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Background: Thalassaemia major (TM) patients need regular blood transfusions that lead to accumulation of iron and death from heart failure. Deferiprone has been reported to be superior to deferoxamine for the removal of cardiac iron and improvement in left ventricular (LV) function but little is known of their relative effects on the right ventricle (RV), which is being increasingly recognised as an important prognostic factor in cardiomyopathy. Therefore data from a prospective randomised controlled trial (RCT) comparing these chelators was retrospectively analysed to assess the RV responses to these drugs. Methods: In the RCT, 61 TM patients were randomised to receive either deferiprone or deferoxamine monotherapy, and CMR scans for T2* and cardiac function were obtained. Data were re-analysed for RV volumes and function at baseline, and after 6 and 12 months of treatment. Results: From baseline to 12 months, deferiprone reduced RV end systolic volume (ESV) from 37.7 to 34.2 mL (p = 0.014), whilst RV ejection fraction (EF) increased from 69.6 to 72.2% (p = 0.001). This was associated with a 27% increase in T2* (p < 0.001) and 3.1% increase in LVEF (p < 0.001). By contrast, deferoxamine showed no change in RVESV (38.1 to 39.1 mL, p = 0.38), or RVEF (70.0 to 69.9%, p = 0.93) whereas the T2* increased by 13% (p < 0.001), but with no change in LVEF (0.32%; p = 0.66). Analysis of between drugs treatment effects, showed significant improvements favouring deferiprone with a mean effect on RVESV of-1.82 mL (p = 0.013) and 1.16% for RVEF (p = 0.008). Using regression analysis the improvement in RVEF at 12 months was shown to be greater in patients with lower baseline EF values (p < 0.001), with a significant difference in RVEF of 3.5% favouring deferiprone over deferoxamine (p = 0.012). Conclusion: In this retrospective analysis of a prospective RCT, deferiprone monotherapy was superior to deferoxamine for improvement in RVEF and end-systolic volume. This improvement in the RV volumes and function may contribute to the improved cardiac outcomes seen with deferiprone.

Transfusion and iron chelation therapy revolutionised survival and reduced morbidity in patients with transfusion-dependent beta thalassaemia major. Despite these improvements, cardiac disease remained the most common cause of death in those patients. Recently the ability to determine the degree of cardiac iron overload, through cardiac magnetic resonance imaging (CMR) has allowed more logical approaches to iron removal, particularly from the heart. The availability of two oral chelators, deferiprone and deferasirox has reduced the need for the injectable chelator deferrioxamine and an additional benefit has been that deferiprone has been shown to be more cardioprotective than deferrioxamine. This review on the prevention of cardiac disease makes recommendations on the chelation regime that would be desirable for patients according to their cardiac iron status as determined by CMR determined by CMR. It also discusses approaches to chelation management should CMR not be available.

BACKGROUND: beta-Thalassemia is an inherited hemoglobin disorder characterized by reduced synthesis of beta-globin chain. The severity of clinical course distinguishes this heterogeneous disease in two main subtypes: thalassemia major (TM) and thalassemia intermedia (TI). TI has a later clinical onset with a milder anemia that does not require transfusions at least during the first few years of life. The clinical picture of TI patients who have not received transfusions or have occasionally received transfusions is dominated by the consequences of chronic hemolytic anemia, tissue hypoxia, and their compensatory reactions, such as bone deformities and fractures, extramedullary hemopoiesis, spleen and liver enlargement, hypercoagulability, and pulmonary hypertension. These complications, especially the latter two, are getting more frequent and severe over the years. Nowadays, although TI patients have almost no changes in the course of the disease, well-treated TM patients with regular transfusion-chelation therapy showed suppression of the anemia-related disorders in parallel to prolongation of life. The new oral iron chelators and the magnetic resonance imaging application for early detection of heart iron load are promising for further improvement on survival. CONCLUSIONS: Considering the current cost-benefit balance of regular treatment in TM as well as the frequency and severity of complications in TI, it seems that the majority of TI patients will be benefited if this kind of treatment is applied targeting prevention and not palliation of the anemia-induced complications.

Objectives: Brucellosis, whether in an endemic region or not, remains a diagnostic puzzle due to occasional misleading unusual presentations and non-specific symptoms. Presented herein is our 14-year experience with acute brucellosis at Sparta General Hospital, Lakonia, Greece. Methods: A case series of 144 patients admitted to the internal medicine, pediatrics, and urology departments, through evaluation of history, occupational data, serological. tests, cultures of blood and other body fluids, and imaging studies. Patients were treated with a 21-day course of intramuscular streptomycin and a prolonged two-month course of doxycycline with a six-month follow-up. Results: Infected patients had a relevant occupational history in fewer than 20% of cases. Clinical manifestations included non-specific symptoms (fever, malaise, sweats, arthralgias, lower back pain, headache), findings such as splenomegaly (51%), osteoarticular involvement (42%), cervical lymphadenitis (31%), hepatomegaly (25%), genitourinary involvement (13% of men), chotecystitis (2%), breast abscess (0.7%), and acute abdomen (0.7%). Ninety-five percent of the patients had a serotogical titer >= 1/160 with culture-proven brucellosis. Overall, 82% of blood cultures and 100% of other body fluid cultures (synovial, bite) were positive. Ninety-seven percent of the patients were cured. Relapse in the follow-up period was observed in four patients who had not complied with treatment. Conclusions: Brucellosis is an infection with multiple presentations, and whether in an endemic region or not, a thorough history of exposure and clinical. suspicion are required since thresholds in serological evaluation may lead to misdiagnosis and withholding of adequate treatment. (c) 2006 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Early detection of cardiac-function impairment by echo-Doppler indices can assist in preventing further cardiac damage by modifying disease progression and treatment. We analyzed our thalassemia major patients database with 10 years cardiac follow-up. Included patients were under constant therapy and should have an initial echo-Doppler study with normal Shortening Fraction ( SF > 30%) and reexamination within the last year. We identified patients who developed impaired left ventricular (LV) function in the last Echo and we attempted to find which measured indices could predict LV function impairment. Three hundred fifteen of the 632 database patients were enrolled. Twelve of them developed LV systolic dysfunction. There were no statistically significant differences in mean age, ferritin, and pretransfusion hemoglobin levels of the two groups. LV-systolic-dysfunction group was presenting statistically significantly higher LF end-systolic diameter (LVESD) index, lower SF, higher early transmitral peak flow velocities/late transmitral peak flow velocities ( A) ratios, lower A value. All other echocardiographic parameters did not differ significantly. By receiver-operating characteristic analysis, we determined systolic and diastolic indices specificity and sensitivity for LV impairment: LVESD 97% specificity, 11% sensitivity ( cutoff value 2.44 cm/m(2)), SF 92.1 and 33.3% ( cutoff value 33%). Regarding diastolic indices, A index was the best criterion ( 97.7% specificity, 25% sensitivity, cutoff value

Background. Thalassemia intermedia has a later clinical onset and a milder anemia than thalassemia major, characterized by high output state, left ventricle remodeling, and age-related pulmonary hypertension. Bone deformities, extramedullary hematopoiesis (EMH), and spleen and liver enlargement are the consequences of hypoxia and enhanced erythropoiesis. The EMH-related pleural effusion is rarely referred to in the literature of thalassemia. Methods. We reviewed the thalassemia patients’ medical records hospitalized for pleural effusion in our Department, within the last 6 years. Results. Eight ( 4 men) thalassemia intermedia patients admitted for symptomatic pleural effusion were identified. Common clinical findings on admission were dyspnea and apyrexia. Their mean hemoglobin level was 7.15 +/- 0.64 g/dL. Radiology revealed intrathoracic EMH and pleural effusion in all patients: exudative in seven patients and massive hemothorax in one. Cytologic fluid analysis was negative for malignancy. Fluid and serum cultures, antibodies, and stains were negative for viral, bacterial, and fungal infection. The hemothorax case was successfully treated with repeated aspirations, transfusions, and hydroxyurea. Although repeated thoracentesis and radiation could not control the effusions in the rest of the cases, pleurodesis was successful in 5 patients, without serious adverse events. Treatment was further accomplished with hydroxyurea. No relapses were observed in the mean 30 month follow-up period. Conclusions. Afebrile, EMH-related pleuritis represents a potentially life-threatening complication in thalassemia. Therapy should be individualized and treatment is emerging. Pleurodesis seems to be an effective and safe therapeutic option for exudative effusions, while transfusion-chelation therapy combined with hydroxyurea may be helpful in suppressing increased erythropoiesis.