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镰状细胞病患儿的可逆性后部白质脑病

Reversible posterior leuko-encephalopathy in children with sickle cell disease.

作者信息

Khademian Zarir, Speller-Brown Barbara, Nouraie Seyed-Medhi, Minniti Caterina P

机构信息

Department of Radiology, George Washington University, Children's National Medical Center, Washington, District of Columbia, USA.

出版信息

Pediatr Blood Cancer. 2009 Mar;52(3):373-5. doi: 10.1002/pbc.21812.

DOI:10.1002/pbc.21812
PMID:19003906
Abstract

Children with sickle cell disease (SCD) have high risk of neurologic morbidity and mortality, such as strokes, silent infarcts and TIA's. A retrospective review of magnetic resonance imaging and magnetic resonance angiography identified eight children with radiological and clinical characteristics of reversible posterior encephalopathy (RPLS). These patients had no evidence of previous cerebral infarcts or vasculopathy. Three have died during the 5-year follow up; one developed a stroke and one a conditional TCD. RPLS needs to be considered in the differential diagnosis of children with SCD that present with acute neurological changes, especially if they are already been hospitalized.

摘要

患有镰状细胞病(SCD)的儿童有较高的神经病变和死亡风险,如中风、无症状性梗死和短暂性脑缺血发作(TIA)。一项对磁共振成像和磁共振血管造影的回顾性研究发现了8名具有可逆性后部脑病(RPLS)放射学和临床特征的儿童。这些患者既往没有脑梗死或血管病变的证据。在5年的随访期间,有3人死亡;1人发生了中风,1人出现了条件性经颅多普勒(TCD)异常。对于出现急性神经功能改变的SCD患儿,尤其是已经住院的患儿,在鉴别诊断时需要考虑RPLS。

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