Bernaudin Françoise, Verlhac Suzanne, Chevret Sylvie, Torres Martine, Coic Lena, Arnaud Cécile, Kamdem Annie, Hau Isabelle, Grazia Neonato Maria, Delacourt Christophe
Reference Center for Sickle Cell Disease, Department of Pediatrics, Intercommunal Créteil Hospital, Créteil, France.
Blood. 2008 Nov 15;112(10):4314-7. doi: 10.1182/blood-2008-03-143891. Epub 2008 Sep 4.
Stroke is predicted by abnormally high cerebral velocities by transcranial doppler (TCD). This study aimed at defining predictive factors for abnormally high velocities (>/= 2 m/sec) based on the Créteil pediatric sickle cell anemia (SCA) cohort composed of 373 stroke-free SCA children. alpha genes and beta-globin haplotypes were determined. Biologic parameters were obtained at baseline. alpha-thalassemia was present in 155 of 325 and G6PD deficiency in 36 of 325 evaluated patients. TCD was abnormal in 62 of 373 patients. Multivariate logistic regression analysis showed that G6PD deficiency (odds ratio [OR] = 3.36, 95% confidence interval [CI] 1.10-10.33; P = .034), absence of alpha-thalassemia (OR = 6.45, 95% CI 2.21-18.87; P = .001), hemoglobin (OR per g/dL = 0.63, 95% CI 0.41-0.97; P = .038), and lactate dehydrogenase (LDH) levels (OR per IU/L = 1.001, 95% CI 1.000-1.002; P = .047) were independent risk factors for abnormally high velocities. This study confirms the protective effect of alpha-thalassemia and shows for the first time that G6PD deficiency and hemolysis independently increase the risk of cerebral vasculopathy.
经颅多普勒（TCD）检测到的大脑速度异常升高可预测中风。本研究旨在基于由373名无中风的镰状细胞贫血（SCA）患儿组成的克雷泰伊儿科队列，确定大脑速度异常升高（≥2米/秒）的预测因素。测定了α基因和β珠蛋白单倍型。在基线时获取生物学参数。325名接受评估的患者中，155名存在α地中海贫血，36名存在葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症。373名患者中62名TCD异常。多因素逻辑回归分析显示，G6PD缺乏症（比值比[OR]=3.36，95%置信区间[CI]1.10 - 10.33；P = 0.034）、无α地中海贫血（OR = 6.45，95%CI 2.21 - 18.87；P = 0.001）、血红蛋白（每克/分升的OR = 0.63，95%CI 0.41 - 0.97；P = 0.038）和乳酸脱氢酶（LDH）水平（每国际单位/升的OR = 1.001，95%CI 1.000 - 1.002；P = 0.047）是大脑速度异常升高的独立危险因素。本研究证实了α地中海贫血的保护作用，并首次表明G6PD缺乏症和溶血独立增加脑血管病变的风险。
