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

Anemia ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-9 ◽  
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.

scholarly journals Correlation of Oxidative Stress with Serum Trace Element Levels and Antioxidant Enzyme Status in Beta Thalassemia Major Patients: A Review of the Literature

Anemia ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Q. Shazia ◽  
Z. H. Mohammad ◽  
Taibur Rahman ◽  
Hossain Uddin Shekhar
Keyword(s):  
Oxidative Stress ◽  
Trace Elements ◽  
Antioxidant Enzymes ◽  
Tissue Injury ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Trace Element Levels ◽  
Serum Trace Elements

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.

scholarly journals Nutritional Biomarkers in Children and Adolescents with Beta-Thalassemia-Major: An Egyptian Center Experience

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Laila M. Sherief ◽  
Sanaa M. Abd El-Salam ◽  
Naglaa M. Kamal ◽  
Osama El safy ◽  
Mohamed A. A. Almalky ◽  
...  
Keyword(s):  
Trace Elements ◽  
Folic Acid ◽  
Serum Zinc ◽  
Serum Levels ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Healthy Children ◽  
Multicenter Studies ◽  
Thalassemic Patient ◽  
Beta Thalassemia Major

Background and Aim. Trace elements and vitamins play a vital role in human body to perform its function properly. Thalassemic patients are at risk of micronutrient deficiency. This study estimated levels of vitamins A, C, E, B12, folic acid, total homocysteine (tHcy), and methylmalonic acid (MMA) along with trace elements, zinc, copper, and selenium in Beta-thalassemia-major patients.Methods. This study included 108 patients with Beta-thalassemia-major and 60 age and sex matched healthy children. Serum levels of vitamin A, E, C, tHcy, and MMA were estimated by high pressure liquid chromatography while serum levels of folic acid and B12 were estimated by thin layer chromatography. Serum zinc, copper, and selenium were determined by atomic absorption spectrometry.Results. There was a significant decrease of vitamins A, C, E, and B12 and trace elements zinc, copper, and selenium in thalassemic patients as compared to controls. tHcy and MMA were significantly elevated in patients. No significant correlations were found between the serum levels of the studied vitamins and trace elements as regards age, frequency of transfusion, duration of transfusion, and serum ferritin.Conclusion. The level of various nutritional biomarkers (vitamins A, C, E, and B12 and trace elements zinc, copper, selenium) was reduced in chronically transfused Egyptian thalassemic patient. These patients should have periodic nutritional evaluation and supplementation. Multicenter studies are highly recommended.

scholarly journals Evaluation of serum trace elements, lipids profile, ferritin and 8-hydroxydeoxyguansine levels in Yemeni patients with beta thalassemia major

2020 ◽  
Vol 6 (1) ◽  
pp. 47-52
Author(s):  
Mansour S. Al-Kobati ◽  
Hassan M. Al-Mahbashi ◽  
Badria Shamsan ◽  
Najla AL-Sonboli ◽  
Nawfal A.M. Numan
Keyword(s):  
Trace Elements ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Serum Trace Elements ◽  
Lipids Profile

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

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.

A case of post-splenectomy transfusion-dependent homozygous beta-thalassemia major complicated with myocardial siderosis and osteoporosis and usage of iron-chelating therapy with deferiprone in pregnancy

2019 ◽  
Vol 39 (2) ◽  
Author(s):  
Mohd Faizal Ahmad ◽  
Nur Zawani Zakaria ◽  
Noorazizah Arsad ◽  
Kah Teik Chew ◽  
Muhammad Azrai Abu ◽  
...  
Keyword(s):  
Well Being ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Induction Agent ◽  
Close Monitoring ◽  
Assisted Reproductive Technique ◽  
Autosomal Recessive Disorders ◽  
Beta Thalassemia Major ◽  
Individual Care ◽  
Endocrine Organs

Abstract Beta-thalassemia major is a subtype component of hemoglobinopathies; autosomal recessive disorders complicated with anemia that affect at least 50,000 babies each year. It contributes to problems in reproductive entities such as infertility due to iron deposition in the endocrine organs, which leads to malfunction of the hypothalamus-pituitary axis. Due to this, there have been very few pregnancies discovered and reported with this type of condition as they usually required an ovulation-induction agent with assisted reproductive technique to achieved pregnancy. We report a successful spontaneous pregnancy in a woman with beta-thalassemia major who underwent splenectomy with lifelong transfusion-dependence complicated with myocardial siderosis and osteoporosis. The close monitoring and regular blood transfusion are a core of successful support to this type of pregnancy. The unintentional consumption of Fosamax, hydroxyurea and deferiprone (Ferriprox) up till 20 weeks of gestation did not show any adverse effects on fetal well-being. As expected, this pregnancy ended with the preterm delivery via cesarean section due to intrauterine growth restriction with oligohydramnios, and currently, this child is thriving. We concluded that pregnancy is not a contraindication in beta-thalassemia major; complex individual care is needed to achieve a safe outcome for the mother.

scholarly journals Oxidative stress and disturbance in antioxidant balance in beta thalassemia major

2008 ◽  
Vol 23 (4) ◽  
pp. 337-340 ◽  
Author(s):  
Rahul A. Ghone ◽  
K. M. Kumbar ◽  
A. N. Suryakar ◽  
R. V. Katkam ◽  
N. G. Joshi
Keyword(s):  
Oxidative Stress ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major
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