Dorie A, Guindo A, Saro Y S, Touré B A, Fané B, Dembelé A K, Diallo D A
Centre de recherche et de lutte contre la drépanocytose (CRLD), 03 BP, 186 BKO 03 Point G, Bamako, Mali.
Service d'hématologie oncologie médicale du CHU du Point G, BP 333, Bamako, Mali.
Arch Pediatr. 2015 Mar;22(3):260-6. doi: 10.1016/j.arcped.2014.12.005. Epub 2015 Jan 27.
Cerebral vasculopathy exposes patients to a high risk of stroke, a major complication of sickle cell disease (SCD) associated with a high risk of death and disability. Transcranial doppler (TCD) ultrasonography used to identify SCD patients at risk of stroke may contribute to significantly reducing morbidity and mortality in these patients by indicating appropriate treatment. From March 2008 to February 2013, we conducted systematic screening for cerebral vasculopathy using TCD in 572 SCD patients (including 375 SS, 144 SC, 26 S/β(0), and 27 S/β(+) thalassemia patients) aged 1-17 years in a comprehensive center for follow-up and research on sickle cell disease in Bamako, Mali. After exclusion of 30 inadequate results and one case of abnormal TCD observed in a multiple organ failure patient, we found an abnormal or conditional TCD in 18% of 541 children examined in a steady state. The highest prevalence of abnormal cases concerned homozygous SS patients (8.1%). No case of abnormal or conditional TCD was observed in children with S/β(+) thalassemia. Hemoglobin concentrations were significantly lower in patients with conditional or abnormal TCD (P<0.01). In a subgroup of 68 patients with conditional TCD, nine (13%) converted to abnormal TCD over 1 year. In this subgroup of 68 conditional TCD patients, a decrease or increase in baseline hemoglobin concentration was predictive of conditional or abnormal TCD at the follow-up visit. Progression towards conditional TCD was observed in four patients (0.9%) who initially had normal TCD. Children with abnormal TCD had, whenever possible, a monthly exchange transfusion program. One case of transient stroke in the context of P. falciparum malaria with low hemoglobin concentration and one death were observed. These findings highlight the need for systematic TCD in sickle cell disease monitoring and implementing regular blood transfusion programs in the context of limited access to regular and secure blood transfusions.
脑血管病变使患者面临中风的高风险，中风是镰状细胞病（SCD）的主要并发症，与高死亡和残疾风险相关。用于识别有中风风险的SCD患者的经颅多普勒（TCD）超声检查，通过指明适当的治疗方法，可能有助于显著降低这些患者的发病率和死亡率。2008年3月至2013年2月，我们在马里巴马科一家镰状细胞病综合随访与研究中心，对572名1至17岁的SCD患者（包括375名SS型、144名SC型、26名S/β(0)型和27名S/β(+)型地中海贫血患者）进行了TCD脑血管病变系统筛查。在排除30份不合格结果以及1例在多器官功能衰竭患者中观察到的TCD异常病例后，我们发现在541名处于稳定状态接受检查的儿童中，18%存在异常或疑似异常的TCD。异常病例的最高患病率出现在纯合子SS型患者中（8.1%）。在S/β(+)型地中海贫血儿童中未观察到异常或疑似异常的TCD病例。疑似异常或异常TCD的患者血红蛋白浓度显著更低（P<0.01）。在68名疑似异常TCD的患者亚组中，9名（13%）在1年内转变为异常TCD。在这个68名疑似异常TCD患者的亚组中，基线血红蛋白浓度的降低或升高可预测随访时的疑似异常或异常TCD。最初TCD正常的4名患者（0.9%）出现了向疑似异常TCD的进展。TCD异常的儿童尽可能接受每月一次的换血治疗方案。观察到1例在血红蛋白浓度低的恶性疟原虫疟疾背景下发生的短暂性中风病例和1例死亡病例。这些发现凸显了在镰状细胞病监测中进行系统TCD检查以及在常规安全输血受限的情况下实施定期输血方案的必要性。
