developmental Medicine and Child Neurology

Date

2017

Subject

behavioral problems; De Lange syndrome; trajectory; characteristics

Description

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 studyselection, 43 papers were included. The Cochrane quality criteria were adjusted to assign quality scores to the included studies. ESULTS 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.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Mulder P A; Huisman S A; Hennekam R C; Oliver C; Van Balkom I D C; Piening S, “Behaviour in Cornelia de Lange syndrome: a systematic review,” Pediatric Palliative Care Library, accessed July 5, 2022, https://pedpalascnetlibrary.omeka.net/items/show/16756.

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