Comparison of effects of oral deferiprone and subcutaneous desferrioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia
Comparison of effects of oral deferiprone and subcutaneous desferrioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia
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DOI：
10.1016/s0140-6736(02)09740-4
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发表时间：
2002-08-17
期刊：
影响因子：
168.9
通讯作者：
Pennell, DJ
中科院分区：
文献类型：
--
作者：
Anderson, LJ;Wonke, B;Pennell, DJ
背景：尽管30多年前就引入了肠外铁络合剂去铁胺，但50%的重型地中海贫血症患者在35岁之前死亡，主要死于铁诱导的心力衰竭。唯一的替代治疗是口服去铁酮，但其对心肌铁浓度的长期疗效尚不清楚。方法比较15例接受长期去铁酮治疗的患者和30例长期服用去铁胺的地中海贫血症患者的心肌铁含量和心功能。用一种新的磁共振T2*技术测量了心肌铁浓度，其值与组织铁浓度成反比。发现去铁组的心肌铁(中位数34.0ms比11.4ms，p=0.02)和更高的射血分数(平均70%[SD6.5]比63%[6.9]，p=0.004)显著低于去铁胺对照组。心肌铁过量(T2*
Background Despite the introduction of the parenteral iron chelator desferrioxamine more than 30 years ago, 50% of patients with thalassaemia major die before the age of 35 years, predominantly from iron-induced heart failure. The only alternative treatment is oral deferiprone, but its long-term efficacy on myocardial iron concentrations is unknown.Methods We compared myocardial iron content and cardiac function in 15 patients receiving long-term deferiprone treatment with 30 matched thalassaemia major controls who were on long-term treatment with desferrioxamine. Myocardial iron concentrations were measured by a new magnetic-resonance T2* technique, which shows values inversely related to tissue iron concentration.Findings The deferiprone group had significantly less myocardial iron (median 34.0 ms vs 11.4 ms, p=0.02) and higher ejection fractions (mean 70% [SD 6.5] vs 63% [6.9], p=0.004) than the desferrioxamine controls. Excess myocardial iron (T2*