【摘要】 目的探讨难治性肾病综合征患儿临床与病理的关系及转归。方法选择难治性肾病51例,其中单纯型31例,肾炎型20例;难治类型有激素耐药25例,频复发20例,激素依赖6例;病理类型微小病变型(MCD)19例,系膜增殖性肾小球肾炎(MsPGN)、IgM肾病(IgMN)各10例,IgA肾病(IgAN)6例,局灶节段性肾小球硬化3例,膜增殖性肾小球肾炎、膜性肾病、肾小球硬化各1例。患儿经糖皮质激素长程疗法,部分加用雷公藤总苷片或环磷酰胺(CTX)针,对其进行随访并分析。结果临床类型与病理类型、难治类型关系密切,有显著统计学意义(χ2=29.91.20.26Pa<0.001);MCD及IgMN近期疗效较MsPGN及IgAN有显著差异(QCMH=21.14P<0.001),远期疗效无显著差异(P>0.05)。持续完全缓解36例(70.6%)。结论激素长程疗法及联合雷公藤总苷片或CTX疗效满意,但长期复发及激素依赖患儿的病因、病理转型及治疗方案仍需关注。更多还原

【Abstract】 Objective To explore the relationship between clinical and pathological manifestations in children with refractory nephrotic syndrome(RNS).Methods Fifty-one patients were recruited in this retrospective study.Of these 51 patients with RNS,31 cases were simple type,and 20 cases of nephritis type;25 cases were steroid RNS,20 cases with frequent relapse and 6 cases with steroid dependence.Renal biopsy results showed that 19 patients with minimal change disease(MCD),10 cases with mesangial prolife-rative glomerulonephritis(MsPGN),10 cases with IgM nephropathy(IgMN),6 cases with IgA nephropathy(IgAN),3 cases with focal segmental glomeruloslecrosis,1 case with membrane proliferative glomerulonephritis,1 case with membrane nephropathy,and 1 case with sclerosis glomerulonephritis,respectively.Clinical and pat-hological manifestations of children were collected and Chi-square test and cochran-mantel-haenszel were used to analyze the results.Results There was significant relationship between the results of clinical manifestations and renal biopsy,refractory types(χ2=29.91,20.26 Pa<0.001).The recent effects of treatment strategy in MCD and IgMN were better than those in MsPGN and IgAN(QCMH=21.14 P<0.001),but the long-term effects were not(P>0.05).For all of,the long remission rate was 70.6%.Conclusion Steroid,triptolide and cyclophosphmide are used well in all patients,but new treatment strategy in RNS children needs to be evaluated in the future.更多还原