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Background: Deferasirox (DFX) is a once-daily oral iron chelator with proven dose-dependent efficacy in patients with thalassemia major (TM). The reason for switching from DFX dispersible tablets (Exjade®) to DFX film-coated tablets (Jadenu®) was intolerance. Many patients also reported that deferasirox® did not taste good. In this study, we compared the effect of Jadenu® and Exjade® on satisfaction, convenience, cardiac/liver MRI T2*, serum ferritin levels, and biochemical profiles in patients with thalassemia major. Method: Sixty-two patients with thalassemia over 2 years of age, who had iron overload indicated by chelation therapy, were randomly divided into two groups. The first group (n = 32) is treated with Exjade®, and the second group (n = 30) is treated with Jadenu®. Laboratory investigations included alkaline phosphatase (ALK), alanine transferase (ALT), aspartate transferase (AST), and serum ferritin levels. Cardiac/liver MRI T2* levels and patient satisfaction and convenience, were assessed before and 1 year after starting therapy. Results: The study found that 53.3% of Jadenu® patients were satisfied with the taste of the medication compared to only 12.5% of Exjade® patients, which was statistically significant (p = 0.001). Additionally, 40% of Jadenu® patients were satisfied with the ease of taking the medication compared to 28.1% of Exjade® patients, and again, the difference was statistically significant (p = 0.047). A comparison of the cardiac MRI T2* levels between the two studied groups showed no significant difference (p = 0.851). Conclusion: Jadenu® offers patients an improved formulation that can be taken on an empty stomach, has a better taste, and presents fewer gastrointestinal tolerability concerns. Overall, patient satisfaction is higher with Jadenu®, which may improve adherence and reduce the frequency and severity of complications associated with iron overload. This, in turn, may help mitigate cardiovascular and hepatic complications from iron overload in the long term. Clinical Trial Registration: https://irct.behdasht.gov.ir/search/result?query=IRCT20210830052346N1 [ABSTRACT FROM AUTHOR]

Background: Given the association of hypomagnesemia with cardiac arrhythmia, the aim of this study was to investigate the relationship between serum magnesium levels with age and T2* magnetic resonance imaging (MRI) findings of the heart and liver in patients with thalassemia major (TM). Materials and Methods: In a descriptive cross-sectional study, a total of 62 patients with ß-thalassemia major aged 11-48 years were selected at the Amir-Kabir Hospital, Arak, Iran. Serum magnesium, ferritin, and iron levels of patients were measured, and the rate of cardiac and hepatic hemosiderosis of patients was extracted according to the routine T2*MRI method. Results: The mean age of the patients at diagnosis was 32.6 years. The comparison of TM patients with and without hepatic/cardiac hemosiderosis demonstrated that mean levels of serum ferritin, serum iron, and age were significantly higher in TM patients with cardiac hemosiderosis than in hepatic/cardiac non-hemosiderosis (P < 0.05); however, there was no significant difference in mean levels of serum magnesium in TM patients with and without hepatic/cardiac hemosiderosis (P = 0.279). Interestingly, the correlation of age with serum magnesium levels in TM patients revealed a statistically significant and moderate inverse correlation (r = -0.56, P = 0.013). Conclusion: Hypomagnesemia may occur in a time-dependent manner. It is recommended that, in addition to cardiac and hepatic T2*MRI, serum magnesium levels be measured by using magnesium replacement if necessary. [ABSTRACT FROM AUTHOR]

Background: Pulmonary arterial hypertension (PAH) may result in cardiomyopathy which is a major cause of death in thalassemia patients. Vitamin D is associated with benefits in cardiovascular disorders. Our purpose was to study effects of vitamin D on pulmonary artery pressure in thalassemia major and intermedia patients. Materials and Methods: This randomized trial was performed on 26 patients with thalassemia major (TM) and intermedia (TI) in Amir-Kabir Hospital, Arak, Iran in 2019-2020. Patients were randomized 1:1 to intervention group (vitamin D 50,000 IU/week) and control group (received no supplement) for 20 weeks. Echocardiography was used to measure pulmonary artery pressure and assess cardiovascular function. The levels of 1,25-dihydroxyvitamin D3, ferritin, and cardiac iron content were measured in study groups. Results: After 20 weeks, pulmonary arterial pressure (PAP), tricuspid regurgitant velocity (TRV), and pulmonary regurgitant velocity (PRV) significantly improved in the intervention group compared to the control group (P= 0.010, P= 0.003, and P= 0.001, respectively). Moreover, ejection fraction (EF) had significant increase in the intervention group compared to the control group (P= 0.008), although vitamin D supplementation had no significant impact on cardiac T2* values (P= 0.827), systolic and diastolic blood pressure (P= 0.388 and P= 0.509, respectively) and serum hemoglobin and ferritin levels (P= 0.557 and P= 0.620) as compared to the control group. However, the levels of 25-OHD3 significantly increased in the intervention group compared to the control group (P= 0.036). Conclusion: This study showed that vitamin D 50000 IU/week can improve PAP in patients with thalassemia. Sufficient intake of vitamin D may prevent cardiomyopathies related to PAH. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension is one of the most common cardiac complications among patients with β-thalassemia major (β-TM). The aim of this study is to investigate the correlation of pulmonary artery pressure (PAP), serum ferritin level, and cardiac T2* MRI in patients with β-TM. Method: This cross-sectional study was performed on 50 patients older than 7 years old. Echocardiography, electrocardiography, and cardiac T2* MRI were performed on all patients and their serum ferritin levels were measured. Based on the echocardiographic the patients were divided into two groups of PAP>30 and <30 mmHg. Results: 40% had PAP higher than 30 mmHg, 32% had abnormal T2* MRI, and 36% had serum ferritin levels higher than 1500ng/dl. there were significant negative correlations between CMRI *T2 and PAP (-0.36) and the P-pulmonary (-0.29). the serum ferritin level was positively correlated with PAP (0.44) and the P pulmonary in ECG (0.30). in the patients with PAP>30, the means of age, serum ferritin level, and P-pulmonary were significantly higher than patients with PAP<30 (P=0.001). In the patients with PAP>30mmHg, CMRI *T2 had higher accuracy (80%), sensitivity (65%), and specificity (90%) compared to the measurement of serum ferritin levels. Conclusion: The PAP was positively correlated with serum ferritin levels and negatively with T2MRI. The accuracy of 80% for T2MRI as a method to diagnose or even anticipate PH among patients with TM suggested that this method is a useful and accurate method of PH diagnosis and can be used as an alternative among clinicians. [ABSTRACT FROM AUTHOR]