[Inherited metabolic liver disease causing copper deposition].

Q3 Medicine
H Jiang, C Liang, H Liu, S J Zheng
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引用次数: 0

Abstract

Copper, as a kind of trace element, is crucial for the physiological functions of various key enzymes in the body, and the liver plays a central role in maintaining copper metabolism. Theoretically, dysfunction in the body's metabolic processes, such as copper absorption, transportation, and excretion, can lead to copper deposition or deficiency in various organs. Wilson's disease's characteristic pathological manifestation is deposition of copper in liver. However, during liver pathological examinations, it has been found in clinical practice that certain patients with non-Wilson's disease and inherited metabolic liver disease may also have copper deposition. This review summarizes the inherited metabolic liver diseases that can cause liver copper deposition, their related pathogenesis, and the differential diagnosis approach from the perspectives of clinical and pathological characteristics.

[引起铜沉积的遗传性代谢性肝病]。
铜作为一种微量元素,对机体各种关键酶的生理功能起着至关重要的作用,而肝脏在维持铜的代谢中起着核心作用。从理论上讲,人体代谢过程中的功能障碍,如铜的吸收、运输和排泄,可导致各器官的铜沉积或缺铜。肝豆状核变性的特征性病理表现是肝内铜的沉积。然而,在肝脏病理检查中,在临床实践中发现,某些非威尔逊氏病和遗传性代谢性肝病患者也可能存在铜沉积。本文从临床和病理特点方面综述了可引起肝铜沉积的遗传性代谢性肝病及其相关发病机制和鉴别诊断方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
中华肝脏病杂志
中华肝脏病杂志 Medicine-Medicine (all)
CiteScore
1.20
自引率
0.00%
发文量
7574
期刊介绍:
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