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Health comorbidities and cognitive abilities across the lifespan in Down syndrome.
Journal of Neurodevelopmental Disorders ( IF 4.1 ) Pub Date : 2020-01-23 , DOI: 10.1186/s11689-019-9306-9
Carla M Startin 1, 2, 3 , Hana D'Souza 3, 4, 5 , George Ball 3, 4 , Sarah Hamburg 1, 2, 3 , Rosalyn Hithersay 1, 2, 3 , Kate M O Hughes 3, 4, 6 , Esha Massand 3, 4 , Annette Karmiloff-Smith 3, 4 , Michael S C Thomas 3, 4 , , Andre Strydom 1, 2, 3, 7
Affiliation  

BACKGROUND Down syndrome (DS) is associated with variable intellectual disability and multiple health and psychiatric comorbidities. The impact of such comorbidities on cognitive outcomes is unknown. We aimed to describe patterns of physical health and psychiatric comorbidity prevalence, and receptive language ability, in DS across the lifespan, and determine relationships with cognitive outcomes. METHODS Detailed medical histories were collected and cognitive abilities measured using standardised tests for 602 individuals with DS from England and Wales (age range 3 months to 73 years). Differences in prevalence rates between age groups and between males and females were determined using chi-squared or Fisher's exact tests. In adults, rates for psychiatric comorbidities were compared to expected population rates using standardised morbidity ratios (SMRs). Adapted ANCOVA functions were constructed to explore age and sex associations with receptive language ability across the lifespan, and regression analyses were performed to determine whether the presence of health comorbidities or physical phenotypes predicted cognitive abilities. RESULTS Multiple comorbidities showed prevalence differences across the lifespan, though there were few sex differences. In adults, SMRs were increased in males and decreased in females with DS for schizophrenia, bipolar disorder, and anxiety. Further, SMRs were increased in both males and females with DS for dementia, autism, ADHD, and depression, with differences more pronounced in females for dementia and autism, and in males for depression. Across the lifespan, receptive language abilities increasingly deviated from age-typical levels, and males scored poorer than females. Only autism and epilepsy were associated with poorer cognitive ability in those aged 16-35 years, with no relationships for physical health comorbidities, including congenital heart defects. CONCLUSIONS Our results indicate the prevalence of multiple comorbidities varies across the lifespan in DS, and in adults, rates for psychiatric comorbidities show different patterns for males and females relative to expected population rates. Further, most health comorbidities are not associated with poorer cognitive outcomes in DS, apart from autism and epilepsy. It is essential for clinicians to consider such differences to provide appropriate care and treatment for those with DS and to provide prognostic information relating to cognitive outcomes in those with comorbidities.

中文翻译:


唐氏综合症患者一生中的健康合并症和认知能力。



背景唐氏综合症(DS)与不同程度的智力障碍以及多种健康和精神合并症有关。此类合并症对认知结果的影响尚不清楚。我们的目的是描述 DS 患者整个生命周期的身体健康和精神合并症患病率以及接受语言能力的模式,并确定与认知结果的关系。方法 我们收集了来自英格兰和威尔士的 602 名 DS 患者(年龄范围为 3 个月至 73 岁)的详细病史,并使用标准化测试测量了认知能力。使用卡方检验或费舍尔精确检验确定年龄组之间以及男性和女性之间患病率的差异。在成人中,使用标准化发病率(SMR)将精神合并症的发生率与预期人口发生率进行比较。构建适应性ANCOVA函数来探索整个生命周期中年龄和性别与接受语言能力的关系,并进行回归分析以确定健康合并症或身体表型的存在是否可以预测认知能力。结果多种合并症在整个生命周期中的患病率存在​​差异,但性别差异很少。在成人中,患有精神分裂症、双相情感障碍和焦虑症的 DS 的男性 SMR 增加,而女性 SMR 减少。此外,患有痴呆、自闭症、多动症和抑郁症 DS 的男性和女性 SMR 均有所增加,其中女性痴呆和自闭症以及男性抑郁症的差异更为明显。在整个生命周期中,接受语言的能力越来越偏离年龄典型水平,男性的得分低于女性。 只有自闭症和癫痫与 16-35 岁人群认知能力较差有关,与身体健康合并症(包括先天性心脏缺陷)无关。结论 我们的结果表明,多种合并症的患病率在 DS 患者的整个生命周期中存在差异,而在成人中,相对于预期人口比率,男性和女性的精神合并症发生率显示出不同的模式。此外,除了自闭症和癫痫之外,大多数健康合并症与 DS 较差的认知结果无关。临床医生必须考虑这些差异,以便为 DS 患者提供适当的护理和治疗,并为患有合并症的患者提供与认知结果相关的预后信息。
更新日期:2020-04-22
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