Subtle brain abnormalities in children with sickle cell disease: Relationship to blood hematocrit
Subtle brain abnormalities in children with sickle cell disease: Relationship to blood hematocrit
复制标题
镰状细胞病儿童的细微大脑异常：与血细胞比容的关系
作者：
R. Steen;Xioping Xiong;R. Mulhern;J. Langston;Winfred C. Wang
我们的目标是验证一个假设，即在临床上没有中风的儿童镰状细胞病(SCD)患者中可能存在轻微的脑异常。我们使用定量磁共振成像，前瞻性地比较了50名患者和52名年龄相仿的健康对照组。先前验证的精确和准确的反转-恢复方法被用于在基底节的切片上测量T1。我们还使用韦氏测验对随机选择的27名患者的智商(IQ)进行了测量。在所评估的每个灰质结构中，患者的脑T1显著降低，但在所有白质结构中均不存在。回归分析表明，4岁时尾状核、枕核和大脑皮质的T1出现异常。心理测试显示，33%的患者处于轻度智力缺陷(IQ，50-70)范围内，而市中心黑人儿童的公开患病率为1.45%。因此，在我们的患者中，SCD与轻度精神缺陷的风险增加了23倍。SCD患者的全量智商是血细胞比容(HCT)的函数，当用Hct对患者进行分层时，Hct<27%的患者的心理测试得分显著低于Hct=27或更高的患者，灰质T1显著低于Hct=27或更高的患者。认知缺陷和微小的T1异常都与低红细胞压积有关，当常规磁共振成像发现正常时，两者都可能存在。我们的研究结果表明，无临床卒中的SCD患者可发生慢性脑组织缺氧。Ann Neurol 1999；45：279-286
Our objective was to test a hypothesis that subtle brain abnormality can be present in pediatric sickle cell disease (SCD) patients who are clinically free of stroke. We prospectively compared 50 patients with 52 healthy age‐similar controls, using quantitative magnetic resonance imaging. A previously validated precise and accurate inversion‐recovery method was used to measure T1 in a slice at the basal ganglia. We also used the Wechsler test to measure intelligence quotient (IQ) in a randomly selected subset of 27 patients. Brain T1 was significantly lower in patients in every gray matter structure evaluated but in none of the white matter structures. Regression suggests that T1 in caudate, nucleus pulvinares, and cerebral cortex was abnormal by age 4 years. Psychometric testing showed that 33% of patients were functioning in the range of mild mental deficiency (IQ, 50–70), compared with a published prevalence of 1.45% in inner‐city black children. Thus, in our patients, SCD was associated with a 23‐fold increase in the risk of mild mental deficiency. Full‐scale IQ of SCD patients was a function of hematocrit (Hct), and when Hct was used to stratify patients, those with an Hct of less than 27% had significantly lower psychometric test scores, and significantly lower gray matter T1, than those with an Hct of 27 or more. Both cognitive deficits and subtle T1 abnormalities were associated with a low Hct, and both could be present when conventional magnetic resonance imaging findings were normal. Our findings suggest that chronic hypoxia of brain tissue can occur in SCD patients free of clinical stroke. Ann Neurol 1999;45:279–286
镰状细胞性视网膜病的免疫组织化学见解。
影响因子：
2
作者：
Lutty,GA;Merges,C;Crone,S;McLeod,DS
通讯作者：
McLeod,DS