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成人继发性低丙种球蛋白血症伴微小病变病:一例报告

Minimal Change Disease in an Adult With Secondary Hypogammaglobulinemia: A Case Report.

作者信息

Agrawal Gautam, Agarwal Bhawna

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 Mar 14;17(3):e80555. doi: 10.7759/cureus.80555. eCollection 2025 Mar.

DOI:10.7759/cureus.80555
PMID:40225525
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11993934/
Abstract

Minimal change disease (MCD) is a common cause of nephrotic syndrome, particularly in children, but it also affects adults, albeit in smaller proportions. MCD presents with edema, severe proteinuria, hypoalbuminemia, and hyperlipidemia. MCD can lead to secondary hypogammaglobulinemia and an increased risk of infections by causing increased loss of immunoglobulins in the urine, impaired IgG synthesis, and from the immunosuppressive therapies used to treat MCD. The mainstay treatment for MCD is corticosteroids, which induce remission in most cases. However, some patients require other immunosuppressive therapies due to persistent relapses or resistance to standard treatment. This case report presents a 60-year-old woman with recurrent infections who was found to have hypogammaglobulinemia, with further workup showing nephrotic syndrome due to MCD. Her immunoglobulin levels normalized as her proteinuria levels improved with the treatment of her MCD.

摘要

微小病变性肾病(MCD)是肾病综合征的常见病因,在儿童中尤为常见,但也会影响成人,尽管比例较小。MCD表现为水肿、严重蛋白尿、低白蛋白血症和高脂血症。MCD可导致继发性低丙种球蛋白血症,并因尿液中免疫球蛋白丢失增加、IgG合成受损以及用于治疗MCD的免疫抑制疗法而增加感染风险。MCD的主要治疗方法是使用皮质类固醇,大多数情况下可诱导缓解。然而,由于持续复发或对标准治疗耐药,一些患者需要其他免疫抑制疗法。本病例报告介绍了一名60岁反复感染的女性,发现患有低丙种球蛋白血症,进一步检查显示因MCD导致肾病综合征。随着MCD治疗后蛋白尿水平改善,她的免疫球蛋白水平恢复正常。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28b8/11993934/7c8f07fe81e1/cureus-0017-00000080555-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28b8/11993934/7c8f07fe81e1/cureus-0017-00000080555-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28b8/11993934/7c8f07fe81e1/cureus-0017-00000080555-i01.jpg

相似文献

1
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本文引用的文献

1
Interventions for minimal change disease in adults with nephrotic syndrome.成人肾病综合征微小病变病的干预措施。
Cochrane Database Syst Rev. 2022 Mar 1;3(3):CD001537. doi: 10.1002/14651858.CD001537.pub5.
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Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: A Work Group Report of the AAAAI Primary Immunodeficiency and Altered Immune Response Committees.继发性低丙种球蛋白血症的诊断和管理实用指南:美国过敏、哮喘和免疫学学会原发性免疫缺陷和免疫反应改变工作组的报告。
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Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases.
KDIGO 2021肾小球疾病管理指南执行摘要。
Kidney Int. 2021 Oct;100(4):753-779. doi: 10.1016/j.kint.2021.05.015.
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BMC Nephrol. 2018 Aug 16;19(1):207. doi: 10.1186/s12882-018-0999-x.
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Minimal Change Disease.微小病变性肾病。
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The treatment of minimal change disease in adults.成人微小病变病的治疗。
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Adult minimal-change disease: clinical characteristics, treatment, and outcomes.成人微小病变病:临床特征、治疗及预后
Clin J Am Soc Nephrol. 2007 May;2(3):445-53. doi: 10.2215/CJN.03531006. Epub 2007 Apr 11.
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Impaired IgG synthesis in patients with the nephrotic syndrome.肾病综合征患者免疫球蛋白G合成受损。
Clin Nephrol. 1982 Sep;18(3):144-7.