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伊拉克巴士拉非输血依赖型地中海贫血青少年的健康相关生活质量

Health-Related Quality of Life of Adolescents With Non-transfusion-Dependent Thalassemia in Basrah, Iraq.

作者信息

Hameed Zahraa M, Hassan Meaad K, Ahmed Bahaa A

机构信息

Department of Pediatrics, Basrah Teaching Hospital, Basrah, IRQ.

Department of Pediatrics, College of Medicine, University of Basrah, Basrah, IRQ.

出版信息

Cureus. 2024 Sep 25;16(9):e70189. doi: 10.7759/cureus.70189. eCollection 2024 Sep.

Abstract

BACKGROUND

Thalassemia is a chronic inherited disease with the potential for serious clinical and psychological effects. In the case of thalassemia, a cure is not currently accessible, and lifelong treatment is required. Health-related quality of life (HRQoL) is considered a crucial health outcome.

OBJECTIVES

This study aims to assess the HRQoL of children and adolescents with non-transfusion-dependent thalassemia (NTDT) and compare it with that of beta-thalassemia major (β-TM) and healthy subjects.

PATIENTS AND METHODS

This case-control study included 88 patients with NTDT and 153 age- and gender-matched healthy children and adolescents. In addition, we included 70 registered patients with β-TM. We used the short-form health survey questionnaire to assess HRQoL.

RESULTS

Of the 88 patients, 41 were diagnosed with alpha-thalassemia intermedia (α-TI; hemoglobin H disease), and 47 were with beta-thalassemia intermedia (β-TI). HRQoL domains were significantly higher in healthy children and adolescents compared to NTDT patients (P<0.001); the role emotion domain was the most affected in NTDT patients (51.92 ± 3.37), followed by general health (52.72 ± 3.05) and role physical (53.59 ± 3.13). α-TI patients had significantly higher HRQoL domains than patients with β-TI. The study also indicated that NTDT patients had significantly better QoL scores compared to β-TM patients (P<0.001) across all domains.

CONCLUSIONS

NTDT patients have a lower HRQoL compared to healthy controls. However, their HRQoL scores are significantly better than those of patients with β-TM. Among NTDT patients, those with α-TI have significantly better HRQoL scores compared to patients with β-TI.

摘要

背景

地中海贫血是一种慢性遗传性疾病,可能产生严重的临床和心理影响。在地中海贫血病例中,目前尚无治愈方法,需要终身治疗。健康相关生活质量(HRQoL)被视为一项关键的健康指标。

目的

本研究旨在评估非输血依赖型地中海贫血(NTDT)儿童和青少年的健康相关生活质量,并将其与重型β地中海贫血(β-TM)患者及健康受试者进行比较。

患者与方法

本病例对照研究纳入了88例NTDT患者以及153名年龄和性别匹配的健康儿童和青少年。此外,还纳入了70例已登记的β-TM患者。我们使用简短健康调查问卷来评估健康相关生活质量。

结果

在88例患者中,41例被诊断为中间型α地中海贫血(α-TI;血红蛋白H病),47例为中间型β地中海贫血(β-TI)。与NTDT患者相比,健康儿童和青少年的健康相关生活质量各领域得分显著更高(P<0.001);在NTDT患者中,角色情感领域受影响最大(51.92±3.37),其次是总体健康(52.72±3.05)和角色身体(53.59±3.13)。α-TI患者的健康相关生活质量各领域得分显著高于β-TI患者。该研究还表明,在所有领域中,NTDT患者的生活质量得分显著优于β-TM患者(P<0.001)。

结论

与健康对照组相比,NTDT患者的健康相关生活质量较低。然而,他们的健康相关生活质量得分显著优于β-TM患者。在NTDT患者中,α-TI患者的健康相关生活质量得分显著高于β-TI患者。

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Thalassaemia in children: from quality of care to quality of life.儿童地中海贫血:从医疗质量到生活质量
Arch Dis Child. 2015 Nov;100(11):1051-7. doi: 10.1136/archdischild-2014-308112. Epub 2015 Aug 19.

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