纤维性肾小球肾炎的发病机制及诊治研究进展

doi:10.12372/jcp.2026.25e0295

摘要/Abstract

摘要：

纤维性肾小球肾炎（FGN）是一种病因不明的罕见肾小球疾病，DNAJ热休克蛋白家族成员B9蛋白（DNAJB9）可能参与其发病。该病临床表现多为肾病综合征，可合并血尿、高血压和肾功能不全，少数表现为急进性肾小球肾炎。肾组织电镜联合DNAJB9免疫组化对其诊断有重要价值。该病尚无特效治疗，预后差，未来亟须发掘该病潜在的治疗靶点以提高患者生存质量。本文对FGN可能的发病机制及临床特征进行了综述，以期引起大家在临床实践中对FGN的重视。

关键词: 纤维性肾小球肾炎, DNAJ热休克蛋白家族成员B9, 肾活检, 电镜, 肾病综合征

Abstract:

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with unknown etiology. Member of DNAJ heat shock protein family B9 (DNAJB9) protein may be involved in its pathogenesis. The clinical manifestations of FGN are mostly nephrotic syndrome, which can be combined with hematuria, hypertension and renal insufficiency. A few patients show rapid progressive glomerulonephritis. It has important diagnostic value by electron microscopic examination of renal tissue combined with immunohistochemistry of DNAJB9 in FGN patients.Unfortunately, there is no specific therapy at present, and the prognosis is poor. In the future, it is urgent to explore the potential therapeutic targets of FGN to improve the quality of life of FGN patients. This article reviews the possible pathogenesis and clinical characteristics of FGN.

Key words: fibrillary glomerulonephritis, member of DNAJ heat shock protein family B9, renal biopsy, electron microscope, nephrotic syndrome

中图分类号:

张媛媛, 赵德安. 纤维性肾小球肾炎的发病机制及诊治研究进展[J]. 临床儿科杂志, 2026, 44(2): 167-174.

ZHANG Yuanyuan, ZHAO Dean. Advances in pathogenesis, diagnosis and treatment of fibrillary glomerulonephritis[J]. Journal of Clinical Pediatrics, 2026, 44(2): 167-174.

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