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肝脏铁过载的病理学。

Pathology of hepatic iron overload.

机构信息

CHU Rennes, Liver Unit, Rennes, France.

出版信息

Semin Liver Dis. 2011 Aug;31(3):260-71. doi: 10.1055/s-0031-1286057. Epub 2011 Sep 7.

Abstract

The growing availability of genetic tests for most inherited iron-overload conditions and our current ability to assess hepatic iron stores, and at a lesser extent, liver fibrosis by noninvasive methods have reduced the need for liver biopsy in patients with hepatic iron excess. Histologic evaluation of the liver remains useful (1) in well-defined genetic iron overload disorders to evaluate associated hepatic damage, (2) in unclassified genetic or acquired iron excess to guide etiologic diagnosis and to establish prognosis, and (3) in research studies for a whole and reliable assessment of the liver. The identification of iron overload, the description of its cellular and lobular distribution, semiquantitative assessment of its amount, and inventory of associated lesions, especially fibrosis, are the pathologist's main objectives.

摘要

越来越多的遗传性铁过载疾病可进行基因检测,并且我们现在能够通过非侵入性方法评估肝铁储存量,在一定程度上还可以评估肝纤维化,这使得肝活检在铁过载患者中的应用减少。肝脏的组织学评估仍然具有重要意义:(1)在明确的遗传性铁过载疾病中,用于评估相关肝损伤;(2)在未分类的遗传性或获得性铁过载中,用于指导病因诊断和确定预后;(3)在研究中,用于全面而可靠地评估肝脏。铁过载的鉴定、其细胞和小叶分布的描述、其数量的半定量评估以及相关病变的清单,特别是纤维化,是病理学家的主要目标。

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