Iron chelation therapy and thalassemia - an overview.
Iron chelation therapy and thalassemia - an overview.
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铁螯合疗法和地中海贫血 - 概述。
DOI：
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发表时间：
2011
期刊：
影响因子：
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通讯作者：
M. Haque
中科院分区：
文献类型：
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作者：
P. Chakrabarty;S. Rudra;M. Hossain;M. Bhuiyan;M. Khaleque;M. Haque
地中海贫血的流行病学、治疗和预后方面的新发展极大地改变了对受影响患者的护理方法。在过去的30年中，主要基于定期输血和去铁胺（DFO）铁螯合疗法的重型β地中海贫血的常规治疗显著改善了疾病的预后。适当的胃肠外DFO给药可减少或防止铁蓄积。DFO（dysferol）对大多数重型地中海贫血患者的不可用性以及处方治疗在其他患者中预防并发症的失败导致了对替代铁螯合剂的研究;其中之一，去铁酮（DFP）已上市。患者可能最终受益于几种螯合剂之间的选择，包括口服活性药物。联合治疗和器官靶向螯合，可能很快就会对地中海贫血患者的治疗结果和生活质量产生相当大的影响。
New developments in the epidemiology, treatment and prognosis of thalassemia have dramatically altered the approach to the care of affected patients. In the last 30 years, conventional treatment of β-thalassemia major, based primarily on regular blood transfusions and iron chelation therapy with desferrioxamine (DFO) has markedly improved the prognosis of the disease. Adequate administration of parenteral DFO reduces or prevents iron accumulation. The unavailability of DFO (dysferol) for most patients with thalassemia major and the failure of prescribed therapy to prevent complications in other patients have led to a search for alternative iron chelators; one of them, deferiprone (DFP) has been commercially available. Patients may ultimately benefit from having a choice between several chelators, including orally active drugs. Combination therapy and organ-targeted chelation, may soon have a considerable impact on the therapeutic outcome and quality of life of patients with thalassemia.