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中间型β地中海贫血的铁过载与去铁胺螯合疗法

Iron overload and desferrioxamine chelation therapy in beta-thalassemia intermedia.

作者信息

Cossu P, Toccafondi C, Vardeu F, Sanna G, Frau F, Lobrano R, Cornacchia G, Nucaro A, Bertolino F, Loi A, De Virgiliis S, Cao A

出版信息

Eur J Pediatr. 1981 Nov;137(3):267-71. doi: 10.1007/BF00443255.

Abstract

This study on serum ferritin levels in urinary iron excretion after 12h subcutaneous infusion of desferrioxamine in 10 thalassemia intermedia patients shows that even nontransfusion-dependent patients may have positive iron balance resulting in iron overload from 5 years of age. However, the iron overload found in these patients appears to be much lower than in age matched patients with transfusion-dependent thalassemia major. Iron overload increases with advancing age, as shown by increasing serum ferritin levels and desferrioxamine-induced urinary iron elimination. After a six month trial of 12h continuous subcutaneous desferrioxamine administration there was a significant decline in serum ferritin levels. From this study it seems that iron chelation is indicated in thalassemia intermedia patients over 5 years of age in order to prevent iron accumulation. However, the appropriate treatment schedule should be tailored to the individual needs of each patients, established by close monitoring of serum ferritin levels and desferrioxamine-induced urinary iron elimination.

摘要

这项针对10名中间型地中海贫血患者皮下注射去铁胺12小时后尿铁排泄及血清铁蛋白水平的研究表明,即使是非输血依赖型患者,从5岁起也可能出现正铁平衡,导致铁过载。然而,这些患者中发现的铁过载似乎远低于年龄匹配的输血依赖型重型地中海贫血患者。正如血清铁蛋白水平升高和去铁胺诱导的尿铁排泄所显示的那样,铁过载随年龄增长而增加。在连续皮下注射去铁胺12小时进行为期6个月的试验后,血清铁蛋白水平显著下降。从这项研究来看,似乎5岁以上的中间型地中海贫血患者需要进行铁螯合治疗,以防止铁蓄积。然而,合适的治疗方案应根据每位患者的个体需求制定,通过密切监测血清铁蛋白水平和去铁胺诱导的尿铁排泄来确定。

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