儿童狼疮相关性足细胞损伤5例分析

Systemic lupus erythematosus related podocyte injury in five children

目的提高对小儿狼疮肾炎(lupus nephritis,LN)中足细胞损伤的认识。方法回顾性分析5例临床表现为肾病综合征而病理改变轻微的小儿LN临床及病理特点,与同期非肾病表现、病理改变轻微的7例小儿LN进行比较,并检索国内外文献进行复习、总结。结果临床表现为肾病的LN患儿与非肾病患儿相比,临床表现除尿蛋白量与血白蛋白水平有统计学差异外,性别、年龄、血尿、高血压、肾功能不全、补体及自身抗体水平均无统计学差异;两组病理改变比较显示,肾病组电镜下表现为弥漫足突融合,而非肾病组则未见此表现;光镜、免疫荧光镜检查两组均为正常或轻度系膜增生及少量免疫复合物沉积。结论与狼疮性肾炎典型的病理类型相比,狼疮相关性足细胞病可能是狼疮肾炎中的一种独立的病理类型,在临床工作中要加以重视。

Objective To improve knowledge of podocyte injury in lupus nephritis（LN）.Methods Five children with LN characteristic of nephrotic syndrome（group 1）and seven children with non-nephrotic manifestation LN（group 2）were included.Both groups presented mild change in pathology.Clinical and pathologic data and therapeutic process were retrospectively studied and related literature was reviewed.Results There were significant difference in level of serum albumin and urine protein between the two groups,however no remarkable differences in age,sex,hematuria,hypertension,renal insufficiency,complement and autoantibodies between the two groups.Compared with group 2,group 1 showed extensive effacement of epithelial foot processes under electron microscope.No significant difference was found under light microscope and immunofluorescence microscope,with completely negative or a little immume complex deposition in the mesangial areas.Conclusions Compared with typical pathologic types,SLE-related podocytopathy is independent in lupus nephritis.Attention should be paid to it during clinical practice.