可逆性后部脑病综合征在儿童肾脏性疾病中的最新研究进展

Recent research progress of reversible posterior encephalopathy syndrome in children with renal diseases

可逆性后部脑病综合征(PRES)是一种临床神经影像学综合征,临床表现为癫痫、头痛、意识障碍和视觉障碍,主要影像学改变以双侧大脑半球后部白质对称性受累为特征性表现。最常见的危险因素为高血压、肾脏疾病和钙调神经磷酸酶抑制剂等。PRES的确切发病机制仍不清楚,目前主要有脑血流灌注突破学说、血管内皮细胞受损学说。PRES的治疗包括控制血压,治疗癫痫,以及减少或停用钙调神经磷酸酶抑制剂。大多数病例预后良好,临床和影像学异常可恢复正常。但是一些严重病例如合并急性脑出血者,常遗留后遗症,甚至导致死亡。早期识别、积极治疗具有十分重要的意义。

Reversible posterior encephalopathy syndrome(PRES)is a kind of clinical neuroimaging syndrome.Its clinical manifestations are epilepsy,headache,disturbance of consciousness and visual impairment.The main imaging changes are symmetrical involvement of the posterior white matter of bilateral cerebral hemispheres.The most common risk factors were hypertension,kidney disease and calcineurin inhibitors.The exact pathogenesis of pres is still unclear.At present,there are mainly cerebral blood perfusion breakthrough theory and vascular endothelial cell damage theory.Treatment of pres includes controlling blood pressure,treating epilepsy,and reducing or stopping calcineurin inhibitors.The prognosis of most cases is good,and the clinical and imaging abnormalities can return to normal.But some serious cases,such as those with acute cerebral hemorrhage,often leave sequelae,even lead to death.Early identification and active treatment are of great significance.