White D A, DeBaun M
Department of Psychology, Washington University, St. Louis, Missouri 63130, USA.
J Pediatr Hematol Oncol. 1998 Sep-Oct;20(5):458-62. doi: 10.1097/00043426-199809000-00009.
A review of the literature regarding cognitive and behavioral function in children with sickle cell disease (SCD) is presented, including a discussion of methodological issues to be considered when evaluating these patients and research in this area.
Sixteen studies were examined that addressed cognition and behavior in children with SCD.
Most studies of children with SCD who were classified as neurologically normal probably included a substantial number of children who had experienced silent strokes; this misclassification error likely resulted in erroneous findings of deficits in children thought to be free of brain injury. In most studies of children with SCD-related infarct, investigators failed to consider that patterns of impairment will vary depending upon lesion location. Many studies relied upon data from inappropriate control groups when examining the function of children with SCD.
Future studies should address a number of methodological factors. Brain imaging must be conducted to verify the presence or absence of cerebral infarcts. In children with imaging-verified infarcts, greater attention must be given to the relationship between lesion location and distinct patterns of cognitive or behavioral deficits. Sibling controls are the best choice in studies of children with SCD.
对有关镰状细胞病（SCD）患儿认知和行为功能的文献进行综述，包括讨论评估这些患者时应考虑的方法学问题以及该领域的研究情况。
审查了16项涉及SCD患儿认知和行为的研究。
大多数将患有SCD的儿童归类为神经功能正常的研究，可能纳入了大量曾经历过无症状性卒中的儿童；这种错误分类可能导致认为未受脑损伤的儿童出现缺陷的错误研究结果。在大多数关于与SCD相关梗死的儿童研究中，研究人员没有考虑到损伤模式会因病变位置而异。许多研究在检查SCD患儿的功能时依赖于来自不适当对照组的数据。
未来的研究应关注一些方法学因素。必须进行脑成像以核实是否存在脑梗死。对于经成像证实有梗死的儿童，必须更加关注病变位置与不同认知或行为缺陷模式之间的关系。在SCD患儿研究中，同胞对照是最佳选择。
