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β 地中海贫血患者的心脏并发症和铁过载:系统评价和荟萃分析。

Cardiac complications and iron overload in beta thalassemia major patients-a systematic review and meta-analysis.

机构信息

Student Research Committee, Department of Epidemiology, School of Public Health and Safety, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Cardiovascular Diseases Research Center & Department of Cardiology, Faculty of Medicine, Birjand University of Medical Sciences, Birjand, Iran.

出版信息

Ann Hematol. 2019 Jun;98(6):1323-1331. doi: 10.1007/s00277-019-03618-w. Epub 2019 Feb 7.

Abstract

Despite the major improvement in therapeutic management of thalassemia major, iron overload is considered a challenging conundrum in these patients and heart disease still remains a major cause of morbidity and mortality in these patients. Therefore, this study aimed to investigate the prevalence of cardiac iron overload and cardiovascular complications in transfusion-dependent thalassemia patients in the worldwide. The following databases were searched: ISI/Web of Science, Embase, PubMed, Scopus, up to February 30, 2018. The quality of the studies was evaluated using the Joanna Briggs Institute Prevalence Critical Appraisal Tool. The random model based on Metaprop was used. One hundred forty-two studies were included. The total number of patients included was 26,893. The mean age of patients was 22.6 (SD = 1.7) years. Based on Metaprop, the overall prevalence of cardiac iron overload/myocardial sidoresis (T2* < 20 ms) and cardiac complications in thalassemia major patients in the worldwide was 25% (95% CI 22-28%) and 42% (95% CI 37-46%), respectively. The results of this study show that the prevalence of cardiac iron overload and cardiovascular complications in patients with thalassemia major is almost high. Therefore, iron chelation and careful monitoring of serum ferritin level will prevent the cardiac iron overload, and interval monitoring of patients with transfusion-dependent thalassemia (TDT) by echocardiography and electrocardiography will help with early detection of cardiovascular complications.

摘要

尽管重型地中海贫血的治疗管理已经取得了重大进展,但铁过载仍然是这些患者面临的一个具有挑战性的难题,心脏病仍然是这些患者发病率和死亡率的主要原因。因此,本研究旨在调查全球依赖输血的地中海贫血患者中心脏铁过载和心血管并发症的患病率。检索了以下数据库:ISI/Web of Science、Embase、PubMed、Scopus,截至 2018 年 2 月 30 日。使用 Joanna Briggs 研究所流行Critical Appraisal 工具评估研究质量。使用基于 Metaprop 的随机模型。共纳入 142 项研究。纳入患者的总数为 26893 例。患者的平均年龄为 22.6(SD=1.7)岁。根据 Metaprop,全球重型地中海贫血患者中心脏铁过载/心肌 siderosis(T2*<20ms)和心脏并发症的总体患病率分别为 25%(95%CI 22-28%)和 42%(95%CI 37-46%)。本研究结果表明,重型地中海贫血患者中心脏铁过载和心血管并发症的患病率相当高。因此,铁螯合和仔细监测血清铁蛋白水平将预防心脏铁过载,通过超声心动图和心电图对依赖输血的地中海贫血(TDT)患者进行间隔监测将有助于早期发现心血管并发症。

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