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特发性血色素沉着症

Idiopathic hemochromatosis.

作者信息

Kinney T B, Deluca S A

机构信息

Department of Radiology, Massachusetts General Hospital, Boston.

出版信息

Am Fam Physician. 1991 Sep;44(3):873-5.

PMID:1877429
Abstract

Hemochromatosis is a rare disorder in which about four times the normal amount of iron is absorbed in the intestine, resulting in the accumulation of iron in the skin, liver, pancreas and heart. Radiographic features may include a small liver on plain abdominal films, increased hepatic density on computed tomographic imaging, and loss of liver signal on magnetic resonance imaging as a result of the strong paramagnetic effect of iron. Management includes phlebotomy and supportive treatment of damaged organs.

摘要

血色素沉着症是一种罕见的疾病,其中肠道吸收的铁量约为正常量的四倍,导致铁在皮肤、肝脏、胰腺和心脏中蓄积。影像学特征可能包括腹部平片上肝脏较小、计算机断层扫描成像显示肝脏密度增加以及磁共振成像上由于铁的强顺磁效应导致肝脏信号丧失。治疗包括放血疗法和对受损器官的支持性治疗。

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