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重型β地中海贫血的口服与肠外联合铁螯合疗法

Combined oral and parenteral iron chelation in beta thalassaemia major.

作者信息

Balveer K, Pyar K, Wonke B

机构信息

Department of Paediatrics, Penang Hospital, Residency Road, 10450 Penang.

出版信息

Med J Malaysia. 2000 Dec;55(4):493-7.

Abstract

Thalassaemics in Malaysia are poorly chelated because desferrioxamine is too expensive and cumbersome for long term compliance. The efficacy and tolerability of the oral chelator deferiprone, and the effects of using a combination therapy in our patients were studied. Ten patients completed the study and the mean serum ferritin reduced from 7066.11 ug/L (2577-12,896 ug/L) to 3242.24 ug/L (955-6120 ug/L). The liver iron concentration did not show a significant drop (19.6 vs 18.2 mg/g dry weight) although 3 patients showed reductions ranging from 30-40%. Concomitant use of desferrioxamine increased the urinary excretion from a mean of 13.66 mg/day to 27.38 mg/day. Main side effects seen were nausea and rashes.

摘要

马来西亚的地中海贫血患者螯合治疗效果不佳,因为去铁胺过于昂贵且长期使用不便,难以保证患者的依从性。我们研究了口服螯合剂地拉罗司的疗效和耐受性,以及联合治疗对我们患者的影响。10名患者完成了研究,血清铁蛋白平均值从7066.11微克/升(2577 - 12,896微克/升)降至3242.24微克/升(955 - 6120微克/升)。肝脏铁浓度虽无显著下降(分别为19.6和18.2毫克/克干重),但有3名患者下降了30% - 40%。同时使用去铁胺使尿铁排泄量从平均每天13.66毫克增加到27.38毫克。主要副作用为恶心和皮疹。

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