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Neurocognitive ability in children with sickle cell disease and its relationship with the hemoglobin value

机译:镰状细胞病患儿的神经认知能力及其与血红蛋白值的关系

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It is known for decades that there is a cognitive deficit in children with sickle cell disease, and anemia has been regarded as one of the causes. Neuropsychological measures were obtained with the Wechsler Intelligence Scale in 119 patients aged 6 to 18 years, and compared with 42 healthy children of similar age and sex. In 87 cases, we obtained the annual averages of the values of hemoglobin (Hb) collected from medical records at follow-up visits until the time of evaluation. It was observed a decrease in the ratios of full-scale intelligence (ET, p= 0.024), the executive level (EE, p= 0.008) and also information on the subscales (p= 0.004), order figures (p= 0.017) and block design (p= 0.003), with respect to the healthy ones. Differences were observed between the results of patients with genotypes considered as more severe (SS and thalassemia Sb0) with less severe (Sb+ thalassemia SC) in ET (p= 0.033) and EE (p= 0.011). We found a significant relationship between Hb annual average of 4 to 6 years of age with ET (p= 0.023), the verbal scale (p= 0.028), sub scales information (p= 0.010), comprehension (p= 0.024), vocabulary (p= 0.022) and block design (p= 0.011). These results confirm the cognitive deficits in patients with sickle cell disease and relate them to the severity of anemia in preschool ages.
机译:几十年来,众所周知,镰状细胞病患儿存在认知缺陷,贫血一直被认为是原因之一。使用韦氏智力量表对119位6至18岁的患者进行了神经心理学测量,并与42名年龄和性别相仿的健康儿童进行了比较。在87例病例中,我们获得了随访期间直至评估之时从病历收集的血红蛋白(Hb)值的年度平均值。观察到全比例智力的比率(ET,p = 0.024),执行水平(EE,p = 0.008)以及子量表的信息(p = 0.004),订单数字(p = 0.017)均下降。相对于健康设计者而言(p = 0.003)。在ET(p = 0.033)和EE(p = 0.011)的较重基因型(SS和地中海贫血Sb0)和较不严重(Sb +地中海贫血SC)的基因型患者的结果之间观察到差异。我们发现4至6岁的Hb年平均水平与ET(p = 0.023),语言量表(p = 0.028),子量表信息(p = 0.010),理解力(p = 0.024),词汇量之间存在显着关系(p = 0.022)和区块设计(p = 0.011)。这些结果证实了镰状细胞疾病患者的认知缺陷,并将其与学龄前儿童贫血的严重程度相关。

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