输血依赖性印尼地中海贫血患者的铁超载。

IF 2.2 Q3 HEMATOLOGY Anemia Pub Date : 2021-04-15 eCollection Date: 2021-01-01 DOI:10.1155/2021/5581831
Pandji Irani Fianza, Anita Rahmawati, Sri Hudaya Widihastha, Shofura Afifah, Mohammad Ghozali, Andre Indrajaya, Dilli Marayuzan Akbar Pratama, Dimmy Prasetya, Teddy Arnold Sihite, Mas Rizky A A Syamsunarno, Djatnika Setiabudi, Suthat Fucharoen, Ramdan Panigoro
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引用次数: 4

摘要

地中海贫血是一种遗传性疾病,由珠蛋白链合成中断导致严重贫血,因此需要定期输血。然而,已知输血相关的后果,包括铁超载和多器官损伤。本研究的目的是评估青年和成人输血依赖型印尼地中海贫血患者的肝功能和心功能,并评估其与血清铁蛋白水平以及T2 *磁共振成像(MRI)的相关性。输血依赖型地中海贫血(TDT)门诊患者(n = 66;平均年龄21.5±7.2岁)进行全面评估,包括血液检查包括肝酶和血清铁蛋白,然后进行心电图和超声心动图检查。根据血清铁蛋白水平将受试者分为< 2500 ng/mL、2500-5000 ng/mL和>5000 ng/mL三组。此外,对T2 * MRI评估患者进行亚组分析。从首次输血年龄来看,铁蛋白>5000 ng/mL的受试者年龄最小。血清铁蛋白≥5000ng /mL组丙氨酸转氨酶(ALT)水平显著高于血清铁蛋白≥5000ng /mL组,三尖瓣环形平面收缩位移(TAPSE)显著低于血清铁蛋白* MRI显著低于无心脏铁素体沉积组。血清铁蛋白与心脏T2 * MRI之间有显著但仅中等程度的相关性。基于这些发现,常规监测铁积累相关并发症,包括青年和成人TDT患者的肝脏和心脏损伤是很重要的。
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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Iron Overload in Transfusion-Dependent Indonesian Thalassemic Patients.

Thalassemia is a genetic disease caused by disruption of globin chain synthesis leading to severe anemia and thus regular blood transfusion is necessary. However, there have been known transfusions-related consequences, including iron overload and multi-organ damage. The aims of this study were to evaluate liver and cardiac function in youth and adult transfusion-dependent Indonesian thalassemic patients and to assess its correlation with serum ferritin levels, as well as T2 magnetic resonance imaging (MRI). Transfusion-dependent thalassemic (TDT) outpatients (n = 66; mean age, 21.5 ± 7.2 years) were carried out for the complete assessment consisting of blood test including liver enzyme and serum ferritin, followed by electrocardiography (ECG) and echocardiography. Subjects were also divided by serum ferritin levels into three groups: < 2500 ng/mL, 2500-5000 ng/mL, and >5000 ng/mL. Additionally, subgroup analysis in patients with T2 MRI assessment was conducted. In terms of age of first blood transfusion, subjects with ferritin >5000 ng/mL were the youngest among others. The alanine aminotransferase (ALT) levels in group with serum ferritin >5000 ng/mL were significantly higher than those of the group with serum ferritin <2500 ng/mL. Additionally, youth and adult TDT patients whose serum ferritin >5000 ng/mL had significantly lower tricuspid annular plane systolic excursion (TAPSE) when compared with those who had serum ferritin <2500 ng/mL. Similarly, TAPSE in patients with moderate cardiac siderosis based on cardiac T2 MRI was significantly lower than those without cardiac siderosis. There was significant, but only moderate correlation between serum ferritin and cardiac T2 MRI. Based on these findings, it is important to routinely monitor iron accumulation-related complications, including liver and cardiac damage in youth and adult TDT patients.

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来源期刊
Anemia
Anemia HEMATOLOGY-
CiteScore
4.80
自引率
3.40%
发文量
11
审稿时长
18 weeks
期刊介绍: Anemia is a peer-reviewed, Open Access journal that publishes original research articles, review articles, and clinical studies on all types of anemia. Articles focusing on patient care, health systems, epidemiology, and animal models will be considered, among other relevant topics. Affecting roughly one third of the world’s population, anemia is a major public health concern. The journal aims to facilitate the exchange of research addressing global health and mortality relating to anemia and associated diseases.
期刊最新文献
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