Department of Pediatrics, Washington University in St. Louis, 660 South Euclid Avenue, St. Louis, MO 63110, USA.
Department of Pediatrics, Washington University in St. Louis, 660 South Euclid Avenue, St. Louis, MO 63110, USA; Department of Neurology, Washington University in St. Louis, 660 South Euclid Avenue, St. Louis, MO 63110, USA.
Hematol Oncol Clin North Am. 2022 Dec;36(6):1167-1186. doi: 10.1016/j.hoc.2022.07.011.
Sickle cell disease (SCD) is complicated by neurologic complications including vasculopathy, hemorrhagic or ischemic overt stroke, silent cerebral infarcts and cognitive dysfunction. Patients with SCD, even in the absence of vasculopathy or stroke, have experience cognitive dysfunction that progresses with age. Transcranial Doppler ultrasound and structural brain MRI are currently used for primary and secondary stroke prevention, but laboratory or imaging biomarkers do not currently exist that are specific to the risk of cognitive dysfunction in patients with SCD. Recent investigations have used advanced MR sequences assessing cerebral hemodynamics, white matter microstructure and functional connectivity to better understand the pathophysiology of cognitive decline in SCD, with the long-term goal of developing neuroimaging biomarkers to be used in risk prediction algorithms and to assess the efficacy of treatment options for patients with SCD.
镰状细胞病 (SCD) 可并发神经并发症，包括血管病变、出血性或缺血性显性中风、无症状性脑梗死和认知功能障碍。即使没有血管病变或中风，SCD 患者也会经历认知功能障碍，且这种障碍会随年龄增长而加重。经颅多普勒超声和结构性脑 MRI 目前用于一级和二级卒中预防，但目前尚不存在针对 SCD 患者认知功能障碍风险的特异性实验室或影像学生物标志物。最近的研究使用先进的磁共振序列评估脑血流动力学、白质微观结构和功能连接，以更好地了解 SCD 认知能力下降的病理生理学，其长期目标是开发神经影像学生物标志物，用于风险预测算法，并评估 SCD 患者治疗选择的疗效。
