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评估口服螯合疗法药物地拉罗司对重型β地中海贫血患者的听觉影响。

Assessing the auditory effects of oral chelation therapy drug Deferasirox in individuals with β-thalassemia major.

作者信息

Tiwana Balwinder S, Aggarwal Ankita, Bhagat Sanjeev, Singh Harjinder, Sahni Dimple, Yadav Vishav

机构信息

Department of Otorhinolaryngology and Head & Neck Surgery Government Medical College Patiala Punjab India.

Department of Paediatrics Government Medical College Patiala Punjab India.

出版信息

World J Otorhinolaryngol Head Neck Surg. 2023 Dec 10;10(4):309-314. doi: 10.1002/wjo2.150. eCollection 2024 Dec.

DOI:10.1002/wjo2.150
PMID:39677049
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11634694/
Abstract

OBJECTIVE

Our study aimed to investigate the ototoxicity associated with the iron chelator deferasirox in patients with β-thalassemia major, who were receiving regular transfusion therapy, along with evaluating the data on audiological tests using appropriate statistical tests.

METHODS

A cross-sectional observational study was conducted on 100 transfusion-dependent β-thalassemia major patients on oral iron chelating agent-deferasirox. Pure tone audiometry (PTA) and distortion product otoacoustic emissions (DPOAE) was carried out in all patients to assess the auditory side effects of the drug. Data was collected, compiled, and analyzed statistically using appropriate statistical tests. The relationship between ototoxicity and various demographic parameters such as age, sex, hemoglobin (Hb) level, S. ferritin, duration, and dose of chelation therapy was also assessed.

RESULTS

Sixteen patients had abnormal DPOAE and the number of patients with pure tone average above 25 dB HL which was taken as hearing deficit on PTA was 13. No statistically significant relationship between hearing loss and age, gender, S. ferritin, duration of therapy, cumulative dose, Hb levels were found.

CONCLUSION

Despite being a lifesaving drug, the advantages of chelating agent-Deferasirox must be weighed against its probable ototoxic effects. We could not find a relationship of ototoxicity with variable parameters (age, gender, Hb level, Ferritin level, duration, and cumulative dose of drug), thus future research is encouraged to form a definitive basis.

摘要

目的

我们的研究旨在调查与去铁胺相关的耳毒性,研究对象为接受定期输血治疗的重型β地中海贫血患者,并使用适当的统计检验评估听力测试数据。

方法

对100名口服铁螯合剂去铁胺的依赖输血的重型β地中海贫血患者进行了一项横断面观察性研究。对所有患者进行纯音听力测定(PTA)和畸变产物耳声发射(DPOAE),以评估该药物的听觉副作用。收集、整理数据,并使用适当的统计检验进行统计学分析。还评估了耳毒性与各种人口统计学参数之间的关系,如年龄、性别、血红蛋白(Hb)水平、血清铁蛋白、螯合治疗的持续时间和剂量。

结果

16例患者DPOAE异常,PTA上纯音平均听阈高于25dB HL(被视为听力损失)的患者有13例。未发现听力损失与年龄、性别、血清铁蛋白、治疗持续时间、累积剂量、Hb水平之间存在统计学显著关系。

结论

尽管去铁胺是一种救命药物,但螯合剂去铁胺的优势必须与其可能的耳毒性作用相权衡。我们未发现耳毒性与可变参数(年龄、性别、Hb水平、铁蛋白水平、药物持续时间和累积剂量)之间的关系,因此鼓励未来的研究形成明确的依据。

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本文引用的文献

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Hearing Loss in Beta-Thalassemia: Systematic Review.β地中海贫血中的听力损失:系统评价
J Clin Med. 2021 Dec 25;11(1):102. doi: 10.3390/jcm11010102.
2
Sensorineural Hearing Loss and Its Relationship with Duration of Chelation Among Major β-Thalassemia Patients.重型β地中海贫血患者的感音神经性听力损失及其与螯合治疗持续时间的关系
Cureus. 2019 Aug 22;11(8):e5465. doi: 10.7759/cureus.5465.
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Bilateral auditory ossicular expansions in a child with beta-thalassemia major: Case report and literature review.一名重型β地中海贫血患儿的双侧听小骨膨大:病例报告及文献复习
Int J Pediatr Otorhinolaryngol. 2018 Sep;112:126-131. doi: 10.1016/j.ijporl.2018.06.046. Epub 2018 Jul 2.
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Clinical Features of β-Thalassemia and Sickle Cell Disease.β地中海贫血和镰状细胞病的临床特征
Adv Exp Med Biol. 2017;1013:1-26. doi: 10.1007/978-1-4939-7299-9_1.
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The Incidence of Ototoxicity in Patients Using Iron Chelators.
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Evaluation of a new tablet formulation of deferasirox to reduce chronic iron overload after long-term blood transfusions.评估一种去铁胺新片剂配方在长期输血后减轻慢性铁过载的效果。
Ther Clin Risk Manag. 2016 Feb 15;12:201-8. doi: 10.2147/TCRM.S82449. eCollection 2016.
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Sensorineural hearing loss in β-thalassemia patients treated with iron chelation.接受铁螯合治疗的β地中海贫血患者的感音神经性听力损失
Ear Nose Throat J. 2015 Dec;94(12):481-5.
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Iron overload in Beta thalassaemia major and intermedia patients.重型和中间型β地中海贫血患者的铁过载
Maedica (Bucur). 2013 Sep;8(4):328-32.
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Audiologic and vestibular assessment in patients with β-thalassemia major receiving long-term transfusion therapy.β-重型地中海贫血患者长期输血治疗的听力学和前庭评估。
Pediatr Blood Cancer. 2013 Dec;60(12):1963-6. doi: 10.1002/pbc.24699. Epub 2013 Aug 19.
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Microcytic hypochromic anemia patients with thalassemia: genotyping approach.地中海贫血的小细胞低色素性贫血患者:基因分型方法
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