小儿原发性肾病综合征病理与临床分析

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

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。

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 exami- nation. Results （1） The pathological types that most frequently occurred were： mesangioproliferative glomeru- lonephritis （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 （x2 = 32.19, P 〈 0.01）. （3） Patients with different pathological types yielded dif- ferent response rates to glucocorticoid. There were statistical significant differences （7,2 = 62.58, P 〈 0. 001）. （4）The pathological types were closely associated with the prognosis of the disease. There were statistical significant differ- ences between prognosis and patients with different pathological types （x2 = 104.66, P 〈 0.05）. Conclusion The pathological manifestations of primary nephrotic syndrome in children are diverse. The pathological type is closely as- sociated 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.