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神经影像学的进展改善了镰状细胞病的治疗效果。

Advances in neuroimaging to improve care in sickle cell disease.

机构信息

Department of Pediatrics, Division of Pediatric Neurology, Vanderbilt University Medical Center, Nashville, TN, USA; Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA; Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, Nashville, TN, USA.

Department of Pediatrics, Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, TN, USA.

出版信息

Lancet Neurol. 2021 May;20(5):398-408. doi: 10.1016/S1474-4422(20)30490-7.

Abstract

Sickle cell disease is associated with progressive and increased neurological morbidity throughout the lifespan. In people with sickle cell anaemia (the most common and severe type of sickle cell disease), silent cerebral infarcts are found in more than a third of adolescents by age 18 years and roughly half of young adults by age 30 years, many of whom have cognitive impairment despite having few or no conventional stroke risk factors. Common anatomical neuroimaging in individuals with sickle disease can assess structural brain injury, such as stroke and silent cerebral infarcts; however, emerging advanced neuroimaging methods can provide novel insights into the pathophysiology of sickle cell disease, including insights into the cerebral haemodynamic and metabolic contributors of neurological injury. Advanced neuroimaging methods, particularly methods that report on aberrant cerebral blood flow and oxygen delivery, have potential for triaging patients for appropriate disease-modifying or curative therapies before they have irreversible neurological injury, and for confirming the benefit of new therapies on brain health in clinical trials.

摘要

镰状细胞病与整个生命周期中进行性和增加的神经发病有关。在镰状细胞贫血患者(最常见和最严重的镰状细胞病类型)中,18 岁时超过三分之一的青少年和 30 岁时近一半的年轻人会出现无症状性脑梗死,尽管他们几乎没有或没有传统的中风危险因素,但其中许多人存在认知障碍。镰状细胞病患者的常见解剖神经影像学可以评估结构性脑损伤,如中风和无症状性脑梗死;然而,新兴的高级神经影像学方法可以为镰状细胞病的病理生理学提供新的见解,包括对脑血管和代谢因素导致的神经损伤的见解。高级神经影像学方法,特别是报告异常脑血流和氧输送的方法,有可能在患者出现不可逆的神经损伤之前,对适当的疾病修正或治愈疗法进行分诊,并且在临床试验中确认新疗法对大脑健康的益处。

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