Search results for "Lymph"

showing 10 items of 4590 documents

Cytoprotective effects of the antioxidant phytochemical indicaxanthin in beta-thalassemia red blood cells

2006

Antioxidant phytochemicals are investigated as novel treatments for supportive therapy in beta-thalassemia. The dietary indicaxanthin was assessed for its protective effects on human beta-thalassemic RBCs submitted in vitro to oxidative haemolysis by cumene hydroperoxide. Indicaxanthin at 1.0-10 microM enhanced the resistance to haemolysis dose-dependently. In addition, it prevented lipid and haemoglobin (Hb) oxidation, and retarded vitamin E and GSH depletion. After ex vivo spiking of blood from thalassemia patients with indicaxanthin, the phytochemical was recovered in the soluble cell compartment of the RBCs. A spectrophotometric study showed that indicaxanthin can reduce perferryl-Hb ge…

congenital hereditary and neonatal diseases and abnormalitiesAntioxidantErythrocytesPyridinesmedicine.medical_treatmentindicaxanthinphytochemicalBiochemistryHemolysisAntioxidantschemistry.chemical_compoundHemoglobinsAntioxidants betalainhemic and lymphatic diseasesSettore BIO/10 - BiochimicamedicineBenzene DerivativesHumansVitamin ETraditional medicineDose-Response Relationship DrugChemistrybeta-ThalassemiaBeta thalassemiaGeneral Medicinemedicine.diseaseLipid MetabolismGlutathionehaemoglobinBetaxanthinsPhytochemicalBiochemistryCytoprotectionSpectrophotometryCase-Control StudiesHeminAntioxidants betalains haemoglobin indicaxanthin phytochemicals red blood cellsIndicaxanthinOxidation-Reductionred blood cells
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B and T lymphocytes are affected in lysosomal disorders--an immunoelectron microscopic study.

1991

Circulating lymphocytes of four patients with mucopolysaccharidoses II and IIIA, four patients with juvenile neuronal ceroid-lipofuscinosis, one patient each with glycogenosis type II, infantile neuronal ceroid-lipofuscinosis, and Gaucher disease were classified by immunoelectron microscopy as B or T lymphocytes. Disease-specific lysosomal inclusions as well as non-specific lysosomal organelles, especially Gall bodies were identified in B and T lymphocytes. These non-quantitative studies indicate that both B and T lymphocytes participate in the lysosomal storage process.

congenital hereditary and neonatal diseases and abnormalitiesPathologymedicine.medical_specialtyHistologyImmunoelectron microscopyMucopolysaccharidosisT-LymphocytesCentral nervous systemVacuoleBiologyPathology and Forensic MedicinePhysiology (medical)OrganellemedicineLysosomal storage diseaseHumansMicroscopy ImmunoelectronB-Lymphocytesnutritional and metabolic diseasesT lymphocytemedicine.diseasemedicine.anatomical_structureNeurologyNeuronal ceroid lipofuscinosisNeurology (clinical)Metabolism Inborn ErrorsNeuropathology and applied neurobiology
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Lonoctocog alfa (rVIII-SingleChain) for the treatment of haemophilia A

2017

Introduction: The administration of factor VIII (FVIII) concentrates on-demand or on long-term prophylaxis is the effective and safe standard of care of patients with hemophilia A (HA). Development of neutralizing antibodies against exogenous FVIII and the short half-life of the current available products remain major challenges. There is currently a great interest towards newer FVIII products with the goal of reducing the inhibitor risk and increasing the half-life. Area covered: In this review, the authors describe the efficacy and safety of rVIII-SingleChain (Lonoctocog alfa), the first and only single chain recombinant FVIII (rFVIII) molecule developed for the prevention and treatment o…

congenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtyHaemophiliaStandard of caresingle-chainHaemophilia AClinical BiochemistrySingle chain030204 cardiovascular system & hematologyHaemophiliaHemophilia A03 medical and health sciences0302 clinical medicineLONOCTOCOG ALFAhemic and lymphatic diseasesDrug DiscoverymedicineHumansPharmacologyClinical Trials as TopicFactor VIIIbusiness.industryCoagulantsDrug Discovery3003 Pharmaceutical ScienceRecombinant Proteinmedicine.diseaseRecombinant ProteinsCoagulantbusiness030215 immunologyHalf-LifeHuman
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Pathogen safety of long-term treatments for bleeding disorders: still relevant to current practice

2013

Hemophilia defines a group of hereditary bleeding disorders: hemophilia A (deficiency of Factor VIII, FVIII), hemophilia B (deficiency of FIX), and para-hemophilia (deficiency of FV). These result from mutations in clotting factor genes. As in the large majority of bleeding disorders ([Table 1][1

congenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtyTime FactorsLong term treatmentBlood transfusionmedicine.medical_treatmentTreatment outcomeEditorials and PerspectivesHemophilia AHemorrhagic DisordersHemorrhagic disorderhemic and lymphatic diseasesBlood-Borne PathogensmedicineHumansBlood TransfusionPathogenClotting factorbusiness.industryHematologyRecombinant ProteinsTreatment OutcomeCurrent practiceImmunologybusiness
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Random Forest Analysis: A New Approach for Classification of Beta Thalassemia

2020

In recent years, Thalassemia care providers started classifying patients as transfusion-dependent-Thalassemia (TDT) or non-transfusion-dependent-Thalassemia (NTDT) owing to the established role of transfusion therapy in defining the clinical complication profile, although this classification was also based on expert opinion and is limited by reliance on patients’current transfusion status. Starting from a vast set of variables indicating severity phenotype, through the use of both classification and clustering techniques we want to explore the presence of two (TDT vs NTDT) or more clusters, in order to approaching to a new definition for the classification of Beta-Thalassemia in Thalassemia…

congenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtybusiness.industryThalassemiaBeta thalassemiamedicine.diseaseRandom foresthemic and lymphatic diseasesExpert opinionTransfusion statusmedicineTransfusion therapybusinessSSRN Electronic Journal
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Bleomycin, a selective inhibitor of DNA-dependent DNA polymerase from oncogenic RNA viruses.

1972

Abstract Bleomycin, an antibiotic, inhibits the DNA-dependent DNA polymerase from Rauscher murine leukemia virus. Higher concentrations of BLM ∗ are required to inhibit it's RNA-dependent DNA polymerase. These inhibition effects of the non-competitive type are not altered by preincubation of the DNA with BLM. Under comparable conditions neither the DNA-dependent DNA polymerase activity from E. coli and mouse liver nor the DNA-dependent RNA polymerase activity from mouse lymphoma cells are affected by BLM.

congenital hereditary and neonatal diseases and abnormalitiesTime FactorsLymphomaDNA polymeraseHepatitis B virus DNA polymeraseUracil NucleotidesDNA polymerase IIBiophysicsRNA-dependent RNA polymeraseCytosine NucleotidesTritiumBiochemistryRauscher VirusCell LineBleomycinMiceEscherichia coliAnimalsMolecular BiologyPolymeraseDNA clampAntibiotics Antineoplasticbiologyurogenital systemnutritional and metabolic diseasesCell BiologyDNAMolecular biologyReverse transcriptaseKineticsReal-time polymerase chain reactionLiverDNA Nucleotidyltransferasesbiology.proteinRNABiochemical and biophysical research communications
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ChemInform Abstract: Tuning the Band Gap of PbCrO4Through High-Pressure: Evidence of Wide-to-Narrow Semiconductor Transitions.

2014

Commercial polycrystalline and cleaved platelets from natural PbCrO4 are studied in a diamond anvil cell at ≤ 21 GPa.

congenital hereditary and neonatal diseases and abnormalitiesbusiness.industryBand gapChemistryA diamondmacromolecular substancesGeneral MedicineSemiconductorstomatognathic systemhemic and lymphatic diseasesHigh pressureparasitic diseasesOptoelectronicsCrystallitebusinessChemInform
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Musculoskeletal ultrasound in hemophilia: Results and recommendations from a global survey and consensus meeting

2021

Abstract Introduction For persons with hemophilia, optimization of joint outcomes is an important unmet need. The aim of this initiative was to determine use of ultrasound in evaluating arthropathy in persons with hemophilia, and to move toward consensus among hemophilia care providers regarding the preferred ultrasound protocols for global adaptation. Methods A global survey of hemophilia treatment centers was conducted that focused on understanding how and why ultrasound was being used and endeavored to move toward consensus definitions of both point‐of‐care musculoskeletal ultrasound (POC‐MSKUS) and full diagnostic ultrasound, terminology to describe structures being assessed by ultrasou…

congenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyDiagnostic ultrasoundMusculoskeletal ultrasoundUnmet needsTerminologysurveyshemic and lymphatic diseaseshemophiliaHealth careMedicineImage acquisitionMedical physicsDiseases of the blood and blood-forming organsClinical caremusculoskeletalbusiness.industryOriginal ArticlesHematologyultrasonographyconsensus hemophilia musculoskeletal surveys ultrasonographyconsensusOriginal ArticlePatient representativesRC633-647.5businessResearch and Practice in Thrombosis and Haemostasis
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Increased susceptibility of microcytic red blood cells to in vitro oxidative stress.

1995

Oxidative damage to erythrocytes in thalassaemia has been related to generation of free radicals by an excess of denaturated alpha- or beta-globin chains, intracellular iron overload and low concentration of normal haemoglobin (HGB). Two good indicators of such oxidative damage are the high red blood cell (RBC) malonyldialdehyde (MDA) production detected following exogenous oxidant stress and the decrease of pyrimidine 5'-nucleotidase (P5N), the most sensitive enzyme to SH-group damage in vivo. Conflicting data, however, have so far accumulated in the literature concerning differences in oxidative damage between the different forms of thalassaemia and iron deficiency anaemia (IDA). In the p…

congenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyErythrocytes Abnormalmedicine.disease_causeSuperoxide dismutasechemistry.chemical_compoundhemic and lymphatic diseasesInternal medicineMalondialdehydemedicineHumans5'-Nucleotidasechemistry.chemical_classificationAnemia HypochromicGlutathione PeroxidasebiologySuperoxide DismutaseGlutathione peroxidaseMicrocytosisHematologyGeneral MedicineGlutathioneIron deficiencymedicine.diseaseGlutathioneRed blood cellOxidative StressEndocrinologymedicine.anatomical_structurechemistryIron-deficiency anemiaImmunologybiology.proteinThalassemiaReactive Oxygen SpeciesOxidative stressEuropean journal of haematology
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A systematic overview of the first pasteurised VWF/FVIII medicinal product, Haemate P/Humate -P: history and clinical performance

2008

Patients with von Willebrand disease (VWD) and haemophilia A (HA) lack, to varying degrees, the von Willebrand factor (VWF) and coagulation factor VIII (FVIII) that are critical for normal haemostasis. These conditions in turn make patients prone to uncontrolled bleeding. Historically, patients with severe forms of VWD or HA were crippled before adulthood and their life expectancy was significantly reduced. Over the past decades, specific coagulation factor replacement therapies including Haemate P, have been developed to help patients achieve and maintain normal haemostasis. Haemate P is a human, plasma-derived VWF/FVIII medicinal product, which was first licensed in Germany in 1981 for th…

congenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyHaemophilia AHemophilia AHaemophiliaHaemate P; Humate-P; von Willebrand disease; von Willebrand factor; haemophilia; factor VIIIVon Willebrand factorhemic and lymphatic diseasesInternal medicinevon Willebrand FactormedicineVon Willebrand diseaseHumansDosingDesmopressinHematologybiologybusiness.industryHematologyGeneral Medicinemedicine.diseasevon Willebrand DiseasesCoagulationImmunologybiology.proteinSafetybusinessSettore MED/15 - Malattie del Sanguemedicine.drug
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