Cognitive and fine motor deficits in a pediatric sickle cell disease cohort of mixed ethnic origin
Cognitive and fine motor deficits in a pediatric sickle cell disease cohort of mixed ethnic origin
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DOI：
10.1007/s00277-016-2861-1
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发表时间：
2017-02-01
影响因子：
3.5
通讯作者：
Driever, Pablo Hernaiz
中科院分区：
文献类型：
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作者：
Burkhardt, Luise;Lobitz, Stephan;Driever, Pablo Hernaiz
脑血管疾病是儿童镰状细胞病(SCD)的一个重要特征，可导致认知和运动障碍。我们的横断面研究考察了柏林混血血统的无临床脑血管事件的儿科队列中这些损害的发生率和严重程度。采用阿姆斯特丹神经心理任务(ANT)程序和伦敦塔(TOL)对32例SCD患者(平均年龄11.14岁，范围7.0~17.25岁；男性14例)进行全面智商(德国版WISC-III)、精细运动功能(数字写字板)和执行功能(计划、注意力、工作记忆和视觉空间能力)的评估。从病历中检索有关临床危险因素的数据。患者的满分智商得到保留，而操作智商显著降低(91.19(SD12.17)d=0.7，p=0.007)。在执行功能和精细运动功能测试中，SCD患者的得分明显低于健康对照组，例如，在TOL中的计划时间(6.73SD3.21)比健康对照组的5.9 S(SD2.33)，d=0.50，p=0.001；在写字板上的频率(平均z得分-0.79，d=0.7，p<；0.001)。没有临床危险因素与认知和运动障碍的发生率和严重程度显著相关。尽管保留了完整的智商，但我们的混合血统的SCD队列显示出较差的执行能力和精细运动技能。我们的研究受到我们队列规模较小以及缺乏对调节较高功能的社会人口和社会经济因素的控制的限制，但强调了对这些缺陷进行早期筛查、预防和具体干预的必要性。
Cerebrovascular disease is an important feature of pediatric sickle cell disease (SCD) and may lead to cognitive and motor impairment. Our cross-sectional study examined the incidence and severity of these impairments in a pediatric cohort without clinical cerebrovascular events from Berlin of mixed ethnic origin. Thirty-two SCD patients (mean age 11.14 years, range 7.0-17.25 years; males 14) were evaluated for full-scale intelligence (IQ) (German version WISC-III), fine motor function (digital writing tablet), and executive function (planning, attention, working memory, and visual-spatial abilities) with the Amsterdam Neuropsychological Tasks (ANT) program and the Tower of London (ToL). Data on clinical risk factors were retrieved from medical records. Full-scale IQ of patients was preserved, whereas performance IQ was significantly reduced (91.19 (SD 12.17) d = 0.7, p = 0.007). SCD patients scored significantly lower than healthy peers when tested for executive and fine motor functions, e.g., planning time in the ToL (6.73 s (SD 3.21) vs. 5.9 s in healthy peers (SD 2.33), d = 0.5, p = < 0.001) and frequency on the writing tablet (mean z score -0.79, d = 0.7, p < 0.001). No clinical risk factors were significantly associated with incidence and severity of cognitive and motor deficits. Despite the preservation of full-scale IQ, our SCD cohort of mixed origin exhibited inferior executive abilities and reduced fine motor skills. Our study is limited by the small size of our cohort as well as the lack for control of sociodemographic and socioeconomic factors modulating higher functions but highlights the need for early screening, prevention, and specific interventions for these deficits.