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肝移植后同种异体 C282Y 纯合子供肝发生肝铁过载:病例报告及文献复习。

Hepatic iron overload following liver transplantation of a C282y homozygous allograft: a case report and literature review.

机构信息

Liver Unit, St Vincent's University Hospital, Elm Park, Dublin 4, Ireland.

出版信息

Liver Int. 2011 Nov;31(10):1589-92. doi: 10.1111/j.1478-3231.2011.02606.x. Epub 2011 Jul 29.

Abstract

Hereditary haemochromatosis is a common genetic disease associated with progressive iron overload and parenchymal organ damage including liver, pancreas and heart. We report a case of inadvertent transplantation of a liver from a haemochromatosis donor to a 56-year-old Asian female. Progressive iron overload occurred over a 2 year follow up as assessed by liver biopsy and iron studies in the absence of a secondary cause of iron overload, supporting a primary role of liver rather than small intestine in the regulation of iron homeostasis in hereditary haemochromatosis.

摘要

遗传性血色素沉着症是一种常见的遗传性疾病,与进行性铁过载和实质器官损伤有关,包括肝脏、胰腺和心脏。我们报告了一例意外将遗传性血色素沉着症供体的肝脏移植到一名 56 岁亚洲女性的病例。在没有继发性铁过载原因的情况下,通过肝活检和铁研究评估,在 2 年的随访中发生了进行性铁过载,这支持肝脏而不是小肠在遗传性血色素沉着症中铁稳态调节中的主要作用。

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