{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T13:09:49Z","timestamp":1776172189044,"version":"3.50.1"},"reference-count":54,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,11,27]],"date-time":"2023-11-27T00:00:00Z","timestamp":1701043200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J intellect Disabil Res"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Angelman syndrome (AS) is a rare neurodevelopmental disorder characterised by severe intellectual disability, movement disorder, epilepsy, sleeping problems, and behavioural issues. Little is known on child health\u2010related quality of life (HRQoL) in AS. AS family studies have reported elevated parenting stress and a high impact of the child's syndrome on the parent. It is unclear which factors influence child HRQoL and parenting stress\/impact in AS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We collected data prospectively through standardised clinical assessments of children with AS at the ENCORE Expertise centre for Angelman Syndrome at the Erasmus MC Sophia Children's Hospital. A linear regression analysis was conducted for the following outcome variables: (1) child HRQoL (Infant and Toddler Quality of Life Questionnaire); (2) the impact of the child's syndrome on the parent (Infant and Toddler Quality of Life Questionnaire); and (3) parenting stress (Parenting Stress Index). Predictor variables were child genotype, epilepsy, sleeping problems (Sleep Disturbance Scale for Children), cognitive developmental level (Bayley Cognition Scale), autistic features (Autism Diagnostic Observation Schedule) and emotional\/behavioural problems (Child Behaviour Checklist). Covariates were sex, age and socio\u2010economic status.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The study sample consisted of 73 children with AS, mean age\u00a0=\u00a09.1\u00a0years, range\u00a0=\u00a02\u201318\u00a0years. Emotional\/behavioural problems were the strongest significant predictor of lowered child HRQoL. Internalising problems were driving this effect. In addition, having the deletion genotype and higher age was related to lower child HRQoL. Sleeping problems were related to a higher impact of the child's syndrome on the parent. Finally, emotional\/behavioural problems were associated with higher parenting stress. Cognitive developmental level, autistic features and epilepsy were not a significant predictor of child HRQoL and parenting stress\/impact.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These results suggest that interventions aimed at increasing child HRQoL and decreasing parenting stress\/impact in AS should focus on child emotional\/behavioural problems and sleeping problems, using a family\u2010centred approach.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jir.13106","type":"journal-article","created":{"date-parts":[[2023,11,28]],"date-time":"2023-11-28T00:37:30Z","timestamp":1701131850000},"page":"248-263","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Child characteristics associated with child quality of life and parenting stress in Angelman syndrome"],"prefix":"10.1111","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-9274-6316","authenticated-orcid":false,"given":"D. A.","family":"Hagenaar","sequence":"first","affiliation":[{"name":"ENCORE Expertise Centre for Neurodevelopmental Disorders Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Child\u2010 and Adolescent Psychiatry\/Psychology Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Paediatrics Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K. G. C. B.","family":"Bindels\u2010de Heus","sequence":"additional","affiliation":[{"name":"ENCORE Expertise Centre for Neurodevelopmental Disorders Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Paediatrics Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Lubbers","sequence":"additional","affiliation":[{"name":"ENCORE Expertise Centre for Neurodevelopmental Disorders Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Child\u2010 and Adolescent Psychiatry\/Psychology Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3122-7713","authenticated-orcid":false,"given":"L. W.","family":"ten Hoopen","sequence":"additional","affiliation":[{"name":"ENCORE Expertise Centre for Neurodevelopmental Disorders Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Child\u2010 and Adolescent Psychiatry\/Psychology Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. B.","family":"Rietman","sequence":"additional","affiliation":[{"name":"ENCORE Expertise Centre for Neurodevelopmental Disorders Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Child\u2010 and Adolescent Psychiatry\/Psychology Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P. F. A.","family":"de Nijs","sequence":"additional","affiliation":[{"name":"ENCORE Expertise Centre for Neurodevelopmental Disorders Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Child\u2010 and Adolescent Psychiatry\/Psychology Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. H. J.","family":"Hillegers","sequence":"additional","affiliation":[{"name":"Department of Child\u2010 and Adolescent Psychiatry\/Psychology Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H. A.","family":"Moll","sequence":"additional","affiliation":[{"name":"ENCORE Expertise Centre for Neurodevelopmental Disorders Erasmus MC  Rotterdam The Netherlands"},{"name":"Department of Paediatrics Erasmus MC  Rotterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. C. 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