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肾脏病与透析肾移植杂志 ›› 2025, Vol. 34 ›› Issue (6): 590-594.DOI: 10.3969/j.issn.1006⁃298X.2025.06.017

• 病例报告 • 上一篇    下一篇

Wilson 病相关膜性肾病

  

  • 出版日期:2025-12-28 发布日期:2025-12-29

Wilson’s disease⁃associated membranous nephropathy: a case report

  • Online:2025-12-28 Published:2025-12-29

摘要: 33 岁女性患者,因 “发现尿检异常 20 年,大量蛋白尿 4 月” 入院。患者 20 年前因神经系统异常伴少量蛋白尿于外院诊断为 Wilson 病,长期予青霉胺驱铜治疗。本次因自行停用驱铜药一年余后 Wilson 病复发,肾损伤从少量蛋白尿,逐渐发展为肾病综合征,血清肌酐正常。完善肾活检检查,病理示肾小球膜性病变,免疫荧光染色示 IgG4、IgA、C3 阳性为主,抗磷脂酶 A2 受体、含血小板反应蛋白 1 型结构域 7A 蛋白等膜性肾病抗体均阴性,电镜下见肾小球足细胞足突广泛融合,诊断为 Wilson 病相关膜性肾病。予以他克莫司治疗后患者肾病综合征达到临床缓解状态。

关键词: font-family:Inter, -apple-system, BlinkMacSystemFont, ", font-size:16px, background-color:#FFFFFF, ">Wilson 病、肝豆状核变性、膜性肾病

Abstract: This is a case of a 33⁃year⁃old female patient who was admitted to our hospital due to "abnormal urine test results detected for 20 years and massive proteinuria for 4 months". Twenty years ago, the patient was diagnosed with Wilson’s disease at another hospital based on abnormal neurological manifestations accompanied by mild proteinuria, and had received long⁃term penicillamine therapy for copper chelation. The current admission was prompted by a relapse of Wilson’s disease after self⁃discontinuation of copper⁃chelating medication for over a year. The renal injury presented with mild proteinuria as the initial manifestation, progressed gradually to nephrotic syndrome, and the serum creatinine level was within the normal range. Renal biopsy revealed membranous nephropathy, with immunofluorescence staining predominantly positive for IgG4, IgA, and C3, while antibodies associated with membranous nephropathy such as anti⁃PLA2R and anti⁃THSD7A were negative. Electron microscopy demonstrated extensive effacement of podocyte foot processes. A diagnosis of Wilson’s disease⁃associated membranous nephropathy was established. The patient achieved clinical remission of nephrotic syndrome following treatment with tacrolimus.

Key words: font-family:Inter, -apple-system, BlinkMacSystemFont, ", font-size:16px, background-color:#FFFFFF, ">Wilson disease、hepatolenticular degeneration、membranous nephropathy