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血红蛋白H病合并铁过载患者中与氧化应激相关的代谢途径。

Metabolic pathways related to oxidative stress in patients with hemoglobin h disease and iron overload.

作者信息

Chiou Shyh-Shin, Tsao Chao-Jung, Tsai Shih-Meng, Wu Ye-Ru, Liao Yu-Mei, Lin Pei-Chin, Tsai Li-Yu

机构信息

Department of Pediatrics, Faculty of Medicine, School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Division of Hematology-Oncology, Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.

出版信息

J Clin Lab Anal. 2014 Jul;28(4):261-8. doi: 10.1002/jcla.21676. Epub 2014 Feb 27.

DOI:10.1002/jcla.21676
PMID:24577940
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6807426/
Abstract

BACKGROUND

Iron overload is a major complication in patients with hemoglobin H (Hb H) disease and causes damage of tissues.

METHODS

We investigated 26 Hb H patients and 75 controls to evaluate their oxidative stress and antioxidant statuses.

RESULTS

There were significantly increased levels of superoxide anion in leucocytes, nitrite (NO2-), and malondialdehyde (MDA) in plasma, and activities of superoxide dismutase (SOD), glutathione peroxidase (GPx), glutathione reductase (GRx) and oxidized glutathione (GSSG) in erythrocytes, decreased levels of nitrate (NO3-) and vitamin C in plasma, and reduced glutathione (GSH) in erythrocytes, in addition to the abnormal iron status in the patients when compared with those in the controls. Meanwhile, levels of serum ferritin were positively correlated with serum iron, plasma MDA, and erythrocyte SOD in the patients. In addition, the activities of SOD were positively correlated with those of GPx and GRx, and the levels of GSSG and MDA, but negatively correlated with those of GSH. Furthermore, the levels of MDA were negatively correlated those of vitamin C.

CONCLUSIONS

These results demonstrate the presence of oxidative stress and decreased levels of antioxidants; moreover, the related metabolic antioxidant pathway is active in Hb H patients with iron overload.

摘要

背景

铁过载是血红蛋白H（Hb H）病患者的主要并发症，会导致组织损伤。

方法

我们调查了26例Hb H患者和75名对照者，以评估他们的氧化应激和抗氧化状态。

结果

与对照组相比，患者白细胞中超氧阴离子水平、血浆中亚硝酸盐（NO2-）和丙二醛（MDA）水平显著升高，红细胞中超氧化物歧化酶（SOD）、谷胱甘肽过氧化物酶（GPx）、谷胱甘肽还原酶（GRx）和氧化型谷胱甘肽（GSSG）活性升高，血浆中硝酸盐（NO3-）和维生素C水平以及红细胞中还原型谷胱甘肽（GSH）水平降低，此外患者铁状态异常。同时，患者血清铁蛋白水平与血清铁、血浆MDA和红细胞SOD呈正相关。此外，SOD活性与GPx和GRx活性以及GSSG和MDA水平呈正相关，但与GSH水平呈负相关。此外，MDA水平与维生素C水平呈负相关。

结论

这些结果表明存在氧化应激且抗氧化剂水平降低；此外，相关的代谢抗氧化途径在铁过载的Hb H患者中活跃。

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