Aydin Sultan, Yeral Hakan, Oktay Gönül, Çoban Yasemin, Akbaş Yilmaz, Köker Alper, Öz Tunçer Gökçen
Departments of Pediatric Hematology and Oncology.
Radiology.
J Pediatr Hematol Oncol. 2021 Oct 1;43(7):249-253. doi: 10.1097/MPH.0000000000002255.
Stroke is one of the major complications of sickle cell disease (SCD). Stroke features either occlusion of, or stenosis at, the origin of one of the large intracerebral arteries, the internal carotid artery (ICA), and/or the middle cerebral artery (MCA).
We sought correlations between cerebral blood flow velocities and the laboratory and clinical findings of patients with SCD.
Fifty-three pediatric SCD patients (39 with HbSS, 14 with HbSβ0) were analyzed. The mean patient age was 12.9±3.9 years. The control group contained 24 healthy individuals. The time-averaged maximum mean velocity (TAMMV) and resistive index (RI) of the MCA, the TAMMVs of the ICA and vertebral artery (VA), and the diameter of the VA were estimated through transcranial Doppler ultrasonography using a 2.5 MHz transducer, in accordance with the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. We evaluated the relationships between the TAMMVs, laboratory parameters, and clinical findings.
The mean±SD MCA TAMMV was 161.2±35.4 cm/s in patients with HbSS and 185.8±62.9 cm/s in patients with HbSβ0. The mean MCA TAMMV, RI, ICA TAMMV, VA TAMMV, and VA diameter were 168.5±43.9 cm/s, 0.63±0.06, 116.8±25.5 cm/s, 69.2±18.5 cm/s, and 3.5±0.61 mm for all SCD patients, respectively. In the control group, the mean MCA TAMMV, RI, ICA TAMMV, VA TAMMV, and VA diameter were 103.8±28.8 cm/s, 0.53±0.04, 96.4±27.8 cm/s, 40.3±12.1 cm/s, and 3.4±0.6 mm, respectively. Although the differences were not significant, TAMMVs were higher in HbSβ0 patients taking hydroxyurea; those with hemoglobin levels <8 g/dL, ferritin levels >1000 ng/dL, mean platelet volume >12 fL, or red cell distribution width >18%; or required chelation, or were below the third percentiles of weight and height. The TAMMV was significantly higher only in SCD patients who complained of headache.
High ferritin and low hemoglobin levels, a high mean platelet volume, a high red cell distribution width, low weight (below the third percentile), and a short height (below the third percentile) may be associated with elevated cerebral blood flow velocities and an increased stroke risk in children with SCD. Children with such features should be closely followed-up through transcranial Doppler ultrasonography examination.
中风是镰状细胞病(SCD)的主要并发症之一。中风的特征是大脑主要动脉之一、颈内动脉(ICA)和/或大脑中动脉(MCA)起始处发生闭塞或狭窄。
我们探寻了SCD患者脑血流速度与实验室检查及临床结果之间的相关性。
分析了53例儿科SCD患者(39例HbSS型,14例HbSβ0型)。患者平均年龄为12.9±3.9岁。对照组包含24名健康个体。根据镰状细胞贫血症预防中风试验(STOP)方案,使用2.5MHz探头通过经颅多普勒超声估计MCA的时间平均最大平均速度(TAMMV)和阻力指数(RI)、ICA和椎动脉(VA)的TAMMV以及VA的直径。我们评估了TAMMV、实验室参数和临床结果之间的关系。
HbSS型患者的平均±标准差MCA TAMMV为161.2±35.4cm/s,HbSβ0型患者为185.8±62.9cm/s。所有SCD患者的平均MCA TAMMV、RI、ICA TAMMV、VA TAMMV和VA直径分别为168.5±43.9cm/s、0.63±0.06、116.8±25.5cm/s、69.2±18.5cm/s和3.5±0.61mm。对照组中,平均MCA TAMMV、RI、ICA TAMMV、VA TAMMV和VA直径分别为103.8±28.8cm/s、0.53±0.04、96.4±27.8cm/s、40.3±12.1cm/s和3.4±0.6mm。尽管差异不显著,但服用羟基脲的HbSβ0型患者的TAMMV较高;血红蛋白水平<8g/dL、铁蛋白水平>1000ng/dL、平均血小板体积>12fL或红细胞分布宽度>18%的患者;或需要螯合治疗的患者;或体重和身高低于第三百分位数的患者。仅主诉头痛的SCD患者的TAMMV显著更高。
高铁蛋白和低血红蛋白水平、高平均血小板体积、高红细胞分布宽度、低体重(低于第三百分位数)和矮身高(低于第三百分位数)可能与SCD儿童脑血流速度升高和中风风险增加有关。具有这些特征的儿童应通过经颅多普勒超声检查进行密切随访。