Oxidative stress and disturbance in antioxidant balance in beta thalassemia major

Beta thalassemia major is an inherited disease resulting from reduction or total lack of beta globin chains. Patients with this disease need repeated blood transfusion for survival. This may cause oxidative stress and tissue injury due to iron overload, altered antioxidant enzymes, and other essential trace element levels. The aim of this review is to scrutinize the relationship between oxidative stress and serum trace elements, degree of damage caused by oxidative stress, and the role of antioxidant enzymes in beta thalassemia major patients. The findings indicate that oxidative stress in patients with beta thalassemia major is mainly caused by tissue injury due to over production of free radical by secondary iron overload, alteration in serum trace elements and antioxidant enzymes level. The role of trace elements like selenium, copper, iron, and zinc in beta thalassemia major patients reveals a significant change of these trace elements. Studies published on the status of antioxidant enzymes like catalase, superoxide dismutase, glutathione, and glutathione S-transferase in beta thalassemia patients also showed variable results. The administration of selective antioxidants along with essential trace elements and minerals to reduce the extent of oxidative damage and related complications in beta thalassemia major still need further evaluation.

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Genetic Variation in SOD1Gene Promoter Ins/Del and Its Influence on Oxidative Stress in Beta Thalassemia Major Patients

International Journal of Hematology-Oncology and Stem Cell Research ◽

10.18502/ijhoscr.v14i2.2674 ◽

2020 ◽

Author(s):

Poonam Tripathi ◽

Sarita Agarwal ◽

Aditya Narayan Sarangi

Keyword(s):

Oxidative Stress ◽

Human Peripheral Blood ◽

In Silico Analysis ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Loss Of Function ◽

Sod Activity ◽

Beta Thalassemia Major ◽

The Status ◽

Polymerase Chain

Background: A genetic polymorphism of 50 bp insertion/deletion (Ins/Del) (rs 36232792) in the promoter region of the SOD1 was reported to influence the enzyme activity. The aims of the present study are to evaluate the status of this polymorphism on the SOD1 levels in human peripheral blood cells and its association with risk of oxidative stress in beta thalassemia major patients. Material and Methods: The study was carried out on 200 thalassemia major patients and 200 healthy controls healthy. The SOD1 genotypes were determined using a polymerase chain reaction (PCR)-based method. Serum SOD activity were assessed using SOD assay kit. In-silico analysis was assessed using loss-of-function (LofTool) (PMID: 27563026). Results: No association was found between the insertion/deletion (Ins/Del) polymorphism of SOD1 and risk of oxidative stress in thalassemia major patients Conclusion: The results of this study indicated that the oxidative stress is not affected by the Ins/ Del polymorphism of SOD1 in thalassemia major patients. Further research with larger sample size and with other genes of antioxidant system is required.

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Beta-Thalassemia Major and Female Fertility: The Role of Iron and Iron-Induced Oxidative Stress

Anemia ◽

10.1155/2013/617204 ◽

2013 ◽

Vol 2013 ◽

pp. 1-9 ◽

Cited By ~ 25

Author(s):

Paraskevi Roussou ◽

Nikolaos J. Tsagarakis ◽

Dimitrios Kountouras ◽

Sarantis Livadas ◽

Evanthia Diamanti-Kandarakis

Keyword(s):

Oxidative Stress ◽

Trace Elements ◽

Tissue Injury ◽

Hypogonadotropic Hypogonadism ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Beta Thalassemia Major ◽

Number Of Patients ◽

Endocrine Organs ◽

Reproductive Techniques

Endocrine complications due to haemosiderosis are present in a significant number of patients with beta-thalassemia major (BTM) worldwide and often become barriers in their desire for parenthood. Thus, although spontaneous fertility can occur, the majority of females with BTM is infertile due to hypogonadotropic hypogonadism (HH) and need assisted reproductive techniques. Infertility in these women seems to be attributed to iron deposition and iron-induced oxidative stress (OS) in various endocrine organs, such as hypothalamus, pituitary, and female reproductive system, but also through the iron effect on other organs, such as liver and pancreas, contributing to the impaired metabolism of hormones and serum antioxidants. Nevertheless, the gonadal function of these patients is usually intact and fertility is usually retrievable. Meanwhile, a significant prooxidants/antioxidants imbalance with subsequent increased (OS) exists in patients with BTM, which is mainly caused by tissue injury due to overproduction of free radicals by secondary iron overload, but also due to alteration in serum trace elements and antioxidant enzymes. Not only using the appropriate antioxidants, essential trace elements, and minerals, but also regulating the advanced glycation end products, could probably reduce the extent of oxidative damage and related complications and retrieve BTM women’s infertility.

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Nigella sativa improves anemia, enhances immunity and relieves iron overload-induced oxidative stress as a novel promising treatment in children having beta-thalassemia major

Journal of Herbal Medicine ◽

10.1016/j.hermed.2018.11.001 ◽

2019 ◽

Vol 16 ◽

pp. 100245 ◽

Cited By ~ 3

Author(s):

Mohamed El-Shanshory ◽

Nahed Mohammed Hablas ◽

Moutasem Salih Aboonq ◽

Ahmed R. Fakhreldin ◽

Mohammed Attia ◽

...

Keyword(s):

Oxidative Stress ◽

Iron Overload ◽

Nigella Sativa ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Beta Thalassemia Major ◽

Promising Treatment

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ATTENUATION OF IRON OVERLOAD AND EFFECT OF ANTIOXIDANTS SUPPLEMENTATION ON OXIDATIVE STRESS IN HOMOZYGOUS β-THALASSEMIA

International Journal of Clinical and Biomedical Research ◽

10.31878/ijcbr.2020.62.06 ◽

2020 ◽

pp. 21-24

Author(s):

Ghone Rahul A ◽

Bhagat Sonali S ◽

Karnik AC

Keyword(s):

Oxidative Stress ◽

Superoxide Dismutase ◽

Serum Iron ◽

Thalassemia Major ◽

Normal Serum ◽

Beta Thalassemia ◽

Regular Treatment ◽

Beta Thalassemia Major ◽

Erythrocyte Catalase ◽

Erythrocyte Superoxide Dismutase

It has been projected that ferritin and iron yoke in homozygous thalassemic children is coupled with the enhanced free radical formation and blemished in antioxidative defense coordination. Aim: The purpose of the current study was to analyze the consequence of serum iron, erythrocyte catalase, and erythrocyte superoxide dismutase (ESOD) in patients with beta-thalassemia major. Method: 60 beta-thalassemia major patients were studied before and after supplementation of A – Z antioxidants for 20 weeks, and status were compared with 60 age and sex-matched healthy normal. Serum Iron estimation was carried out by Ramsay’s Dipyridyl Method. Estimation of erythrocyte superoxide dismutase was done by Kajari Das Method. The levels of Catalase concentration in erythrocytes were analyzed by the Goth method. All the objectives mentioned above were run by using a UV visible Spectrophotometer (Systronix). Results: A marked enhancement was seen in the intensity of serum iron, and superoxide dismutase (p<0.001) with parallel decline was observed in the level of erythrocyte catalase (p<0.001) in homozygous thalassemia patients when compared with healthy subjects. After 20 weeks of regular supplementation of antioxidants A-Z syrup, which consists of multimineral multivitamins, the concentration of catalase was increased whereas iron and ESOD (p<0.001) were reduced significantly when compared with normal and baselines thalassemic patients. Conclusion: Due to multiple blood transfusions, beta-thalassemia major children are at advanced risk due to secondary iron surplus and intense oxidative stress. Such kind of circumstances may be handled with supplementation of antioxidants A-Z syrup with their regular treatment.

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Hyperferritinemia and oxidative stress in the kidney of beta thalassemia major

Bali Medical Journal ◽

10.15562/bmj.v8i2.1413 ◽

2019 ◽

Vol 8 (2) ◽

pp. 596

Author(s):

Dian Ariningrum ◽

Purwanto Adhipireno ◽

Lisyani Budipradigdo Suromo

Keyword(s):

Oxidative Stress ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Beta Thalassemia Major ◽

And Oxidative Stress

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Hemoglobin H Disease and Beta Thalassemia Major Demonstrated Higher Leucocytic DNA Damage

European Journal of Biology and Biotechnology ◽

10.24018/ejbio.2021.2.6.307 ◽

2021 ◽

Vol 2 (6) ◽

pp. 26-28

Author(s):

Yim Tong Szeto ◽

Phyllis Lok Yin Ho ◽

Tommy Tsz Hin Kong

Keyword(s):

Oxidative Stress ◽

Dna Damage ◽

Blood Transfusion ◽

White Blood Cells ◽

Normal Subjects ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Beta Thalassemia Major ◽

Hbh Disease ◽

Hemoglobin H Disease

Hemoglobin H disease and beta thalassemia major are the more severe forms of thalassemia with frequent blood transfusion may be required. Iron chelation therapy is usually needed with blood transfusion to avoid iron overload. Oxidative stress mediated by excess iron via Fenton reaction may contribute to cellular DNA damage. This study was to investigate whether HbH and beta thalassemia major patients were suffered from higher oxidative stress in leucocytes. Comet assay was performed to investigate the DNA damage of 40 normal subjects, 40 hemoglobin H disease patients and beta thalassemia major patients. The UV-induced DNA damages of leucocytes were measured. The comet scores calculated by visual scoring under light microscope represented DNA damage. The mean ± standard deviation comet score for normal subjects; HbH disease and beta thalassemia major were 262.9 ± 8.1, 293.9 ± 15.4 and 293.5 ± 7.2 respectively. Results showed that both HbH disease and beta thalassemia major patients had higher DNA damage in white blood cells.

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