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第18届国际地中海贫血大会在希腊雅典举行:地中海贫血研究取得新突破,实现了铁过载的完全治疗,数百名患者实现并维持了正常的体内铁储备。关于螯合疗法的伦理问题。

The 18th ICOC Proceedings in Athens, Greece: New breakthrough in thalassemia leading to the complete treatment of iron overload and to hundreds of patients achieving and maintaining normal body iron stores. Ethical questions on chelation therapy.

作者信息

Kontoghiorghes George J

机构信息

Postgraduate Research Institute of Science, Technology, Environment and Medicine, Limassol, Cyprus.

出版信息

Hemoglobin. 2010 Jun;34(3):199-203. doi: 10.3109/03630269.2010.484963.

Abstract

A new era in thalassemia and other transfusional iron loading conditions was highlighted during the 18th International Conference on Chelation (ICOC) with reports that all excess iron accumulated from transfusions could be removed using the ICOC combination protocol of deferiprone (L1) (80-100 mg/kg/day) and subcutaneous deferoxamine (DFO) (40-60 mg/kg/day, at least 3 days per week), and that normal range body iron store levels (NRBISL) could be maintained using L1 monotherapy. Hundreds of patients in Cyprus, Greece, Italy, UK and elsewhere, maintain NRBISL, some for more than 9 years, and without complications. This gold standard of complete iron overload treatment is likely to change current practices, aims and protocols because it could prevent and also reverse cardiac, liver, endocrine and other organ complications as well as the incidence of infections and hepatocellular carcinomas. The overall morbidity and mortality in thalassemia and other transfusional iron loading conditions is expected to be substantially reduced. New applications of chelating drugs include renal, neurodegenerative, infectious diseases and ischemia reperfusion injury patients. Ethical questions have been raised on the role of pharmaceutical companies, the clinicians and the Hippocratic oath in relation to chelation therapy.

摘要

在第18届螯合国际会议(ICOC)期间,地中海贫血及其他输血性铁过载病症迎来了一个新时代,会上报告称,采用去铁酮(L1)(80 - 100毫克/千克/天)和皮下注射去铁胺(DFO)(40 - 60毫克/千克/天,每周至少3天)的ICOC联合方案,可以清除因输血积累的所有过量铁,并且采用L1单药疗法能够维持正常范围的机体铁储存水平(NRBISL)。塞浦路斯、希腊、意大利、英国及其他地方的数百名患者维持着NRBISL,部分患者已维持了9年以上,且无并发症。这种完全铁过载治疗的金标准可能会改变当前的治疗实践、目标和方案,因为它可以预防并逆转心脏、肝脏、内分泌及其他器官的并发症,以及感染和肝细胞癌的发生率。地中海贫血及其他输血性铁过载病症的总体发病率和死亡率预计将大幅降低。螯合药物的新应用包括用于肾脏、神经退行性疾病、传染病和缺血再灌注损伤患者。关于制药公司、临床医生以及希波克拉底誓言在螯合疗法中的作用,引发了伦理问题。

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