Patient-reported neurocognitive symptoms influence instrumental activities of daily living in sickle cell disease.

Patient-reported neurocognitive symptoms influence instrumental activities of daily living in sickle cell disease.
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DOI:
10.1002/ajh.26315
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发表时间:
2021-11-01
影响因子:
12.8
通讯作者:
Porter J
Porter J
中科院分区:
医学1区
文献类型:
--
作者:
Longoria JN;Pugh NL;Gordeuk V;Hsu LL;Treadwell M;King AA;Gibson R;Kayle M;Crego N;Glassberg J;Melvin CL;Hankins JS;Porter J

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患有镰状细胞病(SCD)的个体经历神经认知下降,药物依从性低,失业增加,以及工具性日常生活活动(IADL)困难。自我感知的认知困难和IADL之间的关系,包括就业,入学,独立性,参与休闲活动和药物依从性尚不清楚。我们假设,自我报告的神经认知领域的困难将预测较低的IADL技能。多中心镰状细胞病实施联盟(SCDIC)的青少年和成人参与者(n= 2,436)完成了注意力、执行功能、处理速度、学习和理解的患者报告结果(PRO)测量。认知症状作为多变量模型中的预测因子进行分析。结果变量包括:1)IADL复合指标,包括就业、上学、对他人的依赖和休闲活动; 2)羟基脲依从性。参与者报告了注意力(55%),执行功能(51%),处理速度(57%)和阅读理解(65%)领域的认知困难。执行功能障碍(p<0.0001)、有时或经常经历学习困难(p<0.0001和p= 0.0397)和理解力差(p= 0.0002和p= 0.0005),控制年龄(p<0.0001)、疼痛(p<0.0001)和使用羟基脲(p= 0.0001),与IADL技能差相关。执行功能障碍(p= 0.0212),控制年龄(25-34岁p= 0.0129),基因型(p= 0.0006)和血红蛋白(p= 0.0039),预测羟基脲不遵守。PRO测量分析表明,认知功能障碍在SCD青少年和成人中普遍存在。认知功能障碍转化为具有临床意义的结局。认知症状的PRO可用作重要的辅助临床工具,以监测影响功能技能的症状,包括参与社会活动和药物依从性。
Individuals with sickle cell disease (SCD) experience neurocognitive decline, low medication adherence, increased unemployment, and difficulty with instrumental activities of daily living (IADL). The relationship between self-perceived cognitive difficulties and IADLs, including employment, school enrollment, independence, engagement in leisure activities, and medication adherence is unknown. We hypothesized that self-reported difficulties across neurocognitive areas would predict lower IADL skills. Adolescent and adult participants of the multi-site Sickle Cell Disease Implementation Consortium (SCDIC) (n= 2,436) completed patient-reported outcome (PRO) measures of attention, executive functioning, processing speed, learning, and comprehension. Cognitive symptoms were analyzed as predictors in multivariable modeling. Outcome variables included 1) an IADL composite that consisted of employment, participation in school, reliance on others, and leisure pursuits, and 2) hydroxyurea adherence. Participants reported cognitive difficulty across areas of attention (55%), executive functioning (51%), processing speed (57%), and reading comprehension (65%). Executive dysfunction (p<.0001) and sometimes or often experiencing learning difficulties (p<.0001 and p=.0397) and poor comprehension (p=.0002 and p=.0005), controlled for age (p<.0001), pain (p<.0001), and hydroxyurea use (p=.0001), were associated with poor IADL skills. Executive functioning difficulties (p=.0212), controlled for age (p=.0129 for ages 25–34), genotype (p=.0006), and hemoglobin (p=.0039), predicted hydroxyurea non-adherence. Analysis of PRO measures indicated that cognitive dysfunction is prevalent in adolescents and adults with SCD. Cognitive dysfunction translated into clinically meaningful outcomes. PRO of cognitive symptoms can be used as an important adjunct clinical tool to monitor symptoms that impact functional skills, including engagement in societal activities and medication adherence.
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