Parental Perception of Comorbidities in Children With Dravet Syndrome
Parental Perception of Comorbidities in Children With Dravet Syndrome
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DOI：
10.1016/j.pediatrneurol.2017.06.008
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发表时间：
2017-11-01
影响因子：
3.8
通讯作者：
Dempsey, Amanda
中科院分区：
文献类型：
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作者：
Knupp, Kelly G.;Scarbro, Sharon;Dempsey, Amanda
背景：我们假设Dravet综合征患儿除了癫痫发作和认知功能障碍外，可能还有其他共同特征。为了解决这一知识差距，我们对Dravet综合征儿童的照顾者进行了一项调查，以确定和量化他们对这一人群相关症状的看法。方法：在REDcap（研究电子数据采集）中开发了一项电子调查，并通过电子邮件发送给Dravet综合征基金会电子邮件列表中的参与者。问题集中在饮食，睡眠，行为和其他可能与Dravet综合征有关的症状。使用四点李克特量表（例如，强烈反对（强烈反对）。随后将结果二分以供分析。Logistic回归用于计算可能与症状相关的各种人口统计学因素的比值比。采用后向消除法构建多变量模型，以评估各种症状之间的关系。研究结果：共有202名受访者，96%是Dravet综合征儿童的父母（其余为祖父母或监护人）; 90.5%为女性。受影响儿童的中位年龄为8岁（四分位数范围为5至14），50%为男性，90.5%报告有已知的SCN 1A突变。99%的受访者报告了至少一种与食欲相关的症状，82%的受访者报告了睡眠障碍，三分之一的受访者报告了自主神经症状，75%的受访者报告了步态问题。注意力不集中和持续行为比其他行为障碍更常见。重要性：除了先前报道的症状外，护理人员还可以感知Dravet综合征患儿的许多症状，包括食欲、睡眠、步态和行为。其中许多可能会严重影响儿童和照顾者的生活质量。
BACKGROUND: We hypothesized that children with Dravet syndrome may have additional common features beyond seizures and cognitive impairment. To address this gap in knowledge, we conducted a survey of caregivers of children with Dravet syndrome to identify and quantify their perception of associated symptoms in this population. METHODS: An electronic survey was developed in REDcap (Research Electronic Data Capture) and sent via e-mail to the participants on the Dravet Syndrome Foundation e-mail list. Questions focused on eating, sleep, behavior, and other symptoms that might be related to Dravet syndrome. The questions were assessed using a four-point Likert scale (e.g., strongly agree to strongly disagree). Results were later dichotomized for analysis. Logistic regression was used to calculate odds ratios of various demographic factors potentially associated with symptoms. Multivariable models were constructed using backward elimination to assess the relationship among a variety of symptoms. RESULTS: There were 202 respondents, 96% were parents of a child with Dravet syndrome (the remainder were grandparents or guardians); 90.5% were female. The median age of the affected child was eight years (interquartile range five to 14), 50% were male, and 90.5% were reported to have a known SCN1A mutation. At least one symptom associated with appetite was reported in 99% of respondents, 82% reported a disturbance of sleep, one third reported autonomic symptoms, and 75% reported problems with gait. Inattention and perseveration were reported more commonly than other behavioral disturbances. SIGNIFICANCE: Caregivers have the perception of many symptoms in children with Dravet syndrome in addition to those that have been previously reported, including appetite, sleep, gait, and behavior. Many of these can significantly affect quality of life for both the child and the caregiver.