Association of Dementia With Mortality Among Adults With Down Syndrome Older Than 35 Years
Association of Dementia With Mortality Among Adults With Down Syndrome Older Than 35 Years
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DOI：
10.1001/jamaneurol.2018.3616
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发表时间：
2019-02-01
期刊：
影响因子：
29
通讯作者：
Strydom, Andre
中科院分区：
文献类型：
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作者：
Hithersay, Rosalyn;Startin, Carla M.;Strydom, Andre
重要性这项工作量化的致命负担与阿尔茨海默病的个体与唐氏综合征（DS）。目的探讨与阿尔茨海默病相关的痴呆症与死亡率的关联，并检查与痴呆症的成年人与DS。设计，设置和参与者前瞻性的纵向研究在英国的社区设置。数据收集始于2012年3月29日。病例于2017年12月13日删失。潜在样本包括来自伦敦唐氏综合征联盟队列的所有36岁及以上的成人，记录了2个数据时间和痴呆状态（N = 300）; 6例退出研究，28例失访，55例在分析时有一个数据收集点。最终样本包括211名参与者，503.92人-年的随访。暴露痴呆状态，年龄，性别，载脂蛋白E基因型，智力残疾水平，健康变量和生活状况。主要结果和指标粗死亡率，死亡时间，痴呆诊断时间与预测因子的比例风险。96例为女性（45.5%），66例（31.3%）有临床痴呆诊断。27名参与者（11名女性;平均死亡年龄，56.74岁）在研究期间死亡。70%患有痴呆症。痴呆症患者的粗死亡率（1191.85例死亡/10000人-年; 95%CI，1168.49-1215.21）是非痴呆症患者的5倍（232.22例死亡/10000人-年; 95%CI，227.67-236.77）。对于痴呆患者，APOE β 4携带者的死亡风险增加7倍（风险比[HR]，6.91; 95%CI，1.756-27.195）。对于无痴呆的患者，36岁以后发作的癫痫与死亡率相关（HR，9.66; 95%CI，1.59-58.56）。载脂蛋白E β 4携带者（HR，4.91; 95% CI，2.53-9.56），早发性癫痫成人（HR，3.61; 95% CI，1.12-11.60），多种健康合并症（HR，1.956; 95%CI，1.087-3.519），和那些与家人住在一起的人（HR，2.14; 95%CI，1.08-4.20）收到了显着更早的痴呆diagnosis.Conclusions和相关性痴呆与死亡率在70%的老年人与DS。APOE β 4携带者和/或患有多种共病的健康状况的人患痴呆症和死亡的风险增加，突出了良好医疗保健的必要性。对于那些没有痴呆症诊断而死亡的人来说，晚发性癫痫是与死亡相关的唯一重要因素，这引发了对该组潜在未诊断的痴呆症病例的质疑。
IMPORTANCE This work quantifies the fatal burden of dementia associated with Alzheimer disease in individuals with Down syndrome (DS).OBJECTIVE To explore the association of dementia associated with Alzheimer disease with mortality and examine factors associated with dementia in adults with DS.DESIGN, SETTINGS AND PARTICIPANTS Prospective longitudinal study in a community setting in England. Data collection began March 29, 2012. Cases were censored on December 13, 2017. The potential sample consisted of all adults 36 years and older from the London Down Syndrome Consortium cohort with 2 data times and dementia status recorded (N = 300); 6 withdrew from study, 28 were lost to follow-up, and 55 had a single data collection point at time of analysis. The final sample consisted of 211 participants, with 503.92 person-years' follow-up.EXPOSURES Dementia status, age, sex, APOE genotype, level of intellectual disability, health variables, and living situation.MAIN OUTCOMES AND MEASURES Crude mortality rates, time to death, and time to dementia diagnosis with proportional hazards of predictors.RESULTS Of the 211 participants, 96 were women (45.5%) and 66 (31.3%) had a clinical dementia diagnosis. Twenty-seven participants (11 female; mean age at death, 56.74 years) died during the study period. Seventy percent had dementia. Crude mortality rates for individuals with dementia (1191.85 deaths per 10 000 person-years; 95% CI, 1168.49-1215.21) were 5 times higher than for those without (232.22 deaths per 10 000 person-years; 95% CI, 227.67-236.77). For those with dementia, APOE epsilon 4 carriers had a 7-fold increased risk of death (hazard ratio [HR], 6.91; 95% CI, 1.756-27.195). For those without dementia, epilepsy with onset after age 36 years was associated with mortality (HR, 9.66; 95% CI, 1.59-58.56). APOE epsilon 4 carriers (HR, 4.91; 95% CI, 2.53-9.56), adults with early-onset epilepsy (HR, 3.61; 95% CI, 1.12-11.60), multiple health comorbidities (HR, 1.956; 95% CI, 1.087-3.519), and those living with family (HR, 2.14; 95% CI, 1.08-4.20) received significantly earlier dementia diagnoses.CONCLUSIONS AND RELEVANCE Dementia was associated with mortality in 70% of older adults with DS. APOE epsilon 4 carriers and/or people with multiple comorbid health conditions were at increased risk of dementia and death, highlighting the need for good health care. For those who died without a dementia diagnosis, late-onset epilepsy was the only significant factor associated with death, raising questions about potentially undiagnosed dementia cases in this group.