Search results for "Beta-thalassemia"

showing 10 items of 52 documents

Quantitative evaluation of oxidative stress status on peripheral blood in beta-thalassaemic patients by means of electron paramagnetic resonance spec…

2005

Summary High oxidative stress status (OSS) is known to be one of the most important factors determining cell injury and consequent organ damage in thalassaemic patients with secondary iron overload. Using an innovative hydroxylamine ‘radical probe’ capable of efficiently trapping majority of oxygen-radicals including superoxide we measured, by electron paramagnetic resonance (EPR) spectroscopy, OSS in peripheral blood of 38 thalassaemic patients compared with sex-/age-matched healthy controls. Thalassaemic patients showed sixfold higher EPR values of OSS than controls. Significantly higher EPR values of OSS were observed in those with a severe phenotype (thalassaemia major, transfusion-depe…

AdultMaleHemolytic anemiamedicine.medical_specialtyIron Overloadmedicine.disease_causelaw.inventionchemistry.chemical_compoundHydroxylaminelawInternal medicineOXIDATIVE STRESS STATUSmedicineHumansB-THALASSAEMIC PATIENTSElectron paramagnetic resonanceChelating AgentsAnalysis of VarianceHematologySuperoxidebeta-ThalassemiaElectron Spin Resonance SpectroscopyHYDROXYLAMINEHematologyCHELATING THERAPYmedicine.diseaseOxidative StressELECTRON PARAMAGNETIC RESONANCEEndocrinologyHemoglobinopathychemistryCase-Control StudiesImmunologyFemaleDeferiproneOxidative stressBritish Journal of Haematology
researchProduct

Serum and fecal pancreatic enzymes in beta-thalassemia major

1997

This study, using indirect tests, demonstrated that exocrine pancreatic function is impaired in a proportion of patients with beta-thalassemia major (TM), though this impairment is generally mild or moderate.Impaired structure and function of the exocrine pancreas has been reported in patients with Beta-thalassemia major.In this study we measured fecal fats and serum and fecal pancreatic enzymes in 30 patients (13 M, 17 F) with TM, mean age 22.1 yr (range 14-39) and compared them with those of a matched group of healthy controls. Results were correlated with age, serum ferritin, blood transfusion, and various nutritional parameters. Enzymes assays included: serum pancreatic amylase (PA), li…

AdultMaleHemolytic anemiamedicine.medical_specialtyPancreatic diseaseAdolescentHydrolasesGastroenterologyFatsPathogenesisFecesEndocrinologyInternal medicinemedicineChymotrypsinHumansTrypsinFeceschemistry.chemical_classificationPancreatic Elastasebusiness.industrybeta-ThalassemiaGastroenterologyBeta thalassemiaLipasemedicine.diseaseHemoglobinopathyEnzymeEndocrinologyOncologychemistryAmylasesExocrine Pancreatic InsufficiencyFemalebusinessPancreatic enzymesInternational Journal of Pancreatology
researchProduct

MR imaging technique for the diagnosis of pituitary iron overload in patients with transfusion-dependent beta-thalassemia major

1998

To identify the optimal MR imaging technique for diagnosing pituitary iron overload, we compared spin-echo and gradient-echo MR imaging with measurements of pituitary T2 relaxation times in 30 patients with secondary hemochromatosis due to transfusion-dependent beta-thalassemia major and in 10 healthy volunteers. We found that the optimal MR imaging technique to evaluate pituitary iron overload is the gradient-echo T2*-weighted technique, as it best demonstrated signal reduction in the anterior lobe of the pituitary gland.

AdultMaleIron OverloadAdolescentPituitary Glandbeta-ThalassemiaJournal ArticleHumansTransfusion ReactionBlood TransfusionFemaleMagnetic Resonance ImagingHuman
researchProduct

Improved T2* assessment in liver iron overload by magnetic resonance imaging.

2009

In the clinical MRI practice, it is common to assess liver iron overload by T2* multi-echo gradient-echo images. However, there is no full consensus about the best image analysis approach for the T2* measurements. The currently used methods involve manual drawing of a region of interest (ROI) within MR images of the liver. Evaluation of a representative liver T2* value is done by fitting an appropriate model to the signal decay within the ROIs vs. the echo time. The resulting T2* value may depend on both ROI placement and choice of the signal decay model. The aim of this study was to understand how the choice of the analysis methodology may affect the accuracy of T2* measurements. A softwar…

AdultMaleIron OverloadBiomedical EngineeringBiophysicsImage processingSignalSoftwareRegion of interestImage Processing Computer-AssistedMedicineLiver ironHumansRadiology Nuclear Medicine and imagingliver iron overloadObserver VariationReproducibilitymedicine.diagnostic_testbusiness.industrybeta-ThalassemiaReproducibility of ResultsPattern recognitionMagnetic resonance imagingMagnetic Resonance ImagingLiverData Interpretation StatisticalAutomatic segmentationFemaleArtificial intelligencebusinessNuclear medicineAlgorithmsSoftwareMagnetic resonance imaging
researchProduct

Evaluation of the efficacy of oral deferiprone in beta-thalassemia major by multislice multiecho T2*.

2006

Objectives: Oral deferiprone (L1) appears to be promising in the treatment of beta-thalassemia major (TM) patients. T2* magnetic resonance imaging (MRI) with a single measurement in the mid-ventricular septum was validated as a quantitative evaluation of myocardial iron overload. Previous studies suggested a marked heterogeneity of iron distribution in the myocardium. We set up a multislice multiecho T2* MRI for the detection of this heterogeneity. The aim of our study was to investigate differences between the L1 vs. the subcutaneous desferrioxamine (DF)-treated patients using this new approach.Methods: Thirty-six beta-TM patients (age 29 +/- 8 yr) underwent MRI. Eighteen patients received…

AdultMaleIron OverloadPyridonesCoefficient of variationDeferoxamineBETA THALASSEMIA MAJORchemistry.chemical_compoundMagneticsmultislice multiecho T2*MedicineHumansMultisliceDeferiproneReproducibilitymedicine.diagnostic_testbusiness.industryMyocardiumbeta-ThalassemiaBeta thalassemiaMagnetic resonance imagingHematologyGeneral MedicineMiddle Agedmedicine.diseaseMagnetic Resonance Imagingchelation treatmentmedicine.anatomical_structurechemistryVentricleFemalebusinessNuclear medicineDeferiproneEuropean journal of haematology
researchProduct

Multislice multiecho T2* cardiovascular magnetic resonance for detection of the heterogeneous distribution of myocardial iron overload.

2006

Purpose To assess the tissue iron concentration of the left ventricle (LV) using a multislice, multiecho T2* MR technique and a segmental analysis. Materials and Methods T2* multiecho MRI was performed in 53 thalassemia major patients. Three short-axis views of the LV were obtained and analyzed with custom-written software. The myocardium was automatically segmented into 12 segments. The T2* value on each segment as well as the global T2* value were calculated. Cine dynamic images were also obtained to evaluate biventricular function parameters by quantitative analysis. Results For the T2* global value, the coefficient of variation (CoV) for intra-/interobserver and interstudy reproducibili…

AdultMaleLiver Iron ConcentrationthalassemiaAdolescentHeart VentriclesIronCoefficient of variationCardiomyopathyMagnetic Resonance Imaging CineImage Processing Computer-AssistedHumansMedicineDistribution (pharmacology)magnetic resonance imagingRadiology Nuclear Medicine and imagingMultisliceiron overloadChildObserver VariationReproducibilitymedicine.diagnostic_testbusiness.industryMyocardiumbeta-ThalassemiaReproducibility of Resultsrelaxation time (T2*)Magnetic resonance imagingMiddle Agedmedicine.diseasemedicine.anatomical_structureLiverVentricleFerritinsFemalebusinessNuclear medicinecardiomyopathy
researchProduct

Analysis of 55 cases of adenomatoid odontogenic tumor in an Indian population and review of literature

2021

Background This study reviews the demographic, clinical and radiographic features of adenomatoid odontogenic tumor(AOT) diagnosed in an Indian population over 50 years and also evaluate and compare follicular AOT(F-AOT) and extra-follicular AOT(EF-AOT). Material and Methods 55 diagnosed cases of AOT from 1971-2020 were studied retrospectively. The data regarding the age, sex, location, variant of AOT, duration, clinical features, radiographic appearance, treatment and recurrence were collected and analysed. Results Of the 722 odontogenic tumors diagnosed, 7.6% were AOTs with higher prevalence of extra-follicular (67.3%) than follicular (32.7%) variant. All the tumors were intraosseous with …

AdultMaleOral Medicine and PathologyAdolescentbeta-thalassemia majorResearchTooth ImpactedIndiaOdontogenic TumorsAmeloblastomaYoung AdultOtorhinolaryngologyfractalspanoramic radiographyHumansFemaleSurgeryChildGeneral DentistryUNESCO:CIENCIAS MÉDICASRetrospective Studies
researchProduct

MR imaging of pancreatic changes in patients with transfusion-dependent beta-thalassemia major.

1999

The aim of this study was to evaluate MR imaging changes of the pancreas in patients with transfusion-dependent beta-thalassemia major.Twenty patients with transfusion-dependent beta-thalassemia major were examined using MR imaging at 0.5 T, with spin-echo T1-weighted, fast spin-echo T2-weighted, and gradient-echo T2*-weighted sequences. Image analysis was performed to assess pancreas-to-fat signal intensity ratios for all pulse sequences. Pancreatic exocrine and endocrine function and serum ferritin levels were assessed. Twenty healthy volunteers underwent MR imaging with the same three sequences and served as a control group.The pancreas-to-fat signal intensity ratio was significantly dec…

AdultMalePancreatic diseaseAdolescentFecesmedicineChymotrypsinHumansEndocrine systemBlood TransfusionTrypsinRadiology Nuclear Medicine and imagingPancreasPancreatic Elastasemedicine.diagnostic_testbiologybusiness.industryPulse (signal processing)beta-ThalassemiaCase-control studyMagnetic resonance imagingLipaseGeneral Medicinemedicine.diseaseMagnetic Resonance ImagingFerritinPancreatic Function TestsHemoglobinopathymedicine.anatomical_structureAmylasesFerritinsbiology.proteinFemalebusinessNuclear medicinePancreas
researchProduct

Transfusional Hemochromatosis: Quantitative Relation of MR Imaging Pituitary Signal Intensity Reduction to Hypogonadotropic Hypogonadism

2000

To assess the relationship between magnetic resonance (MR) imaging pituitary signal intensity reduction in patients with transfusional hemochromatosis and the clinical manifestation of hypogonadotropic hypogonadism.Pituitary MR imaging at 0.5 T was performed in 38 consecutive patients affected by secondary hemochromatosis and in 20 healthy volunteers. Serum ferritin levels were estimated in the affected population. Twenty (53%) of the 38 patients had hypogonadotropic hypogonadism diagnosed. Pituitary-to-fat signal intensity ratios were calculated from coronal gradient-echo (GRE) T2*-weighted MR images. The relationship between the quantitative reduction of the pituitary-to-fat signal intens…

AdultMalePathologymedicine.medical_specialtyPituitary glandAdolescentHemochromatosiPopulationSensitivity and SpecificityPituitary Gland AnteriorHypogonadotropic hypogonadismmedicineHumansBlood TransfusionRadiology Nuclear Medicine and imagingChildeducationHemochromatosisFerritineducation.field_of_studymedicine.diagnostic_testbusiness.industryHypogonadismbeta-ThalassemiaTransfusion ReactionBeta thalassemiaMagnetic resonance imagingmedicine.diseaseMagnetic Resonance Imagingmedicine.anatomical_structureROC CurveCoronal planeFerritinsFemaleHemochromatosisbusinessNuclear medicineHumanHormoneRadiology
researchProduct

Risk of mortality from anemia and iron overload in nontransfusion-dependent β-thalassemia

2021

AdultMaleRiskPediatricsmedicine.medical_specialtyIron OverloadAnemiabusiness.industryThalassemiabeta-ThalassemiaAnemiaHematologyKaplan-Meier Estimatemedicine.diseaseYoung AdultTransfusion dependencemedicineRisk of mortalityHumansBlood TransfusionFemaleMortalitybusinessHuman
researchProduct