Hand Function and Quality of Life in Children with Epidermolysis Bullosa
Hand Function and Quality of Life in Children with Epidermolysis Bullosa
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DOI：
10.1111/pde.12262
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发表时间：
2014-03-01
影响因子：
1.5
通讯作者：
Cornwall, Roger
中科院分区：
文献类型：
--
作者：
Eismann, Emily A.;Lucky, Anne W.;Cornwall, Roger
患者报告的结果对临床护理变得越来越重要。大疱性表皮病（EB）是一种罕见的遗传性皮肤病，可导致严重的手部损伤，但父母和患者对手部功能的看法从未被评估过。本研究旨在量化父母和患者报告的手功能，并评估其与EB儿童生活质量（QOL）的关系。这项横断面研究包括在跨学科EB中心接受治疗的EB儿童。检索了医院记录中的人口统计学特征和病史。符合条件的家庭被邀请通过邮件或电话完成两项调查。ABILHAND-Kids问卷评估了21种功能的手动能力。大疱性表皮病患者生活质量问卷评估了EB相关的生活质量。使用Mann-Whitney检验比较不同亚型的手功能和生活质量。来自美国20个州的71名父母和2至18岁的EB患者完成了问卷调查。隐性营养不良型EB患儿的手功能和生活质量最差。涉及手指活动性的双手功能对所有EB亚型都是最具挑战性的。QOL与手功能的程度高度相关，与21个单独的手功能中的20个相关，并且大多数与执行单手功能的能力相关。父母和患者报告的手功能可以使用ABILHAND-Kids问卷在EB儿童中测量。手部损伤与较差的生活质量密切相关，可能是由于难以进行日常活动。干预措施的效果，如手外科手术可以使用该问卷进行前瞻性评估。
Patient-reported outcomes are becoming increasingly important to clinical care. Epidermolysis bullosa (EB), a rare genetic skin disorder, can result in severe hand impairment, but parent and patient perceptions of hand function have never been assessed. This study aimed to quantify parent- and patient-reported hand function and assess its relationship with quality of life (QOL) in children with EB. This cross-sectional study included children with EB treated at an interdisciplinary EB center. Hospital records were searched for demographic characteristics and medical history. Eligible families were invited to complete two surveys by mail or telephone. The ABILHAND-Kids questionnaire assessed manual hand ability for 21 functions. The Quality of Life in Epidermolysis Bullosa questionnaire assessed EB-related QOL. Hand function and QOL of various subtypes were compared using Mann-Whitney tests. Seventy-one parents and patients ages 2 to 18 years with EB from 20 states in the United States completed questionnaires. Children with recessive dystrophic EB reported the worst hand function and QOL. Bimanual functions involving finger mobility were the most challenging for all EB subtypes. QOL was highly related to the degree of hand function, being correlated with 20 of the 21 individual hand functions and most associated with the ability to perform unimanual functions. Parent- and patient-reported hand function can be measured in children with EB using the ABILHAND-Kids questionnaire. Hand impairment is strongly associated with worse QOL, probably due to difficulty performing daily activities. The effect of interventions such as hand surgery could be prospectively evaluated using this questionnaire.