Zhang Xinxian, Li Chenglong, Li Qiancheng
Department of Radiology, Xuzhou Children's Hospital, Xuzhou, Jiangsu 221002, P.R. China.
Exp Ther Med. 2016 Aug;12(2):555-558. doi: 10.3892/etm.2016.3417. Epub 2016 Jun 2.
Sickle cell disease is the result of altered genetic make up due to hereditary encounter and its form as homozygous sickle cell anemia is the most common and severe. The disease is characterized by chronic anemia, recurrent pain crises and vascular occlusion. Neurologically, there is a high incidence of stroke in childhood, as well as cognitive dysfunction. Newborn screening programmes and preventative treatments have allowed a much longer lifespan. However, recently, neurological research has shifted to characterizing more subtle aspects of brain development and functioning that may be critically important to the individual's quality of life. The present review article examines the neurological and neurocognitive complications of sickle cell disease, and discusses the importance of magnetic resonance imaging scans in the management of the disease.
镰状细胞病是由于遗传因素导致基因组成改变的结果，其纯合子形式的镰状细胞贫血最为常见且严重。该疾病的特征为慢性贫血、反复疼痛发作和血管阻塞。在神经方面，儿童期中风的发病率很高，同时还存在认知功能障碍。新生儿筛查项目和预防性治疗使患者的寿命得以显著延长。然而，最近神经学研究已转向对大脑发育和功能中更细微方面的特征描述，这些方面可能对个体的生活质量至关重要。本综述文章探讨了镰状细胞病的神经和神经认知并发症，并讨论了磁共振成像扫描在该疾病管理中的重要性。
