Department of Neurology, Division of Pediatric Neurology, Vanderbilt University School of Medicine, Nashville, TN 37232, USA.
Br J Haematol. 2012 Apr;157(1):14-25. doi: 10.1111/j.1365-2141.2011.09005.x. Epub 2012 Jan 9.
This review will focus on the strengths and limitations associated with the current standard of care for primary prevention of ischaemic strokes in children with sickle cell anaemia (SCA) - transcranial Doppler ultrasound (TCD) screening followed by regular blood transfusion therapy when TCD measurement is above a threshold defined by a randomized clinical trial (RCT). The theoretical basis for potential alternative strategies for primary prevention of neurological injury in SCA is also discussed. These strategies will include, but will not be limited to: immunizations to prevent bacterial infections, particularly in low income countries; management of elevated blood pressure; and targeted strategies to increase baseline haemoglobin levels with therapies such as hyroxycarbamide or potentially definitive haematopoietic stem cell transplant.
本篇综述将聚焦于当前镰状细胞贫血（SCA）儿童缺血性脑卒中一级预防的标准治疗——经颅多普勒超声（TCD）筛查，以及当 TCD 测量值超过随机临床试验（RCT）定义的阈值时进行定期输血治疗——的优缺点。本文还讨论了 SCA 神经损伤一级预防的潜在替代策略的理论基础。这些策略将包括但不限于：预防细菌感染的免疫接种，特别是在低收入国家；控制血压升高；以及通过羟基脲或潜在的确定性造血干细胞移植等治疗方法，有针对性地提高基础血红蛋白水平。
