Body Iron Status and Its Complications in Patients With Beta Thalassemia Major: A Cross-Sectional Study

None Shafaq Ismail, None Shaiza Ijaz, None Spenta Kakalia, None Muhammad Ashraf Chaudhry, None Menahil Mukhtar, None Laiba Fayyaz
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Abstract

Introduction: Thalassemia is a genetic condition and is one of the commonest single-gene hereditary disorders. As it is among a group of hemoglobinopathies, it consists of a decrease in either alpha or beta globin chain synthesis. This eventually leads to the inappropriate ratio of alpha or beta globin chains causing ineffective erythropoiesis. Ineffective erythropoiesis thus causes chronic anemia and haemopoietic expansion to compensate for it. Aims & Objectives: This study aimed to investigate the serum ferritin levels of patients with beta thalassemia major presenting in a tertiary care hospital in Lahore, Pakistan and study the complications that occur with an abnormal iron load. Place and Duration of Study: It was conducted at CMH Hospital, Lahore, and completed over a period of one and a half year, from June 2021 to January 2023. Material & Methods: It was a descriptive cross-sectional study. The sample size of 32 was calculated using Raosoft calculator, with 90% Confidence Interval, 9% margin of error and 10.5% response distribution.Data was collected using an interviewer-administered questionnaire that compromised of 4 parts, demographics and socio-economic background of patient and caretakers; medical history and anthropometric measurement of the patient; details regarding serum ferritin levels and chelation; and lastly, laboratory (for example, TSH, T4, HbA1c and ALT for all patients, as well as FSH and LH for only those patients greater than 10 years of age), ultrasound, and cardiac echocardiography findings.Collected data was entered and analyzed in SPSS version 24. P value of <0.05 was taken as significant. Results: In this study, thirty-two patients with beta thalassemia major were included with mean age of 7.79 ± 4.57 years. Mean serum ferritin was 3410±2629µg/l and lack of compliance to chelation had significant association with serum ferritin values (p=0.05). Serum ALT levels showed that hepatic dysfunction was the most common endocrine complication in 17 (53.1%) patients. There was a significant association between compliance and echocardiography findings (p=0.04). No significant association of compliance was found with ultrasound findings, serum TSH and T4 levels, serum ALT levels and serum FSH and LH levels. Conclusion: This study concluded that high ferritin levels ultimately lead to significant complications.
β -地中海贫血患者的体铁状态及其并发症:一项横断面研究
地中海贫血是一种遗传性疾病,是最常见的单基因遗传性疾病之一。由于它是一组血红蛋白病,它包括α或β球蛋白链合成的减少。这最终导致α或β珠蛋白链比例不合适,导致红细胞生成无效。因此,无效的红细胞生成导致慢性贫血和造血扩张来补偿它。的目标是,目的:本研究旨在调查巴基斯坦拉合尔某三级医院乙型地中海贫血患者的血清铁蛋白水平,并研究铁负荷异常引起的并发症。研究地点和时间:在拉合尔CMH医院进行,从2021年6月到2023年1月,历时一年半完成。 材料,方法:采用描述性横断面研究。32人的样本量采用Raosoft计算器计算,置信区间90%,误差幅度9%,响应分布10.5%。使用访谈者管理的问卷收集数据,该问卷包含4个部分,即患者和护理人员的人口统计学和社会经济背景;患者的病史和人体测量;关于血清铁蛋白水平和螯合的细节;最后是实验室检查(例如,所有患者的TSH、T4、HbA1c和ALT,仅10岁以上患者的FSH和LH)、超声和心脏超声心动图检查结果。收集的数据在SPSS version 24中输入和分析。P值为<0.05为显著性。 结果:本研究纳入32例重度地中海贫血患者,平均年龄7.79±4.57岁。血清铁蛋白平均值为3410±2629µg/l,缺乏螯合依从性与血清铁蛋白值有显著相关性(p=0.05)。血清ALT水平显示17例(53.1%)患者以肝功能障碍为最常见的内分泌并发症。依从性与超声心动图结果有显著相关性(p=0.04)。超声检查结果、血清TSH和T4水平、血清ALT水平、血清FSH和LH水平与依从性无显著相关性。 结论:本研究得出高铁蛋白水平最终导致显著并发症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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