Neurocognitive Deficits in Children With Sickle Cell Disease Are Associated With the Severity of Anemia
Neurocognitive Deficits in Children With Sickle Cell Disease Are Associated With the Severity of Anemia
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DOI：
10.1002/pbc.22892
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发表时间：
2011-08-01
影响因子：
3.2
通讯作者：
Fijnvandraat, Karin
中科院分区：
文献类型：
--
作者：
Hijmans, Channa T.;Grootenhuis, Martha A.;Fijnvandraat, Karin
背景。尽管镰状细胞病（SCD）儿童的神经认知缺陷已有充分记录，但这些缺陷的病因尚未完全阐明。本研究的目的是调查疾病严重程度的实验室标志物和放射学参数与 SCD 儿童神经认知功能的关联。设计和方法。参与者是 37 名年龄 6-18 岁的 SCD（(HbSS 或 HbS-beta(0)-地中海贫血）儿童。所有参与者都接受了广泛的神经认知评估。进一步的数据（TCD 值、实验室测试结果和 MRI 数据）从医学图表中获得。通过分层回归分析对关联进行分析。结果。血红蛋白与言语短期记忆下降相关。当控制为患有无症状梗塞的儿童与 MRI 正常的儿童相比，通过连续动脉自旋标记 (CASL) MRI 测量的脑血流不对称的儿童比没有不对称的儿童具有更好的持续注意力。障碍，可能影响他们的学业发展和全面参与社会。2011；57：297302。（C）2010 Wiley-Liss，Inc。
Background. Although neurocognitive deficits in children with sickle cell disease (SCD) have been well documented, the etiology of these deficits has not been completely clarified. The aim of this study was to investigate the association of laboratory markers of disease severity and radiological parameters with neurocognitive functioning in children with SCD. Design and Methods. Participants were 37 children with SCD ((HbSS or HbS-beta(0)-thalassemia) aged 6-18 years. All participants underwent extensive neurocognitive assessment. Further data (TCD values, laboratory test results, and MRI data) were obtained from medical charts. Associations were analyzed by hierarchical regression analysis. Results. Hemoglobin was associated with a decrease in verbal short-term memory. There was no association between TCD velocities and neurocognitive functioning, when controlled for age. Children with silent infarcts did not differ from children with normal MRI in neurocognitive functioning. Children with right-left asymmetries in cerebral blood flow as measured by continuous arterial spin labelling (CASL) MRI had better sustained attention than children without asymmetries. Conclusions. Neurocognitive deficits are associated with the severity of anemia, indicating reduced oxygen delivery to the brain as an etiological mechanism. This implies that children with SCD and normal MRIs may still suffer from neurocognitive impairments, possibly affecting their academic development and full participation in society. Pediatr Blood Cancer. 2011; 57: 297302. (C) 2010 Wiley-Liss, Inc.