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重型地中海贫血多次输血患儿持续皮下输注去铁胺后血清铁蛋白延迟下降

Delayed decrease in serum ferritin in polytransfused children with thalassemia major after continuous subcutaneous infusions of desferrioxamine.

作者信息

Schettini F, Mautone A, Cavallo L, Altomare M, Montagna O, Dell'Edera L

出版信息

Acta Haematol. 1981;66(2):96-101. doi: 10.1159/000207104.

DOI:10.1159/000207104
PMID:6794319
Abstract

A longitudinal study has been conducted on serum ferritin concentrations in children with thalassemia major treated with desferrioxamine (DFO), both intramuscularly and by continuous subcutaneous infusion, in order to evaluate the time interval after which iron chelation becomes effective. In a first group of 19 children, treated intramuscularly with DFO at a dose of 20 mg/kg for 20 days a month, ferritin levels were followed for 739 days and a progressive increase with a significant linear correlation with time was seen. The daily increase was calculated to be 8.53 +/- 1.95 ng/ml. In the second group of 12 children treated with DFO by continuous subcutaneous infusion (greater than or equal to 20 mg/kg/day for 6 days a week), the increase in ferritin was markedly lower, the mean daily value being 4.77 +/- 3.30 ng/ml. Only after the first 360 days of treatment did the serum ferritin decrease, by 1.95 +/- 3.32 ng/ml/day, in spite of a mean daily iron contribution from blood transfusions of 7.09 +/- 2.69 mg/day. These changes in serum ferritin led us to conclude that one can only expect to obtain a decrease in the body's iron overload after more than a year of treatment with subcutaneous DFO.

摘要

为了评估铁螯合治疗开始起效的时间间隔,我们对接受去铁胺(DFO)治疗的重型地中海贫血患儿的血清铁蛋白浓度进行了一项纵向研究,治疗方式分别为肌肉注射和皮下持续输注。在第一组19名儿童中,每月肌肉注射20mg/kg的DFO,持续20天,观察铁蛋白水平739天,发现铁蛋白水平呈进行性升高,且与时间呈显著线性相关。计算得出每日升高值为8.53±1.95ng/ml。在第二组12名通过皮下持续输注DFO治疗的儿童中(每周6天,剂量≥20mg/kg/天),铁蛋白的升高明显较低,平均每日升高值为4.77±3.30ng/ml。尽管输血每日平均补铁量为7.09±2.69mg/天,但仅在治疗的前360天后,血清铁蛋白才开始下降,下降幅度为1.95±3.32ng/ml/天。血清铁蛋白的这些变化使我们得出结论,皮下注射DFO治疗一年以上才有望使体内铁过载降低。

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Delayed decrease in serum ferritin in polytransfused children with thalassemia major after continuous subcutaneous infusions of desferrioxamine.重型地中海贫血多次输血患儿持续皮下输注去铁胺后血清铁蛋白延迟下降
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