D. A. Hagenaar, K. G. C. B. Bindels-de Heus, K. Lubbers, L. W. ten Hoopen, A. B. Rietman, P. F. A. de Nijs, M. H. J. Hillegers, H. A. Moll, M. C. Y. de Wit, G. C. Dieleman, S. E. Mous
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It is unclear which factors influence child HRQoL and parenting stress/impact in AS.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <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-economic status.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>The study sample consisted of 73 children with AS, mean age = 9.1 years, range = 2–18 years. 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.</p>\n </section>\n \n <section>\n \n <h3> Conclusions</h3>\n \n <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-centred approach.</p>\n </section>\n </div>","PeriodicalId":16163,"journal":{"name":"Journal of Intellectual Disability Research","volume":"68 3","pages":"248-263"},"PeriodicalIF":2.1000,"publicationDate":"2023-11-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jir.13106","citationCount":"0","resultStr":"{\"title\":\"Child characteristics associated with child quality of life and parenting stress in Angelman syndrome\",\"authors\":\"D. A. Hagenaar, K. G. C. B. 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引用次数: 0
摘要
背景:Angelman综合征(AS)是一种罕见的神经发育障碍，以严重的智力障碍、运动障碍、癫痫、睡眠问题和行为问题为特征。对AS儿童健康相关生活质量(HRQoL)知之甚少。AS家庭研究报告了育儿压力的增加和儿童综合症对父母的高影响。目前尚不清楚哪些因素影响AS儿童的HRQoL和父母压力/影响。方法:我们通过Erasmus MC Sophia儿童医院ENCORE Angelman综合征专家中心对AS儿童的标准化临床评估收集前瞻性数据。对以下结局变量进行线性回归分析:(1)儿童HRQoL(婴幼儿生活质量问卷);(2)儿童综合症对父母的影响(婴幼儿生活质量问卷);(3)养育压力(养育压力指数)。预测变量为儿童基因型、癫痫、睡眠问题(儿童睡眠障碍量表)、认知发展水平(贝利认知量表)、自闭症特征(自闭症诊断观察表)和情绪/行为问题(儿童行为检查表)。协变量包括性别、年龄和社会经济地位。结果:研究样本为73例AS患儿，平均年龄9.1岁，范围2 ~ 18岁。情绪/行为问题是儿童HRQoL降低的最强显著预测因子。内部化问题推动了这种影响。此外，缺失基因型和年龄越大，儿童HRQoL越低。睡眠问题与孩子的综合症对父母的影响更大有关。最后，情绪/行为问题与较高的养育压力有关。认知发展水平、自闭症特征和癫痫不是儿童HRQoL和父母压力/影响的显著预测因子。结论:这些结果表明，旨在提高儿童HRQoL和减少AS父母压力/影响的干预措施应采用以家庭为中心的方法，重点关注儿童的情绪/行为问题和睡眠问题。
Child characteristics associated with child quality of life and parenting stress in Angelman syndrome
Background
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-related 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.
Methods
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-economic status.
Results
The study sample consisted of 73 children with AS, mean age = 9.1 years, range = 2–18 years. 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.
Conclusions
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-centred approach.
期刊介绍：
The Journal of Intellectual Disability Research is devoted exclusively to the scientific study of intellectual disability and publishes papers reporting original observations in this field. The subject matter is broad and includes, but is not restricted to, findings from biological, educational, genetic, medical, psychiatric, psychological and sociological studies, and ethical, philosophical, and legal contributions that increase knowledge on the treatment and prevention of intellectual disability and of associated impairments and disabilities, and/or inform public policy and practice. Expert reviews on themes in which recent research has produced notable advances will be included. Such reviews will normally be by invitation.
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