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首页> 外文期刊>Journal of stroke and cerebrovascular diseases: The official journal of National Stroke Association >Ectasia of the Basilar Artery in Children With Sickle Cell Disease: Relationship to Hematocrit and Psychometric Measures
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Ectasia of the Basilar Artery in Children With Sickle Cell Disease: Relationship to Hematocrit and Psychometric Measures

机译：镰状细胞病患儿的基底动脉蜕膜：与血细胞比容和心理测度的关系

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Goal: To determine whether children with sickle cell disease (SCD), but without clinical evidence of cerebrovascular disease, have vasculopathy shown by quantitative magnetic resonance angiography (MRA),. Methods: In a retrospective review of MRA films, we compared 47 SCD patients with 49 control patients. Time-of-flight three-dimensional Tl-weighted gradient-echo images were reconstructed, by maximum-intensity projection, to show the basilar artery in coronal view, and basilar volume was calculated from measurements made on films. Basilar volume was correlated with hematocrit and with results of cognitive testing. Findings: Mean basilar artery volume was 74% larger in SCD patients than in controls (P < .001). If the upper limit of normal is defined as mean adult volume 4- 2 SD (<=427 mm3), 2% (1 of 43) of controls but 37% (17 of 46) of SCD patients exceed this value (x2 = 19.0; P < .001). Basilar volume correlated inversely with hematocrit (r = - .60; P < .0001), with full-scale IQ (r = - .62; P < .005), and with freedom from distractability (r = -.61; P < .006) in SCD patients. Analysis of basilar artery tissue from a 5-year-old SCD patient showed that basilar dilatation can be associated with pathological changes typical of hypertension. Conclusions: Approximately 37% of a heterogenous group of pediatric SCD patients had ectasia of the basilar artery. Quantitative MRA is sensitive to subtle vasculopathy that can go undetected in the qualitative analysis more commonly done. Data suggest that there is a substantial elevation of arteriolar blood volume in pediatric SCD patients, and that such patients may share disease features in common with adult hypertension,
机译：目的：确定患有镰状细胞疾病（SCD）但无脑血管疾病临床证据的儿童是否患有定量磁共振血管造影（MRA）显示的血管病变。方法：在回顾性MRA电影中，我们比较了47例SCD患者和49例对照患者。通过最大强度投影重建飞行时间三维T1加权梯度回波图像，以在冠状视图中显示基底动脉，并通过在胶片上进行的测量计算基底体积。基底体积与血细胞比容和认知测试结果相关。结果：SCD患者的平均基底动脉体积比对照组大74％（P <.001）。如果将正常人的上限定义为平均成人体积4- 2 SD（<= 427 mm3），则2％（43人中的1人）的对照组，但37％（46人中的17人）的SCD患者超过该值（x2 = 19.0 ; P <.001）。基底体积与血细胞比容成反比（r =-.60; P <.0001），与全比例IQ（r =-.62; P <.005）和不受分散性影响（r = -.61; P <.006）在SCD患者中。对一名5岁SCD患者的基底动脉组织进行的分析表明，基底扩张可能与高血压的典型病理变化有关。结论：小儿SCD异种患者中约有37％患有基底动脉扩张。定量MRA对细微的血管病变很敏感，在更常见的定性分析中可能无法发现。数据表明，小儿SCD患者的小动脉血容量显着增加，并且此类患者可能与成人高血压有共同的疾病特征，

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