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目的分析儿童肾脏疾病合并脑后部可逆性脑病综合征(PRES)的临床特征,提高对本病的认识。方法报告我院诊断的7例儿童肾脏疾病合并PRES的临床资料,分析儿童肾脏疾病合并PRES的临床表现、影像学检查、治疗及预后情况。结果 7例患儿中女4例,发病年龄12~16岁。7例患儿的肾脏疾病临床诊断分别是狼疮性肾炎(4例)、原发性Ig A肾病(1例)、急进性肾小球肾炎(1例)以及Goodpasture综合征(1例)。7例患儿出现PRES时临床表现均有惊厥发作及头痛,呕吐2例,意识改变5例,视力障碍4例。出现PRES时7例患儿均有血压升高,3例患儿接受糖皮质激素及环磷酰胺治疗。头颅MRI示大脑后循环皮质下白质受累为主。患儿经控制血压、止惊以及治疗原发病后,神经系统症状均在1周内恢复,随访1~4.5年,均未出现神经系统后遗症。结论儿童肾脏疾病合并PRES主要表现为惊厥发作、头痛、意识障碍和视觉障碍。对于有肾脏疾病的患儿血压升高时,尤其同时予大剂量糖皮质激素或环磷酰胺等免疫抑制剂治疗时,应警惕PRES的发生。早期诊断和治疗的PRES预后较好。
Objective To analyze the clinical characteristics of children with renal disease complicated with reversible encephalopathy syndrome (PRES) and to improve the understanding of this disease. Methods The clinical data of 7 cases of children diagnosed as kidney disease combined with PRES in our hospital were reported. The clinical manifestations, imaging examination, treatment and prognosis of children with renal disease combined with PRES were analyzed. Results 7 cases of children in 4 cases, the age of onset of 12 to 16 years old. The clinical diagnosis of kidney disease in 7 children were lupus nephritis (4 cases), primary IgA nephropathy (1 case), acute glomerulonephritis (1 case) and Goodpasture’s syndrome (1 case). 7 cases of PRES present clinical manifestations of both seizures and headache, vomiting in 2 cases, 5 cases of change in consciousness, visual impairment in 4 cases. In the presence of PRES, seven patients had elevated blood pressure and three received glucocorticoid and cyclophosphamide. Head MRI showed cerebral cortex under the white matter involved mainly. Children with controlled blood pressure, only the start of the treatment of shock and the onset of symptoms, neurological symptoms were recovered within 1 week, followed up for 1 to 4.5 years, did not appear nervous system sequelae. Conclusions The main manifestations of PRES in children with kidney disease are seizures, headache, disturbance of consciousness and visual impairment. For children with kidney disease, high blood pressure, especially at the same time to high-dose glucocorticoid or cyclophosphamide and other immunosuppressive therapy, should pay attention to the occurrence of PRES. The prognosis of early diagnosis and treatment of better prognosis.