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首页> 外文期刊>American Journal of Neuroradiology >Cognitive Impairment in Children with Hemoglobin SS Sickle Cell Disease: Relationship to MR Imaging Findings and Hematocrit
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Cognitive Impairment in Children with Hemoglobin SS Sickle Cell Disease: Relationship to MR Imaging Findings and Hematocrit

机译：血红蛋白SS镰状细胞病患儿的认知障碍：与MR成像结果和血细胞比容的关系

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摘要

BACKGROUND AND PURPOSE: Children with hemoglobin SS sickle cell disease are known to suffer cognitive impairment if they have silent infarct, but recent evidence suggests that patients with hemoglobin SS sickle cell disease may be impaired even if they are free of infarction. We test a hypothesis that cognitive impairment in children with hemoglobin SS sickle cell disease is associated with low hematocrit and MR imaging abnormalities. METHODS: A cohort of 49 patients was examined, all of whom had hemoglobin SS sickle cell disease but no history of clinical stroke. The Wechsler scales, which are standardized and age-adjusted, were used to assess cognitive function. Patients also underwent MR imaging examination of the brain, and hematocrit was measured in a subset of 45 patients. MR images were evaluated by at least two readers, and abnormal imaging findings were evaluated by at least three readers. Any lesion was sufficient to be classified as abnormal, with lesions defined to include lacunar infarction, encephalomalacia, or leukoencephalopathy. Hematocrit data were used if obtained within 3 months of psychometric testing and if there were no confounding events in the patients’ charts. Wechsler test scores were then evaluated in relation to imaging findings and hematocrit values. RESULTS: Patients with imaging abnormalities had more cognitive impairment than did patients with normal imaging findings in verbal intelligence quotient (P < .02) and verbal comprehension (P < .01). Patients with low hematocrit had cognitive impairment shown by many performance measures, including full-scale intelligence quotient (P < .006), verbal comprehension (P < .006), and freedom from distractibility (P < .02). Multivariate analysis showed that MR imaging and hematocrit were independent predictors of full-scale intelligence quotient. CONCLUSION: Focal brain injury, revealed by MR imaging, is associated with cognitive impairment, but our data suggest that diffuse brain injury may also contribute to impairment. These findings show that impairment is multifactorial and suggest that chronic brain hypoxia is part of the pathophysiology of sickle cell disease.
机译：背景与目的：已知患有血红蛋白SS镰状细胞 疾病的儿童如果患有 无声梗塞，则患有认知障碍，但最近的证据表明患有 没有梗塞，血红蛋白SS镰状细胞病也可能受损。我们检验了一种假设，即血红蛋白SS镰状细胞病 患儿的认知 受损与低血细胞比容和MR成像异常有关。 方法：一个队列检查了49位患者，所有患者均患有 血红蛋白SS镰状细胞病，但无临床 中风病史。标准化并根据年龄进行了调整的Wechsler量表用于评估认知功能。 还对患者的大脑进行了 MR成像检查，并在45名患者的子集中对 进行了血细胞比容测量。 MR图像至少由两个 评估，并且 至少三个阅读器评估了异常影像学表现。任何病变足以将 归类为异常，病变定义为包括腔隙性脑梗塞， 脑软化或白脑病。如果在心理测验的3个月内获得血细胞比容数据，则 ；如果患者病历表中没有混淆事件，则使用 。 Wechsler测试得分为然后评估影像学检查结果与血细胞比容值的相关性。结果：影像学检查异常的患者比影像学检查正常的患者对认知功能的损害更大。 语言智能商（P <.02）和语言理解 （P <.01）。低血细胞比容患者的认知障碍 通过许多性能指标显示，包括全面智能 商（P <.006），语言理解（P <.006），< sup> 和不受干扰的影响（P <.02）。多元 分析表明，MR成像和血细胞比容是全面智能商的独立 预测因子。 结论：MR成像揭示了局灶性脑损伤，与 与认知障碍有关，但我们的数据表明，弥漫性 脑损伤也可能是造成认知障碍的原因。这些发现 表明损伤是多因素的，表明慢性 缺氧是镰状细胞 疾病的病理生理学的一部分。

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  • 来源
    《American Journal of Neuroradiology》 |2003年第3期|00000382-00000389|共8页
  • 作者

    R. Grant Steen; Mark A. Miles; Kathleen J. Helton; Susan Strawn; Winfred Wang; Xiaoping Xiong; Raymond K. Mulhern;

  • 作者单位

    Departments of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN;

    Behavioral Medicine, St. Jude Children’s Research Hospital, Memphis, TN;

    Departments of Diagnostic Imaging, St. Jude Children’s Research Hospital, Memphis, TN;

    Hematology, St. Jude Children’s Research Hospital, Memphis, TN;

    Hematology, St. Jude Children’s Research Hospital, Memphis, TN;

    Biostatistics, St. Jude Children’s Research Hospital, Memphis, TN;

    Behavioral Medicine, St. Jude Children’s Research Hospital, Memphis, TN;

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