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重型β地中海贫血患者的铁过载与肝功能:一项横断面研究。

Iron overload and liver function in patients with beta thalassemia major: A cross sectional study.

作者信息

Faruqi Amna, Zafar Tooba, Subuctageen Sikander, Mughal Irfan Afzal

机构信息

Amna Faruqi, MBBS, MPhil, FCPS Department of Physiology, NUST School of Health Sciences, Islamabad, Pakistan.

Tooba Zafar, MBBS, FCPS Department of Physiology, NUST School of Health Sciences, Islamabad, Pakistan.

出版信息

Pak J Med Sci. 2024 Oct;40(9):2000-2004. doi: 10.12669/pjms.40.9.8961.

DOI:10.12669/pjms.40.9.8961
PMID:39416629
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11476128/
Abstract

OBJECTIVE

In thalassemia major, repeated blood transfusions result in iron overload causing organ damage. The objective of this study was assessment of liver enzymes in patients with Thalassemia major and to observe their association with ferritin.

METHOD

A cross-sectional study was performed, at Islamabad Medical and Dental College and its affiliated Akbar Niazi Teaching Hospital from November 2021 till August 2022. Serum ferritin, AST, ALT, and total bilirubin levels were determined, in 135 patients of beta thalassemia major receiving transfusions. Data analysis was performed using SPSS Version 20. For categorical variables, calculation of frequencies and percentages was performed. Mean (± standard deviation) was determined for quantitative variables. ANOVA with post hoc Tukey's test was used for determining association between liver enzymes and serum ferritin. A p-value of <0.05 was considered significant. The correlation between ferritin and LFTs was determined by Pearson's correlation coefficient.

RESULTS

Patients had an age range of 7-30 years, and males constituted 51% of sample. Mean level of ferritin was 6062.61 + 3641.79 ng/ml, with an insignificant difference between the genders ( =0.366). The levels of AST, ALT and bilirubin were perceived to show a significant increase in patients with ferritin levels >5000ng/ml, when compared with patients having ferritin levels < 2,500 ng/ml. A significant positive correlation of increasing serum ferritin levels was observed with ALT (r= 0.682), to a lesser extent with AST (r = 0.532), and only a weak correlation with serum bilirubin (r = 0.350).

CONCLUSION

Liver damage was caused by increased iron deposition. LFTs should be performed regularly to detect and reduce liver damage by increasing chelation therapy, thereby reducing morbidity and mortality due to thalassemia.

摘要

目的

在重型地中海贫血中,反复输血会导致铁过载,进而造成器官损伤。本研究的目的是评估重型地中海贫血患者的肝酶,并观察其与铁蛋白的关联。

方法

于2021年11月至2022年8月在伊斯兰堡医学与牙科学院及其附属的阿克巴·尼亚齐教学医院进行了一项横断面研究。测定了135例接受输血的重型β地中海贫血患者的血清铁蛋白、谷草转氨酶(AST)、谷丙转氨酶(ALT)和总胆红素水平。使用SPSS 20版进行数据分析。对于分类变量,计算频率和百分比。对于定量变量,确定均值(±标准差)。采用方差分析及事后Tukey检验来确定肝酶与血清铁蛋白之间的关联。p值<0.05被认为具有统计学意义。铁蛋白与肝功能检查结果之间的相关性通过Pearson相关系数来确定。

结果

患者年龄范围为7至30岁,男性占样本的51%。铁蛋白的平均水平为6062.61±3641.79 ng/ml,性别之间无显著差异(p=0.366)。与铁蛋白水平<2500 ng/ml的患者相比,铁蛋白水平>5000 ng/ml的患者的AST、ALT和胆红素水平明显升高。观察到血清铁蛋白水平升高与ALT呈显著正相关(r=0.682),与AST的相关性稍弱(r=0.532),与血清胆红素仅呈弱相关(r=0.350)。

结论

铁沉积增加导致肝脏损伤。应定期进行肝功能检查,通过加强螯合疗法来检测和减轻肝脏损伤,从而降低地中海贫血所致的发病率和死亡率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dc02/11476128/d60313e902aa/PJMS-40-2000-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dc02/11476128/459f109036f3/PJMS-40-2000-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dc02/11476128/d60313e902aa/PJMS-40-2000-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dc02/11476128/459f109036f3/PJMS-40-2000-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dc02/11476128/d60313e902aa/PJMS-40-2000-g002.jpg

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本文引用的文献

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Mediterr J Hematol Infect Dis. 2023 Sep 1;15(1):e2023048. doi: 10.4084/MJHID.2023.048. eCollection 2023.
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A New Perspective for Potential Organ Damage Due to Iron-Mediated Oxidation in Thalassemia Major Patients.重型地中海贫血患者铁介导氧化导致潜在器官损伤的新视角。
J Clin Med. 2023 Mar 21;12(6):2422. doi: 10.3390/jcm12062422.
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Pulmonary function in patients with transfusion-dependent thalassemia and its associations with iron overload.
输血依赖型地中海贫血患者的肺功能及其与铁过载的关系。
Sci Rep. 2023 Mar 4;13(1):3674. doi: 10.1038/s41598-023-30784-9.
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Lipids profile in children and adolescents with β-thalassemia major.重型β地中海贫血患儿及青少年的血脂谱
Hematol Transfus Cell Ther. 2023 Oct-Dec;45(4):467-472. doi: 10.1016/j.htct.2022.09.1277. Epub 2022 Nov 7.
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Optimized Droplet Digital PCR Assay on Cell-Free DNA Samples for Non-Invasive Prenatal Diagnosis: Application to Beta-Thalassemia.优化的游离 DNA 样本数字液滴 PCR 检测在无创性产前诊断中的应用:β-地中海贫血的应用。
Clin Chem. 2022 Jul 27;68(8):1053-1063. doi: 10.1093/clinchem/hvac076.
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