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突破诊断障碍:在组织样本量有限的病例中,通过检测DNAJB9热休克蛋白诊断纤维性肾小球肾炎

Breaking the Diagnostic Barrier: DNAJB9 Heat Shock Protein Testing for Fibrillary Glomerulonephritis in Cases With Low Tissue Reserves.

作者信息

Qureshi Khadija, Kenmoe Jerry, Kunadi Arvind

机构信息

Internal Medicine, McLaren Hospital, Flint, USA.

Internal Medicine, Michigan State University College of Human Medicine, Flint, USA.

出版信息

Cureus. 2025 Jul 4;17(7):e87305. doi: 10.7759/cureus.87305. eCollection 2025 Jul.

DOI:10.7759/cureus.87305
PMID:40761955
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12319167/
Abstract

Fibrillary glomerulonephritis (FGN) is an uncommon primary glomerular condition with a vague clinical presentation that poses a diagnostic challenge. We present a case of a 73-year-old white female with severe kidney damage, malignant hypertension, and recurring abdominal pain who underwent a series of extensive tests until her renal biopsy showed eosinophilic deposits with mesangial matrix and GBM growth, indicating segmental membranoproliferative characteristics, and global mesangial proliferation of the glomeruli with fibrillary deposits. The DNAJB9 heat-shock protein was present in the tissue sample, supporting the FGN diagnosis. The lack of clear-cut treatment standards makes managing FGN difficult, and the disease has a dismal prognosis with a 50% possibility of developing end-stage renal disease within an average of 10 years post-diagnosis. Improving patient outcomes and halting further decline in renal function depend on early detection and effective care.

摘要

纤维性肾小球肾炎(FGN)是一种不常见的原发性肾小球疾病,临床表现不明确,给诊断带来挑战。我们报告一例73岁白人女性病例,她患有严重肾损害、恶性高血压和反复腹痛,接受了一系列广泛检查,直到肾活检显示有嗜酸性沉积物伴系膜基质和GBM生长,提示节段性膜增生性特征,以及肾小球全球系膜增生伴纤维性沉积物。组织样本中存在DNAJB9热休克蛋白,支持FGN诊断。缺乏明确的治疗标准使得FGN的管理困难,该疾病预后不佳,诊断后平均10年内有50%发展为终末期肾病的可能性。改善患者预后和阻止肾功能进一步下降取决于早期检测和有效护理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/9c3add40ad36/cureus-0017-00000087305-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/9b147d0ab437/cureus-0017-00000087305-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/a3605c8eb1a6/cureus-0017-00000087305-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/e3c1e6dfa1ab/cureus-0017-00000087305-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/380510568260/cureus-0017-00000087305-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/1ce756dc70b3/cureus-0017-00000087305-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/a26250231833/cureus-0017-00000087305-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/9c3add40ad36/cureus-0017-00000087305-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/9b147d0ab437/cureus-0017-00000087305-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/a3605c8eb1a6/cureus-0017-00000087305-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/e3c1e6dfa1ab/cureus-0017-00000087305-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/380510568260/cureus-0017-00000087305-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/1ce756dc70b3/cureus-0017-00000087305-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/a26250231833/cureus-0017-00000087305-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cc8b/12319167/9c3add40ad36/cureus-0017-00000087305-i07.jpg

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Fibrillary and immunotactoid glomerulopathies in the Hunter region: a retrospective cohort study.亨特地区的纤维样和免疫触须样肾小球病:一项回顾性队列研究。
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DNAJB9 Is a Reliable Immunohistochemical Marker of Fibrillary Glomerulonephritis: Evaluation of Diagnostic Efficacy in a Large Series of Kidney Biopsies.
DNAJB9是纤维性肾小球肾炎可靠的免疫组化标志物:对大量肾活检标本诊断效能的评估
Biomedicines. 2022 Aug 27;10(9):2102. doi: 10.3390/biomedicines10092102.
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Fibrillary Glomerulonephritis and DnaJ Homolog Subfamily B Member 9 (DNAJB9).纤维状肾小球肾炎和 DNAJ 同源物亚家族 B 成员 9(DNAJB9)。
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