PDF(Pubmed)
Abstract：
UNASSIGNED: The neurocognitive functions 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.
UNASSIGNED: This cross-sectional study of the neurocognitive functions in 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. Test scores were converted to locally derived age-normalized z-scores. The SCA group underwent a standardized stroke examination for prior stroke and transcranial Doppler ultrasound to determine stroke risk by arterial flow velocity.
UNASSIGNED: The SCA group was younger than their siblings (mean ages 5.46 ± 3.0 vs. 7.11 ± 3.51 years, respectively; p < 0.001), with a lower hemoglobin concentration (7.32 ± 1.02 vs. 12.06 ± 1.42, p < 0.001). The overall cognitive SCA z-scores were lower, -0.73 ± 0.98, vs. siblings, -0.25 ± 1.12 (p < 0.001), with comparable findings for executive function of -1.09 ± 0.94 vs. -0.84 ± 1.26 (p = 0.045), respectively. The attention z-scores for ages 5-12 for the SCA group and control group were similar: -0.37 ± 1.4 vs. -0.11 ± 0.17 (p = 0.09). The overall differences in SCA status were largely driven by the older age group, as the z-scores in the younger subsample 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 < 0.001). The impacts of anemia and SCA were indistinguishable.
UNASSIGNED: Neurocognitive testing in children with SCA compared to non-SCA siblings revealed poorer SCA-associated functioning in children older than age 4. The results indicate the need for trials assessing the impact of disease modification on children with SCA.
UNASSIGNED: This cross-sectional study of the neurocognitive functions in 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. Test scores were converted to locally derived age-normalized z-scores. The SCA group underwent a standardized stroke examination for prior stroke and transcranial Doppler ultrasound to determine stroke risk by arterial flow velocity.
UNASSIGNED: The SCA group was younger than their siblings (mean ages 5.46 ± 3.0 vs. 7.11 ± 3.51 years, respectively; p < 0.001), with a lower hemoglobin concentration (7.32 ± 1.02 vs. 12.06 ± 1.42, p < 0.001). The overall cognitive SCA z-scores were lower, -0.73 ± 0.98, vs. siblings, -0.25 ± 1.12 (p < 0.001), with comparable findings for executive function of -1.09 ± 0.94 vs. -0.84 ± 1.26 (p = 0.045), respectively. The attention z-scores for ages 5-12 for the SCA group and control group were similar: -0.37 ± 1.4 vs. -0.11 ± 0.17 (p = 0.09). The overall differences in SCA status were largely driven by the older age group, as the z-scores in the younger subsample 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 < 0.001). The impacts of anemia and SCA were indistinguishable.
UNASSIGNED: Neurocognitive testing in children with SCA compared to non-SCA siblings revealed poorer SCA-associated functioning in children older than age 4. The results indicate the need for trials assessing the impact of disease modification on children with SCA.
摘要：
■将1-12岁患有镰状细胞性贫血（SCA）的乌干达儿童的神经认知功能与其非SCA兄弟姐妹进行比较，以确定疾病相关损害的危险因素。
■这项对SCA（N=242）和非SCA兄弟姐妹（N=127）儿童的神经认知功能的横断面研究使用了年龄和语言上适当的认知标准化测试，执行功能，以及对1-4岁和5-12岁儿童的关注。将测试分数转换为本地衍生的年龄归一化z分数。SCA组接受了标准化的卒中检查，以检查先前的卒中和经颅多普勒超声检查，以通过动脉流速确定卒中风险。
■SCA组比他们的兄弟姐妹年轻（平均年龄5.46±3.0vs.7.11±3.51年，分别为；p<0.001)，具有较低的血红蛋白浓度（7.32±1.02vs.12.06±1.42，p<0.001）。总体认知SCAz得分较低，-0.73±0.98，与兄弟姐妹,-0.25±1.12（p<0.001），执行功能的可比发现为-1.09±0.94与-0.84±1.26(p=0.045)，分别。SCA组和对照组5-12岁的注意力z得分相似：-0.37±1.4vs.-0.11±0.17（p=0.09）。SCA状况的总体差异主要是由年龄较大的人群驱动的，因为年轻子样本的z评分与对照没有差异.分析显示，SCA样本中神经认知不良结局的最强预测因子是疾病，年龄,和先前的中风（每个p<0.001）。贫血和SCA的影响无法区分。
与非SCA兄弟姐妹相比，SCA儿童的神经认知测试显示，4岁以上儿童的SCA相关功能较差。结果表明，有必要进行试验评估疾病改变对SCA儿童的影响。
■这项对SCA（N=242）和非SCA兄弟姐妹（N=127）儿童的神经认知功能的横断面研究使用了年龄和语言上适当的认知标准化测试，执行功能，以及对1-4岁和5-12岁儿童的关注。将测试分数转换为本地衍生的年龄归一化z分数。SCA组接受了标准化的卒中检查，以检查先前的卒中和经颅多普勒超声检查，以通过动脉流速确定卒中风险。
■SCA组比他们的兄弟姐妹年轻（平均年龄5.46±3.0vs.7.11±3.51年，分别为；p<0.001)，具有较低的血红蛋白浓度（7.32±1.02vs.12.06±1.42，p<0.001）。总体认知SCAz得分较低，-0.73±0.98，与兄弟姐妹,-0.25±1.12（p<0.001），执行功能的可比发现为-1.09±0.94与-0.84±1.26(p=0.045)，分别。SCA组和对照组5-12岁的注意力z得分相似：-0.37±1.4vs.-0.11±0.17（p=0.09）。SCA状况的总体差异主要是由年龄较大的人群驱动的，因为年轻子样本的z评分与对照没有差异.分析显示，SCA样本中神经认知不良结局的最强预测因子是疾病，年龄,和先前的中风（每个p<0.001）。贫血和SCA的影响无法区分。
与非SCA兄弟姐妹相比，SCA儿童的神经认知测试显示，4岁以上儿童的SCA相关功能较差。结果表明，有必要进行试验评估疾病改变对SCA儿童的影响。
