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血红蛋白相关疾病中铁的生理学与病理生理学

Physiology and pathophysiology of iron in hemoglobin-associated diseases.

作者信息

Coates Thomas D

机构信息

Children׳s Center for Cancer and Blood Diseases, Children׳s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, CA 90027, USA.

出版信息

Free Radic Biol Med. 2014 Jul;72:23-40. doi: 10.1016/j.freeradbiomed.2014.03.039. Epub 2014 Apr 12.

DOI:10.1016/j.freeradbiomed.2014.03.039
PMID:24726864
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4940047/
Abstract

Iron overload and iron toxicity, whether because of increased absorption or iron loading from repeated transfusions, can be major causes of morbidity and mortality in a number of chronic anemias. Significant advances have been made in our understanding of iron homeostasis over the past decade. At the same time, advances in magnetic resonance imaging have allowed clinicians to monitor and quantify iron concentrations noninvasively in specific organs. Furthermore, effective iron chelators are now available, including preparations that can be taken orally. This has resulted in substantial improvement in mortality and morbidity for patients with severe chronic iron overload. This paper reviews the key points of iron homeostasis and attempts to place clinical observations in patients with transfusional iron overload in context with the current understanding of iron homeostasis in humans.

摘要

铁过载和铁毒性,无论是由于铁吸收增加还是反复输血导致的铁负荷增加,都可能是多种慢性贫血发病和死亡的主要原因。在过去十年中,我们对铁稳态的理解取得了重大进展。与此同时,磁共振成像技术的进步使临床医生能够无创地监测和量化特定器官中的铁浓度。此外,现在已有有效的铁螯合剂,包括可以口服的制剂。这使得严重慢性铁过载患者的死亡率和发病率有了显著改善。本文回顾了铁稳态的要点,并试图将输血性铁过载患者的临床观察结果与目前对人体铁稳态的理解相结合。