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成功治疗伴有急性间质性肾炎的塌陷性局灶节段性肾小球硬化症肾病综合征。

Successful Treatment of Nephrotic Syndrome Due to Collapsing Focal Segmental Glomerulosclerosis Accompanied by Acute Interstitial Nephritis.

机构信息

Department of Kidney Disease, Kawashima Hospital, Japan.

Department of Laboratory, Kawashima Hospital, Japan.

出版信息

Intern Med. 2022 Jun 15;61(12):1863-1867. doi: 10.2169/internalmedicine.8258-21. Epub 2021 Nov 20.

Abstract

A 39-year-old woman was hospitalized for nephrotic syndrome. Laboratory test results showed increased serum creatinine levels and urinary excretions of beta-2-microglobulin, and N-acetyl-beta-D-glucosaminidase. A renal biopsy revealed collapsing focal segmental glomerulosclerosis (FSGS) and acute interstitial nephritis. Despite treatment with pulse steroid followed by oral high-dose glucocorticoids and cyclosporines, heavy proteinuria persisted. After low-density lipoprotein apheresis (LDL-A) therapy was initiated, her proteinuria gradually decreased, leading to complete remission. A repeat renal biopsy after treatment revealed no collapsing glomeruli. Immediate LDL-A should be performed to treat cases of collapsing FSGS poorly responding to other treatments.

摘要

一位 39 岁女性因肾病综合征住院。实验室检查结果显示血清肌酐水平升高,β-2-微球蛋白和 N-乙酰-β-D-氨基葡萄糖苷酶尿排泄增加。肾活检显示局灶性节段性肾小球硬化(FSGS)伴急性间质性肾炎。尽管接受了脉冲类固醇治疗,随后又口服大剂量糖皮质激素和环孢菌素治疗,但仍持续存在大量蛋白尿。开始进行低密度脂蛋白吸附(LDL-A)治疗后,患者的蛋白尿逐渐减少,病情完全缓解。治疗后重复肾活检显示没有塌陷的肾小球。对于其他治疗反应不佳的塌陷 FSGS 病例,应立即进行 LDL-A 治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/375e/9259812/bcebac98d500/1349-7235-61-1863-g001.jpg

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