天津医药

天津医药 ›› 2019, Vol. 47 ›› Issue (1): 51-54.doi: 10.11958/20181128

• 临床研究 • 上一篇    下一篇

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

吴瑕 1，张碧丽 1△，王文红 1，陈欣 2   

  1. 作者单位：1天津市儿童医院肾内科（邮编300134），2影像科 作者简介：吴瑕（1981），女，硕士在读，主治医师，主要从事儿科肾内科的基础与临床研究 △通讯作者 E-mail：zhangbili218@163.com
  • 收稿日期:2018-07-25 修回日期:2018-10-25 出版日期:2019-01-15 发布日期:2019-01-15
  • 通讯作者: 吴瑕 E-mail:pipisweetxia@163.com

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

WU Xia1, ZHANG Bi-li1△, WANG Wen-hong1, CHEN Xin2   

  1. 1 Department of Renal Medicine, 2 Department of Radiology, Tianjin Children’s Hospital, Tianjin 300134, China △Corresponding Author E-mail：zhangbili218@163.com
  • Received:2018-07-25 Revised:2018-10-25 Published:2019-01-15 Online:2019-01-15

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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。

关键词: 肾病综合征, 儿童, 可逆性后部脑病综合征

Abstract: 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.

Key words: nephrotic syndrome, child, posterior reversible encephalopathy syndrome