临床儿科杂志 ›› 2026, Vol. 44 ›› Issue (3): 202-208.doi: 10.12372/jcp.2026.25e1036

• 论著 • 上一篇    下一篇

8例儿童IgA肾病合并膜性肾病的临床病理学特征及预后分析:回顾性病例系列报告

林媛, 李华荣, 陈朝英()   

  1. 首都医科大学附属首都儿童医学中心肾脏内科 (北京 100020)
  • 收稿日期:2025-08-25 录用日期:2026-01-06 出版日期:2026-03-15 发布日期:2026-03-06
  • 通讯作者: 陈朝英 电子信箱:chenchaoying484@aliyun.com
  • 基金资助:
    北京市属医院科研培育计划项目(PX2023050)

Clinicopathological features and prognosis of eight children with coexisting IgA nephropathy and membranous nephropathy: retrospective case series report

LIN Yuan, LI Huarong, CHEN Chaoying()   

  1. Capital Center for Children’s Health, Capital Medical University, Beijng 100020, China
  • Received:2025-08-25 Accepted:2026-01-06 Published:2026-03-15 Online:2026-03-06

摘要:

目的 总结并分析8例儿童IgA肾病(IgAN)合并膜性肾病(MN)的临床与病理特征及预后。方法 本研究为回顾性病例系列,纳入2014年1月至2024年12月在首都医科大学附属首都儿童医学中心经肾活检确诊的8例IgAN合并MN(IgAN+MN)患儿,收集临床与病理资料,并结合国内外文献进行对比分析。结果 8例患儿中男4例、女4例,中位发病年龄7岁。主要表现为肉眼血尿和/或蛋白尿,50%伴肾病综合征,87.5%出现血尿。病理多为系膜增生性肾小球肾炎伴膜性病变,免疫荧光均示IgA系膜沉积及IgG基底膜沉积。所有患儿接受糖皮质激素联合免疫抑制剂治疗,完全缓解率100%,中位缓解时间2(1~5)个月。结论 儿童 IgAN+MN兼具IgAN+MN的临床表型,对激素及免疫抑制治疗反应良好,短期预后佳。早期诊断与积极治疗可改善预后,长期随访仍需关注复发及慢性进展。

关键词: IgA肾病, 膜性肾病, 临床病理特征, 预后, 儿童

Abstract:

Objective To summarize and analyze the clinical and pathological characteristics and short-term outcomes of eight children diagnosed with concomitant IgA nephropathy (IgAN) and membranous nephropathy (MN). Methods This retrospective case series included eight pediatric patients with biopsy-proven IgAN+MN between January 2014 and December 2024. Clinical manifestations, laboratory findings, and renal histopathology were reviewed and summarized, and the results were compared with those reported in the literature. Results The cohort comprised eight children (4 boys and 4 girls), with a median age at onset of 7 years. The main clinical presentations were gross hematuria and/or proteinuria. Nephrotic syndrome occurred in 50% of patients, and hematuria was observed in 87.5%. Renal pathology predominantly demonstrated mesangial proliferative glomerulonephritis accompanied by early- to intermediate-stage membranous lesions. Immunofluorescence showed IgA deposition in the mesangium and IgG deposition along the glomerular basement membrane. All patients were treated with glucocorticoids in combination with immunosuppressive agents. Complete remission was achieved in all cases (100%), with a median time to remission of 2 months (range, 1-5 months). Conclusion Pediatric IgAN+MN shows overlapping clinical phenotypes of IgAN and MN. Treatment with glucocorticoids plus immunosuppressants appears to be highly effective, and short-term prognosis is favorable. Early diagnosis and active intervention may improve outcomes; however, long-term follow-up is needed to monitor relapse and chronic progression.

Key words: IgA nephropathy, membranous nephropathy, clinicopathological features, prognosis, child

中图分类号: 

  • R72