Abstract: Objective　To investigate the clinical and pathological characteristics and prognosis of diffuse endocapillary proliferative IgA nephropathy（DEP-IgAN）in children. Methods　A total of 36 children diagnosed with DEP-IgAN by renal biopsy at the First Affiliated Hospital of Zhengzhou University from January 2011 to January 2024 were enrolled. Another 72 age- and sex-matched children with IgA nephropathy（IgAN）without diffuse endocapillary proliferation were included as the non-DEP-IgAN group. The clinical，pathological，and prognostic features between the two groups were compared. Results　Compared with the non-DEP-IgAN group，children with DEP-IgAN had a shorter disease duration before renal biopsy［17.0（12.0，24.8）days vs. 30.0（20.0，61.5）days，P<0.001］ and higher incidences of edema and hypertension. Serum albumin，complement C3，IgG levels，and estimated glomerular filtration rate were significantly lower，whereas 24-hour urinary protein excretion，serum creatinine，and total cholesterol levels were significantly higher（all P<0.05）. Pathologically，the DEP-IgAN group exhibited higher proportion of mesangial hypercellularity，tubular atrophy/interstitial fibrosis and crescents（all P<0.05）. In terms of treatment，more patients in the DEP-IgAN group received immunosuppressive agents（26/36 vs. 37/72，P=0.038），particularly cyclophosphamide and methylprednisolone（P<0.05）. Kaplan-Meier analysis revealed comparable overall remission rates between the two groups；however，the time to complete remission of proteinuria was longer in the DEP-IgAN group［7.0（5.8，9.0） months vs. 4.0（2.0，7.8） months，P<0.05］. Long-term outcomes showed no significant difference in cumulative renal survival between the two groups（P>0.05）. Conclusion　Pediatric DEP-IgAN is characterized by more severe clinical manifestations and pathological injury. Clinicians should pay greater attention to this subtype，as early and intensive immunosuppressive therapy may achieve outcomes comparable to those of non-DEP-IgAN.

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摘要： 目的　探讨儿童弥漫性毛细血管内增生型IgA肾病（diffuse endocapillary proliferative IgA nephropathy，DEP-IgAN）的临床病理特征及预后。方法　选取2011年1月至2024年1月于郑州大学第一附属医院经肾活检确诊的36例DEP-IgAN患儿，并以同时期性别、年龄相匹配但无弥漫性毛细血管内增生病变的72例IgAN患儿作为非DEP-IgAN组，比较两组临床病理特征及预后。结果　与非DEP-IgAN组相比，DEP-IgAN组肾活检前病程更短［17.0（12.0，24.8）d vs. 30.0（20.0，61.5）d，P<0.001］，水肿及高血压发生率更高，血清白蛋白、补体C3、血清IgG水平及肾小球滤过率显著降低，而24 h尿蛋白定量、血清肌酐及总胆固醇水平明显升高（均P<0.05）。病理上，DEP-IgAN组系膜细胞增生、肾小管萎缩或间质纤维化及新月体发生比例显著增加（均P<0.05）。在治疗上，DEP-IgAN组患儿更多接受免疫抑制药物（26/36 vs. 37/72，P=0.038），尤其使用环磷酰胺和甲泼尼龙频率更高（P<0.05）。Kaplan-Meier分析显示，两组最终缓解率相似，但DEP-IgAN组尿蛋白完全缓解时间延迟［7.0（5.8，9.0）个月 vs. 4.0（2.0，7.8）个月，P<0.05］。远期结局示两组肾脏累积存活率差异无统计学意义（P>0.05）。结论　儿童DEP-IgAN临床表现及病理损伤重，临床上应提高对此类型IgAN的重视，早期积极免疫治疗可能取得与非DEP-IgAN组相似的预后。

关键词: IgA肾病, 弥漫性, 毛细血管内增生, 预后, 儿童