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去铁酮薄膜包衣片在输血依赖型地中海贫血患儿中的应用:单中心经验

Use of Deferasirox Film-Coated Tablets in Pediatric Patients with Transfusion Dependent Thalassemia: A Single Center Experience.

作者信息

Adramerina Alkistis, Printza Nikoleta, Hatzipantelis Emmanouel, Symeonidis Symeon, Tarazi Labib, Teli Aikaterini, Economou Marina

机构信息

1st Pediatric Department, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece.

2nd Pediatric Department, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, 54621 Thessaloniki, Greece.

出版信息

Biology (Basel). 2022 Feb 5;11(2):247. doi: 10.3390/biology11020247.

Abstract

Thalassemic syndromes are characterized by clinical heterogenicity. For severe disease forms, lifelong blood transfusions remain the mainstay of therapy, while iron overload monitoring and adequate chelation treatment are required in order to ensure effective disease management. Compared to previous chelators, the new deferasirox film-coated tablet (DFX FCT) is considered to offer a more convenient and well-tolerated treatment scheme, aiming at better treatment-related and patient-related outcomes. The present study's objective was to prospectively evaluate the safety and efficacy of DFX FCT in children and adolescents with transfusion-dependent thalassemia. Data collected included patient demographics, hematology and biochemistry laboratory work up, magnetic resonance imaging of heart and liver for iron load, as well as ophthalmological and audiological examination prior to and a year following drug administration. Study results confirmed DFX FCT safety in older children in a manner similar to adults, but demonstrated increased frequency of adverse events in younger patients, mainly, involving liver function. With regards to efficacy, study results confirmed the preventive role of DFX FCT in iron loading of liver and heart, however, higher doses than generally recommended were required in order to ensure adequate chelation.

摘要

地中海贫血综合征具有临床异质性。对于严重的疾病形式,终身输血仍然是主要的治疗方法,同时需要进行铁过载监测和充分的螯合治疗,以确保有效的疾病管理。与以前的螯合剂相比,新型去铁斯若薄膜包衣片(DFX FCT)被认为提供了一种更方便且耐受性良好的治疗方案,旨在实现更好的与治疗相关和与患者相关的结果。本研究的目的是前瞻性评估DFX FCT在依赖输血的地中海贫血儿童和青少年中的安全性和有效性。收集的数据包括患者人口统计学信息、血液学和生物化学实验室检查、心脏和肝脏的铁负荷磁共振成像,以及给药前和给药后一年的眼科和听力检查。研究结果证实,DFX FCT在大龄儿童中的安全性与成人相似,但在低龄患者中不良事件的发生率增加,主要涉及肝功能。关于疗效,研究结果证实了DFX FCT在预防肝脏和心脏铁负荷方面的作用,然而,为了确保充分螯合,需要比一般推荐剂量更高的剂量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3bfc/8869542/d62108af9fa1/biology-11-00247-g001.jpg

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