Ebselen: A promising therapy protecting cardiomyocytes from excess iron in iron-overloaded thalassemia patients

Iron-overload-associated cardiomyopathy has been one of the primary causes of mortality in thalassemia patients with iron burden. There is growing evidence citing the beneficial effects of ebselen as an antioxidant selectively blocking the divalent metal transporter 1 (DMT-1) to deter iron ingress into cardiomyocytes, raising internets in viewing this component in this population in order to treat and even prevent cardiomyopathy occurring from iron surplus. In this article, we reviewed the potential advantageous effects of ebselen in thalassemia patients who suffer from iron excess, susceptible to cardiomyopathy induced by iron overload. A systematic search in several databases, including PubMed, Scopus, and Web of Science, was conducted to explore the role of ebselen in controlling iron-overload-related cardiomyopathy in thalassemia patients by the keywords of Ebselen AND iron. The inclusion criteria were English-written preclinical and clinical studies investigating the efficacy and side effects of ebselen in an iron-overload context. After searching the databases, 44 articles were found. Next, of 19 published articles, 3 were included in this article. After reviewing the references of the included studies, no articles were added. In conclusion ebselen can be a promising adjuvant therapy in patients with thalassemia alongside the standard treatment with iron chelators, particularly in severe cases with cardiomyopathy, due to falling iron inflow by inhibiting DMT-1 and increasing ferroportin-1 expression and antioxidant properties. However, clinical studies need to be carried out to reach a definite conclusion

Two risk factors for hypozincemia in diabetic β-thalassemia patients: Hepatitis C and deferasirox.

Background and aimHypozincemia is a prevalent adverse consequence in diabetes mellitus (DM) and β-Thalassemia patients. We aimed to evaluate the level of serum zinc in β-thalassemia patients with DM and a risk assessment for hypozincemia.MethodsThe study population included transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT) with overt DM (fasting plasma glucose (FPG) ≥126 mg/dL, and/or 2-h plasma glucose≥200 mg/dL). Serum zinc concentration was measured by the colorimetric method, and the values below 70 μg/dL were defined as hypozincemia. Myocardial and liver T2*-weighted magnetic resonance imaging (MRI T2*, millisecond [ms]) were valued by a free contrast MRI. The demographic, clinical, paraclinical, and laboratory data were also recorded. The data belonged to the period from December 2018 until December 2020.ResultsOf 64 diabetic β-thalassemia patients, 41 cases had zinc data in their medical files (aged 38 ± 9 years, 48.8% female). 78.05% of patients (n = 32) were TDT, and 21.95% were NTDT (n = 9). The mean ± standard deviation of zinc level was 110.2 ± 127.6 μg/dL. The prevalence of hypozincemia was 9.76%, 95% confidence interval [CI] 0.27 to 19.24 (four cases). After controlling age, the odds of hypozincemia for using deferasirox (DFX) was 8.77, 95% CI 0.60 to 127.1. In β-thalassemia patients, the age-adjusted risk of hypozincemia was calculated at 15.85, 95% CI 0.47 to 529.3 for hepatitis C. The adjusted risk of hypozincemia based on age for antacid use was 6.34, 95% CI 0.39 to 102.7.ConclusionIn light of this study, as well as hepatitis C, using DFX and antacids is associated with a high risk of hypozincemia amid diabetic β-thalassemia cases. However, upward bias should be taken into consideration

Factors Related to Reduced Bone Density in β-Thalassemia Major Patients: Mazandaran Thalassemia Registry

Introduction: OsteoPenia and osteoPorosis are known to be one of the main comPlications of β-thalassemia major (β-TM). The aim of this study was to determine the factors associated with decreased bone density of these Patients. Methods: Lumbar and femoral neck bone mineral density (BMD) in β-TM Patients characterized via Dual-energy X-ray absorPtiometry (DXA) to PinPoint the association of iron chelators, diabetes mellitus, serum ferritin above 3000 ng/mL, hemoglobin below 8 gr/dl, using hydroxyurea, liver siderosis and moderate to severe hePatic and cardiac siderosis with BMD. The information was collected from the thalassemia registry in 2019 and statistical analysis was Performed by STATA-13 software. Results: Out of 1959 ParticiPants with β-thalassemia major registered in the registry, 139 ones (7.09%) had bone mineral density (53 males and 86 females). The Prevalence of abnormal bone density in the lumbar and femoral neck was (52.48 to 47.66) 57.5 and (40.18 to 57.52) 48.85 Percent, resPectively. After adjusting for the effects of Possible interventions, the odds ratio of abnormal bone density in the Patients consuming hydroxyurea and deferasirox were 0.24 (0.03 to 1.73) and 0.25 (0.03 to 1.69), resPectively. The highest odds were 8.21 (0.18 to 360.22), and 6.72 (0.60 to 74.83) for moderate to severe cardiac and hePatic dePosition, resPectively. Conclusion: The Prevalence of osteoPenia and osteoPorosis is high among thalassemia major Patients. ConsumPtion of hydroxyurea and deferasirox is the most imPortant Protective factor and moderate to severe cardiac and hePatic siderosis are the most imPortant risk factors for abnormal bone density