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使用口服铁螯合剂1,2 - 二甲基 - 3 - 羟基吡啶 - 4 - 酮(L1)对输血性血色素沉着症患者进行长期治疗

[Long-term treatment of patients with transfusion hemosiderosis using oral iron chelator 1,2-dimethyl-3-hydroxypyrid-4-one (L1)].

作者信息

Goudsmit R

机构信息

Academisch Medisch Centrum, afd. Hematologie, Amsterdam.

出版信息

Ned Tijdschr Geneeskd. 1991 Nov 9;135(45):2133-6.

PMID:1944690
Abstract

A new oral iron chelator, L1, was given for 14 to 16 months to four patients with transfusion haemosiderosis. These patients could no longer be treated with deferoxamine because of allergic reactions or psychological problems. Serum ferritin values at the start of the treatment ranged from 2220 to 4530 microgram/l. L1 was given in a total daily dose of 3 g, at first in a single dose, later 3 times 1 g daily. The 24-hour urinary excretion of iron varied greatly from day to day. The mean urinary iron excretion of the four patients ranged from 19.3 to 45.7 mg/day. In two patients an important decrease of ferritin was found. These patients had been given no more or only sporadic transfusions. In one patient transient agranulocytosis was seen which was probably not caused by L1.

摘要

一种新型口服铁螯合剂L1给予4例输血性血色素沉着症患者服用14至16个月。这些患者因过敏反应或心理问题无法再用去铁胺治疗。治疗开始时血清铁蛋白值在2220至4530微克/升之间。L1的每日总剂量为3克,起初为单次给药,后来为每日3次,每次1克。铁的24小时尿排泄量每天变化很大。4例患者的平均尿铁排泄量在19.3至45.7毫克/天之间。在2例患者中发现铁蛋白显著下降。这些患者未再输血或仅偶尔输血。1例患者出现短暂性粒细胞缺乏症,可能并非由L1引起。

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