Brain imaging findings in pediatric patients with sickle cell disease
Brain imaging findings in pediatric patients with sickle cell disease
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DOI：
10.1148/radiol.2281020943
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发表时间：
2003-07-01
期刊：
影响因子：
19.7
通讯作者：
Helton, KJ
中科院分区：
文献类型：
--
作者：
Steen, RG;Emudiabughe, T;Helton, KJ
目的：了解镰状细胞病(SCD)患儿脑部影像异常的发生率，探讨影响SCD发病率估计的临床和方法学因素。材料和方法：回顾分析1993年以来在圣裘德儿童研究医院就诊的185例SCD患儿的磁共振(MR)和磁共振血管成像(MR)表现。至少有两位读者独立审阅了图片。标准磁共振成像标准用于识别腔隙、白质体积丢失、脑软化症或白质脑病。对患者进行分级，以显示有限的或广泛的异常。标准的MR血管成像标准被用来识别动脉弯曲(局限性血管病变)和狭窄或闭塞(广泛血管病变)。根据患者的临床状态(包括中风)和诊断对结果进行评估。结果：在平均年龄10岁时，SCD患者总的梗塞、缺血或萎缩的发生率为44%(82/185)，血管病变的发生率为55%(102/185)。27例临床卒中患者中有26例有影像异常表现，但即使排除卒中患者，也有35%(56/158)出现“无症状性脑梗塞”(无临床卒中的MR影像可见损伤)，49%(78/158)有MR血管造影异常表现。与病情较轻的患者相比，临床病情较重的患者在磁共振成像(P<；.001)和磁共振血管成像(P<；.004)上有更多的异常。严重血管病变在血红蛋白SS患者中比在血红蛋白SC患者中更常见(P<；.001)。最新的成像方法比旧的方法显示更多的异常(P<；.01)。在新方法下，43%(29/67)的患者有广泛的异常，而在旧的方法下，28%(33/116)有广泛的异常。结论：儿童SCD患者缺血性脑损伤的发生率显著高于先前报道，部分原因是成像方法的改进。
PURPOSE: To determine prevalence of imaging abnormalities in the brain of children with sickle cell disease (SCD) and to identify clinical and methodological factors that influence prevalence estimate.MATERIALS AND METHODS: Magnetic resonance (MR) imaging and MR angiographic findings for 185 patients with SCD examined at St Jude Children's Research Hospital since 1993 were reviewed. At least two readers independently reviewed images. Standard MR imaging criteria were used to identify lacunae, loss of white matter volume, encephalomalacia, or leukoencephalopathy. Patients were assigned grades to indicate limited or extensive abnormalities. Standard MR angiographic criteria were used to identify arterial tortuosity (limited vasculopathy) and stenosis or occlusion (extensive vasculopathy). Findings were evaluated as a function of patient clinical status (including stroke) and diagnosis. Recent methods (T1- and T2- weighted MR imaging plus fluid-attenuated inversion recovery [FLAIR] at 3-mm section thickness) were compared with older methods (T1-T2-weighted MR imaging without FLAIR at 5-mm section thickness).RESULTS: At mean age of 10 years, overall prevalence of infarction, ischemia, or atrophy in patients with SCD was 44% (82 of 185), and prevalence of vasculopathy was 55% (102 of 185), without evidence of a significant referral bias. Twenty-six of 27 patients with clinical stroke had abnormal findings at imaging, but even if patients with stroke were excluded, 35% (56 of 158) had a "silent infarction" (MR imaging-visible injury without clinical stroke), and 49% (78 of 158) had abnormal findings at MR angiography. Patients with clinically severe disease had more abnormalities at MR imaging (P < .001) and MR angiography (P < .004) than did patients with milder disease. Severe vasculopathy was more prevalent in patients with hemoglobin SS than in those with hemoglobin SC (P < .001). Recent imaging methods showed more abnormalities than did older methods (P < .01). With newer methods, 43% (29 of 67) of patients had extensive abnormalities, whereas with older methods, 28% (33 of 116) had extensive abnormalities.CONCLUSION: Prevalence of ischemic brain injury in pediatric patients with SCD is substantially higher than was previously reported, in part because of improvements in imaging methods.