威尔逊病的肾脏并发症及其治疗方法:病例报告和文献综述。

IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Clinical nephrology Pub Date : 2024-10-01 DOI:10.5414/CN111402
Maria Salman, Ayub Akbari, Gregory L Hundemer
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引用次数: 0

摘要

威尔逊病是一种罕见的常染色体隐性遗传性铜代谢紊乱病,会导致铜蓄积,进而引起器官功能障碍。虽然威尔逊病通常被认为是一种主要影响肝脏和神经系统的疾病,但威尔逊病及其治疗也可导致多种肾脏并发症。我们介绍了一例 31 岁的女性病例,她患有威尔逊病已有 10 年以上的病史,在从锌过渡到 D-青霉胺进行铜螯合治疗后,出现了急性肾病综合征,包括大量蛋白尿、低白蛋白血症和水肿。在简单停用 D-青霉胺(未使用任何免疫抑制剂,包括皮质类固醇)后,肾病综合征得到了显著改善,包括在数月内完全缓解。本综述全面总结了与威尔逊氏病有关的肾脏并发症及其治疗方法。
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Kidney complications of Wilson disease and its treatments: A case report and literature review.

Wilson disease is a rare autosomal recessive genetic disorder of copper metabolism that leads to copper accumulation and subsequent organ dysfunction. While classically considered a condition that primarily affects the liver and nervous system, Wilson disease and its treatments can also result in a wide range of kidney complications as well. We present the case of a 31-year-old female with a longstanding (> 10 year) history of Wilson disease who developed acute-onset nephrotic syndrome including heavy proteinuria, hypoalbuminemia, and edema after being transitioned from zinc to D-penicillamine for copper chelation therapy. Following simple cessation of D-penicillamine (and without any immunosuppressive therapies including corticosteroids), the nephrotic syndrome showed remarkable improvement including complete remission within several months. This review comprehensively summarizes the kidney complications associated with Wilson disease and its treatments.

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来源期刊
Clinical nephrology
Clinical nephrology 医学-泌尿学与肾脏学
CiteScore
2.10
自引率
9.10%
发文量
138
审稿时长
4-8 weeks
期刊介绍: Clinical Nephrology appears monthly and publishes manuscripts containing original material with emphasis on the following topics: prophylaxis, pathophysiology, immunology, diagnosis, therapy, experimental approaches and dialysis and transplantation.
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