Department of Psychology and Human Development, Vanderbilt University.
Department of Pediatrics, Vanderbilt University Medical Center.
J Pediatr Psychol. 2019 Sep 1;44(8):948-958. doi: 10.1093/jpepsy/jsz031.
To provide a comprehensive quantitative review of neurocognitive function in sickle cell disease (SCD) across multiple domains, cerebral infarct status, and the lifespan.
One hundred and ten studies were identified in PubMed, MedLine, and PsycINFO involving 110 studies of 3,600 participants with SCD and 1,127 sibling or health controls.
Meta-analytic findings indicate significant deficits across all neurocognitive domains, age groups, and infarct status. Significant deficits relative to the normative mean ranged from Hedges' g = -.39 to g = -.63 in preschool children, g = -.83 to g = -1.18 in school-aged children and adolescents, and g = -.46 to g = -.86 in adults. Deficits in full scale IQ (FSIQ), verbal reasoning, perceptual reasoning, and executive function increased from preschool to school-aged samples. However, findings also showed that deficits were smaller in adult samples relative to school-aged samples, likely due to sampling bias in adult studies. Findings across infarct status in sickle cell anemia showed that deficits ranged from g = -.54 to g = -.65 in samples without infarcts, g = -.52 to g = -1.03 in samples with silent cerebral infarct, and g = -1.35 to g = -1.82 in samples with stroke. Deficits in each domain increased in magnitude from no infarct or stroke, to silent cerebral infarct, to overt stroke.
Individuals with SCD are at risk for cognitive deficits across domains, infarct status, and the lifespan. More research is necessary to determine unbiased effects for cognitive function in adults with SCD.
全面评估镰状细胞病（SCD）患者在多个领域、脑梗死状态和整个生命周期中的神经认知功能。
在 PubMed、MedLine 和 PsycINFO 中确定了 110 项研究，共涉及 3600 名 SCD 患者和 1127 名兄弟姐妹或健康对照者的 110 项研究。
荟萃分析结果表明，所有神经认知领域、年龄组和梗死状态均存在显著缺陷。与正常均值相比，学龄前儿童的显著缺陷范围为 Hedges'g=-.39 至 g=-.63，学龄儿童和青少年为 g=-.83 至 g=-1.18，成年人为 g=-.46 至 g=-0.86。全量表智商（FSIQ）、言语推理、知觉推理和执行功能的缺陷从学龄前儿童到学龄儿童和青少年样本逐渐增加。然而，研究结果还表明，成年样本的缺陷相对于学龄样本较小，这可能是由于成年研究中的抽样偏差。镰状细胞贫血的不同梗死状态的研究结果表明，无梗死样本的缺陷范围为 g=-.54 至 g=-.65，无症状性脑梗死样本为 g=-.52 至 g=-1.03，中风样本为 g=-1.35 至 g=-1.82。各领域的缺陷从无梗死或中风、无症状性脑梗死到显性中风逐渐增大。
SCD 个体在多个领域、梗死状态和整个生命周期中均存在认知缺陷的风险。需要进一步研究以确定 SCD 成年患者认知功能的无偏影响。
