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用去铁胺进行铁螯合疗法治疗库利贫血。

Iron chelation therapy with deferoxamine in Cooley anemia.

作者信息

Cohen A, Schwartz E

出版信息

J Pediatr. 1978 Apr;92(4):643-7. doi: 10.1016/s0022-3476(78)80314-x.

Abstract

The iron-chelating agent, deferoxamine, was studied in 16 patients with thalassemia major. Urinary excretion of iron in response to 0.75 gm of deferoxamine, intramuscularly, ranged from 2.2 to 44.8 mg Fe/24 hours. In response to a subcutaneous infusion of 1.5 gm deferoxamine for 18 hours, iron excretion increased by an average of 240%. The intravenous infusion of large doses of deferoxamine for 18 hours resulted in the highest rate of iron excretion, as much as 447.5 mg Fe/24 hours in response to 16 gm of deferoxamine. Administration of vitamin C increased chelation-induced excretion of iron in most patients more than five years of age. Preliminary evidence suggests that further iron accumulation can be prevented and excessive iron stores can be depleted by the intramuscular, subcutaneous, or intravenous administration of deferoxamine.

摘要

对16名重型地中海贫血患者进行了铁螯合剂去铁胺的研究。肌肉注射0.75克去铁胺后,24小时尿铁排泄量为2.2至44.8毫克铁。皮下输注1.5克去铁胺18小时后,铁排泄量平均增加240%。静脉输注大剂量去铁胺18小时导致铁排泄率最高,输注16克去铁胺时,24小时铁排泄量高达447.5毫克铁。维生素C的给药使大多数5岁以上患者因螯合作用导致的铁排泄增加。初步证据表明,通过肌肉注射、皮下注射或静脉注射去铁胺,可以预防进一步的铁蓄积,并消耗过量的铁储存。

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