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去铁胺强化铁螯合疗法治疗铁过载性贫血。

Intensive iron-chelation therapy with desferrioxamine in iron-loading anaemias.

作者信息

Pippard M J, Callender S T, Weatherall D J

出版信息

Clin Sci Mol Med. 1978 Jan;54(1):99-106. doi: 10.1042/cs0540099.

Abstract
  1. Urinary iron excretion after desferrioxamine has been examined in nine patinets with different iron-loading anaemias. Particular attention has been paid to individual variation in response and the kinetics of iron removal in order to determine the most efficient and convenient method of administration. 2. Twelve-hour subcutaneous infusions of desferrioxamine were comparable with intravenous infusions and gave a mean value of 62% more iron excretion than similar intramuscular bolus doses (range 20--125%). 3. Increasing doses as 12 h subcutaneous infusions produced a linear increase in iron excretion, which was followed by a tendency to reach a plateau. Iron excretion varied greatly between patients, was not related solely to age or estimated iron load, and in most cases was increased by ascorbic acid saturation. 4. Maximum iron-excretion rates were achieved after 3--6 and then maintained throughout an infusion. With bolus injections excretion rates declined rapidly after the first 6 h, during which approximately 60% of the total iron excretion occurred. 5. The dose and method of administration should be 'tailor-made' for each patient. Overnight 12 h subcutaneous infusions can be both as effective as similar doses given over 24 h and a practical way of achieving substantial negative iron balance. 6. Since children receiving regular blood transfusions for congenital anaemias such as thalassaemia usually die at the end of the second decade, this approach to iron chelation offers the possibility of alleviating what have hitherto been fatal-iron loading states.
摘要
  1. 已对9例患有不同类型铁过载贫血的患者进行了去铁胺治疗后尿铁排泄情况的研究。特别关注了个体反应差异及铁清除动力学,以确定最有效且便捷的给药方法。2. 去铁胺12小时皮下输注与静脉输注效果相当,铁排泄平均值比类似剂量的肌肉注射大丸剂多62%(范围为20%-125%)。3. 随着12小时皮下输注剂量增加,铁排泄呈线性增加,随后有达到平台期的趋势。患者间铁排泄差异很大,不仅与年龄或估计的铁负荷无关,且多数情况下因抗坏血酸饱和而增加。4. 最大铁排泄率在输注3-6小时后达到,然后在整个输注过程中维持。大丸剂注射后,排泄率在前6小时后迅速下降,在此期间约60%的总铁排泄量发生。5. 给药剂量和方法应针对每位患者“量身定制”。12小时夜间皮下输注既与24小时给予的类似剂量一样有效,也是实现显著负铁平衡的实用方法。6. 由于因先天性贫血如地中海贫血接受定期输血的儿童通常在第二个十年末死亡,这种铁螯合方法提供了缓解迄今致命的铁过载状态的可能性。

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