Oxidative stress and disturbance in antioxidant balance in beta thalassemia major

Ghone, Rahul A; Kumbar, K. M.; Suryakar, Adinath N.; Katkam, R. V.; Joshi, N G

doi:10.1007/s12291-008-0074-7

Cited by 88 publications

(76 citation statements)

References 8 publications

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“…This is in agreement with Ghone et al, 24 who stated that, the majority of the beta thalassemia major patients had significant increase of serum iron with significant decrease of total iron binding capacity and have severe anemia due to ineffective erythropoiesis which is a primary reason for iron overload. Thus, increased iron may increase the potential of oxidative injury to erythrocyte and cell organelles.…”

supporting

confidence: 92%

Therapeutic Value of Combined Therapy with Deferasirox and Silymarin on Iron Overload in Children with Beta Thalassemia

Hagag¹,

Elfarargy²,

El-Lateef³

2013

JAC

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Abstract. Background: Beta thalassemia is an inherited hemoglobin disorder resulting in a severe, chronic anemia requiring life-long blood transfusion that induces iron overload. Silymarin is a flavonoid complex isolated from Silybin marianum with a strong antioxidant activity, inducing an hepatoprotective action, and probably, a protective effect on iron overload. The aim of this work was to determine the silymarin value in improving iron chelation in thalassemic patients with iron overload treated with Deferasirox. Patients and Methods: This study was conducted on 40 children with beta thalassemia major under follow-up at Hematology Unit, Pediatric Department, Tanta University Hospital with serum ferritin level more than 1000 ng/ml and was divided into two groups. Group IA: Received oral Deferasirox (Exjade) and silymarin for 6 months. Group IB: Received oral Deferasirox (Exjade) and placebo for 6 months and 20 healthy children serving as a control group in the period between April 2011 and August 2012 and was performed after approval from research ethical committee center in Tanta University Hospital and obtaining an informed written parental consent from all participants in this study. Results: Serum ferritin levels were markedly decreased in group IA cases compared with group IB (P= 0.001). Conclusion: From this study we concluded that, silymarin in combination with Exjade can be safely used in the treatment of iron-loaded thalassemic patients as it showed good iron chelation with no sign of toxicity. Recommendations: We recommend extensive multicenter studies in a large number of patients with longer duration of follow-up and more advanced techniques of assessment of iron status in order to clarify the exact role of silymarin in reducing iron overload in children with beta thalassemia.

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supporting

confidence: 92%

Therapeutic Value of Combined Therapy with Deferasirox and Silymarin on Iron Overload in Children with Beta Thalassemia

Hagag¹,

Elfarargy²,

El-Lateef³

2013

JAC

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“…In patients with BTM, serum glycoprotein levels are lower, and desialylated serum glycoprotein quantities are higher than in controls [18]. Oxidative stress increases in both BTM and HBT [18,19,20]. Increased oxidative stress may disrupt the structure of the membrane glycoproteins on platelets and cause reduced platelet function [17,18,19,20].…”

Section: Discussionmentioning

confidence: 99%

Mean Platelet Volume in Heterozygous Beta Thalassaemia

Çıkrıkçıoğlu¹,

Çelik²,

Ekinci³

et al. 2017

Acta Haematol

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Background/Aim: It is not known why cerebrovascular and cardiovascular ischaemic events are less frequently observed in heterozygous beta thalassaemia (HBT) patients than in the general population. However, we previously reported that serum levels of some platelet function markers, i.e. soluble CD40 ligand and soluble P-selectin, are lower in patients with HBT than in controls. A high mean platelet volume (MPV) is an indicator of in vivo platelet activation and may indicate a tendency to thrombosis. We investigated whether MPV is lower in HBT patients than in controls. Methods: Forty-eight patients with HBT were compared with 51 controls matched for gender, age, and BMI for MPV in a cross-sectional study. Results: The MPV was within the normal range and higher in the HBT group (9.64 ± 1.20 vs. 9.07 ± 082 fL, p = 0.006). The 2 groups were similar in terms of atherosclerosis risk factors and medications. After linear regression analysis, the MPV was correlated with HBT, sensitive CRP, and BMI. Conclusion: The higher MPV in patients with HBT could indicate platelet activation, and this may represent a dilemma. Higher MPV in the HBT group might have resulted from higher sympathetic nervous system activity, mild ineffective erythropoiesis, and haemolysis.

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“…Their defense mechanism in biological system involves chain breaking and preventive mechanisms. 2,3 Same studies had shown that the erythrocytes of thalassemia patients are exposed to higher oxidative stress and a possible consequential accelerated apoptosis because of the high concentration of ROS. 4 ROS elimination involves glutathione transferase own, so these enzymes play an important physiological role.…”

Section: Introductionmentioning

confidence: 99%

Glutathione S Transferase Polymorphisms Influence on Iron Overload in β-Thalassemia Patients

Sclafani

Calvaruso

Agrigento

et al. 2013

Thalassemia Reports

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In patients with β-thalassemia iron overload that leads to damage to vital organs is observed. Glutathione S transferase (GST) enzymes have an antioxidant role in detoxification processes of toxic substances. This role is determined genetically. In this study, we correlated GSTT1 and GSTM1 genotypes with iron overload measured with direct and indirect non-invasive methods; in particular, we used serum ferritin and signal intensity of the magnetic resonance image (MRI) in 42 patients with β-thalassemia, which were regularly subjected to chelation and transfusion therapy. Multiplex polymerase chain reaction was used to determine the genotype. The loss of both alleles leads to a decreased value of liver and heart MRI-signal intensity with a consequent iron accumulation in these organs; the loss of only one allele doesn't lead to relevant overload. Serum ferritin doesn't appear to be correlated to iron overload instead.

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Oxidative stress and disturbance in antioxidant balance in beta thalassemia major

Cited by 88 publications

References 8 publications

Therapeutic Value of Combined Therapy with Deferasirox and Silymarin on Iron Overload in Children with Beta Thalassemia

Therapeutic Value of Combined Therapy with Deferasirox and Silymarin on Iron Overload in Children with Beta Thalassemia

Mean Platelet Volume in Heterozygous Beta Thalassaemia

Glutathione S Transferase Polymorphisms Influence on Iron Overload in β-Thalassemia Patients

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