22例儿童难治性肾病的临床及病理分析

Clinical and pathological analysis of refractory nephritic syndrome in 22 Children.

目的探讨儿童难治性肾病的临床与病理改变之间的关系。方法对22例符合难治性肾病诊断标准的住院患儿的病理诊断及临床类型、临床疗效进行分析。结果小儿难治性肾病的病理类型中以系膜增生性肾炎(mesangial proliferative glomerulonephritis,MsPGN)(12/22)为最多见,其次为微小病变性肾病(minimal change disease,MCD)(4/22)、局灶节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)(3/22)、IgA肾病(IgA nephropathy,IgAN)(2/22)、膜增生性肾炎(membranoproliferative GN,MPNG)(1/22)。临床表现为激素耐药/频反复者,MsPGN 5/4例,MCD 0/3例,FSGS 3/0例,IgAN 1/0例,MPGN 1/0例;临床疗效中,获得完全缓解者,MsPGN 7/12例,MCD 3/4例,FSGS 1/3例,IgAN 1/2例。结论小儿难治性肾病中,病理改变以MsPGN、MCD及FSGS较多见,经激素联合免疫抑制剂治疗,MsPGN和MCD较易获得完全缓解。

Objective To find the correlations between the clinical presentation and pathological changes in refractory nephritic syndrome（RNS） of children. Methods 22 children with RNS were performed renal biopsy. The correlation between the pathologial changes and the clinical types or the curative effect were analyzed. Results ①The results shoed that MsPGN were 12/22,MCD 4/22, FSGS 3/22, IgAN 2/22 and MPGN 1/22. ②Clinically MsPGN 5/4, MCD 0/3, FSGS 3/0 ,IgANN 1/1 and MPGN 1/0 were classified to steroid-resistant（ SR）/frequently-relapsing（ FR）. ③ MsPGN 7/12, MCD 4/5 AND fsgs 2/3, IgAN 1/2 were acquired entire remission. Conclusion MCS, MsPGN and FSGS were more of the histological changes of RNS in Children. The reactioh to cure was different by their pathological changes.