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本文引用的文献

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Molecular Characterization and Disease-Related Morbidities of β-Thalassemia Patients from the Northeastern Part of Iraq.伊拉克东北部β地中海贫血患者的分子特征及疾病相关并发症
Int J Gen Med. 2020 Dec 9;13:1453-1467. doi: 10.2147/IJGM.S277947. eCollection 2020.
2
Quality of life in patients with β-thalassemia: A prospective study of transfusion-dependent and non-transfusion-dependent patients in Greece, Italy, Lebanon, and Thailand.β地中海贫血患者的生活质量:希腊、意大利、黎巴嫩和泰国依赖输血与非依赖输血患者的前瞻性研究
Am J Hematol. 2019 Oct;94(10):E261-E264. doi: 10.1002/ajh.25584. Epub 2019 Aug 13.
3
Factors affecting quality of life in children and adolescents with thalassemia in Iraqi Kurdistan.影响伊拉克库尔德斯坦地区地中海贫血患儿及青少年生活质量的因素。
Saudi Med J. 2018 Aug;39(8):799-807. doi: 10.15537/smj.2018.8.23315.
4
Health-related quality of life in pediatric and adolescent patients with transfusion-dependent ß-thalassemia in upper Egypt (single center study).埃及上埃及依赖输血的儿童和青少年β-地中海贫血患者的健康相关生活质量(单中心研究)。
Health Qual Life Outcomes. 2018 Apr 10;16(1):59. doi: 10.1186/s12955-018-0893-z.
5
Non-Transfusion-Dependent Thalassemia: An Update on Complications and Management.非输血依赖型地中海贫血:并发症与治疗的最新进展。
Int J Mol Sci. 2018 Jan 8;19(1):182. doi: 10.3390/ijms19010182.
6
Iron Overload and Chelation Therapy in Non-Transfusion Dependent Thalassemia.非输血依赖型地中海贫血中的铁过载与螯合疗法。
Int J Mol Sci. 2017 Dec 20;18(12):2778. doi: 10.3390/ijms18122778.
7
Health-Related Quality of Life and Health Utility Values in Beta Thalassemia Major Patients Receiving Different Types of Iron Chelators in Iran.伊朗接受不同类型铁螯合剂治疗的重型β地中海贫血患者的健康相关生活质量和健康效用值
Int J Hematol Oncol Stem Cell Res. 2016 Oct 1;10(4):224-231.
8
Prevalence and Risk Factors for Complications in Patients with Nontransfusion Dependent Alpha- and Beta-Thalassemia.非输血依赖型α和β地中海贫血患者并发症的患病率及危险因素
Anemia. 2015;2015:793025. doi: 10.1155/2015/793025. Epub 2015 Nov 18.
9
Non-transfusion-dependent thalassemia and thalassemia intermedia: epidemiology, complications, and management.非输血依赖型地中海贫血和中间型地中海贫血:流行病学、并发症及管理
Curr Med Res Opin. 2016;32(1):191-204. doi: 10.1185/03007995.2015.1110128. Epub 2015 Nov 25.
10
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.

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.

DOI:10.7759/cureus.70189
PMID:39463582
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11508038/
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。