去铁素和去铁素联合螯合治疗重度乙型地中海贫血儿童的疗效:来自发展中国家一个单位的审计。

IF 3.5 4区 医学 Q2 MEDICINE, RESEARCH & EXPERIMENTAL
Vinson James, Anand Prakash
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引用次数: 0

摘要

有效的铁螯合对预防输血依赖性-地中海贫血的发病率和死亡率至关重要。虽然口服螯合是首选的给药方式,但严重铁超载的患者通常需要联合治疗。虽然去铁胺和去铁酮通常被推荐,但两种口服螯合剂-去铁胺和去铁酮的组合,提供了一个更方便的选择。本研究评估联合口服螯合治疗严重铁超载患儿的疗效和安全性。输注依赖型重度β -地中海贫血患儿,尽管使用最大剂量去铁素(40 mg/kg/天),但血清铁蛋白水平持续高(> 2500µg/dL)超过6个月,开始联合去铁素螯合治疗。每隔6个月监测血清铁蛋白水平以评估治疗效果。在130例定期随访的患者中,27例符合联合螯合标准。血清铁蛋白水平显著降低,从基线时的4277±1885µg/dL降至6个月时的3242±1110µg/dL (p = 0.003), 12个月时进一步降至2985±1116µg/dL (p = 0.018)。在研究期间没有发现明显的不良反应。去铁铁素和去铁素的联合螯合是治疗重度地中海贫血儿童严重铁超载的有效且耐受性良好的策略。这种方法为注射治疗提供了一种实用的替代方法,并可能改善依从性和治疗结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Efficacy of combination chelation with deferasirox and deferiprone in children with beta-thalassemia major: an audit from a unit in the developing world.

Efficacy of combination chelation with deferasirox and deferiprone in children with beta-thalassemia major: an audit from a unit in the developing world.

Efficacy of combination chelation with deferasirox and deferiprone in children with beta-thalassemia major: an audit from a unit in the developing world.

Effective iron chelation is crucial for preventing morbidity and mortality in transfusion-dependent beta-thalassemia major. While oral chelation is the preferred mode of administration, heavily iron-overloaded patients often require combination therapy. Although desferoxamine and deferiprone are commonly recommended, a combination of two oral chelators-deferasirox and deferiprone, offers a more convenient alternative. This study evaluates the efficacy and safety of combination oral chelation in pediatric patients with severe iron overload. Children with transfusion-dependent beta-thalassemia major and persistently high serum ferritin levels (> 2500 µg/dL) for more than six months despite maximum-dose deferasirox (40 mg/kg/day) were initiated on combination chelation with deferiprone. Serum ferritin levels were monitored at six-month intervals to assess treatment efficacy. Among 130 regularly followed patients, 27 met the criteria for combination chelation. A significant reduction in serum ferritin levels was observed, decreasing from 4277 ± 1885 µg/dL at baseline to 3242 ± 1110 µg/dL at six months (p = 0.003) and further to 2985 ± 1116 µg/dL at twelve months (p = 0.018). No significant adverse effects were noted during the study period. Combination chelation with deferasirox and deferiprone is an effective and well-tolerated strategy for managing severe iron overload in children with beta-thalassemia major. This approach provides a practical alternative to injectable therapies and may improve adherence and treatment outcomes.

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来源期刊
Clinical and Experimental Medicine
Clinical and Experimental Medicine 医学-医学:研究与实验
CiteScore
4.80
自引率
2.20%
发文量
159
审稿时长
2.5 months
期刊介绍: Clinical and Experimental Medicine (CEM) is a multidisciplinary journal that aims to be a forum of scientific excellence and information exchange in relation to the basic and clinical features of the following fields: hematology, onco-hematology, oncology, virology, immunology, and rheumatology. The journal publishes reviews and editorials, experimental and preclinical studies, translational research, prospectively designed clinical trials, and epidemiological studies. Papers containing new clinical or experimental data that are likely to contribute to changes in clinical practice or the way in which a disease is thought about will be given priority due to their immediate importance. Case reports will be accepted on an exceptional basis only, and their submission is discouraged. The major criteria for publication are clarity, scientific soundness, and advances in knowledge. In compliance with the overwhelmingly prevailing request by the international scientific community, and with respect for eco-compatibility issues, CEM is now published exclusively online.
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