Brewin John, Kaya Banu, Chakravorty Subarna
Department of Haematology, King's College London, London, UK.
Department of Haematology, Royal London Hospital, London, UK.
Br J Haematol. 2017 Nov;179(3):377-388. doi: 10.1111/bjh.14850. Epub 2017 Aug 2.
Stroke is one of the most severe complications to affect children with sickle cell anaemia (SCA). Transcranial doppler (TCD) is an accurate and non-invasive method to determine stroke risk. Randomised controlled trials have demonstrated the efficacy of chronic transfusion therapy in stroke prevention based on risk stratification determined by TCD velocities. This has led to the regular use of TCD monitoring for children with SCA in order to determine stroke risk. Significant resource allocation is necessary to facilitate training, quality assurance and failsafe arrangements for non-attenders. In a subgroup of patients, chronic transfusions for primary stroke prevention can be replaced by hydroxycarbamide therapy, provided careful monitoring is undertaken; including repeat TCD studies at frequent intervals. The authors propose an evidence-based algorithm for the management of abnormal TCD velocities and discuss the role of this test in other clinical contexts, such as in Haemoglobin SC disease.
中风是影响镰状细胞贫血(SCA)患儿的最严重并发症之一。经颅多普勒(TCD)是一种确定中风风险的准确且非侵入性的方法。随机对照试验已证明,基于TCD速度确定的风险分层,长期输血疗法在预防中风方面具有疗效。这导致对SCA患儿定期进行TCD监测以确定中风风险。为促进培训、质量保证以及对未就诊者的故障安全安排,需要大量资源分配。在一部分患者中,只要进行仔细监测,包括频繁重复TCD研究,用于一级预防中风的长期输血可被羟基脲疗法替代。作者提出了一种基于证据的算法,用于管理异常TCD速度,并讨论了该测试在其他临床情况中的作用,例如在血红蛋白SC病中的作用。