Two trade names of deferasirox (Osveral® and Exjade®) in reduction of iron overload parameters in major beta-thalassemia patients: A randomized open labeled clinical trial.

BACKGROUND: Beta-thalassemia major patients typically require chronic transfusion and iron-chelating agents to reduce serum iron overload. Osveral® is an available Iranian brand name of deferasirox used by majority of thalassemic patients. The aim of this study was to compare the efficacy of Osveral® vs. Exjade® in major beta- thalassemia patients. METHODS: In this randomized clinical trial, all patients received a single daily dose of 30 mg/kg either of Osveral® or Exjade® for 6 months. Primary outcome was the mean of bimonthly changes in serum ferritin concentration and secondary outcomes included mean changes of heart and liver MRI T2* after a year. RESULTS: Finally, 80 patients completed the study. The mean serum ferritin level at the end of sixth month significantly decreased in Osveral® and Exjade® groups (p<0.01). After a year, means cardiac MRI T2* in Osveral® group were changed from 25.9±9.6 ms to 25.4±9.7 ms and in Exjade® group from 24.8±9.2 ms to 26.9±5.9 ms, with no significant difference (P=0.43). Mean liver MRI T2* for Osveral® and Exjade® groups were 8.6±6.4 ms (baseline 6.3±4.7) and 6.3±4 ms (baseline 4.9±3.5), respectively and there was no significant difference between two study arms (P=0.1). CONCLUSION: Osveral® decreased significantly the serum ferritin level and improved heart and liver iron overload as efficient as Exjade®. It can be a suitable cost-effective alternative agent in beta-thalassemia major patients.

A double-blind, controlled, crossover trial of amlodipine on iron overload status in transfusion dependent ß-thalassemia patients.

BACKGROUND AND AIM: This study examined whether administration of amlodipine could improve myocardial iron loading status in patients with transfusion dependent ß-thalassemia (TDT), through a placebo-controlled, crossover study. METHODS: Amlodipine (5 mg, daily) or placebo were prescribed to all patients (n = 19) for 6 months, and after a 2-week washout period, patients were crossed over to the other group. The efficacy of amlodipine on iron loading was assessed by measuring myocardial T2*-weighted magnetic resonance imaging (MRI T2*, millisecond [ms]) and serum ferritin (ng/mL). RESULTS: Seventeen patients completed the study. The mean ± standard deviation [SD] of myocardial MRI T2* at baseline was 9.83 ± 2.67 ms Myocardial MRI T2* value rose to 11.44 ± 4.14 ms post amlodipine treatment in all patients. After placebo, myocardial MRI T2* value reached 10.29 ± 4.01 ms After controlling the baseline measures, Hedges's g for ferritin and myocardial MRI T2* outcomes were estimated 3.84 (95% confidence interval [CI] 2.68 to 4.97) and -1.80 (95% CI -2.58 to -0.10), respectively. CONCLUSION: Amlodipine might improve myocardial MRI T2* and serum ferritin level compared to placebo. However, larger clinical studies are needed to confirm the results.

The improvement of pulmonary artery pressure after bosentan therapy in patients with ß-thalassemia and Doppler-defined pulmonary arterial hypertension.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is relatively prevalent in patients with thalassemia. PAH treatment is necessary as the prevalence of Doppler-estimated PAH and the resultant mortality is high in such patients. MATERIALS AND METHODS: This study aimed at evaluating the effect of bosentan therapy on patients with thalassemia suspected of PAH. Based on pulsed Doppler echocardiography, all the cases were suspected of severe PAH. Consequently, bosentan was initiated at a dose of 62.5 mg twice a day for 4 weeks, which was increased to 62.5-125 mg twice a day, if no adverse side effects were observed. RESULTS: The results of this study showed that pulmonary artery pressure (PAP) decreased after the administration of bosentan in three cases, from 160 to 120, 110 to 65, and 60 to 25 mmHg; in other words, the PAP reduced in the mentioned cases by 25%, 36.4%, and 58.4%, respectively. CONCLUSION: In this study, PAP improved after bosentan therapy in patients with ß-thalassemia suspected of PAH; however, further studies are required to confirm the findings.

The Role of Doppler Imaging in the Assessment of Right Ventricular Function: a Case-control Study of Acute Inferior Wall Infarction.

BACKGROUND: Right ventricular infarction (RVI) develops in 30-50% of patients with inferior wall infarction (IWI). The rates of mortality, morbidity, and complications in these patients are greater than in the patients without RVI. We compared the tissue Doppler imaging (TDI) indices between a group of patients with IWI and RVI, with a similar group of patients who had IWI alone to investigate the application of TDI indices in the evaluation and detection of right ventricular function. MATERIAL AND METHODS: We studied 49 patients with first acute IWI in two groups. Group 1 (N=24) were patients with IWI and RVI while group 2 consisted of patients with IWI alone (N=25), based on standard electrocardiogram criteria. The peak systolic (Sm), peak early (Em) and late (Am) diastolic velocities, and Em/Am ratio were obtained from the apical four chamber view, at the lateral side of the tricuspid annulus. We measured trans-tricuspid early (ET) and peak (AT) filling velocity, ET/AT ratio, right ventricular end diastolic diameter (RVEDD), and tricuspid annular plane systolic excursion (TAPSE) by M-mode TDI projected at the long axis of parasternal view. RESULTS: The RVEDD and E/Em ratio were increased, while the TAPSE was significantly decreased in the patients with RVI as compared to those without RVI (4.7± 0.6 vs. 3.1±0.2 cm; p < 0.005, 5.6±2.21 Vs 4.5±1.2; p<0.006 and 1.7±0.4 vs. 2.3±0.5 cm; p <0.0001, respectively). However, the other statistically measured parameters were not significantly different between these groups. CONCLUSION: The measurement of RVEDD, E/Em ratio, and TAPSE, as right ventricular myocardial systolic and diastolic parameters by pulse wave TDI could be used to objectively assess the status of RV condition in patients with first acute IWI.

Cardiac Structural and Functional Changes Evaluated by Transthoracic and Tissue Doppler Echocardiography in Adult Patients with Sickle Cell Disease.

One of the most common genetic blood disorders, resulting from inherited red blood cell disorders, is sickle cell disease (SCD) which is responsible for high death in adult patients with left ventricular diastolic dysfunction and pulmonary hypertension. Tissue Doppler Echocardiography (TDE) and transthoracic echocardiography (TTE) are two useful tools to assess the risk of SCD. The present study was conducted to evaluate the cardiac functions and structure using TDE and TTE among adult patients suffering from sickle cell anemia compare to normal samples. METHODS: The current study was performed on 30 SCD patients with a mean age 18-40 years and healthy cases at Mazandaran Heart Center, Iran. The left and right ventricular functions were assessed using M-mode, two-dimensional (2D), and tricuspid regurgitation jet velocity (TRJV) data and TDE derived myocardial velocity measurements in: SCD patients compared to control. RESULTS: According to the findings, SCD group showed significantly higher E and E' waves of left ventricle inflow compare to the control group (p<0.03 and p<0.01, respectively). No significant difference was observed for E/E' ratio between two groups: case and control, but there was significant differences between the groups in E and E' waves respectively equal to 8.55 and 8.14 cm/s. We obtained significant differences for peak early and late LV diastolic velocity between two groups and other indicators showed no significant differences. CONCLUSION: The evaluation of LV systolic and diastolic function via TDE did not indicate significant differences between SCD patients compared to healthy subjects. High E and E' waves in these patients, could indicate progression towards cardiac disorders and pulmonary hypertension in future.

Hyaline vascular-type Castleman's disease in the hilum of liver: a case report.

BACKGROUND: Castleman's disease or angiofollicular lymphoid hyperplasia is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. The disease can present at any extra thoracic site with lymphoid tissue such as retroperitoneal, mesentery, axilla, and pelvis. Hepatic localization castleman disease is very rare in children. Herein, we reported a case of Castleman's disease arising from the lymph node in hilum of liver. CASE PRESENTATION: A 5 -year-old girl with chief complaint of abdominal pain for two months which exaggerated in last three days was referred to the hospital. On routine physical examination, only a generalized abdominal pain was noticed. Routine laboratory investigations and Chest X-Ray were normal. Abdominal Sonography revealed a 3.7 x 3.1 cm solid mass in the hilum of the liver. On the MRI images, a lobulated mass in the portal hepatic associated with mass effect on the portal vein was visible. Histological examination revealed expansion of mantle zone in lymphatic nodules accompanied by burnt out germinal centers. This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease. The patient underwent a laparotomy. The patient had an uneventful postoperative course. CONCLUSION: This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease.