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Showing papers by "Alberto Roghi published in 2014"


Journal ArticleDOI
TL;DR: The availability of three iron chelators improved the scenario of chelation therapy for transfusion-dependent thalassemia (TDT) patients, allowing tailoring of drugs according to the goals expected for each patient, and the efficacy, tolerability and safety of combined DFX/DFO was investigated.
Abstract: The availability of three iron chelators improved the scenario of chelation therapy for transfusion-dependent thalassemia (TDT) patients, allowing tailoring of drugs according to the goals expected for each patient. The use of Deferiprone/Deferoxamine (DFP/DFO) combined in different schemes has been reported since many years. Only recently data from combination of Deferasirox/Deferoxamine (DFX/DFO) have been reported showing that it can be safe and efficacious to remove iron overload, particularly in patients who do not respond adequately to a single chelating agent. We investigated the efficacy, tolerability and safety of combined DFX/DFO in thalassemia major patients. Ten TDT patients have started DFX/DFO for different reasons: 1) lack of efficacy in removing liver/cardiac iron with monotherapy; 2) agranulocytosis on DFP; and 3) adverse events with elevated doses of monotherapies. The study design included: cardiac and hepatic T2* magnetic resonance (CMR), transient elastography evaluation (Fibroscan), biochemical evaluation, and audiometric and ocular examinations. The drugs' starting doses were: DFO 32 ± 4 mg/kg/day for 3-4 days a week and DFX 20 ± 2 mg/kg/day. Seven patients completed the one-year follow-up period. At baseline the mean pre-transfusional Hb level was 9.4 ± 0.4 g/dl, the mean iron intake was 0.40 ± 0.10mg/kg/day, the median ferritin level was 2254 ng/ml (range 644-17,681 ng/ml). Data available at 1 year showed no alteration of renal/hepatic function and no adverse events. A marked reduction in LIC (6.54 vs 11.44 mg/g dw at baseline) and in median ferritin (1346 vs 2254 ng/ml at baseline) was achieved. A concomitant reduction of non-transferrin-bound iron (NTBI) at six months was observed (2.1 ± 1.0 vs 1.7 ± 1.2 μM). An improvement in cardiac T2* values was detected (26.34 ± 15.85 vs 19.85 ± 12.06 at baseline). At 1 year an increased dose of DFX was administered (27 ± 6 mg/kg/day vs 20 ± 2 mg/kg/day at baseline, p=0.01) with a stable dose of DFO (32 ± 4 mg/kg/day). Combined or alternated DFX/DFO can be considered when monotherapy is not able to remove the iron overload or in the presence of adverse events.

41 citations



Journal ArticleDOI
TL;DR: Echocardiography remains the first-line imaging technique, but an integrated multimodality evaluation with clinical, biochemical and hemodynamic parameters, and cardiovascular magnetic resonance imaging can provide a more comprehensive way to choose the most appropriate treatment for patients with heart failure associated with right ventricular dysfunction.
Abstract: The role of the right ventricle has often been underestimated in heart failure. It has been thought that the right cavity has a less prominent impact on symptoms, therapeutic approach, and prognosis. Right ventricular dysfunction is a complex issue and its diagnosis has acquired a relevant role, in particular with the improvement of new therapeutic options such as ventricular assist devices. The complex geometry of the right ventricle and its interaction with the left ventricle are still a matter of debate, leaving several open questions about the best therapeutic approach to manage right ventricular dysfunction. Echocardiography remains the first-line imaging technique, but an integrated multimodality evaluation with clinical, biochemical and hemodynamic parameters, and cardiovascular magnetic resonance imaging can provide a more comprehensive way to choose the most appropriate treatment for patients with heart failure associated with right ventricular dysfunction.

3 citations