University of Vermont School of Medicine, Burlington, Vermont.
Developmental Neurosciences Section and Biomedical Research Unit, Clinical and Experimental Sciences, University of Southampton, UCL Great Ormond Street Institute of Child Health, London, UK.
Pediatr Neurol. 2019 Jun;95:34-41. doi: 10.1016/j.pediatrneurol.2019.02.018. Epub 2019 Feb 27.
Cerebral infarction is a common complication of sickle cell disease and may manifest as overt stroke or cognitive impairment associated with "silent" cerebral infarction on magnetic resonance imaging. Vasculopathy may be diagnosed on transcranial Doppler or magnetic resonance angiography. The risk factors in sickle cell disease for cognitive impairment, overt ischemic stroke, silent cerebral infarction, overt hemorrhagic stroke, and vasculopathy defined by transcranial Doppler or magnetic resonance angiography overlap, with severe acute and chronic anemia, acute chest crisis, reticulocytosis, and low oxygen saturation reported with the majority. However, there are differences reported in different cohorts, which may reflect age, geographic location, or neuroimaging techniques, for example, magnetic resonance imaging field strength. Regular blood transfusion reduces, but does not abolish, the risk of neurological complications in children with sickle cell disease and either previous overt stroke or silent cerebral infarction or abnormal transcranial Doppler. There are relatively few data on the use of hydroxyurea or other management strategies. Early assessment of the risk of neurocognitive complications is likely to become increasingly important in the management of sickle cell disease.
脑梗死是镰状细胞病的常见并发症，可能表现为磁共振成像上显性卒中或认知障碍与“沉默性”脑梗死相关。血管病可通过经颅多普勒或磁共振血管造影诊断。镰状细胞病认知障碍、显性缺血性卒中、沉默性脑梗死、显性出血性卒中及经颅多普勒或磁共振血管造影定义的血管病的危险因素重叠，大多数报道有严重急性和慢性贫血、急性胸部危象、网织红细胞增多和低氧饱和度。然而，不同队列报道有差异，这可能反映年龄、地理位置或神经影像学技术（例如磁共振成像场强）的不同。定期输血可降低镰状细胞病儿童的神经并发症风险，但不能消除有既往显性卒中或沉默性脑梗死或经颅多普勒异常的儿童的风险。关于羟基脲或其他治疗策略的数据相对较少。早期评估神经认知并发症的风险可能在镰状细胞病的管理中变得越来越重要。
