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
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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。
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, CYP3 A5 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. W hen 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 CYP3 A5 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.
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, CYP3 A5 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. W hen 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 CYP3 A5 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.
引文
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[2]穆青,刘戈力.儿童可逆性后部脑病综合征的研究现状与进展[J].医学综述,2015,21(6):1053-1055. Mu Q,Liu GL. Researthstatus and progress of reversible potdtedor encepludopathysyndrome in Children[J]. Medical Recapitulate,2015,21(6):1053-1055. doi:10.3969/j.issn.1006-2084.2015.06.036.
[3] Fugate JE,Rabinstein AA. Posterior reversible encephalopathysyndrome:clinical and radiological manifestations,pathophysiology,and outstanding questions[J]. Lancet Neurol,2015,14(9):914-925. doi:10.1016/S1474-4422(15)00111-8.
[4] Fischer M,Schmutzhard E. Posterior reversible encephalopathy syndrome[J]. J Neurol,2017,264(8):1608-1616. doi:10.1007/s00115-018-0565-6.
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[6] Ishikura K,Ikeda M,Hamasaki Y,et al. Nephrotic state as a riskfactor for developing posterior eversible encephalopathy syndromein paediatric patients with nephritic syndrome[J]. Nephrol DialTransplant,2008,23(8):2531-2636.
[7]朱红敏,丁娟娟,毛冰,等.儿童肾脏疾病并可逆性后部白质脑病综合征6例及文献复习[J].中华实用儿科临床杂志,2017,32(6):465-468. Zhu HM,Ding JJ,Mao B,et al. Kidney diseasecombined with posterior reversible encephalopathy syndrome inchildren:a report of 6 cases and literature review[J]. Journal ofApplied Clinical Pediatrics,2017,32(6):465-468. doi:10.3760/cma.j.issn.2095-428X.2017.06.016.
[8]黄力,周岩,张志强,等.原发性肾病综合征相关的可逆性后部脑白质综合征[J].肾脏病与透析肾移植杂志,2016,25(1):8-13. Huang L,Zhou Y,Zhang ZQ,et al. Primary nephritic syndromecomplicated posterior leukoencephalopathy syndrome[J]. ChineseJournal of Nephrology Dialysis&Transplantation,2016,25(1):8-13. doi:10.3969/cndt.j.issn.1006-298X.2016.01.002.
[9]何旭,陈随,夏正坤.激素抵抗型肾病综合征合并可逆性后部白质脑病一例[J].中华肾脏病杂志,2014,30(1):73. He X,Che S,Xia ZK. Hormone resistant nephrotic syndrome with reversibleposterior leukoencephalopathy[J]. Chinese Journal of Nephrology,2014,30(1):73. doi:10.3760/cma.j.issn.1001-7097.2014.01.017.
[10]阚璇,杨箐岩,刘戈力.环孢素A致儿童可逆性后部白质脑病综合征1例报告[J].临床儿科杂志,2012,30(11):1080-1081.Kan X,Yang JY,Liu GL. Ring spore element to A reversibleposterior leukoencephalopathy syndrome in children 1 case report[J]. J Clin Pediatr,2012,30(11):1080-1081. doi:10.3969/j.issn.1000-3606.2012.11.023.
[11]朱伟雪,于力,郝志宏,等.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[J].中华肾脏病杂志,2017,33(11):825-830. Zhu WX,Yu L,Hao ZH,et al. Clinicalanalysis of children having primary nephrotic syndromecomplicated with posterior reversible encephalopathy syndrome ontreatment of immunosuppressants[J]. Chinese Journal ofNephrology,2017,33(11):825-830. doi:10.3760/cma. j.issn.1001-7097.2017.11.004.
[12]Li M,Xu M,Liu W,et al. Effect of CYP3 A4,CYP3 A5 andABCB1 gene polymorphisms on the clinical efficacy of tacrolimus inthe treatment of nephritic syndrome[J]. BMC Pharmacol Toxicol,2018,319(1):14. doi:10.1186/s40360-018-0202-9.
[13]杨敏,魏传梅,刘乃国,等. CYP3A5基因多态性对膜性肾病患者他克莫司初始剂量的预测价值[J].中华肾脏病杂志,2015,31(10):736-742. Yang M,Wei CM,Liu NG,et al. Prediction ofinitial dosage of tacrolimus in patients with idiopathic membranousnephropathy by testing CYP3A5 genetic polymorphisms[J].Chinese Journal of Nephrology,2015,31(10):736-742. doi:10.3760/cma.j.issn.1001-7097.2015.10.003.
[2]穆青,刘戈力.儿童可逆性后部脑病综合征的研究现状与进展[J].医学综述,2015,21(6):1053-1055. Mu Q,Liu GL. Researthstatus and progress of reversible potdtedor encepludopathysyndrome in Children[J]. Medical Recapitulate,2015,21(6):1053-1055. doi:10.3969/j.issn.1006-2084.2015.06.036.
[3] Fugate JE,Rabinstein AA. Posterior reversible encephalopathysyndrome:clinical and radiological manifestations,pathophysiology,and outstanding questions[J]. Lancet Neurol,2015,14(9):914-925. doi:10.1016/S1474-4422(15)00111-8.
[4] Fischer M,Schmutzhard E. Posterior reversible encephalopathy syndrome[J]. J Neurol,2017,264(8):1608-1616. doi:10.1007/s00115-018-0565-6.
[5] Gungor S,Kilic B,Tabel Y. Clinical and imaging findings inchildhood posterior reversible encephalopathy syndrome[J]. Iran JChild Neurol,2018,12(1):16-25.
[6] Ishikura K,Ikeda M,Hamasaki Y,et al. Nephrotic state as a riskfactor for developing posterior eversible encephalopathy syndromein paediatric patients with nephritic syndrome[J]. Nephrol DialTransplant,2008,23(8):2531-2636.
[7]朱红敏,丁娟娟,毛冰,等.儿童肾脏疾病并可逆性后部白质脑病综合征6例及文献复习[J].中华实用儿科临床杂志,2017,32(6):465-468. Zhu HM,Ding JJ,Mao B,et al. Kidney diseasecombined with posterior reversible encephalopathy syndrome inchildren:a report of 6 cases and literature review[J]. Journal ofApplied Clinical Pediatrics,2017,32(6):465-468. doi:10.3760/cma.j.issn.2095-428X.2017.06.016.
[8]黄力,周岩,张志强,等.原发性肾病综合征相关的可逆性后部脑白质综合征[J].肾脏病与透析肾移植杂志,2016,25(1):8-13. Huang L,Zhou Y,Zhang ZQ,et al. Primary nephritic syndromecomplicated posterior leukoencephalopathy syndrome[J]. ChineseJournal of Nephrology Dialysis&Transplantation,2016,25(1):8-13. doi:10.3969/cndt.j.issn.1006-298X.2016.01.002.
[9]何旭,陈随,夏正坤.激素抵抗型肾病综合征合并可逆性后部白质脑病一例[J].中华肾脏病杂志,2014,30(1):73. He X,Che S,Xia ZK. Hormone resistant nephrotic syndrome with reversibleposterior leukoencephalopathy[J]. Chinese Journal of Nephrology,2014,30(1):73. doi:10.3760/cma.j.issn.1001-7097.2014.01.017.
[10]阚璇,杨箐岩,刘戈力.环孢素A致儿童可逆性后部白质脑病综合征1例报告[J].临床儿科杂志,2012,30(11):1080-1081.Kan X,Yang JY,Liu GL. Ring spore element to A reversibleposterior leukoencephalopathy syndrome in children 1 case report[J]. J Clin Pediatr,2012,30(11):1080-1081. doi:10.3969/j.issn.1000-3606.2012.11.023.
[11]朱伟雪,于力,郝志宏,等.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[J].中华肾脏病杂志,2017,33(11):825-830. Zhu WX,Yu L,Hao ZH,et al. Clinicalanalysis of children having primary nephrotic syndromecomplicated with posterior reversible encephalopathy syndrome ontreatment of immunosuppressants[J]. Chinese Journal ofNephrology,2017,33(11):825-830. doi:10.3760/cma. j.issn.1001-7097.2017.11.004.
[12]Li M,Xu M,Liu W,et al. Effect of CYP3 A4,CYP3 A5 andABCB1 gene polymorphisms on the clinical efficacy of tacrolimus inthe treatment of nephritic syndrome[J]. BMC Pharmacol Toxicol,2018,319(1):14. doi:10.1186/s40360-018-0202-9.
[13]杨敏,魏传梅,刘乃国,等. CYP3A5基因多态性对膜性肾病患者他克莫司初始剂量的预测价值[J].中华肾脏病杂志,2015,31(10):736-742. Yang M,Wei CM,Liu NG,et al. Prediction ofinitial dosage of tacrolimus in patients with idiopathic membranousnephropathy by testing CYP3A5 genetic polymorphisms[J].Chinese Journal of Nephrology,2015,31(10):736-742. doi:10.3760/cma.j.issn.1001-7097.2015.10.003.