{"title":"[Inherited metabolic liver disease causing copper deposition].","authors":"H Jiang, C Liang, H Liu, S J Zheng","doi":"10.3760/cma.j.cn501113-20240825-00391","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":24006,"journal":{"name":"中华肝脏病杂志","volume":"33 7","pages":"697-703"},"PeriodicalIF":0.0000,"publicationDate":"2025-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"中华肝脏病杂志","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.3760/cma.j.cn501113-20240825-00391","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
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.