Department of Immunochemistry, Institution of Chemical Engineering, Ural Federal University, Yekaterinburg, Russia; Laboratory for Brain and Neurocognitive Development, Department of Psychology, Institution of Humanities, Ural Federal University, Yekaterinburg, Russia.
Laboratory for Brain and Neurocognitive Development, Department of Psychology, Institution of Humanities, Ural Federal University, Yekaterinburg, Russia.
Prev Med. 2024 Sep;186:108079. doi: 10.1016/j.ypmed.2024.108079. Epub 2024 Jul 23.
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.
This study explores HRQOL in IEI patients, identifies impacting factors, and advocates for increased research focus on their quality of life.
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.
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.
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.
先天免疫缺陷（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。目前的研究缺乏对这一关键问题的全面综合。
本研究探讨 IEI 患者的 HRQOL，确定影响因素，并倡导更多关注他们的生活质量。
按照系统评价和荟萃分析指南，对 Scopus 和 PubMed 进行检索，截至 2023 年 11 月 15 日，共检索到 1633 篇文献。我们评估了文献，评估了研究质量，并比较了 IEI 患者与健康人群和其他慢性病患者的 HRQOL。
在 90 篇文章和 10971 名 IEI 患者中，研究质量各不相同（9 篇为优，63 篇为良，18 篇为差）。短表 36 项（SF-36）和儿科生活质量量表通用核心量表（PedsQL）分别是成人和儿童中使用最广泛的通用工具，各有 12 项研究使用疾病特异性工具。荟萃分析显示，IEI 患者在一般健康、身体和心理健康以及社会和情感角色方面的评分明显低于健康人群。我们注意到自我报告和代理报告之间存在显著差异，表明照顾者的焦虑和感知差异。
尽管存在样本量小和依赖通用工具等局限性，但这项研究强调了 IEI 患者 HRQOL 明显较低，强调需要采用以患者为中心的多学科方法来提高他们的生活质量，并呼吁更加关注 IEI 患者及其照顾者的 HRQOL。
