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Body iron metabolism and pathophysiology of iron overload

机译:体内铁代谢与铁超负荷的病理生理

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摘要

Iron is an essential metal for the body, while excess iron accumulation causes organ dysfunction through the production of reactive oxygen species. There is a sophisticated balance of body iron metabolism of storage and transport, which is regulated by several factors including the newly identified peptide hepcidin. As there is no passive excretory mechanism of iron, iron is easily accumulated when exogenous iron is loaded by hereditary factors, repeated transfusions, and other diseased conditions. The free irons, non-transferrin-bound iron, and labile plasma iron in the circulation, and the labile iron pool within the cells, are responsible for iron toxicity. The characteristic features of advanced iron overload are failure of vital organs such as liver and heart in addition to endocrine dysfunctions. For the estimation of body iron, there are direct and indirect methods available. Serum ferritin is the most convenient and widely available modality, even though its specificity is sometimes problematic. Recently, new physical detection methods using magnetic resonance imaging and superconducting quantum interference devices have become available to estimate iron concentration in liver and myocardium. The widely used application of iron chelators with high compliance will resolve the problems of organ dysfunction by excess iron and improve patient outcomes.
机译:铁是人体必需的金属,而过多的铁积累会通过产生活性氧而导致器官功能障碍。人体铁代谢的储存和运输之间存在复杂的平衡,这受几个因素的调节,其中包括新近鉴定出的肽铁调素。由于没有铁的被动排泄机制,当外源铁受遗传因素,反复输血和其他疾病影响时,铁很容易积聚。循环中的游离铁,未与运铁蛋白结合的铁和不稳定的血浆铁,以及细胞内不稳定的铁池是造成铁毒性的原因。铁超负荷的特征是除了内分泌功能障碍外,还有重要器官如肝脏和心脏衰竭。对于人体铁的估计,有直接和间接的方法。血清铁蛋白是最方便,使用最广泛的方法,尽管有时它的特异性存在问题。最近,使用磁共振成像和超导量子干涉装置的新物理检测方法已可用于估计肝脏和心肌中的铁浓度。具有高度依从性的铁螯合剂的广泛应用将解决铁过量引起的器官功能障碍问题并改善患者预后。

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