Bangirana Paul, Boehme Amelia K, Birabwa Annet, Opoka Robert O, Munube Deogratias, Mupere Ezekiel, Kasirye Phillip, Muwanguzi Grace, Musiimenta Maxencia, Ru George, Green Nancy S, Idro Richard
Department of Psychiatry, Makerere University College of Health Sciences, Kampala, Uganda.
Global Health Uganda, Kampala, Uganda.
medRxiv. 2024 Jan 29:2023.11.09.23298329. doi: 10.1101/2023.11.09.23298329.
Neurocognitive function in Ugandan children aged 1-12 years with sickle cell anemia (SCA) were compared to their non-SCA siblings to identify risk factors for disease-associated impairment.
This cross-sectional neurocognitive function study of children with SCA (N=242) and non-SCA siblings (N=127) used age- and linguistically-appropriate standardized tests of cognition, executive function and attention for children ages 1-4 and 5-12 years. Test scores were converted to locally derived age-normalized z-scores. The SCA group underwent standardized stroke examination for prior stroke and transcranial doppler ultrasound (TCD) to determine stroke risk by arterial flow velocity.
The SCA group was younger than siblings (mean ages 5.46±3.0 versus 7.11±3.51 years, respectively; p <.001), with lower hemoglobin concentration (7.32±1.02 vs. 12.06±1.42, p <.001). Overall cognitive SCA z-scores were lower: -0.73 ±0.98 vs. siblings -0.25 ±1.12 (p<.001), with comparable findings for executive function of -1.09±0.94 versus -0.84±1.26 (p=0.045), respectively. Attention z-scores for ages 5-12 for the SCA group and controls were similar: -0.37±1.4 vs. -0.11±0.17 (p=.09). Overall differences by SCA status were largely driven by the older age group, as z-scores in the younger sub-sample did not differ from controls. Analyses revealed the strongest predictors of poor neurocognitive outcomes among the SCA sample to be the disease, age and prior stroke (each p<.001). Impact from anemia and SCA were indistinguishable.
Neurocognitive testing in children with SCA compared to non-SCA siblings revealed poorer SCA-associated functioning in children older than age 4. Results indicate need for trials assessing impact from disease modification for children with SCA.
将1至12岁患镰状细胞贫血（SCA）的乌干达儿童的神经认知功能与其非SCA的兄弟姐妹进行比较，以确定疾病相关损伤的风险因素。
这项针对SCA儿童（N = 242）和非SCA兄弟姐妹（N = 127）的横断面神经认知功能研究，对1至4岁以及5至12岁的儿童使用了适合其年龄和语言的标准化认知、执行功能和注意力测试。测试分数被转换为本地得出的年龄标准化z分数。SCA组接受了标准化的中风检查以确定既往中风情况，并进行经颅多普勒超声（TCD）检查以通过动脉血流速度确定中风风险。
SCA组比其兄弟姐妹年龄小（平均年龄分别为5.46±3.0岁和7.11±3.51岁；p <.001），血红蛋白浓度较低（7.32±1.02对12.06±1.42，p <.001）。SCA组的总体认知z分数较低：-0.73±0.98对比其兄弟姐妹的-0.25±1.12（p<.001），执行功能方面的结果类似，分别为-1.09±0.94对比-0.84±1.26（p = 0.045）。SCA组和对照组5至12岁儿童的注意力z分数相似：-0.37±1.4对比-0.11±0.17（p =.09）。SCA状态导致的总体差异在很大程度上由年龄较大的组驱动，因为较年轻子样本中的z分数与对照组没有差异。分析显示，SCA样本中神经认知结果较差的最强预测因素是疾病、年龄和既往中风（各p<.001）。贫血和SCA的影响难以区分。
与非SCA兄弟姐妹相比，对SCA儿童进行神经认知测试发现，4岁以上儿童中与SCA相关的功能较差。结果表明需要进行试验来评估疾病修正对SCA儿童的影响。
