ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2016, Vol. 25 ›› Issue (1): 8-13.DOI: 10.3969/cndt.j.issn.1006-298X.2016.01.002

• 论文 • 上一篇    下一篇

原发性肾病综合征相关的可逆性后部脑白质综合征

  

  • 出版日期:2016-02-26 发布日期:2016-02-04

Primary nephritic syndrome complicated posterior leukoencephalopathy syndrome

  • Online:2016-02-26 Published:2016-02-04

摘要:

目的:分析原发性肾病综合征(NS)合并可逆性后部脑白质综合征(PRLS)的特点,探讨NS合并PRLS可能的发病机制和危险因素。
方法:回顾性分析7例儿童NS合并PRLS患者的临床、实验室检查及影像学资料。
结果:7例患者中,男性4例,女性3例,年龄6~17岁,平均(127±353)岁,3例未行肾活检,2例行肾活检诊断肾小球足细胞病,2例膜性肾病。尿蛋白定量(41±54) g/24h,血清白蛋白(218±27) g/L。发生PRLS前3例(43%)血压正常,4例(57%)发病前有轻度高血压。发生PRLS时,6例临床癫痫大发作,1例为复杂部分性癫痫发作。5例患者行头部CT检查, 4例见双侧斑片状低密度灶,1例头部CT正常。7例患者MRI平扫均出现双侧对称的额、顶及枕叶大脑皮层浅部异常信号,在T1WI上呈低信号、T2WI高信号略模糊影,DWI上病变呈低信号。4例(66%)合并急性肾损伤,电解质紊乱者4例(66%),合并感染者4例(66%)。
结论:本组发生7例PRLS的患者均为原发性肾病综合征,儿童、轻度高血压、合并电解质紊乱、血清肌酐升高及感染。

Abstract:

Objective:To investigated the clinical and neuroimaging features of patients with primary nephritic syndrome who developed reversible posterior leukoencephalophathy syndrome (PRLS), and to characterize the risk factors of PRLS in these patients.
Methodology:The clinical and neuroimaging features of seven patients with PRLS were analyzed retrospectively.
Results:They were 4 males and 3 females with an average age of (127±353) years old. The mean urine protein was (41±54) g/24h, and serum albumin was (218±27) g/L. Among them, 4 cases accompanied with acute kidney injury, 4 cases had electrolyte disorders, and 4 cases complicated with infection. 4 cases received renal biopsy, and the histological diagnosis was minimal change disease in 2 cases, and membranous nephropathy in other 2 cases. 3 patients had normal blood pressure, and the other 4 cases have mild hypertension. 6 cases manifested as tonicclonic seizures, and one case showed as complex partial seizure. 5 patients underwent CT examination, 4 cases showed bilateral patchy low density foci, and one showed no abnormity. For the first MRI scan, all the 7 cases showed bilateral symmetry abnormal signal on the cerebral cortex of frontal lobe, parietal lobe and occipital lobe, with low signal on T1WI, high signal on T2WI.
Conclusion:Our data suggested that the idiopathic nephritic syndrome patients with younger or children, with high pressure, with electrolyte disturbance, with elevated serum creatinine, and with infection are more susceptible to PRLS.