狼疮相关性微小病变性肾病2例报告并文献复习  

Two Case Reports of Systemic Lupus Erythematosus Related Minimal-Change Nephropathy and Its Literature Reviews

作　　者：黄建萍[1] 赵晓燕[1] 都娟[1] 闫果林[1] 肖丽丽[1] 

机构地区：[1]北京军区总医院附属八一儿童医院肾病和免疫科,北京100700

出　　处：《实用儿科临床杂志》2011年第19期1511-1512,1516,共3页Journal of Applied Clinical Pediatrics

摘　　要：目的提高临床医师对小儿狼疮性肾炎多样性的认识,加强对小儿狼疮相关性微小病变性肾病的了解。方法回顾性分析本院收治的2例狼疮相关性微小病变性肾病患儿的临床资料、病理特点及诊疗经过,并结合文献进行分析。结果 2例患儿均为女童,年龄分别为12岁和13岁,病程为1～2个月。均符合典型的SLE诊断标准。狼疮活动的同时肾脏损害表现均以肾病综合征为主,但肾脏病理表现为微小病变。其病理表现为:光镜下2例患儿肾脏均未见明显改变;免疫荧光镜检例1显示阴性,例2仅见系膜区节段微量沉积,均无明显的肾小球毛细血管壁沉积;电镜均偶见少量的电子致密物沉积,但突出表现为弥散性肾小球上皮细胞足突融合。2例患儿经激素或联合免疫抑制剂治疗后尿蛋白均转阴。结论临床医师在工作中应对狼疮活动并同时出现肾病蛋白尿的患儿进行认真分析,肾活检病理非常必要,对于认识新的狼疮性肾炎类型,提高临床医师对狼疮性肾炎的发病机制的了解,以及正确诊断、治疗极为重要。Objective To improve the knowledge of diversity of lupus nephritis and strengthen the understanding of systemic lupus erythematosus related minimal - change nephropathy. Methods The 2 children with systemic lupus erythematosus related minimal - change nephropathy were included. Clinical data, pathologic type and therapeutic process were retrospectively studied and related literatures were reviewed. Results The 2 cases were female teenagers who were diagnosed as typical systemic lupus erythematosus, their renal damage mainly presented as nephrotie syndrome during lupus activity. No significant change of kidney tissue was found under light microscope in 2 patients. Immunofluoreseence microscope showed completely negative in case 1 and little segment mesangial deposition in case 2 ,with no deposition in glomerular capillary wall. The prominent change was extensive fusion of epithelial foot process under electron microscope. Urinary protein all disappeared after treatment with glueoeorticoid or compound immunosuppressive agent. Conclusions Doctors shall pay attention to active systemic lupus erythematosus with nephrotic proteinuria. Renal biopsy is very necessary. It is very important to know the new type of lupus nephritis and improve the understanding about the pathogenesis of lupus nephritis.

关 键 词：狼疮 微小病变 肾病综合征 儿童 

分 类 号：R726.9[医药卫生—儿科]