免疫抑制剂治疗肾病综合征儿童并发可逆性后部脑病综合征的临床分析  

Clinical analysis of children having primary nephrotic syndrome complicated with posterior reversible encephalopathy syndrome on treatment of immunosuppressants

作　　者：朱伟雪 于力[1] 郝志宏[1] 于生友[1] 

机构地区：[1]广州医科大学附属广州市第一人民医院儿科,510180

出　　处：《中华肾脏病杂志》2017年第11期825-830,共6页Chinese Journal of Nephrology

基　　金：国家自然科学基金（81670652、81273205）;广东省科技计划项目（2016A020215010）

摘　　要：目的探讨免疫抑制剂（如环孢素A、他克莫司）治疗与肾病综合征患儿并发可逆性后部脑病综合征（PRES）之间的关系。方法通过收集2014年6月至2017年5月期间在广州市第一人民医院儿科住院确诊肾病综合征并给予免疫抑制剂治疗而并发PRES的病例，分析患儿PRES的临床特点、头颅的影像学改变、治疗措施及预后。结果共纳入23例患儿，其中环孢素A治疗13例，他克莫司治疗10例。20例在并发PRES时处于肾病综合征活动期，有大量尿蛋白、明显水肿、低蛋白血症和高脂血症等临床表现；3例处于肾病综合征缓解期。并发PRES时主要表现为血压升高、头痛、抽搐、意识障碍、视觉障碍等症状。69．6％患儿使用高剂量免疫抑制剂，78．3％患儿存在高药物浓度。17例患儿行头颅核磁共振成像（MRI）检查，结果显示顶枕叶或合并额叶、基底核等部位T1加权成像（T1WI）呈低或等信号，T2加权成像（T2WI）及液体衰减反转恢复像（FLAIR）呈高信号，弥散加权成像（DWI）呈现弥散信号；6例行计算机断层成像（CT）检查，结果显示顶枕叶低密度灶。所有患儿发生PRES症状后，立即给予镇静止惊、降颅内压、改善微循环等对症治疗，减少或停用免疫抑制剂。21例患儿1周内临床症状和体征消失；2例患儿治疗1周内再次出现抽搐，1个月内临床症状消失。3个月后5例复查头颅MRI或CT，脑部原有病灶消失。结论PRES的发生可能与免疫抑制剂的剂量及血药浓度有关。对肾病综合征患儿使用环孢素A、他克莫司要从小剂量开始，逐渐加量，使用过程中如出现神经系统的症状须警惕PRES的发生。Objective To explore the relationship between posterior reversible encephalopathy syndrome （PRES） and the treatment of immunosuppressants such as cyclosporine A （CsA） and tacrolimus （FK506） in children with nephrotic syndrome. Methods The clinical data of nephrotie syndrome children with PRES caused by immunosuppressants who were hospitalized in Guangzhou First People＇s Hospital from June 2014 to May 2017 were collected. Their clinical characteristics, imaging features, treatments and prognosis were analyzed. Results A total of 23 children were enrolled, including 13 children with CsA and 10 children with FKS06. In the concurrent of PRES 20 cases were in the activity stage of nephrotic syndrome, with large amounts of urinary protein, obvious edema, hypoalbuminemia and hyperlipidemia; while 3 cases were in the remission of nephrotie syndrome. The main clinical symptoms of PRES were hypertension, headache, epileptic attack, consciousness disorder, visual disorder and so on. Sixty-nine point six percent of children were using high dose immunosuppressive agents, and 78.3% had high drug concentration. The cranial magnetic resonance imaging （MRI） results of 17 patients showed that they had T1 weighted （T1WI） hypointense, T2 weighted （T2WI） and fluid-attenuated inversion recovery （FLAIR） images hyperintense, as well as iso-and slight hypointense of diffusion-weighted image （DWI） in parietal-occipital regions or complicated with frontal lobes or basal nuclei region. Computer tomography （CT） examinations of 6 cases showed low- density focus of the occipital lobes. Children were relieved muscular spasm, debased intracranial hypertension, improved circulation, discontinued or reduced immunosuppressants at the onset of PRES. After these treatments, 21 patients＂ symptoms and signs disappeared within one week; two patients suffered convulsions 2 times in one week, but recovered after one month. After three months 5 children had MRI and CT re-examination and it showed that their brain les

关 键 词：.肾病综合征 后部白质脑病综合征 免疫抑制剂 儿童 

分 类 号：R726.9[医药卫生—儿科] R742[医药卫生—临床医学]