Department of Pediatrics, Pediatric Hematology & Bone Marrow Transplantation (BMT) Unit, Cairo University, Cairo, Egypt.
Department of Diagnostic and Interventional Radiology, Cairo University, Cairo, Egypt.
Hemoglobin. 2020 Nov;44(6):418-422. doi: 10.1080/03630269.2020.1843483. Epub 2020 Nov 8.
Transcranial Doppler (TCD) screening is an established tool to identify children with sickle cell disease at high risk of stroke. Our objective was to study TCD velocities among sickle cell disease patients while in a steady state. This cross-sectional study included 78 steady state sickle cell disease patients [31 Hb SS (β/β) (sickle cell anemia), 47 Hb S/β-thalassemia (: c.20A>T/β-thal)], attending the Pediatric Hematology Clinic at Cairo University Children's Hospital, Cairo, Egypt. All patients underwent TCD velocity assessment as per the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. In our cohort, TCD velocities were comparable among Hb S/β-thal SS patients. Hemolysis indicators correlated significantly to TCD velocities in Hb S/β-thal patients; positive correlation was found between total bilirubin level and right middle cerebral artery (MCA) and right distal internal carotid artery (dICA) TCD velocities ( = 0.428, = 0.00, = 0.360, = 0.01), respectively as well as between reticulocyte count and right MCA, right dICA and right anterior cerebral artery (ACA) TCD velocities ( = 0.424, = 0.01), ( = 0.40, = 0.00), ( = 0.303, = 0.04), respectively. On the other hand, statistically significant negative correlations were found between hemoglobin (Hb) level and right ACA, right dICA TCD velocities ( = -0.290, = 0.05), ( = -0.324, = 0.03). Although Hb F is considered an ameliorating factor for disease severity; hemolysis stands as an indicator of risk for TCD velocity elevation, and in turn, risk for stroke among sickle cell disease patients.
经颅多普勒（TCD）筛查是一种用于识别镰状细胞病患者发生卒中风险较高的既定工具。我们的目的是研究处于稳定状态的镰状细胞病患者的 TCD 速度。本横断面研究纳入了 78 例稳定状态的镰状细胞病患者[31 例 Hb SS（β/β）（镰状细胞贫血），47 例 Hb S/β-地中海贫血（c.20A>T/β-地中海贫血）]，他们均在埃及开罗大学儿童医院儿科血液科就诊。所有患者均按照镰状细胞贫血卒中预防试验（STOP）方案接受 TCD 速度评估。在我们的队列中，Hb S/β-地中海贫血患者的 TCD 速度在 SS 患者之间无差异。溶血指标与 Hb S/β-地中海贫血患者的 TCD 速度呈显著正相关；总胆红素水平与右侧大脑中动脉（MCA）和右侧颈内动脉终末段（dICA）TCD 速度呈正相关（r = 0.428，P = 0.00，r = 0.360，P = 0.01），网织红细胞计数与右侧 MCA、右侧 dICA 和右侧大脑前动脉（ACA）TCD 速度呈正相关（r = 0.424，P = 0.01），（r = 0.40，P = 0.00），（r = 0.303，P = 0.04）。另一方面，血红蛋白（Hb）水平与右侧 ACA、右侧 dICA TCD 速度呈显著负相关（r = -0.290，P = 0.05），（r = -0.324，P = 0.03）。尽管 Hb F 被认为是疾病严重程度的改善因素；但溶血是 TCD 速度升高的风险指标，继而也是镰状细胞病患者发生卒中的风险指标。
