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预防依赖输血的地中海贫血中的铁过载。

Prevention of iron loading in transfusion-dependent thalassaemia.

作者信息

Pippard M J, Letsky E A, Callender S T, Weatherall D J

出版信息

Lancet. 1978 Jun 3;1(8075):1178-81. doi: 10.1016/s0140-6736(78)90968-6.

Abstract

Urinary iron excretion after single intramuscular (i.m.) bolus injections or 12 h subcutaneous (s.c.) infusions of desferrioxamine (D.F.) was determined in sixteen homozygous beta-thalassaemia patients whose ages ranged from 10 months to 23 years. At all ages the s.c. infusions resulted in greater iron loss than identical i.m. doses. With doses of 0.5-1 g of D.F. as s.c. infusions eight out of nine children aged less than 6 years with a total transfusion iron load of less than 10 g excreted sufficient iron to achieve iron balance. These results suggest that iron loading in transfusion-dependent thalassaemics may be preventable by the use of an appropriate chelation regimen started early in life.

摘要

在16名年龄从10个月至23岁的纯合子β地中海贫血患者中,测定了单次肌内推注或12小时皮下输注去铁胺(D.F.)后的尿铁排泄量。在所有年龄段,皮下输注导致的铁流失均多于相同剂量的肌内注射。对于皮下输注0.5 - 1克去铁胺的情况,9名年龄小于6岁、总输血铁负荷小于10克的儿童中有8名排泄出足够的铁以实现铁平衡。这些结果表明,对于依赖输血的地中海贫血患者,通过在生命早期开始使用适当的螯合方案,铁过载可能是可以预防的。

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