scholarly journals Oxidative Stress (Malondialdehyde) in Adults Beta-Thalassemia Major and Intermedia: Comparison Between Before and After Blood Transfusion and Its Correlation with Iron Overload

2021 ◽  
Vol Volume 14 ◽  
pp. 6455-6462
Author(s):  
Tubagus Djumhana Atmakusuma ◽  
Intan Russiana Nasution ◽  
Noorwati Sutandyo
Keyword(s):  
Oxidative Stress ◽  
Blood Transfusion ◽  
Iron Overload ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Before And After

scholarly journals Protective Effects of Selenium in Patients with Beta-Thalassemia Major

2020 ◽  
Vol 26 (1) ◽  
pp. 25-31
Author(s):  
Ajand Aboutalebi ◽  
Abolghasem Jouyban ◽  
Hadi Chavoshi ◽  
Aliakbar Movassaghpour Akbari ◽  
Elnaz Shaseb ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Iron Overload ◽  
Kidney Function ◽  
Hair Loss ◽  
Binding Capacity ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Severe Renal Failure ◽  
Beta Thalassemia Major ◽  
Liver And Kidney

Background : Beta-thalassemia major patients require repeated blood transfusion which is associated with iron overload in different organs such as heart, liver, kidney and their related complications. In this study the effects of selenium in iron overload related complications of patients with beta-thalassemia major were assessed. Methods: In this clinical trial, 34 beta-thalassemia major patients over 12 years old were enrolled. Patients with severe renal failure, history of selenium consumption over the last three months, change of blood transfusion pattern, and any change of chelating agent were excluded from the study. For all patients, tablet of selenium 200 µg/day was administered for a month. Blood samples were taken at baseline and after one-month to assess the level of ferritin, total iron-binding capacity (TIBC), aspartate aminotransferase (AST), alanine aminotransferase (ALT), serum creatinine (Scr), selenium. Hair loss was assessed by questionnaire before and after intervention. Results: From 34 patients, 27 (79.4%) had deficient level of selenium at baseline. The selenium level was increased after intervention (p=0.005). The level of serum ALT and Scr decreased remarkably after one-month selenium consumption (p=0.007 for both). In addition, the AST level decreased remarkably after intervention (p=0.053). Severe hair loss profile has improved significantly after supplementation (p=0.004). Conclusion: One-month selenium consumption improved liver and kidney function related markers remarkably. Moreover, selenium improved hair profile and severe hair loss in thalassemia patients. Further studies are needed on the effect of selenium administration on liver and kidney function.

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

2019 ◽  
Vol 16 ◽  
pp. 100245 ◽  
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

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

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.

scholarly journals Left Atrial Strain Identifies Increased Atrial Ectopy in Patients with Beta-Thalassemia Major

Diagnostics ◽  
2020 ◽  
Vol 11 (1) ◽  
pp. 1
Author(s):  
Maria Vlachou ◽  
Vasileios Kamperidis ◽  
Efthymia Vlachaki ◽  
Georgios Tziatzios ◽  
Despoina Pantelidou ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Left Atrial ◽  
Systolic Pressure ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Tape Recording ◽  
Blood Transfusions ◽  
Systolic Strain ◽  
Peak Systolic Strain ◽  
Beta Thalassemia Major

Patients with beta-thalassemia major (β-ΤΜ) may develop cardiac arrhythmias through a multifactorial mechanism. The current study evaluated the association of cardiac structure and function on echocardiography with atrial ectopic burden on 24-hour tape recording in β-ΤΜ patients. This prospective study included consecutive β-ΤΜ patients. Demographic, laboratory, echocardiographic, cardiac magnetic resonance (CMR) T2* and 24-hour tape recording data were prospectively collected. The patients were classified according to the median value of premature atrial contractions (PACs) on 24-hour tape. In total, 50 β-TM patients (37.6 ± 9.1 years old, 50% male) were divided in 2 groups; PACs ≤ 24/day and > 24/day. Patients with PACs > 24/day were treated with blood transfusion for a longer period of time (39.0 ± 8.6 vs. 32.0 ± 8.9 years, p < 0.007), compared to their counterparts. Older age (OR: 1.121, 95% CI: 1.032–1.217, p = 0.007), longer duration of blood transfusion (OR:1.101, 95% CI:1.019–1.188, p = 0.014), larger LV end-diastolic diameter (OR: 4.522, 95% CI:1.009–20.280, p = 0.049), higher values of LA peak systolic strain (OR: 0.869, 95% CI: 0.783–0.964, p = 0.008), higher MV E/E′ average (OR: 1.407, 95% CI: 1.028–1.926, p = 0.033) and higher right ventricular systolic pressure (OR: 1.147, 95% CI: 1.039–1.266, p = 0.006) were univariably associated with PACs > 24/day. LA peak systolic strain remained significantly associated with PACs > 24/day after adjusting for the duration of blood transfusions or for CMR T2*. The multivariable model including blood transfusion duration and LA peak systolic strain was the most closely associated with PACs > 24/day. Receiver operating characteristic curve analysis identified a left atrial peak systolic strain of 31.5%, as the best cut-off value (83% sensitivity, 68% specificity) for prediction of PACs > 24/day. In β-TM patients, LA peak systolic strain was associated with the atrial arrhythmia burden independently to the duration of blood transfusions and CMR T2*.

scholarly journals Liver Enzymes in Children with beta-Thalassemia Major: Correlation with Iron Overload and Viral Hepatitis

2015 ◽  
Vol 3 (2) ◽  
pp. 287-292 ◽  
Author(s):  
Khaled M. Salama ◽  
Ola M. Ibrahim ◽  
Ahmed M. Kaddah ◽  
Samia Boseila ◽  
Leila Abu Ismail ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Iron Overload ◽  
Viral Hepatitis ◽  
Serum Ferritin ◽  
Liver Enzymes ◽  
Transferrin Saturation ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Elevated Liver Enzymes ◽  
Hcv Antibody

BACKGROUND: Beta Thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%) with an estimated carrier rate of 9-10.2%. Injury to the liver, whether acute or chronic, eventually results in an increase in serum concentrations of Alanine transaminase (ALT) and Aspartate transaminase (AST).AIM: Evaluating the potentiating effect of iron overload & viral hepatitis infection on the liver enzymes.PATIENTS AND METHODS: Eighty (80) thalassemia major patients were studied with respect to liver enzymes, ferritin, transferrin saturation, HBsAg, anti-HCV antibody and HCV-PCR for anti-HCV positive patients.RESULTS: Fifty % of the patients were anti-HCV positive and 55% of them were HCV-PCR positive. Patients with elevated ALT and AST levels had significantly higher mean serum ferritin than those with normal levels. Anti-HCV positive patients had higher mean serum ferritin, serum ALT, AST and GGT levels and higher age and duration of blood transfusion than the negative group. HCV-PCR positive patients had higher mean serum ferritin and serum ALT and also higher age and duration of blood transfusion than the negative group.CONCLUSION: Iron overload is a main leading cause of elevated liver enzymes, and presence of HCV infection is significantly related to the increased iron overload.

The Oxidative Status of Valinomycin-Resistant Normal and Thalassemic Red Cells (RBCs).

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3191-3191
Author(s):  
Johnny Amer ◽  
Zipora Etzion ◽  
Robert M. Bookchin ◽  
Eitan Fibach
Keyword(s):  
Oxidative Stress ◽  
Oxidant Stress ◽  
Thalassemia Major ◽  
Oxidative Status ◽  
Normal Blood ◽  
Beta Thalassemia ◽  
Globin Genes ◽  
Before And After ◽  
Mercury Orange

Abstract Normal high-K+, low-Na+ RBCs, suspended in low-K+ media and permeabilized to K+ with valinomycin, become dehydrated from net loss of KCl and water. A very small fraction of light, normal RBC and larger fractions of light, sickle cell anemia (SCA) and beta-thalassemia RBC were found to be “valinomycin-resistant” (val-res) due to their Na+/K+ gradient dissipation (PNAS2000;97: 8050; BLOOD2000;96:24b). In thalassemia and SCA, although the primary lesions involve the globin genes, the major damage to the RBC membranes is mediated by oxidative stress. We previously showed (Cytometry2004;60:73) that thalassemic RBC have higher reactive oxygen species (ROS) and lower reduced glutathione (GSH) levels than normal RBC before or after in vitro oxidant stress (treatment with hydrogen peroxide). Here, we examined the oxidative status of val-res RBC from normal and beta-thalassemia major blood. RBC suspended in a plasma-like buffer containing 15 mM KCl and 10 mM valinomycin for 45 min were then layered on arabinogalactone (Larex) with density δ=1.091, and spun at 15,000 g for 30 min. Val-res cells were identified as the low density (δ<1.091 g/ml) RBCs recovered from the interphase layer. The percent val-res RBC in beta-thalassemic samples (n = 10), was 84-fold higher (4.2 ± 0.4% (mean ± SD), range 2.5 to 6.0%) than in normal samples (0.05 ± 0.06%, range 0.02 to 0.1%) (n =10). To determine the oxidative status of the RBC, the cells were washed with PBS and stained for intracellular contents of ROS and GSH, using 2′-7′-dichlorofluoresein and Mercury Orange, respectively. RBC were analyzed by flow cytometry, using gating based on size and granularity. The Mean Fluorescence Channel (MFC) for each fluorochrome was computed. The results showed that valinomycin treatment, per se, did not affect ROS and GSH contents: MFC of the stained un-fractionated RBC was similar before and after treatment with valinomycin, indicating that large changes in MCHC had little or no effect on these measurements. In addition, the unfractionated RBC had ROS and GSH values comparable to those of the high density (val-sensitive) RBC which were recovered from the pellet of valinomycin-treated RBC following Larex fractionation. Measurements on six normal and six beta-thalassemic blood samples indicated that in each case val-res RBC had higher ROS (3.5-10 fold) and lower GSH (2.5-8 fold) levels than the unfractionated RBC or the val-sensitive RBC of the same sample. Compared with val-res cells from normal blood, thalassemic val-res RBC had higher capacity to produce ROS (1.7-fold) and had a lower GSH level (1.5-fold) compared with normal val-res RBC. These results confirm that, as with SCA, beta-thalassemia blood contains a higher percent of val-res RBC than normal blood. They show, further, that (i) both normal and thalassemic val-res RBC have higher oxidative status than other cells (val-sensitive) in the same sample; and that (ii) thalassemic val-res RBC have higher oxidative status than val-res RBC in normal blood. The present results are consistent with the possibility that oxidative stress may contribute to the generation of val-res RBCs, but do not establish a cause-effect relationship. Further studies will be needed to elucidate the origin and significance of these cells.

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