• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

去铁酮或去铁胺对铁过载的重型地中海贫血患者右心室功能的影响。

Effect of deferiprone or deferoxamine on right ventricular function in thalassemia major patients with myocardial iron overload.

机构信息

CMR Unit, Royal Brompton & Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, UK.

出版信息

J Cardiovasc Magn Reson. 2011 Jul 6;13(1):34. doi: 10.1186/1532-429X-13-34.

DOI:10.1186/1532-429X-13-34
PMID:21733147
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3148987/
Abstract

BACKGROUND

Thalassaemia major (TM) patients need regular blood transfusions that lead to accumulation of iron and death from heart failure. Deferiprone has been reported to be superior to deferoxamine for the removal of cardiac iron and improvement in left ventricular (LV) function but little is known of their relative effects on the right ventricle (RV), which is being increasingly recognised as an important prognostic factor in cardiomyopathy. Therefore data from a prospective randomised controlled trial (RCT) comparing these chelators was retrospectively analysed to assess the RV responses to these drugs.

METHODS

In the RCT, 61 TM patients were randomised to receive either deferiprone or deferoxamine monotherapy, and CMR scans for T2* and cardiac function were obtained. Data were re-analysed for RV volumes and function at baseline, and after 6 and 12 months of treatment.

RESULTS

From baseline to 12 months, deferiprone reduced RV end systolic volume (ESV) from 37.7 to 34.2 mL (p=0.008), whilst RV ejection fraction (EF) increased from 69.6 to 72.2% (p=0.001). This was associated with a 27% increase in T2* (p<0.001) and 3.1% increase in LVEF (p<0.001). By contrast, deferoxamine showed no change in RVESV (38.1 to 39.1 mL, p=0.38), or RVEF (70.0 to 69.9%, p=0.93) whereas the T2* increased by 13% (p<0.001), but with no change in LVEF (0.32%; p=0.66). Analysis of between drugs treatment effects, showed significant improvements favouring deferiprone with a mean effect on RVESV of -1.82 mL (p=0.014) and 1.16% for RVEF (p=0.009). Using regression analysis the improvement in RVEF at 12 months was shown to be greater in patients with lower baseline EF values (p<0.001), with a significant difference in RVEF of 3.5% favouring deferiprone over deferoxamine (p=0.012).

CONCLUSION

In this retrospective analysis of a prospective RCT, deferiprone monotherapy was superior to deferoxamine for improvement in RVEF and end-systolic volume. This improvement in the RV volumes and function may contribute to the improved cardiac outcomes seen with deferiprone.

摘要

背景

重型地中海贫血(TM)患者需要定期输血,这会导致铁的积累和心力衰竭死亡。据报道,去铁酮在去除心脏铁和改善左心室(LV)功能方面优于去铁胺,但对于右心室(RV)的相对影响知之甚少,而 RV 作为心肌病的一个重要预后因素,其重要性正日益受到关注。因此,回顾性分析了一项比较这两种螯合剂的前瞻性随机对照试验(RCT)的数据,以评估这些药物对 RV 的影响。

方法

在 RCT 中,61 名 TM 患者被随机分配接受去铁酮或去铁胺单药治疗,并获得 T2*和心脏功能的心脏磁共振(CMR)扫描。在基线、治疗 6 个月和 12 个月时,对 RV 容积和功能进行重新分析。

结果

从基线到 12 个月,去铁酮使 RV 收缩末期容积(ESV)从 37.7 毫升减少到 34.2 毫升(p=0.008),而 RV 射血分数(EF)从 69.6%增加到 72.2%(p=0.001)。这与 T2增加 27%(p<0.001)和 LVEF 增加 3.1%(p<0.001)有关。相比之下,去铁胺对 RVESV(38.1 至 39.1 毫升,p=0.38)或 RVEF(70.0 至 69.9%,p=0.93)均无变化,而 T2增加 13%(p<0.001),但 LVEF 无变化(0.32%;p=0.66)。药物治疗效果的分析表明,去铁酮具有明显的优势,RVESV 平均降低 1.82 毫升(p=0.014),RVEF 增加 1.16%(p=0.009)。回归分析显示,在基线 EF 值较低的患者中,RVEF 的改善更大(p<0.001),去铁酮比去铁胺在 RVEF 方面的优势为 3.5%(p=0.012)。

结论

在这项前瞻性 RCT 的回顾性分析中,去铁酮单药治疗在改善 RVEF 和收缩末期容积方面优于去铁胺。RV 容积和功能的这种改善可能有助于改善去铁酮治疗的心脏结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f663/3148987/d9df8e923f6d/1532-429X-13-34-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f663/3148987/ca4d34e591c9/1532-429X-13-34-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f663/3148987/d9df8e923f6d/1532-429X-13-34-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f663/3148987/ca4d34e591c9/1532-429X-13-34-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f663/3148987/d9df8e923f6d/1532-429X-13-34-2.jpg

相似文献

1
Effect of deferiprone or deferoxamine on right ventricular function in thalassemia major patients with myocardial iron overload.去铁酮或去铁胺对铁过载的重型地中海贫血患者右心室功能的影响。
J Cardiovasc Magn Reson. 2011 Jul 6;13(1):34. doi: 10.1186/1532-429X-13-34.
2
Effects of combined deferiprone with deferoxamine on right ventricular function in thalassaemia major.联合去铁酮和去铁胺对重型地中海贫血患者右心室功能的影响。
J Cardiovasc Magn Reson. 2012 Jan 25;14(1):8. doi: 10.1186/1532-429X-14-8.
3
Cardiac and hepatic iron and ejection fraction in thalassemia major: multicentre prospective comparison of combined deferiprone and deferoxamine therapy against deferiprone or deferoxamine monotherapy.地中海贫血症患者的心脏和肝脏铁含量与射血分数:联合使用去铁酮和去铁胺与单独使用去铁酮或去铁胺的多中心前瞻性比较。
J Cardiovasc Magn Reson. 2013 Jan 16;15(1):1. doi: 10.1186/1532-429X-15-1.
4
Combined chelation therapy in thalassemia major for the treatment of severe myocardial siderosis with left ventricular dysfunction.重型地中海贫血联合螯合疗法治疗重度心肌铁沉着症伴左心室功能障碍。
J Cardiovasc Magn Reson. 2008 Feb 25;10(1):12. doi: 10.1186/1532-429X-10-12.
5
Desferrioxamine mesylate for managing transfusional iron overload in people with transfusion-dependent thalassaemia.甲磺酸去铁胺用于治疗依赖输血的地中海贫血患者的输血性铁过载。
Cochrane Database Syst Rev. 2013 Aug 21;2013(8):CD004450. doi: 10.1002/14651858.CD004450.pub3.
6
Treatment of heart failure in adults with thalassemia major: response in patients randomised to deferoxamine with or without deferiprone.重型地中海贫血成年患者的心力衰竭治疗:随机接受去铁胺治疗(加或不加去铁酮)的患者的反应
J Cardiovasc Magn Reson. 2013 May 20;15(1):38. doi: 10.1186/1532-429X-15-38.
7
A randomized, placebo-controlled, double-blind trial of the effect of combined therapy with deferoxamine and deferiprone on myocardial iron in thalassemia major using cardiovascular magnetic resonance.一项使用心血管磁共振评估去铁胺与去铁酮联合治疗对重型地中海贫血患者心肌铁影响的随机、安慰剂对照、双盲试验。
Circulation. 2007 Apr 10;115(14):1876-84. doi: 10.1161/CIRCULATIONAHA.106.648790. Epub 2007 Mar 19.
8
Oral deferiprone for iron chelation in people with thalassaemia.口服去铁酮用于地中海贫血患者的铁螯合治疗。
Cochrane Database Syst Rev. 2013 Aug 21;2013(8):CD004839. doi: 10.1002/14651858.CD004839.pub3.
9
Long-term treatment with deferiprone enhances left ventricular ejection function when compared to deferoxamine in patients with thalassemia major.与去铁胺相比,长期使用地拉罗司可增强重型地中海贫血患者的左心室射血功能。
Blood Cells Mol Dis. 2013 Aug;51(2):85-8. doi: 10.1016/j.bcmd.2013.04.002. Epub 2013 Apr 28.
10
MRI multicentre prospective survey in thalassaemia major patients treated with deferasirox versus deferiprone and desferrioxamine.MRI 多中心前瞻性研究:地拉罗司治疗重型地中海贫血患者与去铁酮和去铁胺的对比。
Br J Haematol. 2018 Dec;183(5):783-795. doi: 10.1111/bjh.15595. Epub 2018 Oct 18.

引用本文的文献

1
ATH434, a promising iron-targeting compound for treating iron regulation disorders.ATH434,一种有前途的铁靶向化合物,可用于治疗铁调节紊乱。
Metallomics. 2024 Oct 4;16(10). doi: 10.1093/mtomcs/mfae044.
2
Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia.改善镰状细胞病或地中海贫血患者铁螯合治疗依从性的干预措施。
Cochrane Database Syst Rev. 2023 Mar 6;3(3):CD012349. doi: 10.1002/14651858.CD012349.pub3.
3
HIF-1α Regulates Bone Homeostasis and Angiogenesis, Participating in the Occurrence of Bone Metabolic Diseases.

本文引用的文献

1
Extent of RV dysfunction and myocardial infarction assessed by CMR are independent outcome predictors early after STEMI treated with primary angioplasty.经心脏磁共振评估的 RV 功能障碍和心肌梗死范围是 STEMI 患者行直接经皮冠状动脉介入治疗后早期的独立预后预测因素。
JACC Cardiovasc Imaging. 2010 Dec;3(12):1237-46. doi: 10.1016/j.jcmg.2010.09.018.
2
Continued improvement in myocardial T2* over two years of deferasirox therapy in β-thalassemia major patients with cardiac iron overload.在β-地中海贫血伴心脏铁过载患者中,地拉罗司治疗两年后心肌 T2*持续改善。
Haematologica. 2011 Jan;96(1):48-54. doi: 10.3324/haematol.2010.031468. Epub 2010 Nov 11.
3
HIF-1α 调节骨稳态和血管生成,参与骨代谢疾病的发生。
Cells. 2022 Nov 10;11(22):3552. doi: 10.3390/cells11223552.
4
Pathogenesis, Diagnosis, and Clinical Implications of Hereditary Hemochromatosis-The Cardiological Point of View.遗传性血色素沉着症的发病机制、诊断及临床意义——心脏病学视角
Diagnostics (Basel). 2021 Jul 16;11(7):1279. doi: 10.3390/diagnostics11071279.
5
Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia.改善镰状细胞病或地中海贫血患者铁螯合疗法依从性的干预措施。
Cochrane Database Syst Rev. 2018 May 8;5(5):CD012349. doi: 10.1002/14651858.CD012349.pub2.
6
Cardiac complications in beta-thalassemia: From mice to men.β地中海贫血的心脏并发症:从小鼠到人类
Exp Biol Med (Maywood). 2017 Jun;242(11):1126-1135. doi: 10.1177/1535370217708977. Epub 2017 May 9.
7
Comparative efficacy and safety of deferoxamine, deferiprone and deferasirox on severe thalassemia: a meta-analysis of 16 randomized controlled trials.去铁胺、去铁酮和地拉罗司治疗重型地中海贫血的疗效和安全性比较:16项随机对照试验的荟萃分析
PLoS One. 2013 Dec 23;8(12):e82662. doi: 10.1371/journal.pone.0082662. eCollection 2013.
8
Review of Journal of Cardiovascular Magnetic Resonance 2012.《心血管磁共振杂志》2012 年回顾。
J Cardiovasc Magn Reson. 2013 Sep 4;15(1):76. doi: 10.1186/1532-429X-15-76.
9
Oral deferiprone for iron chelation in people with thalassaemia.口服去铁酮用于地中海贫血患者的铁螯合治疗。
Cochrane Database Syst Rev. 2013 Aug 21;2013(8):CD004839. doi: 10.1002/14651858.CD004839.pub3.
10
Desferrioxamine mesylate for managing transfusional iron overload in people with transfusion-dependent thalassaemia.甲磺酸去铁胺用于治疗依赖输血的地中海贫血患者的输血性铁过载。
Cochrane Database Syst Rev. 2013 Aug 21;2013(8):CD004450. doi: 10.1002/14651858.CD004450.pub3.
Right ventricular volumes and function in thalassemia major patients in the absence of myocardial iron overload.
无心肌铁过载的重型地中海贫血患者的右心室容量和功能。
J Cardiovasc Magn Reson. 2010 Apr 23;12(1):24. doi: 10.1186/1532-429X-12-24.
4
Relation of myocardial T2* to right ventricular function in thalassaemia major.铁过载对重型β地中海贫血患者右心室功能的影响
Eur Heart J. 2010 Jul;31(13):1648-54. doi: 10.1093/eurheartj/ehq106. Epub 2010 Apr 22.
5
Effects of right ventricular ejection fraction on outcomes in chronic systolic heart failure.右心室射血分数对慢性收缩性心力衰竭结局的影响。
Circulation. 2010 Jan 19;121(2):252-8. doi: 10.1161/CIRCULATIONAHA.109.887570. Epub 2010 Jan 4.
6
Efficacy of deferasirox in reducing and preventing cardiac iron overload in beta-thalassemia.地拉罗司降低和预防β-地中海贫血心脏铁过载的疗效。
Blood. 2010 Mar 25;115(12):2364-71. doi: 10.1182/blood-2009-04-217455. Epub 2009 Dec 8.
7
Risk/benefit assessment, advantages over other drugs and targeting methods in the use of deferiprone as a pharmaceutical antioxidant in iron loading and non iron loading conditions.去铁酮在铁过载和非铁过载情况下作为药物抗氧化剂使用时的风险/效益评估、与其他药物及靶向方法相比的优势。
Hemoglobin. 2009;33(5):386-97. doi: 10.3109/03630260903217141.
8
Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major.心脏T2*磁共振成像预测重型地中海贫血患者心脏并发症的研究
Circulation. 2009 Nov 17;120(20):1961-8. doi: 10.1161/CIRCULATIONAHA.109.874487. Epub 2009 Oct 2.
9
Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance.英国重型地中海贫血患者生存率的提高及其与T2*心血管磁共振成像的关系
J Cardiovasc Magn Reson. 2008 Sep 25;10(1):42. doi: 10.1186/1532-429X-10-42.
10
Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation.药物介导的铁重分布可恢复因铁调素缺乏而受损的细胞功能。
Blood. 2008 Dec 15;112(13):5219-27. doi: 10.1182/blood-2008-06-161919. Epub 2008 Sep 16.