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目的：研究难治性肾病综合征的临床与病理特点。方法：对９０例难治性肾病综合征患者进行肾穿术，取肾活组织进行免疫荧光、光镜、电子显微镜检查，同时将临床与病理进行对照分析。结果：难治性肾病综合征的病理类型为：局灶节段性肾小球硬化（ＦＳＧＳ）、膜性肾病（ＭＧＮ）、膜增生性肾炎（ＭＰＧＮ）、ＩｇＭ肾病（ＩｇＭＧＮ）、ＩｇＡ肾病（ＩｇＡＧＮ）及新月体肾炎（ＲＰＧＮ），临床与病理之间无肯定的因果关系。多元相关分析：病程长短、年龄（＞５０岁）、高血压、肾小球硬化、新月体形成、间质炎症细胞浸润及纤维化是促使肾小球滤过率下降的重要因素，间质炎症细胞浸润及纤维化是直接影响患者预后的一个重要因素。不同病理类型存在小管功能不全：ＦＳＧＳ５３．３％、ＭＧＮ３０％、ＭＰＧＮ６６．７％、ＩｇＭＧＮ２３％、ＩｇＡＧＮ３５．７％、ＲＰＧＮ６０％。结论：对于难治性肾病综合征不能盲目地依靠临床进行治疗，应尽早肾穿，明确诊断，同时要重视小管间质的损害程度，选择合理的治疗方案，提高疗效改善预后。
Objective: To study the clinical and pathological features of refractory nephrotic syndrome. Methods: Ninety patients with refractory nephrotic syndrome were treated with renal perforation. Immunofluorescence, light microscopy and electron microscopy were performed on living tissues of the kidney, and the clinical and pathological changes were analyzed. Results: The pathological types of refractory nephrotic syndrome were focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MGN), membranoproliferative nephritis (MPGN), IgM nephropathy (IgMGN), IgA nephropathy IgAGN) and crescentic glomerulonephritis (RPGN), there is no positive causal relationship between clinical and pathological. Multivariate correlation analysis showed that the duration of disease, age (> 50 years), hypertension, glomerulosclerosis, crescent formation, interstitial inflammatory cell infiltration and fibrosis were the important factors that led to the decrease of glomerular filtration rate. Inflammatory cell infiltration and fibrosis is a direct impact on the prognosis of patients is an important factor. Different pathological types of tubule insufficiency: FSGS53.3%, MGN30%, MPGN66.7%, IgMGN23%, IgAGN35.7%, RPGN60%. Conclusion: For refractory nephrotic syndrome can not blindly rely on clinical treatment, kidney as soon as possible, a clear diagnosis, while attention should be paid to the degree of tubulointerstitial damage, select a reasonable treatment plan to improve the efficacy and improve prognosis.