Liu Elina, Twilt Marinka, Tyrrell Pascal N, Dropol Anastasia, Sheikh Shehla, Gorman Mark, Kim Susan, Cabral David A, Forsyth Rob, Van Mater Heather, Li Suzanne, Huber Adam M, Stringer Elizabeth, Muscal Eyal, Wahezi Dawn, Toth Mary, Dolezalova Pavla, Kobrova Katerina, Ristic Goran, Benseler Susanne M
Rheumatology, Department of Pediatrics, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.
School of Medicine, Queen's University, Kingston, ON, Canada.
Pediatr Rheumatol Online J. 2018 Nov 20;16(1):73. doi: 10.1186/s12969-018-0291-4.
To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning.
This was a multicenter, observational cohort study of children (< 18 years of age) diagnosed with inflammatory brain disease (IBrainD). Patients were included if they had completed at least one Health Related Quality of Life Questionnaire (HRQoL). HRQoL was measured using the Pediatric Quality of Life Inventory Version 4.0 (PedsQL) Generic Core Scales, which provided a total score out of 100. Analyses of trends were performed using linear regression models adjusted for repeated measures over time.
In this study, 145 patients were included of which 80 (55%) were females. Cognitive dysfunction was the most common presenting symptoms (63%), and small vessel childhood primary angiitis of the CNS was the most common diagnosis (33%). The mean child's self-reported PedsQL total score at diagnosis was 68.4, and the mean parent's proxy-reported PedsQL score was 63.4 at diagnosis. Child's self-reported PedsQL scores reflected poor HRQoL in 52.9% of patients at diagnosis. Seizures or cognitive dysfunction at presentation was associated with statistically significant deficits in HRQoL.
Pediatric IBrainD is associated with significantly diminished health-related quality of life. Future research should elucidate why these deficits occur and interventions should focus on improving HRQoL in the most affected subdomains, in particular for children presenting with seizures and cognitive dysfunction.
量化儿科人群中炎症性脑病对健康相关生活质量的影响，包括身体、情绪、学校和社交功能等子领域。
这是一项针对诊断为炎症性脑病（IBrainD）的18岁以下儿童的多中心观察性队列研究。如果患者至少完成了一份健康相关生活质量问卷（HRQoL），则纳入研究。使用儿童生活质量量表第4.0版（PedsQL）通用核心量表测量HRQoL，该量表满分为100分。使用针对时间重复测量进行调整的线性回归模型进行趋势分析。
本研究纳入了145例患者，其中80例（55%）为女性。认知功能障碍是最常见的症状（63%），中枢神经系统小血管儿童原发性血管炎是最常见的诊断（33%）。诊断时儿童自我报告的PedsQL总分平均为68.4，父母代理报告的PedsQL评分平均为63.4。诊断时，52.9%的患者儿童自我报告的PedsQL评分反映出健康相关生活质量较差。发病时的癫痫发作或认知功能障碍与健康相关生活质量的统计学显著缺陷相关。
儿科炎症性脑病与健康相关生活质量显著下降有关。未来的研究应阐明这些缺陷发生的原因，干预措施应侧重于改善受影响最严重的子领域的健康相关生活质量，特别是对于出现癫痫发作和认知功能障碍的儿童。
