儿童肾脏相关疾病并可逆性后部白质脑病综合征单中心分析  

A single center study of kidney diseases combined with posterior reversible encephalopathy syndrome in children

作　　者：黄惠梅[1] 王治静[2] 刘勃[3] 李志娟[1] 张敏[1] 骞佩 包瑛[1] 张小鸽 Huang Huimei;Wang Zhijing;Liu Bo;Li Zhijuan;Zhang Min;Qian Pei;Bao Ying;Zhang Xiaoge(Department of Nephrology,Xi′an Children′s Hospital,Xi′an 710002,China;Department of Neurology,Xi′an Children′s Hospital,Xi′an 710002,China;Department of Radiology,Xi′an Children′s Hospital,Xi′an 710002,China;Department of Nephrology,Northwest Women and Children′s Hospital,Xi′an 710061,China)

机构地区：[1]西安市儿童医院肾脏科,710002 [2]西安市儿童医院神经科,710002 [3]西安市儿童医院影像科,710002 [4]西北妇女儿童医院肾脏内科,西安710061

出　　处：《中华实用儿科临床杂志》2020年第12期912-916,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：陕西省社会发展科技攻关项目(2016SF-288)。

摘　　要：目的分析儿童肾脏相关疾病并可逆性后部脑白质综合征(PRES)的特点,探究其可能的发病因素,提高认识,早期诊治并改善预后。方法回顾性分析2016年11月至2018年8月于西安市儿童医院肾脏科住院10例儿童肾脏相关疾病并PRES的临床、实验室检查及影像学资料,并随访其预后。结果10例患儿中,男1例,女9例;年龄4岁3个月~13岁[(8.53±3.09)岁]。原发基础疾病分别为肾病综合征6例,狼疮性肾炎、乙型肝炎病毒相关性肾炎、多发性大动脉炎和肾综合征出血热各1例。其中8例口服激素,4例联合应用其他免疫抑制剂。均急性起病,其中9例并感染。10例患儿发生PRES时血压均升高(100.0%),头痛、头晕5例(50.0%),恶心、呕吐5例(50.0%),视物模糊3例(30.0%),抽搐9例(90.0%)且均表现为全身强直大发作。神经系统查体均未见异常。眼底检查均未见异常。9例患儿头颅CT均表现为低密度灶,无特异性。10例患儿头颅磁共振成像(MRI)示病灶以顶、枕、额叶大脑皮质为主呈异常信号。液体衰减反转恢复序列均高信号,4例轴位弥散加权成像高信号表明PRES已由可逆的血管源性水肿进展为不可逆的细胞毒性水肿,提示预后不佳,但4例患儿经及时血液净化、脱水降颅压等治疗后病情恢复。大多数确诊PRES后经及时治疗后症状消失,未再复发,预后较好。结论PRES在儿童肾脏疾病发作时常伴高血压,可能与应用免疫制剂有关,对疑似患儿应尽早行头颅MRI协助诊断PRES。Objective To investigate the clinical features of children with kidney diseases who developed posterior reversible encephalopathy syndrome(PRES),explore the risk factors of PRES in these children,improve the understanding of the diseases,and help early diagnosis and effective treatment of the diseases.Methods The clinical manifestations,laboratory inspection results,magnetic resonance imaging(MRI)material as well as the prognosis of 10 children with kidney diseases complicated by PRES who were admitted to the Department of Nephrology,Xi′an Children′s Hospital from November 2016 to August 2018 were analyzed retrospectively.Results A total of 10 children were recruited,including 1 boy and 9 girls,with the onset age ranging from 4 years and 3 months to 13 years[(8.53±3.09)years].The diagnosed kidney diseases in these patients were primary nephritic syndrome(6 cases),lupus nephritis(1 case),Hepatitis B-related nephritis(1 case),polyarteritis(1 case)and hemorrhagic fever with renal syndrome(1 case).Eight children received corticosteroids and 4 of them received other immunosuppressants simultaneously.Nine children suffered from the infections.All of them had acute onset,and the main symptoms were hypertension(10/10 cases,100.0%),headache and dizziness(5/10 cases,50.0%),nausea and vomiting(5/10 cases,50.0%),visual disturbance(3/10 cases,30.0%)and convulsions by the ways of seizures definitely(9/10 cases,90.0%).There was nothing positive in the examinations of the nervous system and fundus.Computer tomography examinations of 9 cases showed nonspecific low-density foci.The cranial MRI scan showed abnormal signals on the cerebral cortex of frontal lobe,parietal lobe and occipital lobe in all these 10 cases.The hyperintensities were observed on the fluid-attenuated inversion recovery sequences of all the 10 cases.Slight hyperintensities on diffusion-weighted images of 4 cases indicated that PRES progressed from reversible angiogenic edema to irreversible cytotoxic edema,meaning a poor prognosis.After blood purification

关 键 词：可逆性后部脑白质综合征 肾脏疾病 磁共振成像 儿童 

分 类 号：R726.9[医药卫生—儿科] R742[医药卫生—临床医学]