Ghanavat Majid, Haybar Habib, Pezeshki Seyed Mohammad Sadegh, Shahjahani Mohammad, Jodat Hosein, Elyasi Milad, Saki Najmaldin
Child Growth & Development Research Center, Isfahan University of Medical Sciences, Isfahan, Iran.
Atherosclerosis Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
Lab Med. 2020 Mar 10;51(2):143-150. doi: 10.1093/labmed/lmz052.
Cardiomyopathic manifestations induced by continuous blood transfusion are the leading cause of death among patients with thalassemia major (TM). Despite introduction of chelation therapy, heart failure after cardiomyopathic manifestations is still a major threat to patients.
We performed a search of relevant English-language literature, retrieving publications from the PubMed database and the Google Scholar search engine (2005-2018). We used "thalassemia major", "cardiomyopathy", "iron overload", "cardiac magnetic resonance T2" "chelation therapy", and "iron burden" as keywords.
The results of the studies we found suggest that cardiac hepcidin is a major regulator of iron homeostasis in cardiac tissue. Unlike previous assumptions, the heart appears to have a limited regeneration capability, originating from a small population of hypoxic cardiomyocytes.
Oxygen levels determine cardiomyocyte gene-expression patterns. Upregulation of cardiac hepcidin in hypoxia preserves cardiomyocytes from forming out of reactive oxygen species catalyzed by free cellular iron in cardiomyocytes. Using the limited regeneration capacity of cardiac cells and gaining further understanding of the cellular aspects of cardiomyopathic manifestations may help health care professionals to develop new therapeutic strategies.
持续输血诱发的心肌病表现是重型地中海贫血（TM）患者死亡的主要原因。尽管引入了螯合疗法，但心肌病表现后的心力衰竭仍是患者面临的主要威胁。
我们检索了相关英文文献，从PubMed数据库和谷歌学术搜索引擎（2005 - 2018年）中获取出版物。我们使用“重型地中海贫血”“心肌病”“铁过载”“心脏磁共振T2”“螯合疗法”和“铁负荷”作为关键词。
我们发现的研究结果表明，心脏铁调素是心脏组织中铁稳态的主要调节因子。与先前的假设不同，心脏似乎具有有限的再生能力，源于一小部分缺氧心肌细胞。
氧水平决定心肌细胞基因表达模式。缺氧时心脏铁调素的上调可保护心肌细胞免受心肌细胞内游离细胞铁催化产生的活性氧的影响。利用心脏细胞有限的再生能力并进一步了解心肌病表现的细胞层面，可能有助于医护人员制定新的治疗策略。
