雷特综合征的临床表现与生活质量及家庭功能的关系。
Clinical presentation of Rett syndrome in relation to quality of life and family functioning.
作者信息
Rozensztrauch Anna, Sebzda Agnieszka, Śmigiel Robert
机构信息
Department of Paediatrics, Division of Neonatology, Faculty of Health Science, Wroclaw Medical University, Wroclaw, Poland.
Department of Paediatrics, Department of Propaedeutic of Paediatrics and Rare Disorders, Faculty of Health Science, Wroclaw Medical University, Wroclaw, Poland.
出版信息
J Int Med Res. 2021 Apr;49(4):3000605211007714. doi: 10.1177/03000605211007714.
OBJECTIVE
Rett syndrome (RTT) is a chronic condition that manifest in young children, with concomitant comorbidities such as respiratory problems, scoliosis, epilepsy, and malnutrition, which may affect children's quality of life (QoL) and family functioning. The objective of this cross-sectional descriptive correlation study was to understand the clinical presentation of RTT in relation to QoL and family functioning.
METHODS
We included 23 parents of children with RTT. In this study, we used the PedsQL™ Family Impact Module, the Pediatric Quality of Life Inventory 4.0 generic core scales (PedsQL™ 4.0), and an author-designed questionnaire to assess QoL and family functioning.
RESULTS
A significant relationship was observed between PedsQL™ 4.0 score and child's age in the physical functioning dimension. Children aged 8 to 12 years demonstrated significantly higher scores than those in the other age groups. Malnutrition in the child significantly affected functioning of the family in the family relationships dimension. Children receiving 5 hours of rehabilitation treatment a week had significantly higher QoL in the school functioning dimension.
CONCLUSIONS
QOL in children with RTT, as perceived by their parents, is reduced. RTT has a significant negative correlation with family functioning.
目的
雷特综合征（RTT）是一种在幼儿期出现的慢性疾病，伴有呼吸问题、脊柱侧弯、癫痫和营养不良等合并症，这些可能会影响儿童的生活质量（QoL）和家庭功能。这项横断面描述性相关性研究的目的是了解与生活质量和家庭功能相关的RTT临床表现。
方法
我们纳入了23名RTT患儿的家长。在本研究中，我们使用了儿童生活质量量表家庭影响模块、儿童生活质量量表4.0通用核心量表（PedsQL™ 4.0）以及作者设计的问卷来评估生活质量和家庭功能。
结果
在身体功能维度上，观察到PedsQL™ 4.0得分与儿童年龄之间存在显著关系。8至12岁的儿童得分显著高于其他年龄组。儿童营养不良在家庭关系维度上显著影响家庭功能。每周接受5小时康复治疗的儿童在学校功能维度上的生活质量显著更高。
结论
家长认为RTT患儿的生活质量有所下降。RTT与家庭功能存在显著负相关。
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