目的　分析儿童激素耐药型肾病综合征（SRNS）伴不可逆性脑白质病变的诊断和治疗。方法　回顾性分析1例确诊为SRNS伴不可逆性脑白质病变患儿的临床、实验室及影像学资料，并复习相关文献。结果　患儿临床诊断SRNS后给予激素、免疫抑制剂、血液透析等治疗10个月，病程中患儿反复出现抽搐、视力下降、高血压，头颅MRI显示双侧枕顶叶以及右侧额颞叶长T2信号，T2-Flair相可见双侧枕顶叶低信号影，提示脑软化灶伴胶质增生。结论　多种原因可能诱发儿童脑白质病变，在SRNS治疗过程中需高度警惕不可逆性脑白质病变的发生。

Objectives To analyze the diagnosis and management of steroid-resistant nephrotic syndrome (SRNS) accompanied with irreversible leukoencephalopathy in children. Methods The clinical, laboratory and imaging data were retrospectively analyzed in a SRNS child accompanied with irreversible leukoencephalopathy. A literature review was performed. Results After clinical diagnosis of SRNS, glucocorticoid, immunosuppressant, and hemodialysis were administrated for 10 months. During the course of treatment, the seizures, visual problems, and hypertension were repeatedly occured. The cranial MRI showed bilateral occipital parietal lobe hyperintensity and right frontotemporal lobe hyperintensity on T2-weighted imaging and bilateral occipital parietal lobe hypointensity on T2-Flair imaging, which indicated that encephalomalacia was accompanied with gliosis. Conclusions A variety of reasons may induce leukoencephalopathy in children. The accompanied irreversible leukoencephalopathy should be strongly considered in management of SRNS.