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铁过载综合征与肝脏

Iron overload syndromes and the liver.

作者信息

Batts Kenneth P

机构信息

Pathology Lab, Division of Gastrointestinal Pathology, Minnesota Gastroenterology, Abbott Northwestern Hospital, Minneapolis, MN, USA.

出版信息

Mod Pathol. 2007 Feb;20 Suppl 1:S31-9. doi: 10.1038/modpathol.3800715.

DOI:10.1038/modpathol.3800715
PMID:17486050
Abstract

Iron can accumulate in the liver in a variety of conditions, including congenital, systemic iron-loading conditions (hereditary hemochromatosis), conditions associated with systemic macrophage iron accumulation (transfusions, hemolytic conditions, anemia of chronic disease, etc), in some hepatitidies (hepatitis C, alcoholic liver disease, porphyria cutanea tarda), and liver-specific iron accumulation of uncertain pathogenesis in cirrhosis. The anatomic pathologist will be faced with the task of determining whether iron accumulation in the liver is significant and, if so, the nature of the disease that lead to the accumulation (ie diagnosis). The tools available to the pathologist include (most importantly) histologic examination with iron stain, quantitative iron analysis, clinical history, laboratory iron tests (serum iron and iron-binding capacity, serum ferritin) and germline genetic analysis for mutations in genes known to be associated with hemochromatosis (HFE, ferroportin, hepcidin, hemojuvelin, transferrin receptor-2). This article provides an overview of the above.

摘要

铁可在多种情况下在肝脏中蓄积,包括先天性、全身性铁负荷情况(遗传性血色素沉着症)、与全身性巨噬细胞铁蓄积相关的情况(输血、溶血性疾病、慢性病贫血等)、某些肝炎(丙型肝炎、酒精性肝病、迟发性皮肤卟啉症)以及肝硬化中病因不明的肝脏特异性铁蓄积。解剖病理学家将面临确定肝脏中铁蓄积是否显著的任务,如果是,则要确定导致蓄积的疾病性质(即诊断)。病理学家可用的工具包括(最重要的是)铁染色组织学检查、定量铁分析、临床病史、实验室铁检测(血清铁和铁结合能力、血清铁蛋白)以及对已知与血色素沉着症相关基因(HFE、铁转运蛋白、铁调素、血色素沉着蛋白、转铁蛋白受体2)突变的种系基因分析。本文对上述内容进行概述。

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