Tarazi Reem A, Grant Mitzie L, Ely Elizabeth, Barakat Lamia P
Department of Psychology, Drexel University, Philadelphia, PA 19104, USA.
Child Neuropsychol. 2007 Mar;13(2):155-72. doi: 10.1080/09297040600611312.
Cognitive and academic deficits have been identified in school-aged children with sickle cell disease (SCD). However, there have been very few identified studies that examine neuropsychological functioning in preschool-age children with SCD. It is important to understand effects of SCD from a developmental perspective and to consider the contribution of environmental factors in this at-risk population. Neuropsychological functioning of preschool-age children with SCD and no history of overt stroke (n = 26) was examined across several domains (language, immediate memory/brief attention, visuospatial/visuoconstructional, motor/visuomotor). The mean Full Scale IQ for the sample was 89.0. Performance on the Immediate Memory/ Brief Attention domain was significantly higher than the other domains, although the pattern of performance was relatively consistent, with mean standard scores ranging from 88.0 to 95.0. Disease severity was not significantly related to cognitive functioning in this group of young children with SCD. Socioeconomic status (SES) was significantly correlated with most domain scores and, based on regression analyses, accounted for 18% to 47% of the variance in functioning. Psychosocial factors, such as number of children living in the home and parental stress levels, were negatively associated with Motor/Visuomotor skills, and weekly hours in school/day care was positively associated with language-related skills. Results suggest that, at this young age, psychosocial risk factors appear to be appropriate targets for intervention, with the goal of improving long-term outcome in children with SCD. Further investigations should include comparison to a matched control group.
患有镰状细胞病（SCD）的学龄儿童已被发现存在认知和学业缺陷。然而，很少有已确定的研究考察患有SCD的学龄前儿童的神经心理功能。从发育角度理解SCD的影响，并考虑环境因素对这一高危人群的作用非常重要。对26名无明显中风病史的患有SCD的学龄前儿童的神经心理功能在几个领域（语言、即时记忆/短暂注意力、视觉空间/视觉构建、运动/视觉运动）进行了检查。样本的平均全量表智商为89.0。即时记忆/短暂注意力领域的表现显著高于其他领域，尽管表现模式相对一致，平均标准分数在88.0至95.0之间。在这组患有SCD的幼儿中，疾病严重程度与认知功能无显著相关性。社会经济地位（SES）与大多数领域分数显著相关，基于回归分析，其在功能差异中占18%至47%。心理社会因素，如家庭中孩子的数量和父母的压力水平，与运动/视觉运动技能呈负相关，而每周在学校/日托中心的时长与语言相关技能呈正相关。结果表明，在这个年幼阶段，心理社会风险因素似乎是合适的干预目标，目的是改善患有SCD儿童的长期预后。进一步的研究应包括与匹配对照组的比较。
