Silent cerebral infarcts in sickle cell anemia: A risk factor analysis
Silent cerebral infarcts in sickle cell anemia: A risk factor analysis
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DOI：
10.1542/peds.103.3.640
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发表时间：
1999-03-01
期刊：
影响因子：
8
通讯作者：
Miller, ST
中科院分区：
文献类型：
--
作者：
Kinney, TR;Sleeper, LA;Miller, ST
背景。据报道，17%的年轻镰状细胞病患者存在无症状性梗死，并与标准化心理测试成绩受损有关。这些病变发展的危险因素尚未确定。镰状细胞病合作研究的研究人员对5.9岁及以上的镰状细胞性贫血患者进行了脑磁共振成像扫描，这些患者自出生以来一直按照合作研究的方案进行随访。有脑血管意外病史的个体被排除在本分析之外。在临床和实验室参数方面，比较了有和没有隐匿性梗死的患者。研究样本包括42例无症状性梗死患者（18.3%）。无症状性梗死的患者更有可能有癫痫发作的临床病史，疼痛事件发生率也更低。血红蛋白水平降低，白细胞计数增加，红细胞计数升高，SEN β (s)珠蛋白基因单倍型也与无症状性梗死的存在相关。无症状性梗死与血小板计数、胎儿血红蛋白水平、网状红细胞百分比、血清天冬氨酸转氨酶水平、总胆红素浓度、血压、生长参数或有无地中海贫血没有关系。无症状性梗死的多变量模型确定了以下危险因素：低疼痛事件发生率，癫痫发作史，白细胞计数大于或等于11.8 x 10(9)/L, SEN β (s)珠蛋白基因单倍型。有无症状性梗死危险因素的患者应评估是否患有脑血管疾病。如果发现梗死的证据，必须考虑治疗干预。目前，适当的治疗方法尚未确定。
Background. Silent infarcts have been reported in 17% of young patients with sickle cell disease and are associated with impaired performance on standardized psychometric tests. Risk factors for the development of these lesions have not been identified.Methods. Investigators in the Cooperative Study of Sickle Cell Disease performed a brain magnetic resonance imaging scan on sickle cell anemia patients age 5.9 years and older who had been followed according to the protocols of the Cooperative Study since birth. Individuals with a known history of cerebrovascular accident were excluded from this analysis. Patients with and without silent infarctions were compared with regard to clinical and laboratory parameters.Results. The study sample included 42 patients (18.3%) with silent infarcts. Patients who had silent infarcts were significantly more likely to have a clinical history of seizure and a lower painful event rate. Lower hemoglobin level, increased leukocyte count, elevated pocked red blood cell count, and SEN beta(s) globin gene haplotype were associated also with the presence of silent infarcts. There was no relationship between silent infarcts and platelet count, fetal hemoglobin level, reticulocyte percentage, serum aspartate aminotransferase level, total bilirubin concentration, blood pressure, growth parameters, or presence of cu-thalassemia. A multivariate model for silent infarction identified the following as risk factors: low pain event rate, history of seizure, leukocyte count greater than or equal to 11.8 x 10(9)/L, and the SEN beta(s) globin gene haplotype.Conclusions. Patients with risk factors for silent infarcts should be evaluated for cerebrovascular disease. If evidence of infarction is found, consideration must be given to therapeutic intervention. At present, the appropriate treatment has not been determined.