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首页> 外文期刊>Current opinion in gastroenterology >Inherited metabolic liver disease.
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Inherited metabolic liver disease.

机译：遗传性代谢性肝病。

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PURPOSE OF REVIEW: The purpose of this review is to identify and discuss recent findings related to inherited metabolic disorders of the liver that increase our understanding of the pathophysiology and treatment for hemochromatosis and other iron overload disorders, Wilson disease and alpha one antitrypsin deficiency. RECENT FINDINGS: The main theme in the recent discoveries for both iron overload disorders and Wilson disease is our increasing understanding that the phenotypic expression of these disorders are greatly influenced by genes involved in the metabolic pathways for these metals, or influence the progression of liver disease independent of metal metabolism. For example, the role of hepcidin dysregulation in hemochromatosis has been a surprising discovery that provides some mechanistic understanding for the increased iron absorption that is present in this disorder. SUMMARY: Given the recent explosion of information on iron and copper metabolism and the cellular processing of alpha one antitrypsin, the highlights reviewed in this article will help the reader keep up to date with the current understanding of these diseases and potential future approaches to their treatment.
机译：审查的目的：这次审查的目的是鉴定和讨论与肝脏遗传性代谢疾病有关的最新发现，这些发现增加了我们对血色素沉着病和其他铁超负荷疾病，威尔逊病和α一抗胰蛋白酶缺乏症的病理生理学和治疗的理解。最近的发现：关于铁超负荷疾病和威尔逊病的最新发现的主要主题是我们对这些疾病的表型表达在很大程度上受这些金属的代谢途径中涉及的基因的影响，或影响肝脏疾病的进展，这是我们日益加深的理解。独立于金属代谢。例如，铁调素在血色素沉着症中的失调作用是一个令人惊讶的发现，它为这种疾病中铁吸收的增加提供了一些机理上的理解。摘要：鉴于最近有关铁和铜代谢的信息以及α一抗胰蛋白酶的细胞加工信息激增，本文中重点介绍的内容将有助于读者了解这些疾病的最新知识以及潜在的未来治疗方法。

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