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目的：观察儿童肾病综合征的临床病理与远期疗效。方法：对１９７８年２月～１９９３年１２月经肾穿刺确诊病理类型的２４４例儿童肾病综合征进行随访，停用强的松２年１个月～８年６个月，平均４２．８个月。结果：经强的松，氮芥和中药联合治疗近、远期疗效较好。停用强的松时完全缓解２２２例（９１％），随访时基本治愈１３１例（５３．７％），完全缓解８０例（３２．８％），共８６．５％，复发１９例（８．６％）（其中微小病变复发１４．８％），部分缓解７例（２．９％），激素依赖４例（１．６％），恶化３例（１．２％）。结论：肾病综合征的远期疗效大多数良好，临床分型和病理类型及病变程度与疗效密切相关，单纯性肾病的近、远期疗效较肾炎性肾病好（Ｐ＜０．０５），中度系膜增生性肾炎，中度系膜增生性ＩｇＭ肾病与轻度者相比疗效相差显著（Ｐ＜０．０５），膜增生性肾炎疗效较差，其它病理类型疗效差异不明显（Ｐ＞０．０５）。
Objective: To observe the clinical pathology and long-term effect of nephrotic syndrome in children. Methods: A total of 244 children with nephrotic syndrome diagnosed by renal biopsy from February 1978 to December 1993 were followed up. The prednisone was stopped for 2 months from 1 month to 8 years and 6 months with an average of 42.8 months . Results: The combination of prednisone, nitrogen mustard and traditional Chinese medicine near and long-term effect is better. 222 cases (91%) were relieved when prednisone was stopped, 131 cases (53.7%) were cured basically, 80 cases (32.8%) were completely relieved, 86.5% .6%) (of which 14.8% were minor lesion), 7 (2.9%) partially relieved, 4 (1.6%) hormone dependent and 3 (1.2%) worsened. Conclusion: The long-term curative effect of nephrotic syndrome is mostly good. The clinical classification and pathological type and degree of lesion are closely related to curative effect. The short-term and long-term curative effect of nephrotic nephropathy is better than that of nephritic nephropathy (P <0.05) Mesangial proliferative glomerulonephritis, moderate mesangial proliferative IgM nephropathy compared with mild effect was significantly different (P <0.05), the efficacy of membranoproliferative glomerulonephritis is poor, the effect of other pathological types was not significantly different (P> 0.05).