儿童肾脏疾病伴可逆性后部脑病综合征的临床表现、病因及治疗分析  

Clinical manifestations, etiology and treatment of kidney disease combined with posterior reversible encephalopathy syndrome in children

作　　者：吴瑕[1] 张碧丽[1] 王文红[1] 陈欣[2] WU Xia;ZHANG Bi-li;WANG Wen-hong;CHEN Xin(Department of Renal Medicine,Tianjin Children's Hospital,Tianjin 300134,China;Department of Radiology,Tianjin Children’s Hospital,Tianjin 300134,China)

机构地区：[1]天津市儿童医院肾内科,300134 [2]天津市儿童医院影像科,300134

出　　处：《天津医药》2019年第1期51-54,共4页Tianjin Medical Journal

摘　　要：目的分析4例肾脏疾病伴可逆性后部脑病综合征(PRES)患儿的临床资料,以提高对该病的认识。方法总结、分析2014年5月—2018年4月本院4例肾脏疾病伴PRES患儿临床症状和治疗过程。结果 4例均诊断为肾病综合征,伴头痛的3例患儿均于48 h内出现其他临床表现,4例患儿均存在抽搐,其中1例存在急性肾损伤,2例应用他克莫司治疗患儿发病时均伴肝功能损害,他克莫司血药浓度不同程度升高,2例中1例CYP3A5基因检测表现为杂合突变(AG型),予减停他克莫司。4例经积极治疗,临床症状很快好转,随访PRES均未再复发。结论头痛为PRES的首发症状,肾病综合征合并抽搐时,应注意与PRES相鉴别,高血压致高灌注及他克莫司致内皮损伤可为病因,应用他克莫司治疗的患儿颅MRI影像学可见细胞毒性水肿表现,应尽早行CYP3A5基因检测,密切监测肝功能及他克莫司血药浓度。Objective To analyze clinical data of 4 cases of renal disease with reversible posterior encephalopathy syndrome(PRES),and improve the understanding of the disease.Methods Data of clinical manifestations,etiology and treatment of 4 cases of PRES hospitalized in our hospital from May 2014-April 2018 were summarized and analyzed.Results Four cases were diagnosed with nephrotic syndrome.In 3 cases with headache,other clinical manifestations were found in 48 hours.There were convulsions in 4 children,including 1 case with acute kidney injury,2 cases with liver function damage in the application of tacrolimus therapy.The tacrolimus blood concentrations increased in varying degrees.In 1 case,CYP3A5 gene was detected as hybrid mutant(AG),tacrolimus was decreased/stop.Clinical symptoms were quickly improved in 4 cases after active treatment.No recurrence of PRES was found in the follow-up period.Conclusion Headache is the first symptom of PRES.When nephrotic syndrome is associated with convulsion,it should be differentiated from PRES.Hyperperfusion caused by high blood pressure and tacrolimus induced endothelial injury can be the cause.The CYP3A5 gene detection should be performed as early as possible,to closely monitor liver function and tacrolimus blood concentrations in children with cytotoxic edema on cranial magnetic resonance imaging.

关 键 词：肾病综合征 儿童 可逆性后部脑病综合征 

分 类 号：R334.1[医药卫生—人体生理学] R725.9[医药卫生—基础医学]