Neurocognitive Deficits in Children With Sickle Cell Disease: A Comprehensive Profile
Neurocognitive Deficits in Children With Sickle Cell Disease: A Comprehensive Profile
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DOI：
10.1002/pbc.22879
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发表时间：
2011-05-01
影响因子：
3.2
通讯作者：
Oosterlaan, Jaap
中科院分区：
文献类型：
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作者：
Hijmans, Channa T.;Fijnvandraat, Karin;Oosterlaan, Jaap
背景镰状细胞病（SCD）可导致严重的脑损伤，并伴有神经认知缺陷。本研究的目的是评估与SES匹配的对照组相比，SCD儿童的广泛神经认知功能，以便更深入地了解这些患者的具体缺陷。方法.对41例纯合子SCD（HbSS或HbS-beta 0-地中海贫血）儿童和38例对照儿童进行了一套全面的神经认知功能的明确和有效的测量。除了一般智力，我们还广泛评估了执行功能（包括反应抑制，持续注意力，计划，视觉空间工作记忆和言语工作记忆）以及视觉运动功能。结果SCD明显与较低的IQ分数相关。超过三分之一的SCD儿童的全量表智商低于75。此外，患有SCD的儿童表现出视觉运动功能的缺陷。执行功能障碍的一些证据被发现：患有SCD的儿童表现出视觉空间工作记忆差，以及持续注意力和计划的微妙缺陷。在反应抑制和言语工作记忆方面，SCD儿童与对照儿童之间没有显著差异。结论.患有SCD的儿童智力低下、视觉运动障碍和执行功能障碍的风险增加。这些神经认知缺陷可能是这些儿童学业障碍发生率高的原因。本研究结果进一步阐明了定期神经认知评估和未来神经认知康复计划对SCD儿童的重要性。儿科血液癌症2011;56：783-788。(c)2010 Wiley-Liss，Inc.
Background. Sickle cell disease (SCD) can lead to profound cerebral damage, associated with neurocognitive deficits. The aim of the current study was to evaluate a broad range of neurocognitive functions in children with SCD compared to a SES-matched control group, in order to gain more insight into the specific deficits of these patients. Methods. Forty-one children with homozygous SCD(HbSS or HbS-beta 0-thalassemia) and 38 controls were assessed on a comprehensive set of well-defined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuo-spatial working memory, and verbal working memory) as well as visuo-motor functioning. Results. SCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Full-scale IQ below 75. Furthermore, children with SCD showed deficits in visuo-motor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuo-spatial working memory, as well as subtle deficits in sustained attention and planning. No significant differences were found between children with SCD and controls in terms of response inhibition and verbal working memory. Conclusions. Children with SCD are at increased risk of lower intelligence, visuo-motor impairments, and executive dysfunction. These neurocognitive deficits may underlie high rates of scholastic impairments in these children. The present findings further illuminate the importance of regular neurocognitive evaluations and future neurocognitive rehabilitation programs for children with SCD. Pediatr Blood Cancer 2011;56:783-788. (c) 2010 Wiley-Liss, Inc.