Eligibility criteria.

Background
As the life expectancy of adults (aged ≥ 18 years) with Down syndrome increases for a plethora of reasons including recognition of rights, access, and technological and medical advances, there is a need to collate evidence about their quality of life.
Objective
Using Schalock and Verdugo’s multidimensional quality of life assessment model, this systematic review aimed to identify, synthesise and integrate the quantitative and qualitative evidence on quality of life in adults with Down syndrome via self-and proxy-reporting.
Methods
Five databases were systematically searched: MEDLINE, CINAHL, PsycINFO, Scopus, and Web of Science to identify relevant articles published between 1980 and 2022 along with grey literature and reference lists from relevant studies. A mixed methods systematic review was performed according to the Joanna Briggs Institute methodology using the convergent integrated approach. The review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.
Results
Thirty-nine studies were included: 20 quantitative, 17 qualitative, and 2 mixed methods studies. The synthesised findings were grouped into the 8 core domains of quality of life: personal development, self-determination, interpersonal relations, social inclusion, rights, emotional, physical and material well-being. Of the 39 studies, 30 (76.92%) reported on emotional well-being and 10 (25.64%) on rights. Only 7 (17.94%) studies reported that adults with Down syndrome have a good quality of life centred around self-determination and interpersonal relations. Most adults with Down syndrome wanted to become more independent, have relationships, participate in the community, and exercise their human rights. Self-reported quality of life from adults with Down syndrome was rated higher than proxy reported quality of life. Discrepancies in quality of life instruments were discovered.
Conclusion
This review highlighted the need for a better systematic approach to improving the quality of life in adults with Down syndrome in targeted areas. Future research is required to evaluate self-and proxy-reporting methods and culture-specific quality of life instruments that are more appropriate for adults with Down syndrome. In addition, further studies should consider including digital assistive technologies to obtain self-reported quality of life data in adults with Down syndrome.
International prospective register of systematic reviews registration number
CRD42019140056.

背景
随着权利认可、权益保障、技术与医学进步等多重因素的推动，唐氏综合征成人（年龄≥18岁）的预期寿命不断延长，当前亟需整合关于其生活质量的相关研究证据。

目的
本研究基于沙洛克（Schalock）与韦尔杜戈（Verdugo）提出的多维生活质量评估模型，旨在通过自我报告与代理报告两种方式，识别、综合并整合唐氏综合征成人生活质量的定量与定性研究证据。

方法
本研究系统检索了MEDLINE、CINAHL、PsycINFO、Scopus及Web of Science五大数据库，辅以灰色文献检索以及相关研究的参考文献列表，筛选出1980年至2022年间发表的符合要求的文献。本研究遵循乔安娜·布里格斯研究所（Joanna Briggs Institute）方法学框架，采用收敛整合法开展混合研究系统综述，并严格遵循《系统综述与荟萃分析优先报告条目》（Preferred Reporting Items for Systematic Reviews and Meta-Analyses, PRISMA）指南进行报告。

结果
本研究共纳入39项研究，其中定量研究20项、定性研究17项、混合研究2项。综合后的研究结果被划分为生活质量的8个核心维度：个人发展、自我决定、人际关系、社会包容、权利保障、情感健康、躯体健康与物质福祉。在39项研究中，30项（76.92%）聚焦于情感健康维度，10项（25.64%）聚焦于权利保障维度。仅有7项（17.94%）研究表明，唐氏综合征成人的生活质量以自我决定与人际关系为核心且处于良好水平。多数唐氏综合征成人希望获得更高独立性、建立人际关系、参与社区活动并行使自身人权。唐氏综合征成人的自我报告生活质量评分高于代理报告评分。本研究同时发现不同生活质量评估工具间存在差异。

结论
本综述凸显了针对特定领域改善唐氏综合征成人生活质量的系统性优化路径的必要性。未来需开展研究以评估自我报告与代理报告方法，以及更适配唐氏综合征成人的文化特异性生活质量评估工具。此外，后续研究应考虑引入数字辅助技术，以获取唐氏综合征成人的自我报告生活质量数据。

国际系统综述前瞻性注册编号
CRD42019140056