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[原发性血色素沉着症和威尔逊氏病中的肝硬化]

[Liver cirrhosis in primary hemochromatosis and Wilson's disease].

作者信息

Kobune M, Kato J, Kohgo Y, Niitsu Y

机构信息

Department of Internal Medicine, Sapporo Medical University School of Medicine.

出版信息

Nihon Rinsho. 1994 Jan;52(1):209-14.

PMID:8114295
Abstract

Iron is an essential element in all living cells because it serves machineries for biological oxidation including hemoglobin, cytochrome c oxidase, etc. Copper is also essential for mammalian life since copper is the prosthetic element of several life-essential enzymes. Although intracellular excessive iron and copper were usually sequestrated in ferritin and metallothionein molecules, accumulation of excess iron and copper may also cause severe tissue injury by including oxyradicals and lipid peroxidation and eventually bring about tissue fibrosis such as liver cirrhosis. Hemochromatosis and Wilson's disease are known as iron and copper accumulation disorders, respectively. In this chapter, we review the cirrhosis in hemochromatosis and Wilson's disease.

摘要

铁是所有活细胞中的必需元素,因为它为包括血红蛋白、细胞色素c氧化酶等在内的生物氧化机制提供支持。铜对哺乳动物的生命也至关重要,因为铜是几种生命必需酶的辅基元素。尽管细胞内过量的铁和铜通常会被隔离在铁蛋白和金属硫蛋白分子中,但过量铁和铜的积累也可能通过产生氧自由基和脂质过氧化作用导致严重的组织损伤,并最终引发组织纤维化,如肝硬化。血色素沉着症和威尔逊氏病分别被认为是铁和铜积累紊乱疾病。在本章中,我们将综述血色素沉着症和威尔逊氏病中的肝硬化。

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