Search results for " thalassemia"

showing 4 items of 34 documents

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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Raman Spectroscopy Technology to Monitor the Carotenoids in Skin of Thalassemia Patients: A Novel Non-Invasive Tool Relating Oxidative Stress with Ir…

2014

In this work we approach the relationship between redox state and iron overload by noninvasive instrumental techniques. Intracardiac, liver iron and liver fibrosis have been monitored in transfusion-dependent thalassemia patients by magnetic resonance imaging and hepatic transient elastography examinations. These measurements have been matched with a non-invasive, and yet unexplored in clinical practice, evaluation of body’s oxidative stress through measurement of antioxidant carotenoids in skin, by a spectroscopic method based on Raman technology (RRS). The global body’s antioxidant status results from a balance between the level of antioxidants in cells and body fluids, including blood, a…

medicine.medical_specialtyPathologyAntioxidantmedicine.medical_treatmentThalassemiathalassemia raman spectroscopy body antioxidant statusManagement of thalassemiamedicine.disease_causeGastroenterologySettore MED/15 - Malattie Del SangueSettore BIO/10 - BiochimicaInternal medicinemedicineDiseases of the blood and blood-forming organsAdverse effectRaman spectroscopy technology skin carotenoids thalassemia oxidative stress.Carotenoidchemistry.chemical_classificationChemistryfood and beveragesRaman spectroscopy technology; skin carotenoids; thalassemia; oxidative stressmedicine.diseaseBiomarker (medicine)RC633-647.5Transient elastographyOxidative stressThalassemia Reports; Volume 4; Issue 2; Pages: 1967
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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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Hematopoietic Peripheral Circulating Blood Stem Cells As an Independent Marker of Good Transfusion Management in Patients with Beta-Thalassemia

2015

Abstract Aim Aim of the current study was to prospectively evaluate the potential role of peripheral circulating CD34+ stem cells as new independent marker of appropriate hemopoietic balance in patients with thalassemia major and intermedia. Materials and methods Peripheral blood samples from patients with thalassemia major (TM) and intermedia (TI) were drawn. Peripheral circulating CD34+ stem cells, CF-GEMM, CFU-GM and BFU-GM were assayed with monoclonal antibodies for CD34 and clonogenic tests, according to standard procedures and ISHAGE method (BD stem cell enumeration kit, Becton Dickinson; H4434, Stem Cell Technology). Demographic and clinical data were recorded from each enrolled subj…

medicine.medical_specialtybusiness.industryThalassemiamedicine.medical_treatmentImmunologySplenectomyCD34Becton dickinsonBeta thalassemiaCell BiologyHematologymedicine.diseaseBiochemistryGastroenterologyHaematopoiesisInternal medicineCirculating Hematopoietic stem cells beta-thalassemiatrasfusionmedicineStem cellClonogenic assaybusinessBlood
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