Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia
Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia
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DOI：
10.1182/blood-2014-09-599852
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发表时间：
2015-03-05
期刊：
影响因子：
20.3
通讯作者：
Pondarre, Corinne
中科院分区：
文献类型：
--
作者：
Bernaudin, Francoise;Verlhac, Suzanne;Pondarre, Corinne
对克雷泰伊镰状细胞贫血（SCA）新生儿队列进行早期经颅多普勒（TCD）筛查，并迅速启动输血程序，成功预防了明显卒中，但无症状性脑梗死（SCI）的累积风险仍然很高，这表明TCD筛查不能识别所有有SCI风险的SCA患者。我们假设，在急性胸部综合征或急性贫血事件（AAE）期间观察到的低灌注/缺氧发作，以及通过颌下多普勒超声和颈部磁共振血管造影（MRA）检测到的颅外颈内动脉（eICA）狭窄，也可能是SCI的危险因素。本研究纳入了来自克雷泰伊新生儿队列（1992-2010年）的189例无卒中SCA患者，纵向随访磁共振成像/MRA，包括末次评估时的颈部MRA。所有TCD异常和/或颅内狭窄的患者均接受输血治疗。平均随访时间为9.9年（范围：2.2-19.9年; 1844患者年）。计算临床事件的年发生率。到18岁时，SCI的累积风险为39.1%（95%置信区间[CI]，23.5%-54.7%），无平台期。我们证实，3岁前基线血红蛋白水平低于7 g/dL是SCI的高度显著的预测风险因素（风险比，2.97; 95%CI，1.43-6.17; P = .004）。此外，我们发现AAE发生率（比值比，每单位增加2.64; 95%CI，1.09-6.38; P= 0.031）和孤立性eICA狭窄（比值比，3.19; 95%CI，1.18-8.70; P = 0.023）是SCI的重要独立危险因素。
Early transcranial Doppler (TCD) screening of the Creteil sickle cell anemia (SCA)newborn cohort, and rapid initiation of transfusion programs, resulted in successful prevention of overt strokes, but a high cumulative risk of silent cerebral infarcts (SCI) remained, suggesting that TCD screening does not identify all patients with SCA at risk for SCI. We hypothesized that episodes of hypoperfusion/hypoxia, as observed during acute chest syndromes or acute anemic events (AAE), and extracranial internal carotid artery (eICA) stenoses, detectable via submandibular Doppler sonography and cervical magnetic resonance angiography (MRA), could also be risk factors for SCI. This study includes 189 stroke-free patients with SCA from the Creteil newborn cohort (1992-2010) followed longitudinally by magnetic resonance imaging/MRA, including cervical MRA at the last assessment. All patients with abnormal TCD and/or intracranial stenoses were placed on a transfusion program. Mean follow-up was 9.9 years (range, 2.2-19.9 years; 1844 patient-years). Annual rates of clinical events were calculated. The cumulative risk for SCI was 39.1% (95% confidence interval [CI], 23.5%-54.7%) by age 18 years, with no plateau. We confirm that baseline hemoglobin level lower than 7 g/dL before age 3 years is a highly significant predictive risk factor for SCI (hazard ratio, 2.97; 95% CI, 1.43-6.17; P = .004). Furthermore, we show that AAE rate (odds ratio, 2.64 per unit increase; 95% CI, 1.09-6.38; P=.031) and isolated eICA stenosis (odds ratio, 3.19; 95% CI, 1.18-8.70; P = .023) are significant and independent risk factors for SCI.