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首页> 外文期刊>British Journal of Haematology >Risk factor analysis of cerebral white matter hyperintensities in children with sickle cell disease
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Risk factor analysis of cerebral white matter hyperintensities in children with sickle cell disease

机译：镰状细胞病患儿脑白质高发的危险因素分析

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摘要

Sickle cell disease (SCD) is complicated by silent cerebral infarcts, visible as white matter hyperintensities (WMHs) on magnetic resonance imaging (MRI). Both local vaso-occlusion, elicited by endothelial dysfunction, and insufficiency of cerebral blood flow (CBF) have been proposed to be involved in the aetiology. We performed an explorative study to investigate the associations between WMHs and markers of endothelial dysfunction and CBF by quantifying WMH volume on 30Tesla MRI. We included 40 children with HbSS or HbS(0)thalassaemia, with a mean age of 121 +/- 26years. Boys demonstrated an increased risk for WMHs (odds ratio 45, 95% confidence interval 12-174), unrelated to glucose-6-phosphate dehydrogenase deficiency. In patients with WMHs, lower fetal haemoglobin (HbF) was associated with a larger WMH volume (regression coefficient=-062, R-2=025, P=004). Lower ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels were associated with lower CBF in the white matter (regression coefficient=007, R-2=015, P=003), suggesting that endothelial dysfunction could potentially hamper CBF. The findings of our explorative study suggest that a high level of HbF may be protective for WMHs and that endothelial dysfunction may contribute to the development of WMHs by reducing CBF.
机译：镰状细胞病（SCD）伴随着无声的脑梗塞，在磁共振成像（MRI）上可见为白质高信号（WMH）。已提出由内皮功能障碍引起的局部血管闭塞和脑血流不足（CBF）均与病因有关。我们进行了一项探索性研究，通过在30Tesla MRI上定量WMH量来研究WMH与内皮功能障碍和CBF标记之间的关联。我们纳入了40名患有HbSS或HbS（0）地中海贫血的儿童，平均年龄为121 +/- 26岁。男孩表现出增加的WMH风险（比值45，95％置信区间12-174），与6-磷酸葡萄糖脱氢酶缺乏症无关。在WMH患者中，较低的胎儿血红蛋白（HbF）与较大的WMH量相关（回归系数= -062，R-2 = 025，P = 004）。较低的ADAMTS13（具有血小板反应蛋白1型基序的整合素和金属蛋白酶，成员13）水平与白质中较低的CBF相关（回归系数= 007，R-2 = 015，P = 003），提示内皮功能障碍可能阻碍CBF。我们的探索性研究结果表明，高水平的HbF可能对WMH具有保护作用，并且内皮功能障碍可能通过降低CBF促进WMH的发展。

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