输血依赖型β-地中海贫血患者的体能受损:有规律的体育锻炼可以作为对策吗?

IF 5.1 2区 医学 Q1 HEMATOLOGY British Journal of Haematology Pub Date : 2024-11-19 DOI:10.1111/bjh.19847
Mathilde Noguer, Phanélie Berthon, Caroline Makowski, Laurent A Messonnier
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引用次数: 0

摘要

输血依赖型β-地中海贫血症(TDβT)是一种遗传性疾病,其特征是β-球蛋白链合成减少或缺失,从而导致慢性贫血。治疗包括定期输血和螯合疗法,以限制铁超载及其对器官(如心脏、肺和肝脏)的负面影响。尽管输血和螯合疗法能延长患者的寿命,但 TDβT 患者的生活质量较差,运动能力受损(主要是由于肌肉衰竭和心脏肌力下降)。本综述的目的是:(i) 讨论器官功能障碍在 TDβT 患者运动能力受损和生活质量下降中的作用;(ii) 讨论将体育锻炼作为一种潜在的治疗策略,以减轻该疾病的多因素影响。
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Impaired physical ability in patients with transfusion-dependent β-thalassaemia: Can regular physical activity be a countermeasure?

Transfusion-dependent β-thalassaemia (TDβT) is a genetic disorder characterised by reduced or absent β-globin chain synthesis, resulting in chronic anaemia. Treatment consists of regular blood transfusions and chelation therapy to limit iron overload and its negative effects on organs (e.g. heart, lungs and liver). Despite improved life expectancy with transfusions and chelation therapy, TDβT patients experience poor quality of life and impaired exercise capacity (mainly due to muscle deconditioning and reduced cardiac inotropism). The aims of this review are (i) to discuss the role of organ dysfunction in the impairment of exercise capacity and reduced quality of life of TDβT patients; and (ii) to discuss physical activity as a potential therapeutic strategy to alleviate the multifactorial impact of the disease.

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来源期刊
CiteScore
8.60
自引率
4.60%
发文量
565
审稿时长
1 months
期刊介绍: The British Journal of Haematology publishes original research papers in clinical, laboratory and experimental haematology. The Journal also features annotations, reviews, short reports, images in haematology and Letters to the Editor.
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