摘要
目的提高对儿童肾脏疾病合并后部可逆性脑病综合征(PRES)的临床表现、影像学特征和治疗策略的认识。方法回顾性分析2010年7月至2018年6月首都儿科研究所附属儿童医院肾脏内科收治的4例肾脏疾病合并PRES患儿的临床资料及影像学结果,结合文献分析其临床特点。结果患儿原发病分别为急性链球菌感染后肾小球肾炎1例、肾病综合征2例、非典型溶血尿毒综合征1例,起病前有血压控制不良或细胞毒性药物使用史,以抽搐为首发症状,伴有头痛、头晕、视物模糊、血压急剧增高。典型头颅磁共振成像(MRI)示双侧大脑顶枕叶皮质或皮质下白质片状长T1T2信号,对称或不对称,严重者可累及侧脑室旁、小脑、脑干。4例PRES经过及时降血压和对症治疗后,1~2 d症状缓解,2~3周影像学异常完全消失。结论高血压、药物引起的血管源性脑水肿是发生儿童肾脏疾病合并PRES的主要原因,PRES的诊断依赖于头颅MRI的特征性改变,及时有效的降压和对症治疗可在短期内有效控制病情,治疗中应积极控制高血压,及时停用或减量诱发PRES的细胞毒性药物。
Objective To improve recognition of the clinical manifestation,imaging features and managements of posterior reversible encephalopathy syndrome(PRES)in children with kidney diseases.Methods Four children with kidney diseases complicating PRES admitted to our hospital from July 2010 to June 2018 were included.Clinical data and imaging results were retrospectively studied and related literatures were reviewed.Results The primary diseases of the 4 patients were acute post-streptococcal glomerulonephritis(1 case),nephritic syndrome(2 cases)and atypical hemolytic uremic syndrome(aHUS,1 case)respectively.Before onset there was history of poor control of hypertension or using cytotoxic drugs,and the primary symptom was seizures.They all developed an acute onset of headache,dizziness,visual changes and hypertension.Cranial MRI showed bilateral parietal,and occipital cortical or subcortical lesions with hypointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging,which was symmetrical or asymmetrical and might involve lateral ventricle,cerebel and brainstem in serious conditions.Clinical symptoms resolved soon and radiographic recovery occurred within 2 to 3 weeks with prompt anti-hypertension treatment and supportive care.Conclusion The vasogenic cerebral edema caused by acute elevated blood pressure and drugs seems to be the most important mechanism of PRES.Characteristic imaging features are very useful for diagnosis.Prompt antihypertension treatment and symptomatic treatment usually can reverse the PRES lesion soon.In the treatment,hypertension should be controlled,and the cytotoxic drugs inducing PRES should be withdrawn or reduced in dosage.
作者
于晓宁
陈朝英
YU Xiao-ning;CHEN Chao-ying(Department of Nephrology,Children's Hospital Affiliated to Capital Institute of Pediatrics,Beijing 100020,China)
出处
《中国实用儿科杂志》
CSCD
北大核心
2020年第1期21-25,共5页
Chinese Journal of Practical Pediatrics
