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评估重型地中海贫血患儿铁螯合疗效的尿铁排泄。

Urinary iron excretion for evaluating iron chelation efficacy in children with thalassemia major.

机构信息

Department of Child Health, Faculty of Medicine, Universitas Indonesia - Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia.

Department of Child Health, Faculty of Medicine, Universitas Indonesia - Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia.

出版信息

Blood Cells Mol Dis. 2019 Jul;77:67-71. doi: 10.1016/j.bcmd.2019.03.007. Epub 2019 Apr 1.

DOI:10.1016/j.bcmd.2019.03.007
PMID:30978615
Abstract

BACKGROUND

In patients with thalassemia major, examination routinely used for the evaluation of iron load in Indonesia is serum ferritin, but it is strongly influenced by other factors such as infections, inflammation and vitamin C levels. Evaluation of urinary iron excretion is an important and easy method to indicate iron chelation efficacy.

OBJECTIVE

To determine the efficacy of iron chelation therapy by urinary iron examination and to evaluate its correlation with the time of transfusion, serum ferritin level, transferrin saturation and T2* MRI.

METHODS

Prospective cohort study was conducted in children with thalassemia major aged 7-<18 years old who received DFP therapy. Twenty-four-hour urine collections were examined through inductively coupled plasma - mass spectrometry (ICP-MS). Patient's serum ferritin, transferrin saturation, peripheral blood, differential count and T2* MRI was documented during the study. Data analysis is based on urine iron level, body iron balance and the correlation between urine iron level, serum ferritin, transferrin saturation and T2* MRI and dosage of DFP.

RESULTS

Thirty (55%) subjects showed a higher urine iron level on the day prior to transfusion (mean: 12,828 SD ±12,801 μg/24 h) in comparison to post transfusion (mean: 10,985 SD ±10,023 μg/24 h). All subjects had positive iron balance (mean 524 SD ±230 mg). There were positive correlation between urine iron level and transferrin saturation (r = 0.559, p = 0.01) and serum ferritin (r = 0.291, p = 0.03), no correlation found with T2* MRI results.

CONCLUSIONS

There is a relationship to urinary iron excretion in response to chelation therapy and the degree of iron load.

摘要

背景

在重型地中海贫血患者中,印度尼西亚常规用于评估铁负荷的检查是血清铁蛋白,但它受到感染、炎症和维生素 C 水平等其他因素的强烈影响。尿铁排泄的评估是一种重要且简便的方法,可指示铁螯合治疗的效果。

目的

通过尿铁检查确定铁螯合治疗的效果,并评估其与输血时间、血清铁蛋白水平、转铁蛋白饱和度和 T2*MRI 的相关性。

方法

对 7-<18 岁接受 DFP 治疗的重型地中海贫血患儿进行前瞻性队列研究。通过电感耦合等离子体质谱法(ICP-MS)对 24 小时尿液进行检测。在研究期间记录患者的血清铁蛋白、转铁蛋白饱和度、外周血、分类计数和 T2MRI。数据分析基于尿铁水平、体内铁平衡以及尿铁水平与血清铁蛋白、转铁蛋白饱和度和 T2MRI 以及 DFP 剂量的相关性。

结果

30 名(55%)受试者在输血前一天的尿铁水平较高(平均值:12828 ±12801μg/24h),而输血后(平均值:10985 ±10023μg/24h)较低。所有受试者均存在正铁平衡(平均值 524 ±230mg)。尿铁水平与转铁蛋白饱和度(r=0.559,p=0.01)和血清铁蛋白(r=0.291,p=0.03)呈正相关,与 T2*MRI 结果无相关性。

结论

铁螯合治疗与铁负荷程度与尿铁排泄有关。

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