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以急性肾损伤和肾病综合征为表现的原发性局灶节段性肾小球硬化症:一例持续缓解的病例报告

Primary Focal Segmental Glomerulosclerosis Presenting With Acute Kidney Injury and Nephrotic Syndrome: A Case Report With Sustained Remission.

作者信息

Agrawal Gautam, Agarwal Bhawna, Sonavane Kunal, Shirsat Pallavi

机构信息

Nephrology, Independence Health System, Greensburg, USA.

Internal Medicine, University of Pittsburgh Medical Center McKeesport Hospital, McKeesport, USA.

出版信息

Cureus. 2025 Jun 7;17(6):e85499. doi: 10.7759/cureus.85499. eCollection 2025 Jun.

DOI:10.7759/cureus.85499
PMID:40625481
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12230872/
Abstract

Focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome in adults, characterized by segmental scarring of the glomeruli. It can present with proteinuria, hypoalbuminemia, edema, and varying degrees of renal dysfunction. Early diagnosis and treatment are critical to prevent progression to end-stage kidney disease. We report the case of a 53-year-old female who presented with worsening generalized edema, abdominal distension, and significant weight gain. Laboratory workup revealed significant proteinuria, hypoalbuminemia, and acute kidney injury, and kidney biopsy confirmed the diagnosis of FSGS. She was treated with high-dose corticosteroids, followed by initiation of losartan, and over a two-year follow-up period, she achieved and maintained complete remission with stable renal function and no recurrence of proteinuria. This case highlights the importance of prompt diagnosis and early intervention in FSGS. High-dose corticosteroids remain the cornerstone of treatment for primary FSGS, with adjunctive renin-angiotensin system blockade playing a key supportive role. Long-term follow-up is essential to monitor treatment response and maintain remission.

摘要

局灶节段性肾小球硬化(FSGS)是成人肾病综合征的常见病因,其特征为肾小球节段性瘢痕形成。它可表现为蛋白尿、低白蛋白血症、水肿及不同程度的肾功能不全。早期诊断和治疗对于预防进展至终末期肾病至关重要。我们报告一例53岁女性患者,其出现全身水肿加重、腹胀及体重显著增加。实验室检查显示大量蛋白尿、低白蛋白血症及急性肾损伤,肾活检确诊为FSGS。她接受了大剂量糖皮质激素治疗,随后开始使用氯沙坦,在两年的随访期内,她实现并维持了完全缓解,肾功能稳定且蛋白尿未复发。该病例凸显了FSGS快速诊断和早期干预的重要性。大剂量糖皮质激素仍然是原发性FSGS治疗的基石,肾素 - 血管紧张素系统阻滞剂作为辅助治疗发挥关键支持作用。长期随访对于监测治疗反应和维持缓解至关重要。

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本文引用的文献

1
Sparsentan versus Irbesartan in Focal Segmental Glomerulosclerosis.司帕生坦与厄贝沙坦治疗局灶节段性肾小球硬化症的比较
N Engl J Med. 2023 Dec 28;389(26):2436-2445. doi: 10.1056/NEJMoa2308550. Epub 2023 Nov 3.
2
KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases.KDIGO 2021肾小球疾病管理临床实践指南。
Kidney Int. 2021 Oct;100(4S):S1-S276. doi: 10.1016/j.kint.2021.05.021.
3
Therapeutic trials in adult FSGS: lessons learned and the road forward.成人 FSGS 的治疗试验:经验教训与前进道路。
Nat Rev Nephrol. 2021 Sep;17(9):619-630. doi: 10.1038/s41581-021-00427-1. Epub 2021 May 20.
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Advanced therapeutics in focal and segmental glomerulosclerosis.局灶节段性肾小球硬化的先进治疗方法
Nephrology (Carlton). 2018 Oct;23 Suppl 4:57-61. doi: 10.1111/nep.13463.
5
Protecting Podocytes: A Key Target for Therapy of Focal Segmental Glomerulosclerosis.保护足细胞:局灶节段性肾小球硬化症治疗的关键靶点。
Am J Nephrol. 2018;47 Suppl 1(Suppl 1):14-29. doi: 10.1159/000481634. Epub 2018 May 31.
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Focal Segmental Glomerulosclerosis.局灶节段性肾小球硬化症
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Focal segmental glomerulosclerosis.局灶节段性肾小球硬化症
N Engl J Med. 2011 Dec 22;365(25):2398-411. doi: 10.1056/NEJMra1106556.
8
Twenty-one-year trend in ESRD due to focal segmental glomerulosclerosis in the United States.美国因局灶节段性肾小球硬化导致的终末期肾病的21年趋势。
Am J Kidney Dis. 2004 Nov;44(5):815-25.