Comparative Efficacy and Safety Between Deferiprone and Deferasirox with Special Reference to Serum Ferritin Level and Cardiac Function in Bengali β-Thalassemia Major Children

Tripathy, Isita; Panja, Amrita; Dolai, Tuphan Kanti; Mallick, Asim Kumar

doi:10.1080/03630269.2021.1999258

Cited by 5 publications

(2 citation statements)

References 26 publications

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“…Furthermore, since it has a deleterious impact on extra-hepatic lipolytic activity, anemia has been identified as a risk factor for hypertriglyceridemia [ 31 ]. Significantly high plasma ferritin was found among B-TM patients in our study; this conclusion was consistent with those of many earlier researchers [ 32 , 33 ]. Iron overload was affected by a combination of crucial and presumably correctable factors, including poor compliance with iron chelation.…”

Section: Discussionsupporting

confidence: 94%

The Value of SIRT1/FOXO1 Signaling Pathway in Early Detection of Cardiovascular Risk in Children with β-Thalassemia Major

et al. 2022

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Background: Atherosclerosis represents one of the major causes of morbidity in children with β-thalassemia major (β-TM). Aim: This study was designed to investigate SIRT1-FOXO1 signaling in β-TM children and their role in early detection of premature atherosclerosis. Methods: We equally subdivided 100 Egyptian children aged 6–14 years with β-TM according to carotid intima media thickness (CIMT) into 50 with CIMT < 0.5 mm and 50 with CIMT ≥ 0.5 mm, and 50 healthy children of matched age were included. They were subjected to evaluation of SIRT1, heat shock protein 72 (HSP72), and hepcidin levels via ELISA and forkhead box protein 1 (FOXO1) mRNA expression using real-time PCR in PBMCs; meanwhile, malondialdehyde (MDA), superoxide dismutase (SOD), and catalase activities were evaluated spectrophotometrically. Results: Our results show significantly high values for CIMT, β-stiffness, atherogenic index of plasma (AIP), MDA, HSP72 and FOXO1, ferritin with significantly low hepcidin, SOD, catalase, and SIRT1 in β-TM as compared to controls with a more significant difference in β-TM with CIMT ≥ 0.5 mm than those with CIMT < 0.5 mm. A significant positive correlation between CIMT and MDA, HSP72, and FOXO1 gene expression was found, while a significant negative correlation with hepcidin, SOD, catalase, and SIRT1 was found. FOXO1 gene expression and HSP72 levels were the strongest independent determinants of CIMT. Conclusion: In β-TM, FOXO1 signaling is activated with low levels of SIRT1, and this is attributed to accelerated atherosclerosis in β-TM, which would be crucial in prediction of atherosclerosis.

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Section: Discussionsupporting

confidence: 94%

The Value of SIRT1/FOXO1 Signaling Pathway in Early Detection of Cardiovascular Risk in Children with β-Thalassemia Major

et al. 2022

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“…It was also observed that adherence to the treatment regimen was highest with DFX, followed by DFP, and finally, DFO [127]. This is heartening to note, considering the fact DFX has been reported to have greater effectiveness in the reduction in iron overload and chelator-related side effects in TDT patients, when compared to DFP, as reported in a study conducted on children from West Bengal (Eastern India) in 2021 [128]. Furthermore, in 2021, another Indian study reported that combined oral chelation therapy conducted with DFO and DFX significantly reduces serum ferritin levels in TDT children with severe iron overload [129] These findings indicate that oral iron chelation therapy and, in particular, combined oral chelation therapy with both DFP and DFX, may yield the optimal results for the treatment of iron overload in β-thalassemic patients, although further studies in larger cohorts are warranted.…”

Section: Guidelines For Usage Of Iron Chelators In Indiamentioning

confidence: 75%

Understanding the Intricacies of Iron Overload Associated with β-Thalassemia: A Comprehensive Review

Basu

Rahaman

Dolai

et al. 2023

Thalassemia Reports

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β-thalassemia, a congenital genetic hematological disorder characterized by the decrease or absence of β-globin chains, leads to a decrease in levels of Hemoglobin A. The affected individuals can be categorized into two cohorts based on transfusion dependency: transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). Remarkably, despite the primary pathology lying in β-globin chain depletion, β-thalassemia also exhibits an intriguing association with iron overload. Iron metabolism, a tightly regulated physiological process, reveals a complex interplay in these patients. Over time, both cohorts of β-thalassemic individuals develop iron overload, albeit through distinct mechanisms. Addressing the diverse complications that arise due to iron overload in β-thalassemic patients, the utilization of iron chelators has gained a lot of significance. With varying efficacies, routes of administration, and modes of action, different iron chelators offer unique benefits to patients. In the Indian context, three commercialized iron chelators have emerged, showcasing a high adherence rate to iron chelator-based treatment regimens among β-thalassemic individuals. In this review, we explore the intriguing connection between β-thalassemia and iron overload, shedding light on the intricate mechanisms at play. We delve into the intricacies of iron metabolism, unveiling the distinct pathways leading to iron accumulation in these patients. Additionally, the therapeutic efficacy of different iron chelators in managing iron overload complications is mentioned briefly, along with the guidelines for their usage in India. Through this comprehensive analysis, we aim to deepen our understanding of β-thalassemia and iron overload, paving the way for optimized treatment strategies. Ultimately, our findings provide valuable insights into improving the care and outcomes of individuals affected by β-thalassemia.

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Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia

Geneen

Dorée

Estcourt

2023

Cochrane Database of Systematic Reviews

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Comparative Efficacy and Safety Between Deferiprone and Deferasirox with Special Reference to Serum Ferritin Level and Cardiac Function in Bengali β-Thalassemia Major Children

Cited by 5 publications

References 26 publications

The Value of SIRT1/FOXO1 Signaling Pathway in Early Detection of Cardiovascular Risk in Children with β-Thalassemia Major

The Value of SIRT1/FOXO1 Signaling Pathway in Early Detection of Cardiovascular Risk in Children with β-Thalassemia Major

Understanding the Intricacies of Iron Overload Associated with β-Thalassemia: A Comprehensive Review

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia

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