重型β地中海贫血的早期铁过载：何时开始螯合治疗？
Early iron overload in beta-thalassaemia major: when to start chelation therapy?
作者信息
Fargion S, Taddei M T, Gabutti V, Piga A, Di Palma A, Capra L, Fontanelli G, Avanzini A
出版信息
Arch Dis Child. 1982 Dec;57(12):929-33. doi: 10.1136/adc.57.12.929.
Abstract
Twenty-eight children with beta-thalassaemia major aged between 11 and 48 months were given intensive transfusions. Serum iron, transferrin saturation, serum ferritin, non-transferrin iron, and subcutaneous desferrioxamine-induced urinary iron excretion were measured. The results showed that even children with a limited number of transfusions had severe iron overload as indicated, in particular, by the raised serum ferritin levels and the high excretion rates after subcutaneous infusion of desferrioxamine. The desferrioxamine test was useful, even in very young children, in assessing response to chelation therapy thus enabling such treatment to be started early to prevent harm from iron overload.
摘要
对28名年龄在11至48个月之间的重型β地中海贫血患儿进行了强化输血治疗。检测了血清铁、转铁蛋白饱和度、血清铁蛋白、非转铁蛋白结合铁以及皮下注射去铁胺诱导的尿铁排泄量。结果显示，即使是输血次数有限的患儿也存在严重的铁过载，尤其表现为血清铁蛋白水平升高以及皮下注射去铁胺后的高排泄率。去铁胺试验即使在非常年幼的儿童中也有助于评估螯合疗法的反应，从而能够尽早开始此类治疗以预防铁过载造成的损害。
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