Ohene-Frempong K, Weiner S J, Sleeper L A, Miller S T, Embury S, Moohr J W, Wethers D L, Pegelow C H, Gill F M
The University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
Blood. 1998 Jan 1;91(1):288-94.
Cerebrovascular accident (CVA) is a major complication of sickle cell disease. The incidence and mortality of and risk factors for CVA in sickle cell disease patients in the United States have been reported only in small patient samples. The Cooperative Study of Sickle Cell Disease collected clinical data on 4,082 sickle cell disease patients enrolled from 1978 to 1988. Patients were followed for an average of 5.2 +/- 2.0 years. Age-specific prevalence and incidence rates of CVA in patients with the common genotypes of sickle cell disease were determined, and the effects of hematologic and clinical events on the risk of CVA were analyzed. The highest rates of prevalence of CVA (4.01%) and incidence (0.61 per 100 patient-years) were in sickle cell anemia (SS) patients, but CVA occurred in all common genotypes. The incidence of infarctive CVA was lowest in SS patients 20 to 29 years of age and higher in children and older patients. Conversely, the incidence of hemorrhagic stroke in SS patients was highest among patients aged 20 to 29 years. Across all ages the mortality rate was 26% in the 2 weeks after hemorrhagic stroke. No deaths occurred after infarctive stroke. Risk factors for infarctive stroke included prior transient ischemic attack, low steady-state hemoglobin concentration and rate of and recent episode of acute chest syndrome, and elevated systolic blood pressure. Hemorrhagic stroke was associated with low steady-state hemoglobin and high leukocyte count.
脑血管意外（CVA）是镰状细胞病的主要并发症。美国镰状细胞病患者中CVA的发病率、死亡率及危险因素仅在小样本患者中有所报道。镰状细胞病合作研究收集了1978年至1988年登记的4082例镰状细胞病患者的临床数据。患者平均随访5.2±2.0年。确定了镰状细胞病常见基因型患者中CVA的年龄特异性患病率和发病率，并分析了血液学和临床事件对CVA风险的影响。CVA患病率最高（4.01%）和发病率最高（每100患者年0.61例）的是镰状细胞贫血（SS）患者，但所有常见基因型患者均会发生CVA。梗死性CVA的发病率在20至29岁的SS患者中最低，在儿童和老年患者中较高。相反，SS患者出血性中风的发病率在20至29岁患者中最高。在所有年龄段，出血性中风后2周内的死亡率为26%。梗死性中风后无死亡病例。梗死性中风的危险因素包括既往短暂性脑缺血发作、低稳态血红蛋白浓度及急性胸综合征的发生率和近期发作，以及收缩压升高。出血性中风与低稳态血红蛋白和高白细胞计数有关。
