Towards Understanding Behaviour and Emotions of Children with CLN3 Disease (Batten Disease): Patterns, Problems and Support for Child and Family.
Towards Understanding Behaviour and Emotions of Children with CLN3 Disease (Batten Disease): Patterns, Problems and Support for Child and Family.
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DOI：
10.3390/ijerph19105895
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发表时间：
2022-05-12
影响因子：
--
通讯作者：
Sterkenburg, Paula S.
中科院分区：
文献类型：
--
作者：
Honingh, Aline K.;Kruithof, Yvonne L.;Kuper, Willemijn F. E.;van Hasselt, Peter M.;Sterkenburg, Paula S.
神经元蜡样脂褐质沉积症（CLN 3病/Batten病）的青少年变体是儿童和年轻人中罕见的进行性脑部疾病，其特征是视力丧失，认知和运动能力下降以及癫痫。患有CLN 3疾病的儿童经常表现出行为和情绪不安。本研究的目的是更好地了解CLN 3疾病儿童的行为和情绪，并检查儿童及其父母正在接受的支持。采用定性和定量相结合的方法分析患者档案和家长访谈。采用框架分析方法，制定了一个编码簿，对来源进行了编码，并对数据进行了分析。分析结果概述了（1）CLN 3疾病导致儿童的典型行为和情绪，（2）CLN 3疾病儿童获得的支持，（3）这些儿童的父母获得的支持，以及（4）这些父母面临的问题。对少数儿童来说，他们的视觉、身体或认知能力的退化导致了特定的情绪和行为。定量分析表明，所有儿童都报告了焦虑。所提出的概述支持包含隐性知识的卫生保健专业人员，已明确本研究。这些概述可能会为CLN 3疾病儿童及其父母提供适应性支持模块。
The juvenile variant of Neuronal Ceroid Lipofuscinosis (CLN3 disease/Batten disease) is a rare progressive brain disease in children and young adults, characterized by vision loss, decline in cognitive and motor capacities and epilepsy. Children with CLN3 disease often show disturbed behaviour and emotions. The aim of this study is to gain a better understanding of the behaviour and emotions of children with CLN3 disease and to examine the support that the children and their parents are receiving. A combination of qualitative and quantitative analysis was used to analyse patient files and parent interviews. Using a framework analysis approach a codebook was developed, the sources were coded and the data were analysed. The analysis resulted in overviews of (1) typical behaviour and emotions of children as a consequence of CLN3 disease, (2) the support children with CLN3 disease receive, (3) the support parents of these children receive, and (4) the problems these parents face. For a few children their visual, physical or cognitive deterioration was found to lead to specific emotions and behaviour. The quantitative analysis showed that anxiety was reported for all children. The presented overviews on support contain tacit knowledge of health care professionals that has been made explicit by this study. The overviews may provide a lead to adaptable support-modules for children with CLN3 disease and their parents.
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