小儿原发性肾病综合征病理与临床分析

目的分析小儿原发性肾病综合征(PNS)的病理与临床特点,初步探讨其病理类型与临床的关系。方法回顾性分析218例小儿PNS患儿肾穿刺活组织检查的病理和临床资料。结果①最常见的4种病理类型是MsPGN(48.17%)、FSGS(16.51%)、IgAN(13.30%)、MCD(11.47%);②MCD以单纯型肾病综合征为主,MsPGN、FSGS及I-gAN以肾炎型肾病综合征为主,差异有高度显著性(χ2=32.19,P<0.01)。③病理类型对GC反应的比较差异有高度显著性(χ2=62.58,P<0.001);④病理类型与预后密切有关,差异有显著性(χ2=104.66,P<0.05)。结论小儿原发性肾病综合征的病理类型多种多样,其病理类型与临床类型关系密切,但没有一对一的对应关系;病理类型对GC反应及疾病预后的判断具有一定的指导价值。

The analysis of pathological and clinical characteristic in children with primary nephrotic syndrome

Objective To A preliminary investigation of the relationship between pathological typing and clinical characteristic in children with primary nephrotic syndrome(PNS) was performed by analyzing the clinical records of their pathological and clinical features.Methods A retrospective analysis was conducted to analyze the pathological and clinical data of 218 in-patient children with PNS and all of them had received kidney puncture biopsy examination.Results ①The pathological types that most frequently occurred were: mesangioproliferative glomerulonephritis(MsPGN)(48.17%),focal segmental glomeralosclerosis(FSGS)(16.51%),immunoglobulin A nephropathy(IgAN)(13.30%),minimal change disease(MCD)(11.47%).② The principal manifestation of MCD was simple nephrotic syndrome;MsPGN,FSGS and IgAN mainly presented as nephritis associated with nephrotic syndrome.Compared MCD to MsPGN,FSGS and IgAN,there were statistically significant differences in nephrotic syndrome presentation(χ2=32.19,P<0.01).③ Patients with different pathological types yielded different response rates to glucocorticoid.There were statistical significant differences(χ2=62.58,P<0.001).④The pathological types were closely associated with the prognosis of the disease.There were statistical significant differences between prognosis and patients with different pathological types(χ2=104.66,P<0.05).Conclusion The pathological manifestations of primary nephrotic syndrome in children are diverse.The pathological type is closely associated with the clinical type;but no one-to-one correspondence relationship is observed between them.The pathological typing has significances to guide the grading of glucocorticoid response and to predict the prognosis of disease.