Development and validation of an epidermolysis bullosa family/parental burden score
Development and validation of an epidermolysis bullosa family/parental burden score
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DOI：
10.1111/bjd.14072
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发表时间：
2015-12-01
影响因子：
10.3
通讯作者：
Bodemer, C.
中科院分区：
文献类型：
--
作者：
Dufresne, H.;Hadj-Rabia, S.;Bodemer, C.
与疾病相关的个人负担的概念已被引入，以确定最广泛意义上的“残疾”：心理、社会、经济和身体。亚型大疱性表皮松解症（EB）是罕见的，危及生命的，无法治疗的慢性遗传性皮肤病。目的开发并验证一份评估EB儿童家庭负担的问卷：大疱性表皮松解症疾病负担（EB- bod）。方法采用EB患儿家长的逐字报告生成项目。随后，实施了一项心理测量分析研究。根据问题间的相关性、专家的共识和探索性因素分析，通过项目缩减来完善大疱性表皮松解症疾病负担问卷。通过计算Cronbach’s a来确定内部一致性。通过计算EB-BoD与SF-12问卷之间的相关性来确定并发效度。结果通过探索性因素分析，EB-BoD问卷从最初的30个项目缩减为20个项目，涵盖疾病的4个方面。结构效度得到验证，EB-BoD问卷具有良好的内部一致性（Cronbach’s alpha = 0.9）。EB-BoD评分与SF-12的心理维度显著相关（r = -0.61），但与SF-12的身体维度不相关（r = 0.04）。EB- bod评分在EB亚型之间存在显著差异。EB- bod问卷似乎是EB患者及其家庭医疗和社会经济问题的有用评估工具。EB- bod评分与EB患者家庭所经历的家庭/父母负担有很好的相关性。
Background The notion of the individual burden associated with a disease has been introduced to determine 'disability' in the broadest sense: psychological, social, economic and physical. Subtypes of epidermolysis bullosa (EB) are rare, life-threatening, untreatable chronic genodermatoses.Objectives To develop and validate a specific questionnaire assessing the burden on families of children with EB: Epidermolysis Bullosa Burden of Disease (EB-BoD).Methods Items were generated by a verbatim report from parents of children with EB. Subsequently, a study was implemented for psychometric analysis. An epidermolysis bullosa burden-of-disease questionnaire was refined via item reduction according to inter-question correlations, consensus among experts and exploratory factor analysis. Internal consistency was determined by calculating Cronbach's a. Concurrent validity was determined by calculating the correlation between EB-BoD and the Short-Form 12 items (SF-12) questionnaire.Results From a primary list of 30 items, EB-BoD was reduced to a 20-item questionnaire, covering four disease aspects based on the exploratory factor analysis. Construct validity was demonstrated and the EB-BoD questionnaire showed good internal consistency (Cronbach's alpha = 0.9). The resulting EB-BoD score was significantly correlated to the mental dimension of SF-12 (r = -0.61), but it was not correlated to it's physical dimension (r = 0.04). EB-BoD scores were significantly discriminating between EB subtypes.Conclusions The EB-BoD questionnaire appears to be a useful assessment tool regarding medical and socioeconomic issues in patients with EB and their families. EB-BoD scores correlate well with the family/parental burden experienced by the families of patients with EB.