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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 understanding of clinicians on the diversity of children with lupus nephritis and to strengthen the understanding of children with lupus-related minimal nephropathy. Methods The clinical data, pathological features, diagnosis and treatment of 2 cases of lupus-related minimal nephropathy patients admitted to our hospital were retrospectively analyzed and analyzed. Results Both of the 2 children were girls, aged 12 and 13 years old respectively. The course of disease ranged from 1 to 2 months. All meet the typical SLE diagnostic criteria. Lupus activity while kidney damage performance are mainly nephrotic syndrome, but the renal pathology showed minor lesions. The pathological manifestations were as follows: No significant changes were observed in the kidneys in 2 cases under the light microscope. Immunofluorescence microscopy in case 1 showed negative results. In case 2, only subtle deposition of mesangial segments was found, with no obvious glomerular capillary wall deposition ; Electron microscopy are rare occasional deposition of electron dense material, but prominent for diffuse glomerular epithelial cell foot process fusion. Urine protein was negative in 2 patients treated with hormone or combined immunosuppressive drugs. Conclusion Clinicians in the work of lupus activity and at the same time appear nephropathy proteinuria careful analysis of the pathology of renal biopsy is necessary for the understanding of the new type of lupus nephritis and improve clinicians understanding of the pathogenesis of lupus nephritis, And the correct diagnosis, treatment is extremely important.