背景:先天性免疫错误(IEI)显著影响患者的健康相关生活质量(HRQOL),比健康人群和其他慢性病患者面临更大的挑战。当前的研究缺乏对这一关键问题的全面整合。
目的:本研究探讨IEI患者的HRQOL,确定影响因素,和倡导者增加研究关注他们的生活质量。
方法:遵循系统综述和荟萃分析指南,直到2023年11月15日,对Scopus和PubMed的搜索产生了1633种出版物。我们评估了文献,评估研究质量,并将IEI患者的HRQOL与健康个体和其他慢性病患者的HRQOL进行了比较。
结果:分析了90篇文章和10,971例IEI患者,学习质量各不相同(九个好,63中度,和18个穷人)。简表-36(SF-36)和儿科生活质量量表通用核心量表(PedsQL)是成人和儿童使用的主要通用工具,分别,12项研究均使用疾病特异性仪器。Meta分析显示IEI患者的一般健康状况评分明显较低,身心健康,与健康人群相比,社会和情感角色。我们注意到自我报告和代理报告之间的显著差异,表明护理人员焦虑和感知差异。
结论:尽管存在小样本量和对通用仪器的依赖等限制,这项研究强调了IEI患者的HRQOL显著较低,强调需要以病人为中心,多学科的方法,以提高他们的生活质量,并呼吁更多关注IEI患者及其照顾者的HRQOL。
BACKGROUND: Inborn Errors of Immunity (IEI) significantly affect patients\' health-related quality of life (HRQOL), presenting greater challenges than those faced by the healthy population and other chronic disease sufferers. Current research lacks comprehensive integration of this critical issue.
OBJECTIVE: This study explores HRQOL in IEI patients, identifies impacting factors, and advocates for increased research focus on their quality of life.
METHODS: Following systematic review and meta-analysis guidelines, a search of Scopus and PubMed until November 15, 2023, yielded 1633 publications. We evaluated the literature, assessed study quality, and compared the HRQOL of IEI patients to that of healthy individuals and other chronic disease patients.
RESULTS: Of 90 articles and 10,971 IEI patients analyzed, study quality varied (nine good, 63 moderate, and 18 poor). The Short Form-36 (SF-36) and Pediatric Quality of Life Inventory generic core scales (PedsQL) were the primary generic instruments used among adults and children, respectively, with 12 studies each using the disease-specific instruments. Meta-analysis showed IEI patients have significantly lower scores in general health, physical and mental health, and social and emotional roles compared to healthy populations. We noted significant differences between self and proxy reports, indicating caregiver anxiety and perception disparities.
CONCLUSIONS: Despite limitations like small sample sizes and reliance on generic instruments, this research underscores the substantially lower HRQOL among IEI patients, emphasizing the need for a patient-centered, multidisciplinary approach to improve their life quality and calling for more focused attention on IEI patients and their caregivers\' HRQOL.