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不同铁鳌合剂治疗重型β地中海贫血铁过载的临床研究
A 5-vears clinical trial in iron-overload patients with β-thalassemia major treated by different iron chelators
【摘要】 目的比较去铁胺(DFO)、去铁酮(DFP)、地拉罗司(DFX)三种不同铁螯合剂治疗重型β地中海贫血(β-TM)铁过载患者5年的临床疗效。方法随机选择规律输血的β-TM铁过载患者31人,按DFO、DFP和DFX分为三个治疗组,分别对血清铁蛋白(SF)、心脏MRIT2*、肝脏MRIT2*值变化进行分析。结果服药5年后,三组SF均明显降低、心脏MRIT2*、肝脏MRIT2*明显升高(P<0.05);组间比较,三组SF水平5年连续监测差异无显著性(P>0.05);心脏MRIT2*组间比较差异无显著性(P>0.05),但与基线相比,第2年DFP组心脏MRIT2*值上升幅度明显高于DFX组、DFO组(P<0.05),后3年差异无显著性;5年连续监测肝脏MRIT2*DFO组高于DFP组、DFX组(P<0.05),与基线相比,DFO组第2年肝脏MRIT2*值上升幅度明显高于DFP组、DFX组(P<0.05),后3年差异无显著性。结论三种铁螯合剂均能有效降低重型β-TM患者SF浓度、去除心脏和肝脏铁沉积;短期内DFP降低心脏铁负荷优于DFO及DFX,DFO降低肝脏铁负荷优于DFP及DFX。
【Abstract】 Objective To explore effectiveness of different iron chelators( deferoxamine, DFO;deferiprone, DFP; deferasirox, DFX) in 5 years treatment of iron overlord in children with(3 thalassemia major( p-TM). Methods Thirty-one β-thalassemia major children with iron overload who received regular blood transfusion were randomly enrolled to three groups. Serum ferritin(SF), liver MRI T2 *and cardial MRI T2 * were detected and compared among the three groups. Results There were no significant differences in the SF level and the cardiac MRI T2 * value among the three treatment groups(P < 0.05) in five year’s observation. Compared with baseline, the cardiac MRI T2 * value were higher in DFP than in both of DFO and DFX during the second year(P <0. 05), but no difference for the rest of three years. The liver MRI T2 * value were higher in DFO group than DFP and DFX groups after 5 years(P <0.05). Compared with baseline, the liver MRI T2 * value were higher in DFO than in both of DFP and DFX during second year(P <0. 05), but no difference for the rest of three years.Conclusions Three chelators all can effectively reduce SF levels and lower liver and cardiac overload;However, Removing heart iron was significantly effective by DFP versus DFO and DFX in short time;DFO had better effect in removing liver iron overload than DFP and DFX.
【Key words】 β-thalassemia major; Iron overload; Iron chelator; MRIT2*;
- 【文献出处】 中国小儿血液与肿瘤杂志 ,Journal of China Pediatric Blood and Cancer , 编辑部邮箱 ,2017年04期
- 【分类号】R725.5
- 【被引频次】19
- 【下载频次】203