Search results for "Lymphatic"

showing 10 items of 1179 documents

Umbilical cord blood transplantation from unrelated donors in patients with Philadelphia chromosome-positive acute lymphoblastic leukemia

2014

Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't; There are very few disease-specific studies focusing on outcomes of umbilical cord blood transplantation for Philadelphia chromosome-positive acute lymphoblastic leukemia. We report the outcome of 45 patients with Philadelphia chromosome-positive acute lymphoblastic leukemia who underwent myeloablative single unit cord blood transplantation from unrelated donors within the GETH/GITMO cooperative group. Conditioning regimens were based on combinations of thiotepa, busulfan, cyclophospamide or fludarabine, and antithymocyte globulin. At the time of transplantation, 35 patients (78%) were in first complete remission, four (8%) …

MaleTransplantation Conditioning:Analytical Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Case-Control Studies::Retrospective Studies [Medical Subject Headings]Leucemia-linfoma linfoblástico de células precursorasGraft vs Host Disease:Named Groups::Persons::Age Groups::Adult::Middle Aged [Medical Subject Headings]Supervivencia sin EnfermedadGastroenterology:Anatomy::Body Regions::Transplants::Allografts [Medical Subject Headings]:Chemicals and Drugs::Chemical Actions and Uses::Pharmacologic Actions::Therapeutic Uses::Antineoplastic Agents::Myeloablative Agonists [Medical Subject Headings]:Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings]hemic and lymphatic diseasesMedicineCumulative incidenceTasa de SupervivenciaPhiladelphia ChromosomeChildCromosoma Filadelfia:Diseases::Pathological Conditions Signs and Symptoms::Pathologic Processes::Disease Attributes::Chronic Disease [Medical Subject Headings]:Named Groups::Persons::Age Groups::Child::Child Preschool [Medical Subject Headings]ArticlesHematologyMiddle AgedPrecursor Cell Lymphoblastic Leukemia-LymphomaAllografts:Analytical Diagnostic and Therapeutic Techniques and Equipment::Therapeutics::Biological Therapy::Immunomodulation::Immunotherapy::Immunosuppression::Transplantation Conditioning [Medical Subject Headings]FludarabineSurvival RateChild Preschool:Named Groups::Persons::Age Groups::Adolescent [Medical Subject Headings]:Diseases::Pathological Conditions Signs and Symptoms::Pathologic Processes::Chromosome Aberrations::Translocation Genetic::Philadelphia Chromosome [Medical Subject Headings]:Analytical Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Statistics as Topic::Survival Analysis::Disease-Free Survival [Medical Subject Headings]FemaleAcondicionamiento para el trasplanteCord Blood Stem Cell TransplantationTrasplante de células madre de la sangre del cordónmedicine.drugAloinjertosAdultmedicine.medical_specialtyAdolescent:Check Tags::Male [Medical Subject Headings]Cord Blood Stem Cell TransplantationPhiladelphia chromosomeEnfermedad injerto contra huéspedDisease-Free SurvivalEstudios retrospectivosInternal medicine:Analytical Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Cohort Studies::Longitudinal Studies::Follow-Up Studies [Medical Subject Headings]:Named Groups::Persons::Age Groups::Adult [Medical Subject Headings]HumansPreschoolSurvival rate:Named Groups::Persons::Age Groups::Child [Medical Subject Headings]Retrospective Studies:Analytical Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Data Collection::Vital Statistics::Mortality::Survival Rate [Medical Subject Headings]business.industryUmbilical Cord Blood TransplantationEnfermedad crónicaAgonistas mieloablativosMyeloablative Agonistsmedicine.diseaseSettore MED/15SurgeryTransplantation:Diseases::Immune System Diseases::Graft vs Host Disease [Medical Subject Headings]:Diseases::Hemic and Lymphatic Diseases::Lymphatic Diseases::Lymphoproliferative Disorders::Leukemia Lymphoid::Precursor Cell Lymphoblastic Leukemia-Lymphoma [Medical Subject Headings]:Check Tags::Female [Medical Subject Headings]Estudios de SeguimientoChronic Disease:Analytical Diagnostic and Therapeutic Techniques and Equipment::Surgical Procedures Operative::Transplantation::Cell Transplantation::Stem Cell Transplantation::Cord Blood Stem Cell Transplantation [Medical Subject Headings]Adolescent; Adult; Allografts; Child; Child Preschool; Chronic Disease; Disease-Free Survival; Female; Follow-Up Studies; Graft vs Host Disease; Humans; Male; Middle Aged; Myeloablative Agonists; Retrospective Studies; Survival Rate; Cord Blood Stem Cell Transplantation; Philadelphia Chromosome; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Transplantation ConditioningbusinessBusulfanFollow-Up Studies
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Endothelial Dysfunction in Tristetraprolin-deficient Mice Is Not Caused by Enhanced Tumor Necrosis Factor-α Expression

2014

Cardiovascular events are important co-morbidities in patients with chronic inflammatory diseases like rheumatoid arthritis. Tristetraprolin (TTP) regulates pro-inflammatory processes through mRNA destabilization and therefore TTP-deficient mice (TTP(-/-) mice) develop a chronic inflammation resembling human rheumatoid arthritis. We used this mouse model to evaluate molecular signaling pathways contributing to the enhanced atherosclerotic risk in chronic inflammatory diseases. In the aorta of TTP(-/-) mice we observed elevated mRNA expression of known TTP targets like tumor necrosis factor-α (TNF-α) and macrophage inflammatory protein-1α, as well as of other pro-atherosclerotic mediators, l…

MaleVasculitismedicine.medical_specialtyMRNA destabilizationRNA StabilityTristetraprolinInflammationBiochemistryNitric oxideMicechemistry.chemical_compoundOrgan Culture TechniquesTristetraprolinhemic and lymphatic diseasesInternal medicinemedicineAnimalsEndothelial dysfunctionMolecular BiologyAortaReactive nitrogen speciesMice KnockoutMembrane GlycoproteinsNADPH oxidasebiologyTumor Necrosis Factor-alphaEndothelial CellsNADPH OxidasesMolecular Bases of DiseaseCell Biologyrespiratory systemAtherosclerosismedicine.diseaseReactive Nitrogen SpeciesMice Inbred C57BLOxidative StressCholesterolEndocrinologychemistryMice Inbred DBAChronic DiseaseNADPH Oxidase 2biology.proteinFemaleTumor necrosis factor alphamedicine.symptomReactive Oxygen SpeciesJournal of Biological Chemistry
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Expression and differential localization of xenobiotic transporters in the rat olfactory neuro-epithelium.

2011

International audience; Transporters, such as multidrug resistance P-glycoproteins (MDR), multidrug resistance-related proteins (MRP) and organic anion transporters (OATs), are involved in xenobiotic metabolism, particularly the cellular uptake or efflux of xenobiotics (and endobiotics) or their metabolites. The olfactory epithelium is exposed to both inhaled xenobiotics and those coming from systemic circulation. This tissue has been described as a pathway for xenobiotics to the brain via olfactory perineural space. Thereby, olfactory transporters and xenobiotic metabolizing enzymes, dedicated to the inactivation and the elimination of xenobiotics, have been involved in the toxicological p…

Male[ SDV.AEN ] Life Sciences [q-bio]/Food and NutritionMESH : Multidrug Resistance-Associated Proteinsp glycoproteinATP-binding cassette transporterMESH : HepatocytesReceptors OdorantMESH : P-GlycoproteinMESH: HepatocytesMESH : Lymphatic Vessels0302 clinical medicineMESH : Protein Transportugt2a1MESH: SmellMESH: Receptors OdorantMESH: AnimalsReceptorxenobiotic metabolizingmucosa0303 health sciencesMESH : Gene Expression RegulationMESH : RatsGeneral NeuroscienceMESH : OdorsMESH: Gene Expression RegulationSmellProtein Transportmedicine.anatomical_structureBiochemistryLivertransporterbarrierEffluxMultidrug Resistance-Associated ProteinsMESH: Multidrug Resistance-Associated ProteinsMESH: XenobioticsMESH: Protein TransportMESH: P-GlycoproteinMESH: RatsMESH: Lymphatic VesselsMESH : Maleodorant clearancebrainMESH : XenobioticsxenobioticBiologysystemMESH : Rats WistarOlfactory Receptor NeuronsXenobiotics03 medical and health sciencesbulbOlfactory Mucosamultidrug resistanceMESH : Receptors OdorantmedicineAnimalsATP Binding Cassette Transporter Subfamily B Member 1Rats WistardetoxificationMESH: Olfactory Mucosa030304 developmental biologyLymphatic VesselsMESH : Olfactory MucosaMESH: OdorsMESH : LiverTransporterMESH: Rats WistarMESH: Olfactory Receptor NeuronsEpitheliumMESH: MaleOlfactory bulbRatsenzymeGene Expression RegulationOdorantsHepatocytesMESH : SmellMESH : Olfactory Receptor NeuronsMESH : Animalsolfactory epitheliumOlfactory epitheliumperireceptor event[SDV.AEN]Life Sciences [q-bio]/Food and Nutrition030217 neurology & neurosurgeryDrug metabolismMESH: Liver
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Anemia during treatment with peginterferon Alfa-2b/ribavirin and boceprevir: Analysis from the serine protease inhibitor therapy 2 (SPRINT-2) trial

2013

International audience; Boceprevir (BOC) added to peginterferon alfa-2b (PegIFN) and ribavirin (RBV) significantly increases sustained virologic response (SVR) rates over PegIFN/RBV alone in previously untreated adults with chronic hepatitis C genotype 1. We evaluate the relationship of incident anemia with triple therapy. A total of 1,097 patients received a 4-week lead-in of PegIFN/RBV followed by: (1) placebo plus PegIFN/RBV for 44 weeks (PR48); (2) BOC plus PegIFN/RBV using response-guided therapy (BOC/RGT); and (3) BOC plus PegIFN/RBV for 44 weeks (BOC/PR48). The management of anemia (hemoglobin [Hb]<10 g/dL) included RBV dose reduction and/or erythropoietin (EPO) use. A total of 1,080…

Male[SDV]Life Sciences [q-bio]MedizinGastroenterologyPolyethylene GlycolsPlaceboschemistry.chemical_compoundHemoglobins0302 clinical medicinehemic and lymphatic diseasesMedicine030212 general & internal medicineChronicSettore MED/12 - GastroenterologiaInterferon-alpha; Serine Proteinase Inhibitors; Proline; Recombinant Proteins; Hematinics; Humans; Ribavirin; Anemia; Antiviral Agents; Drug Therapy Combination; Erythropoietin; Hemoglobins; Adult; Treatment Outcome; Placebos; Polyethylene Glycols; Hepatitis C Chronic; Female; MaleAnemiaHepatitis CHepatitis CRecombinant Proteins3. Good health[SDV] Life Sciences [q-bio]Treatment OutcomeCombinationPeginterferon alfa-2b030211 gastroenterology & hepatologyDrug Therapy CombinationFemalemedicine.drugAdultmedicine.medical_specialtySerine Proteinase InhibitorsProlineAnemiaInterferon alpha-2PlaceboAntiviral Agentsprotease inhibitor03 medical and health sciencesDrug TherapyInternal medicineBoceprevirRibavirinHumansAdverse effectErythropoietinHepatologybusiness.industryRibavirinInterferon-alphaHepatitis C Chronicmedicine.diseaseSurgerychemistryErythropoietinHematinicsbusiness
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Using the Hemophilia Joint Health Score for assessment of children: Reliability of the Spanish version

2018

Introduction: Numerous measuring instruments for the evaluation of hemophilic arthropathy have been developed. One of the most used systems is the Hemophilia Joint Health Score (HJHS) given its sensitivity to clinical changes appearing in the joints because of recurrent hemarthrosis. Objective: Assessing the interrater reliability, using the Spanish version of the HJHS (version 2.1) in children with hemophilia. Design: Reliability study to assess the interrater reliability of the Spanish version of HJHS. Methods: A sample of 36 children aged 7–13 years diagnosed with hemophilia A or B was used. Two physiotherapists performed physical assessments with the Spanish version of the HJHS. Descrip…

Malecongenital hereditary and neonatal diseases and abnormalities030506 rehabilitationmedicine.medical_specialtyAdolescentInterobserver reliabilityHealth StatusHemophilic arthropathyPhysical Therapy Sports Therapy and RehabilitationHemophilia AHemophilia BHemofilia03 medical and health sciences0302 clinical medicinePredictive Value of Testshemic and lymphatic diseasesHemarthrosisHealth Status IndicatorsHumansMedicineHealth scoreChildJoint (geology)Reliability (statistics)Observer Variationbusiness.industryAge FactorsReproducibility of ResultsSpanish versionHemartrosisCross-Sectional StudiesSpainNiñoArtropatíasJoint damagePhysical therapyJointsLucha contra las enfermedades0305 other medical sciencebusiness030217 neurology & neurosurgeryPhysiotherapy Theory and Practice
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Two new cases of Thrombocytopenia Absent Radius (TAR) syndrome: Clinical, Genetic and Nosologic Features

1988

Two unrelated children affected by TAR syndrome, autosomic recessive disease with congenital thrombocytopenia and bilateral radial aplasia, are described. In the first case a mild thrombocytopenia has been compatible with a fairly normal life until the second year of age. The other child shows radial aplasia associated with other anomalies of the upper limbs, severe thrombocytopenia and leukemoid reaction. The relationship among TAR syndrome, Fanconi's anemia and Roberts' syndrome are briefly discussed.

Malecongenital hereditary and neonatal diseases and abnormalitiesPathologymedicine.medical_specialtyEctromeliaAnemiaChromosome DisordersGenes RecessiveRadial aplasiaBiologyConsanguinityBilateral radial aplasiahemic and lymphatic diseasesmedicineClinical geneticHumansAbnormalities MultipleChromosome AberrationsPlatelet CountTAR syndromeInfantSyndromemedicine.diseaseThrombocytopeniaDermatologySevere thrombocytopeniaRadiusPediatrics Perinatology and Child HealthFemaleAbsent radiusLeukemoid reactionKlinische Pädiatrie
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Efficacy and safety of Wilate in paediatric VWD patients under 6 years of age - results of a prospective multicentre clinical study including recover…

2013

Treatment with exogenous von Willebrand factor (VWF) is indicated in patients with von Willebrand disease (VWD) in whom treatment with 1-deamino-8-d-arginine vasopressin/desmopressin is contraindicated. Wilate is a new generation plasma-derived concentrate of native VWF and coagulation factor VIII (FVIII) (in a physiological 1:1 ratio) developed for the treatment of VWD. This is the first study to report safety, efficacy and in vivo recovery (IVR) data from 15 paediatric patients less than 6 years of age who received Wilate for either prophylaxis, on-demand treatment or for treatment in surgical procedures during a prospective open-label trial (VWD type 1: 5, type 2A: 1, type 2B: 2, type 3:…

Malecongenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtyHemorrhageClinical studyVon Willebrand factorhemic and lymphatic diseasesvon Willebrand FactormedicineVon Willebrand diseaseHumansProspective StudiesChildDesmopressinGenetics (clinical)Paediatric patientsBleeding episodesFactor VIIIbiologyCoagulantsbusiness.industryInfantHematologyGeneral Medicinemedicine.diseasevon Willebrand DiseasesPhenotypeCoagulationTolerabilityChild Preschoolbiology.proteinFemalebusinessHalf-Lifemedicine.drugHaemophilia
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2p15-p16.1 microdeletions encompassing and proximal to BCL11A are associated with elevated HbF in addition to neurologic impairment.

2015

Elevated fetal hemoglobin (HbF) ameliorates the clinical severity of hemoglobinopathies such as β-thalassemia and sickle cell anemia. Currently, the only curative approach for individuals under chronic transfusion/chelation support therapy is allogeneic stem cell transplantation. However, recent analyses of heritable variations in HbF levels have provided a new therapeutic target for HbF reactivation: the transcriptional repressor BCL11A. Erythroid-specific BCL11A abrogation is now actively being sought as a therapeutic avenue, but the specific impact of such disruption in humans remains to be determined. Although single nucleotide polymorphisms in BCL11A erythroid regulatory elements have …

Malecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyAdolescentImmunologyBiologyBiochemistrySettore MED/38 - Pediatria Generale E SpecialisticaRed Cells Iron and ErythropoiesisInternal medicinehemic and lymphatic diseasesFetal hemoglobinmedicineGene silencingHumansChildNervous System DiseaseFetal HemoglobinNuclear ProteinHematologyNuclear ProteinsCell BiologyHematologymedicine.diseasePhenotypeSickle cell anemiaUp-RegulationTransplantationRepressor ProteinsSettore MED/03 - Genetica MedicaChromosomes Human Pair 22p15-p16.1 microdeletions BCL11A HbF neurologicImmunologyFemaleStem cellChromosome DeletionNervous System DiseasesCarrier ProteinHaploinsufficiencyCarrier ProteinsHumanBlood
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ABO Blood Group and Inhibitor Risk in Severe Hemophilia A Patients: A Study from the Italian Association of Hemophilia Centers

2021

AbstractConsidering the profound influence exerted by the ABO blood group system on hemostasis, mainly through the von Willebrand factor and factor VIII (FVIII) complex, we have conducted a study evaluating the possible role of blood type on the risk of inhibitor development in hemophilia A. A total of 287 consecutive Caucasian patients with severe hemophilia A (202 without FVIII inhibitors and 85 with FVIII inhibitors) followed at seven Italian Hemophilia Treatment Centers belonging to the Italian Association of Hemophilia Centers (AICE) were included in the study. A higher prevalence of O blood group was detected in patients without inhibitors as compared in inhibitor patients (55 vs. 30.…

Malecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyHemophilia ASevere hemophilia AABO Blood-Group SystemVon Willebrand factorhemic and lymphatic diseasesInternal medicineABO blood group systemABO blood group; hemophilia; inhibitors; risk factorshemophiliainhibitorsHumansMedicinerisk factorsIn patientBlood typebiologybusiness.industryABO blood groupHematologyinhibitorItalyHemostasisbiology.proteinFemaleCardiology and Cardiovascular Medicinebusiness
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Acute renal insufficiency and pancreatitis in a child with atypical Henoch–Schönlein purpura: efficacy of a single dose of cyclophosphamide

2018

A 9-year-old boy with petechiae on the legs and abdominal pain was unsuccessfully treated with steroids. He was admitted to our hospital for the onset of fever, ecchymosis, and arthralgia. Skin lesions suggested vasculitis, but they were not typical of Henoch–Schönlein purpura. He showed ecchymosis of the scrotal bursa, diffusion of petechiae to the trunk and arms, vomiting, severe abdominal pain, oliguria with hyponatremia, hypoalbuminemia, low C3 levels, high levels of creatinine, blood urea nitrogen, and tubular enzymes, proteinuria, and glycosuria. The urinary sediment showed macrohaematuria, and hyaline and cellular casts. Ultrasound showed polyserositis. He was treated with intraveno…

MalecorticosteroidMedicine (General)medicine.medical_specialtyAbdominal painHenoch-Schonlein purpuraIgA VasculitisEcchymosisAnti-Inflammatory Agentspancreatitisskin lesionCase ReportsMethylprednisoloneBiochemistryGastroenterology03 medical and health sciencesR5-9200302 clinical medicinehemic and lymphatic diseases030225 pediatricsInternal medicinemedicineHumansChildbusiness.industryBiochemistry (medical)pancreatitiAcute renal insufficiencyCell BiologyGeneral MedicineAcute Kidney InjuryPrognosismedicine.diseasePurpuraMethylprednisolonePancreatitisAcute pancreatitisDrug Therapy Combinationcyclophosphamide030211 gastroenterology & hepatologymedicine.symptomHenoch–Schönlein purpuraHyponatremiabusinessImmunosuppressive Agentsmedicine.drugJournal of International Medical Research
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