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去铁胺在重型地中海贫血中的作用机制及作用位点研究

A study of the mechanisms and sites of action of desferrioxamine in thalassaemia major.

作者信息

Bianco I, Graziani B, Lerone M, Congedo P, Clemente G F, Ingrao G, Ciccone F, Di Nucci G D, Mandelli F, Isacchi G

出版信息

Acta Haematol. 1984;71(2):100-5. doi: 10.1159/000206565.

Abstract

The effects of slow subcutaneous (s.c.) infusions of desferrioxamine (Desferal: DF) on iron metabolism and excretion were studied in 6 thalassaemia major patients in the course of a ferrokinetic study with 59Fe as a label; s.c. DF infusions were performed every 4th day starting 4 days after that of the 59Fe injection. Serum iron and total iron binding capacity (TIBC) increased after s.c. infusion, whereas serum ferritin levels remained unchanged. 59Fe urinary specific activity decreased in all subjects from the first to subsequent infusions, whereas faecal specific activity remained almost constant throughout the experiment. These data support the hypothesis that iron reaching RE cells concentrates initially in a readily chelatable pool from which then it moves to a larger and not readily chelatable pool, whereas iron reaching parenchymal hepatic cells remains permanently available to chelant.

摘要

在一项以59Fe为标记的铁动力学研究过程中,对6例重型地中海贫血患者进行了皮下缓慢输注去铁胺(去铁敏:DF)对铁代谢和排泄影响的研究;在注射59Fe后4天开始,每4天进行一次皮下DF输注。皮下输注后血清铁和总铁结合力(TIBC)升高,而血清铁蛋白水平保持不变。从首次输注到随后的输注,所有受试者尿中59Fe的比活性均下降,而在整个实验过程中粪便比活性几乎保持恒定。这些数据支持以下假设:到达RE细胞的铁最初集中在一个易于螯合的池中,然后从该池转移到一个更大且不易螯合的池中,而到达肝实质细胞的铁仍然可被螯合剂永久利用。

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