儿童肾病综合征合并可逆性后部脑病综合征临床分析  

Clinical Analysis of Childhood Nephrotic Syndrome with Posterior Reversible Encephalopathy Syndrome

作　　者：谢敏 冯仕品 罗苇 王莉 刘喜 刘君 郭丽敏 李佳 Xie Min;Feng Shipin;Luo Wei(Women's and Children's Hospital Affiliated to the Medical College of the University of Electronic Science and Technology,Chengdu Women's and Children's Center Hospital,Chengdu,Sichuan 610000,China)

机构地区：[1]电子科技大学医学院附属妇女儿童医院·成都市妇女儿童中心医院,四川成都610000

出　　处：《四川医学》2020年第3期234-238,共5页Sichuan Medical Journal

基　　金：四川省卫计委科研课题(编号:120513)。

摘　　要：目的分析肾病综合征患儿合并可逆性后部脑病综合征(PRES)的临床及影像学特点。方法回顾性分析5例肾病综合征患儿合并PRES的临床资料,包括病因、临床表现、影像学检查、治疗经过及预后。结果5例肾病综合征合并PRES患儿,女1例,男4例,均行肾活检术,病理类型分别为新月体肾炎1例、IgA肾病1例、ANCA相关性肾炎1例、局灶节段肾小球硬化性肾炎2例。4例应用过细胞毒性药物。临床表现:血压增高5例,剧烈头痛5例,意识障碍2例,视力障碍3例,惊厥持续状态2例,行为异常1例。头颅MRI结果5例均提示顶枕叶皮质及皮质下层受累,其中3例同时伴有额叶受累,1例累及壳核。MRI表现3例为T1WI(长T1加权像)稍低信号,T2WI(长T2加权像)稍高信号,FLAIR(液体衰减反转恢复)高信号、DWI(弥散加权成像)稍高信号。2例为T1WI(长T1加权像)低信号,T2WI(长T2加权像)高信号,FLAIR(液体衰减反转恢复)高信号。经积极治疗原发病、降血压、止惊、降颅压等对症治疗。5例患儿均复查MRI,2例病灶完全消失,2例病灶明显减少,1例患儿其左侧侧脑室前角旁及右侧顶叶局部白质内仍有病灶。4例患儿临床表现及体征恢复正常,1例患儿持续抗癫痫治疗。结论PRES治疗的关键在于早期诊断、控制血压、治疗原发病及对症治疗。提高对PRES的认识,通过详细的病史、体格检查和颅脑影像学的综合分析早期治疗,可将神经系统损伤降至最低。Objective To analyze the clinical and imaging features of posterior reversible encephalopathy syndrome(PRES)in children with nephrotic syndrome.Methods Retrospective analysis of clinical data of 5 children with nephrotic syndrome combined with PRES, including etiology, clinical manifestations, imaging examination, treatment history and prognosis. Results Five children with nephrotic syndrome and PRES included 1 female and 4 males, all of whom underwent renal biopsy.The pathological types were crescentic nephritis, 1 case of IgA nephropathy, 1 case of ANCA-related nephritis, and 2 cases of focal segment Segmental glomerulosclerotic nephritis.Four cases had applied cytotoxic drugs.Clinical manifestations showed increased blood pressure in 5 cases, severe headache in 2 cases, unconsciousness in 2 cases, visual impairment in 3 cases, convulsive status in 2 cases and behavioral abnormality in 1 case.Cranial MRI results showed that the parietal and subcortical layers were involved in the parietal occipital lobe, including 3 cases with frontal lobe involvement and 1 case involving the putamen.Three cases of MRI showed slightly lower signal in T1WI (long T1 weighted image), slightly higher signal in T2WI (long T2 weighted image), high signal in FLAIR (liquid attenuation inversion recovery), and slightly higher signal in DWI (dispersion weighted imaging).MRI manifestations in two cases were T1WI (long T1 weighted image) low signal, T2WI (long T2 weighted image) high signal, FLAIR (liquid attenuation inversion recovery) high signal.After the active treatment of the primary disease, lowering blood pressure, stopping alarm, reducing intracranial pressure and other symptomatic treatment.The MRI was re-examined in 5 children, and the lesions completely disappeared in 2 cases, and the lesions were significantly reduced in 2 cases.In 1 case, there were still lesions in the left white side ventricle near the anterior horn and in the right parietal lobe.The clinical manifestations and signs of 4 children returned to normal, and 1

关 键 词：可逆性后部脑病综合征 肾病综合征 儿童 磁共振成像 

分 类 号：R692[医药卫生—泌尿科学]