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β-地中海贫血特征患儿的动态硫醇-二硫键稳态

Dynamic Thiol-Disulfide Homeostasis in Children With β-Thalassemia Trait.

作者信息

Kurucu Burçak, Fettah Ali, Çapkınoğlu Emre, Öner Nergiz, Eren Funda, Erel Özcan, Yeşil Şule, Şahin Gürses

机构信息

Department of Pediatric Hematology and Oncology, University of Healthy Sciences, Ankara Dr. Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, Ankara, Turkey.

Department of Biochemistry, Faculty of Medicine, Yıldırım Beyazıt University, Ankara City Hospital, Ankara, Turkey.

出版信息

Hemoglobin. 2022 May;46(3):164-167. doi: 10.1080/03630269.2022.2070073. Epub 2022 May 11.

Abstract

In children with β-thalassemia (β-thal) trait, tissue damage occurs with oxidative stress due to oxygen free radicals and reactive oxygen species (ROS) production. Dynamic thiol-disulfide homeostasis (DTDH) is one of the most important indicators showing the pro-oxidant/antioxidant status in the body. In this study, we aimed to examine the status of DTDH by measuring native thiol, disulfide, and total thiol levels in children with β-thal trait. The study included 40 children with β-thal trait and 30 healthy controls (matched by age and gender). The DTDH parameters were measured by an automated method and results were compared between the groups. The levels of native thiol, total thiol, and disulfide in children with β-thal trait group were statistically significantly higher than the control group ( < 0.001). There was no significant difference in disulfide/native thiol, disulfide/total thiol, and native thiol/total thiol levels between the groups. In addition, there was no correlation between hemoglobin (Hb) and serum ferritin levels with the markers of DTDH in children with β-thal trait. In our study, a significant increase was found in native thiol, total thiol, and disulfide levels in response to oxidative stress in children with β-thal trait compared to the healthy control group. Disulfide levels of the children with β-thal trait were higher than the control group, showing oxidative stress is high in β-thal trait. Accordingly, it increases the native thiol and total thiol capacity as compensation.

摘要

在患有β地中海贫血(β-地贫)特征的儿童中,由于氧自由基和活性氧(ROS)的产生,氧化应激会导致组织损伤。动态硫醇-二硫键稳态(DTDH)是显示体内促氧化剂/抗氧化剂状态的最重要指标之一。在本研究中,我们旨在通过测量患有β-地贫特征儿童的天然硫醇、二硫键和总硫醇水平来检查DTDH的状态。该研究纳入了40名患有β-地贫特征的儿童和30名健康对照者(按年龄和性别匹配)。通过自动化方法测量DTDH参数,并在两组之间比较结果。患有β-地贫特征儿童组的天然硫醇、总硫醇和二硫键水平在统计学上显著高于对照组(<0.001)。两组之间的二硫键/天然硫醇、二硫键/总硫醇和天然硫醇/总硫醇水平没有显著差异。此外,患有β-地贫特征儿童的血红蛋白(Hb)和血清铁蛋白水平与DTDH标志物之间没有相关性。在我们的研究中,发现与健康对照组相比,患有β-地贫特征的儿童在氧化应激反应中天然硫醇、总硫醇和二硫键水平显著增加。患有β-地贫特征儿童的二硫键水平高于对照组,表明β-地贫特征中的氧化应激较高。因此,作为补偿,它增加了天然硫醇和总硫醇的能力。

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