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。 Objective: To analyze the characteristics of primary nephrotic syndrome (NS) combined with reversible posterior white matter syndrome (PRLS) and explore the possible pathogenesis and risk factors of NS combined with PRLS. Methods: The clinical, laboratory and imaging data of 7 children with NS and PRLS were retrospectively analyzed. Results: Of the 7 patients, 4 were males and 3 were females, aged 6-17 years (average 12.7 ± 3.53 years), 3 had no renal biopsy, 2 had renal biopsy to diagnose glomerulosclerosis, 2 Case of membranous nephropathy. Urinary protein quantitation (4.1 ± 5.4) g / 24h, serum albumin (21.8 ± 2.7) g / L. The first 3 patients (43%) developed PRLS with normal blood pressure and the other 4 (57%) had mild hypertension before onset. In the presence of PRLS, six cases of clinical epilepsy seizures, one case of complex partial seizures. 5 patients underwent head CT examination, 4 cases of bilateral patchy low density lesions, 1 case of normal CT head. Seven patients had bilateral bilateral symmetrical frontal, parietal and occipital lobe cortical abnormalities in MRI plain scan, low signal on T1WI, high signal on T2WI slightly blurred image, low signal on DWI. Four patients (66%) had acute renal injury, four electrolyte disturbances (66%), and four patients (66%) with infection. CONCLUSIONS: Seven patients with PRLS in our cohort had primary nephrotic syndrome, children with mild hypertension, electrolyte imbalance, elevated serum creatinine, and infection.