Search results for "Deferiprone"

showing 10 items of 20 documents

Cardiac and hepatic iron and ejection fraction in thalassemia major: Multicentre prospective comparison of combined Deferiprone and Deferoxamine ther…

2013

Background: Due to the limited data available in literature, the aim of this multi-centre study was to prospectively compare in thalassemia major (TM) patients the efficacy of combined deferiprone (DFP) and deferoxamine (DFO) regimen versus either DFP and DFO in monotherapy by cardiovascular magnetic resonance (CMR) over a follow up of 18 months. Methods: Among the first 1135 TM patients in the MIOT (Myocardial Iron Overload in Thalassemia) network, we evaluated those who had received either combined regimen (DFO + DFP, N=51) or DFP (N=39) and DFO (N=74) monotherapies between the two CMR scans. Iron overload was measured by T2* multiecho technique. Biventricular function parameters were qua…

MaleLiver Iron ConcentrationTime FactorsThalassemiaVentricular Function Leftchemistry.chemical_compoundMedicineDeferiproneProspective StudiesMedicine(all)Ejection fractionRadiological and Ultrasound TechnologyBeta thalassemiaDeferoxamineTreatment OutcomeItalyLiverCardiologyThalassemiaDrug Therapy CombinationFemaleCardiology and Cardiovascular MedicineDeferiproneCardiomyopathiesmedicine.drugAdultmedicine.medical_specialtyCombination therapyPyridonesChelation therapyMagnetic Resonance Imaging CineDeferoxamineIron Chelating AgentsYoung AdultPredictive Value of TestsInternal medicineHumansRadiology Nuclear Medicine and imagingChelation therapyAnalysis of VarianceChi-Square Distributionbusiness.industryResearchMyocardiumbeta-ThalassemiaStroke Volumemedicine.diseaseSurgerychemistryVentricular Function RightCardiovascular magnetic resonancebusinessJournal of Cardiovascular Magnetic Resonance
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Improving survival with deferiprone treatment in patients with thalassemia major: A prospective multicenter randomised clinical trial under the auspi…

2009

The prognosis for thalassemia major has dramatically improved in the last two decades. However, many transfusion-dependent patients continue to develop progressive accumulation of iron. This can lead to tissue damage and eventually death, particularly from cardiac disease. Previous studies that investigated iron chelation treatments, including retrospective and prospective non-randomised clinical trials, suggested that mortality, due mainly to cardiac damage, was reduced or completely absent in patients treated with deferiprone (DFP) alone or a combined deferiprone-deferoxamine (DFP-DFO) chelation treatment. However, no survival analysis has been reported for a long-term randomised control …

MaleThalassemiaKaplan-Meier Estimatelaw.inventionchemistry.chemical_compoundRandomized controlled triallawCause of DeathNeoplasmsDeferiproneProspective StudiesChildCause of deathHazard ratioHematologyMiddle AgedCombined Modality TherapySurvival RateThalassemia survival chelation treatment trial thalassemia majorCombinationSplenectomyMolecular MedicineDrug Therapy CombinationFemaleDeferiproneAdultmedicine.medical_specialtyAdolescentPyridonesDeferoxamineIron Chelating AgentsYoung AdultDrug TherapyInternal medicinemedicineHumansBlood TransfusionAdolescent; Adult; Blood Transfusion; Cause of Death; Chelation Therapy; Child; Combined Modality Therapy; Deferoxamine; Drug Therapy; Combination; Female; Heart Failure; Humans; Iron Chelating Agents; Kaplan-Meiers Estimate; Male; Middle Aged; Neoplasms; Proportional Hazards Models; Prospective Studies; Pyridones; Splenectomy; Survival Rate; Young Adult; beta-ThalassemiaMolecular BiologySurvival rateKaplan-Meiers EstimateSurvival analysisProportional Hazards ModelsHeart Failurebusiness.industryProportional hazards modelbeta-ThalassemiaCell Biologymedicine.diseaseChelation TherapySurgerychemistrybusiness
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Potential Myocardial Iron Content Evaluation by Magnetic Resonance Imaging in Thalassemia Major Patients Treated with Deferoxamine or Deferiprone Dur…

2003

The purpose of this study was to evaluate if the variations of heart magnetic resonance imaging in beta-thalassemia major patients treated with Deferoxamine B mesylate (DF) or Deferiprone (L1) chelation therapy is a useful tool of the indirect myocardial iron content determination. For this reason, a prospective study was carried out. Seventy-two consecutive patients with beta-thalassemia major (35 treated with DF and 37 with L1) were studied. The main outcome results were laboratory parameters including determination of the liver iron concentration (LIC) and magnetic resonance imaging (MRI) of the heart and liver. The heart to muscle signal intensity ratios (HSIRs) were significantly incre…

Malemedicine.medical_specialtyLiver Iron ConcentrationPyridonesIronThalassemiaClinical BiochemistryAdministration OralDeferoxamineIron Chelating AgentsGastroenterologychemistry.chemical_compoundInternal medicinemedicineHumansDeferiproneInfusions ParenteralChelation therapyProspective cohort studyGenetics (clinical)medicine.diagnostic_testbusiness.industryMyocardiumbeta-ThalassemiaBiochemistry (medical)Beta thalassemiaMagnetic resonance imagingHematologymedicine.diseaseMagnetic Resonance ImagingDeferoxaminechemistryHeart Function TestsPatient ComplianceFemalebusinessDeferipronemedicine.drugHemoglobin
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Prospective Comparison on Cardiac Iron and Liver Iron by MR In Thalassemia Major Patients Treated with Combination Deferipron–Desferrioxamine Versus …

2010

Abstract Abstract 5164 Introduction: Using T2* MR a randomised placebo controlled study from Sardinia demonstrated combination therapy with deferiprone and desferrioxamine (DFP+DFO) significantly more effective than DFO in improving myocardial iron. One non-randomised study from Sardinia and one observational study from Greece seem to confirm for DFP+DFO therapy the most rapid clearance of cardiac iron. No data are available in literature about prospective comparisons on cardiac iron and function and liver iron in TM patients treated with DFP+DFO versus DFP and DFO in monotherapy. The aim of this multi-centre study was to assess prospectively in a large clinical setting the efficacy of the …

medicine.medical_specialtyCombination therapybusiness.industryThalassemiaImmunologyUrologyPlacebo-controlled studyCell BiologyHematologymedicine.diseaseBiochemistryDeferipronSurgeryBiventricular functionchemistry.chemical_compoundchemistrymedicineCardiac ironLiver ironDeferipronebusiness
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Long-Term Use of Deferiprone Enhances Significantly the Left Ventricular Ejection Function in Thalassemia Major

2011

Abstract Abstract 5302 Background: A multicentre randomized controlled trial (RCT) was designed to assess the effectiveness of long-term sequential deferiprone-deferoxamine (DFO-DFP) versus DFP alone to treat thalassaemia major (TM) (Maggio et al.,2009). Effectviness, survival, adverse events and costs were comparable between the groups. These findings were confirmed in a further 21-month follow-up (Pantalone et al., 2011). Moreover, deferiprone-alone has been reported to be superior to deferoxamine for the removal of cardiac iron and improvement in left ventricular ejection function (LVEF). However, little is known of its relative effect on LVEF after long-term treatment. Therefore, data f…

medicine.medical_specialtyEjection fractionbusiness.industryThalassemiaImmunologyDeferasiroxRetrospective cohort studyCell BiologyHematologymedicine.diseaseBiochemistrylaw.inventionchemistry.chemical_compoundchemistryRandomized controlled triallawHeart failureInternal medicinemedicineCardiologybusinessDeferiproneSurvival analysismedicine.drugBlood
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Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative…

2010

Background Oral deferiprone was suggested to be more effective than subcutaneous desferrioxamine for removing heart iron. Oral once-daily chelator deferasirox has recently been made commercially available but its long-term efficacy on cardiac iron and function has not yet been established. Our study aimed to compare the effectiveness of deferasirox, deferiprone and desferrioxamine on myocardial and liver iron concentrations and bi-ventricular function in thalassemia major patients by means of quantitative magnetic resonance imaging. Design and Methods From the first 550 thalassemia subjects enrolled in the Myocardial Iron Overload in Thalassemia network, we retrospectively selected thalasse…

medicine.medical_specialtyLiver Iron ConcentrationthalassemiaThalassemiairon chelation therapycardiac magnetic resonance imagingGastroenterologythalassemia; iron chelation therapy; cardiac magnetic resonance imagingNOchemistry.chemical_compoundCardiac magnetic resonance imagingInternal medicinemedicinemedicine.diagnostic_testbusiness.industryDeferasiroxBeta thalassemiaMagnetic resonance imagingHematologymedicine.diseaseSurgeryDeferoxaminechemistrybusinessDeferipronemedicine.drug
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Cardiac complications in thalassemia: noninvasive detection methods and new directions in the clinical management.

2004

The natural history of thalassemia has shown substantial change during these years. This applies for each aspect of the pathology (for example, endocrinological, hepatological and psychological) and also for the pathology that has presented and still presents the main cause of death: myocardial dysfunction. In this review, the pathophysiology of cardiac complications, possible role of myocarditis, new knowledge on pathogenesis, and noninvasive detection methods for iron overload in the heart are pointed out. Prophylaxis of cardiomyopathy and new therapy strategies of myocardial dysfunction, including the impact of the new chelation treatment, are discussed.

medicine.medical_specialtyMyocarditisThalassemiaCardiomyopathychemistry.chemical_compoundInternal medicineInternal MedicineMedicineHumansCause of deathbusiness.industryGeneral Medicinemedicine.diseaseMagnetic Resonance ImagingPathophysiologyNatural historychemistryEchocardiographyCardiologyThalassemiaCardiac complications in thalassemiaCardiology and Cardiovascular MedicinebusinessDeferiproneCardiomyopathiesHeart damage
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Chelation treatment in sickle-cell-anaemia: much ado about nothing?

2011

Summary Blood transfusions may prevent and treat serious complications related to sickle-cell disease (SCD) when performed according to specific guidelines. However, blood transfusion requirements in SCD inevitably lead to increased body iron burden. An adequate chelation treatment may prevent complications and reduce morbidity and mortality. This review evaluates the effectiveness, safety and costs of chelation treatment. The included trials were examined according to the recommendations of the American College of Cardiology (ACC) and the American Heart Association (AHA). Overall, 14 trials and a total of 502 patients with SCD were included in this review. Deferoxamine alone (s.c. or i.v.)…

medicine.medical_specialtyPediatricsBlood transfusionDrug-Related Side Effects and Adverse Reactionsmedicine.medical_treatmentAnemia Sickle CellDiseaseIron Chelating Agentslaw.inventionBody ironchemistry.chemical_compoundRandomized controlled triallawmedicineHumansChelationIntensive care medicinebusiness.industryDeferasiroxHematologyChelation TherapyDeferoxamineTreatment OutcomechemistryCosts and Cost AnalysisDeferipronebusinessChelation treatment sickle-cell-diseasemedicine.drug
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Iron Chelation Therapy in thalassaemia major: a sistematic review with meta-analyses of 1520 patients included on randomized clinical trials

2011

The effectiveness of deferoxamine (DFO), deferiprone (DFP), or deferasirox (DFX) in thalassemia major was assessed. Outcomes were reported as means±SD, mean differences with 95% CI, or standardized mean differences. Statistical heterogeneity was tested using χ2 (Q) and I2. Sources of bias and Grading of Recommendations Assessment, Development and Evaluation system (GRADE) were considered. Overall, 1520 patients were included. Only 7.4% of trials were free of bias. Overall measurements suggest low trial quality (GRADE). The meta-analysis suggests lower final liver iron concentrations during associated versus monotherapy treatment (p<0.0001), increases in serum ferritin levels during DFX 5, 1…

medicine.medical_specialtyPyridonesIronMEDLINEThalassemiaSiderophoresDeferoxamineIron Chelating AgentsChelation treatment thalassaemia clinical trials iron overload meta-analysisBenzoatesGastroenterologylaw.inventionchemistry.chemical_compoundRandomized controlled triallawInternal medicinemedicineHumansVentricular FunctionDeferiproneMolecular BiologyRandomized Controlled Trials as TopicEjection fractionbusiness.industryMyocardiumbeta-ThalassemiaDeferasiroxBeta thalassemiaCell BiologyHematologyTriazolesmedicine.diseaseChelation TherapySurgeryDeferoxamineDeferasiroxTreatment OutcomeLiverchemistryMeta-analysisFerritinsMolecular MedicineDrug Therapy CombinationbusinessDeferipronemedicine.drug
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A T2* MRI Prospective Survey on Heart and Liver Iron In Thalassemia Major Patients Treated with Deferasirox Versus Deferiprone and Desferrioxamine In…

2010

Abstract Abstract 4267 Introduction: Most deaths in thalassemia major (TM) result from cardiac complications due to iron overload. In thalassaemia available three iron chelation regimes in monotherapy may achieve different changes in cardiac iron and function and liver iron. No data are available in literature about prospective comparisons on cardiac iron and function and liver iron in TM patients treated with deferasirox (DFX) versus deferiprone (DFP) and desferrioxamine (DFO) in monotherapy. Magnetic Resonance (MR) is the unique non invasive suitable technique to evaluated quantitatively this issue. The aim of this multi-centre study was to assess prospectively in the clinical practice th…

medicine.medical_specialtymedicine.diagnostic_testbusiness.industryThalassemiaImmunologyDeferasiroxMagnetic resonance imagingCell BiologyHematologymedicine.diseaseBiochemistryGastroenterologySurgerychemistry.chemical_compoundchemistryInternal medicineMedicineLiver ironMultisliceSiderosisbusinessDeferiproneProspective surveymedicine.drug
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