Hamdule Shifa, Kirkham Fenella J
Developmental Neurosciences Section, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK.
Clinical and Experimental Sciences, University of Southampton, Southampton SO16 6YD, UK.
Children (Basel). 2023 Aug 8;10(8):1360. doi: 10.3390/children10081360.
Cognitive decline is a major problem in paediatric and adult patients with sickle cell anaemia (SCA) and affects the quality of life. Multiple studies investigating the association between quantitative and qualitative neuroimaging findings and cognition have had mixed results. Hence, the aetiology of cognitive decline in this population is not clearly understood. Several studies have established cerebral atrophy in SCA children as well as adults, but the relationship between cognition and brain volumes remains unclear. The purpose of this systematic review was therefore to evaluate the literature on regional brain volumes and their association with cognitive outcomes. We also meta-analysed studies which compared regional brain volumes between patients and controls. Studies report that patients with SCA tend to have lower grey matter volumes, including total subcortical volumes in childhood as compared to controls, which stabilise in young adulthood and may be subjected to decline with age in older adulthood. White matter volumes remain stable in children but are subjected to reduced volumes in young adulthood. Age and haemoglobin are better predictors of cognitive outcomes as compared to regional brain volumes.
认知功能衰退是患有镰状细胞贫血（SCA）的儿童和成人患者面临的一个主要问题，会影响生活质量。多项研究调查了定量和定性神经影像学结果与认知之间的关联，但结果不一。因此，这一人群认知功能衰退的病因尚不清楚。多项研究已证实SCA儿童及成人存在脑萎缩，但认知与脑容量之间的关系仍不明确。因此，本系统评价的目的是评估有关局部脑容量及其与认知结果关联的文献。我们还对比较患者与对照组局部脑容量的研究进行了荟萃分析。研究报告称，与对照组相比，SCA患者往往灰质体积较低，包括儿童期的总皮质下体积，这些体积在青年期稳定下来，在老年期可能会随着年龄增长而下降。白质体积在儿童期保持稳定，但在青年期会减少。与局部脑容量相比，年龄和血红蛋白是认知结果更好的预测指标。
