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接受铁螯合治疗的泰国地中海贫血儿童的健康相关生活质量

Health-related quality of life in Thai thalassemic children treated with iron chelation.

作者信息

Torcharus Kitti, Pankaew Tidarat

机构信息

Department of Pediatrics, Phramongkutklao College of Medicine, Bangkok, Thailand.

出版信息

Southeast Asian J Trop Med Public Health. 2011 Jul;42(4):951-9.

Abstract

Thalassemia is a chronic hereditary disease in which patients with severe disease present with anemia during their first year of life. In Thailand, stem cell transplantation is not an option for most patients. Supportive treatments, such as blood transfusions and iron chelation are used. Little data exists regarding the Health Related Quality of Life (HRQoL) of these patients. We conducted a study of the four dimensions of quality of life: physical, emotional, social, and role (school) functioning, using the PedsQL 4.0 Generic Core Scale to measure the HRQoL among thalassemic patients at the Hematology Unit, Department of Pediatrics, Phramongkutklao Hospital, during December 1, 2006 - November 30, 2007 to evaluate the quality of life in thalassemic patients treated with three iron-chelating agents. Forty-nine thalassemic patients were enrolled and treated with iron-chelating agents. The mean (SD) age of the patients was 10.61 years (4.33). Fifteen thalassemic patients were treated with desferrioxamine, 18 with deferiprone and 16 with deferasirox. The quality of life (QOL) results show the mean (SD) total summary score was 74.35 (12.42). For the psychosocial health summary, the social and school functioning scores were 85.40 (16.67) and 62.14 (15.84), respectively. The QOL scores of the patients who received desferrioxamine, deferiprone and deferasirox were 75.29 (9.09), 73.91 (15.25) and 73.98 (12.32), respectively (p = 0.94). The QOL had no significant differences by age, gender, type of thalassemia or serum ferritin level. Multivariate regression analysis showed no significant differences in clinical severity, age of onset or pre-transfusion hematocrit levels.

摘要

地中海贫血是一种慢性遗传性疾病,重症患者在出生后第一年就会出现贫血症状。在泰国,大多数患者无法选择干细胞移植。采用输血和铁螯合等支持性治疗方法。关于这些患者的健康相关生活质量(HRQoL)的数据很少。我们使用儿童生活质量量表4.0通用核心量表,对2006年12月1日至2007年11月30日期间在佛统皇家医院儿科血液科接受治疗的地中海贫血患者的生活质量的四个维度进行了研究,这四个维度分别为身体、情感、社会和角色(学校)功能,以评估接受三种铁螯合剂治疗的地中海贫血患者的生活质量。49名地中海贫血患者入组并接受铁螯合剂治疗。患者的平均(标准差)年龄为10.61岁(4.33)。15名地中海贫血患者接受去铁胺治疗,18名接受地拉罗司治疗,16名接受地拉罗司治疗。生活质量(QOL)结果显示,平均(标准差)总总结分为74.35(12.42)。心理社会健康总结方面,社会和学校功能得分分别为85.40(16.67)和62.14(15.84)。接受去铁胺、地拉罗司和地拉罗司治疗的患者的QOL得分分别为75.29(9.09)、73.91(15.25)和73.98(12.32)(p = 0.94)。QOL在年龄、性别、地中海贫血类型或血清铁蛋白水平方面无显著差异。多因素回归分析显示,临床严重程度、发病年龄或输血前血细胞比容水平无显著差异。

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