Search results for "FET"

showing 10 items of 3892 documents

What is the impact of a novel MED12 variant on syndromic conotruncal heart defects? Analysis of case report on two male sibs

2020

Abstract Background Syndromic congenital heart disease accounts for 30% of cases and can be determined by genetic, environmental or multifactorial causes. In many cases the etiology remains uncertain. Many known genes are responsible for specific morphopathogenetic mechanisms during the development of the heart whose alteration can determine specific phenotypes of cardiac malformations. Case presentation We report on two cases of association of conotruncal heart defect with facial dysmorphisms in sibs. In both cases the malformations’ identification occurred by ultrasound in the prenatal period. It was followed by prenatal invasive diagnosis. The genetic analysis revealed no rearrangements …

0301 basic medicineAdultHeart Defects CongenitalMaleHeart diseaseFacial dysmorphismCase ReportGenetic analysisFacial dysmorphismsCongenital heart diseases030218 nuclear medicine & medical imagingConotruncal heart defectsMED1203 medical and health sciences0302 clinical medicinePregnancyNext generation sequencingPrenatal DiagnosismedicineHumansGenetic TestingGeneX chromosomeConotruncal heart defectsCongenital heart diseaseGeneticsMediator Complexbusiness.industrylcsh:RJ1-570lcsh:Pediatricsmedicine.diseasePhenotypeMED12Fetal Diseases030104 developmental biologyConotruncal heart defectEchocardiographyEtiologyFemalebusinessItalian Journal of Pediatrics
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Human hippocampal neurogenesis drops sharply in children to undetectable levels in adults.

2018

New neurons continue to be generated in the subgranular zone of the dentate gyrus of the adult mammalian hippocampus(1-5). This process has been linked to learning and memory, stress and exercise, and is thought to be altered in neurological disease(6-10). In humans, some studies have suggested that hundreds of new neurons are added to the adult dentate gyrus every day(11), whereas other studies find many fewer putative new neurons(12-14). Despite these discrepancies, it is generally believed that the adult human hippocampus continues to generate new neurons. Here we show that a defined population of progenitor cells does not coalesce in the subgranular zone during human fetal or postnatal …

0301 basic medicineAdultMaleAdolescentGeneral Science & TechnologyNeurogenesisPopulationHippocampusCell CountBiologyHippocampal formationHippocampusArticleSubgranular zoneFetal Development03 medical and health sciencesEpilepsyYoung Adult0302 clinical medicineNeural Stem CellsmedicineAnimalsHumansYoung adulteducationChildPreschoolCell ProliferationAgedNeuronseducation.field_of_studyMultidisciplinaryEpilepsyDentate gyrusNeurogenesisInfantMiddle Agedmedicine.diseaseNewbornMacaca mulattaHealthy Volunteers030104 developmental biologymedicine.anatomical_structurenervous systemDentate GyrusNeurologicalFemaleNeuroscience030217 neurology & neurosurgery
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Clinical course of sly syndrome (mucopolysaccharidosis type VII).

2016

WOS: 000377110800007

0301 basic medicineAdultMalePediatricsmedicine.medical_specialtyAdolescentMucopolysaccharidosisSly syndromeHepatosplenomegalyMetabolic disordersMucopolysaccharidosis VIIMedical and Health Sciences03 medical and health sciencesYoung Adult0302 clinical medicineHydrops fetalisSurveys and QuestionnairesmedicineGeneticsHumansMedical history1506Clinical geneticsFamily historyPreschoolChildGenetics (clinical)GlucuronidaseGenetics & Hereditybusiness.industryGenotype-Phenotype CorrelationsMucopolysaccharidosis VIIInfantEnzyme replacement therapyBiological Sciencesmedicine.diseaseLysosomal Storage Diseases030104 developmental biologyPhenotypeClinical genetics Genetics Metabolic disordersChild PreschoolFemalemedicine.symptombusiness030217 neurology & neurosurgeryMPS ; lysosomal storage disease ; β-glucuronidase
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KIR2DL3 and the KIR ligand groups HLA-A-Bw4 and HLA-C2 predict the outcome of hepatitis B virus infection.

2017

Killer immunoglobulin-like receptors (KIRs) regulate the activation of Natural Killer cells through their interaction with human leukocyte antigens (HLA). KIR and HLA loci are highly polymorphic and certain HLA-KIR combinations have been found to protect against viral infections. In this study we analyzed whether the KIR/HLA repertoire may influence the course of hepatitis B virus (HBV) infection. Fifty-seven subjects with chronic hepatitis B (CHB), 44 subjects with resolved HBV infection, and 60 healthy uninfected controls (HC) were genotyped for KIR and their HLA ligands. The frequency of the HLA-A-Bw4 ligand group was higher in CHB (58%) than subjects with resolved infection (23%) (crude…

0301 basic medicineAdultMaleSettore MED/09 - Medicina InternaGenotypeSettore MED/17 - Malattie InfettiveKIR LigandHuman leukocyte antigenHLA-C AntigensBiologymedicine.disease_cause03 medical and health sciencesYoung Adult0302 clinical medicineHepatitis B ChronicVirologymedicineHumansGenetic Predisposition to DiseaseAlleleReceptorGeneAgedSettore MED/04 - Patologia GeneraleHepatitis B virusHepatologyHLA-A AntigensMiddle AgedVirologyHLA-AKIRs HLAA HBV030104 developmental biologyInfectious DiseasesTreatment OutcomeHLA-B AntigensReceptors KIR2DL3ImmunologyFemalePredictive variables030215 immunology
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Antiepileptic drug reduction and increased risk of stimulation-evoked focal to bilateral tonic-clonic seizure during cortical stimulation in patients…

2017

Introduction: Stimulation-evoked focal to bilateral tonic-clonic seizure (FBTCS) can be a stressful and possibly harmful adverse event for patients during cortical stimulation (CS). We evaluated if drug load reduction of anti epileptic drugs (AEDs) during CS increases the risk of stimulation-evoked FBTCS. Material and methods: In this retrospective cohort study, we searched our local database for patients with drug resistant epilepsy who underwent invasive video-EEG monitoring and CS in the University Hospital la Fe Valencia from January 2006 to November 2016. The AED drug load was calculated with the defined daily dose. We applied a uni- and multivariate logistic regression model to estima…

0301 basic medicineAdultMalemedicine.medical_specialtyDrug Resistant EpilepsyNeurologyAdolescentDrug-Related Side Effects and Adverse ReactionsStimulation03 medical and health sciencesBehavioral NeuroscienceEpilepsy0302 clinical medicineEpilepsy surgerySeizuresmedicineOdds RatioHumansEpilepsy surgeryAdverse effectRetrospective Studiesbusiness.industryRetrospective cohort studyElectroencephalographyOdds ratioMiddle Agedmedicine.diseaseElectric Stimulation030104 developmental biologyDefined daily doseLogistic ModelsTreatment OutcomeNeurologyElectrical stimulationWithdrawalAnesthesiaDefined daily doseAnticonvulsantsEpilepsy GeneralizedFemaleNeurology (clinical)Epilepsies PartialEpilepsy Tonic-ClonicSafetybusinessGeneralized tonic-clonic seizure030217 neurology & neurosurgeryEpilepsybehavior : EB
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Evolution of major non-HIV-related comorbidities in HIV-infected patients in the Italian Cohort of Individuals, Naïve for Antiretrovirals (ICONA) Fou…

2018

Objectives: The management of HIV disease is complicated by the incidence of a new spectrum of comorbid noncommunicable diseases (NCDs). It is important to document changes in the prevalence of NCDs over time. The aim of the study was to describe the impact of ageing on HIV markers and on the prevalence of NCDs in people living with HIV (PLWHIV) in the Italian Cohort of Individuals, Naïve for Antiretrovirals (ICONA) seen for care in 2004–2014. Methods: Analyses were conducted separately for a closed cohort (same people seen at both times) and an open cohort (all people under follow-up). We used the χ2 test for categorical factors and the Wilcoxon test for quantitative factors to compare pro…

0301 basic medicineAdultMalemedicine.medical_specialtynoncommunicable diseases (NCDs); persons living with HIV (PLWHIV); time trend; Health Policy; Infectious Diseases; Pharmacology (medical)persons living with HIV (PLWHIV)PopulationRenal functionInfectious DiseaseHIV InfectionsDiseaseComorbiditySettore MED/17 - MALATTIE INFETTIVENO03 medical and health sciences0302 clinical medicineInterquartile rangeInternal medicinePrevalenceMedicineHiv infected patientsHumansPharmacology (medical)030212 general & internal medicineeducationDyslipidemiaseducation.field_of_studynoncommunicable diseases (NCDs); persons living with HIV (PLWHIV); time trendFramingham Risk Scorebusiness.industryIncidence (epidemiology)Health Policynoncommunicable diseases (NCDs) persons living with HIV (PLWHIV) time trendMiddle Aged030112 virologytime trendInfectious DiseasesItalyCardiovascular DiseasesCohortHypertensionFemalebusinessnoncommunicable diseases (NCDs)HIV medicine
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Genome-wide DNA methylation study in human placenta identifies novel loci associated with maternal smoking during pregnancy

2016

BACKGROUND: We conducted an epigenome-wide association study (EWAS) of DNA methylation in placenta in relation to maternal tobacco smoking during pregnancy and examined whether smoking-induced changes lead to low birthweight. METHODS: DNA methylation in placenta was measured using the Illumina HumanMethylation450 BeadChip in 179 participants from the INfancia y Medio Ambiente (INMA) birth cohort. Methylation levels across 431 311 CpGs were tested for differential methylation between smokers and non-smokers in pregnancy. We took forward three top-ranking loci for further validation and replication by bisulfite pyrosequencing using data of 248 additional participants of the INMA cohort. We ex…

0301 basic medicineAdultMaleplacentaEpidemiologyMaternal smokingPlacentaEpigenesis GeneticCohort Studies03 medical and health sciencesPregnancyTobacco SmokingMedicineBirth WeightHumansFetal programmingtobacco smokingCotinineDones embarassades -- Consum de tabacDNA methylationepigeneticsbusiness.industryInfant NewbornHuman placentaGeneral MedicineDNA MethylationMendelian Randomization Analysis030104 developmental biologyfetal programmingbirthweightMaternal ExposureSpainLinear ModelsChristian ministryCpG IslandsFemaleDNA -- MetilacióbusinessHumanitiesGenome-Wide Association Study
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Severe pre-eclampsia is associated with alterations in cytotrophoblasts of the smooth chorion.

2016

Pre-eclampsia (PE), which affects ∼8% of first pregnancies, is associated with faulty placentation. Extravillous cytotrophoblasts (CTBs) fail to differentiate properly, contributing to shallow uterine invasion and deficient spiral artery remodeling. We studied the effects of severe PE (sPE) on the smooth chorion portion of the fetal membranes. The results showed a significant expansion of the CTB layer. The cells displayed enhanced expression of stage-specific antigens that extravillous CTBs normally upregulate as they exit the placenta. Transcriptomics revealed the dysregulated expression of many genes (e.g. placental proteins, markers of oxidative stress). We confirmed an sPE-related incr…

0301 basic medicineAdultSpiral arteryTranscription GeneticPlacentaHuman DevelopmentCTBSExtraembryonic MembranesBiology210Andrology03 medical and health sciences0302 clinical medicineDownregulation and upregulationPre-EclampsiaPregnancyPlacentamedicineHumansPregnancy-Associated Plasma Protein-AMolecular BiologyCytotrophoblastPAPPA1Cell ProliferationFetus030219 obstetrics & reproductive medicineCytotrophoblastPlacentationGene Expression Regulation DevelopmentalPreterm birthChorionPlacentationTrophoblastsOxidative Stress030104 developmental biologymedicine.anatomical_structureImmunologyembryonic structuresKeratinsFemaleCytotrophoblastsTranscriptomeDevelopmental BiologyProtein BindingHumanDevelopment (Cambridge, England)
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The close link between the fetal programming imprinting and neurodegeneration in adulthood: The key role of “hemogenic endothelium” programming

2021

The research on neurodegenerative diseases (NeuroDegD) has been traditionally focused on later life stages. There is now an increasing evidence, that they may be programmed during early development. Here, we propose that NeuroDegD are the result of the complex process of imprinting on fetal hemogenic endothelium, from which the microglial cells make to origin. The central role of placenta and epigenetic mechanisms (methylation of DNA, histone modifications and regulation by non-coding RNAs) in mediating the short and long-term effects has been also described. Precisely, it reports their role in impacting plasticity and memory of microglial cells. In addition, we also underline the necessity…

0301 basic medicineAgingHemangioblastsCell PlasticityRisk AssessmentEpigenesis GeneticFetal DevelopmentMolecular Imprinting03 medical and health sciences0302 clinical medicineEpigenetic factors as biomarkers Sex dimorphism Fetal developmental programming Hemogenic endothelium Microglia plasticity and memory Neurodegenerative diseasesmedicineHumansSettore MED/05 - Patologia ClinicaEpigeneticsFetal programmingImprinting (organizational theory)Hemogenic endotheliumSex CharacteristicsBiological Variation Individualbiologybusiness.industryNeurodegenerationGene Expression Regulation DevelopmentalNeurodegenerative Diseasesmedicine.diseaseLife stage030104 developmental biologyHistonePrenatal stressbiology.proteinMicrogliabusinessNeuroscienceBiomarkers030217 neurology & neurosurgeryDevelopmental BiologyMechanisms of Ageing and Development
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Characterization of NO-Induced Nitrosative Status in Human Placenta from Pregnant Women with Gestational Diabetes Mellitus

2017

Dysregulation of NO production is implicated in pregnancy-related diseases, including gestational diabetes mellitus (GDM). The role of NO and its placental targets in GDM pregnancies has yet to be determined. S-Nitrosylation is the NO-derived posttranslational protein modification that can modulate biological functions by forming NO-derived complexes with longer half-life, termed S-nitrosothiol (SNO). Our aim was to examine the presence of endogenous S-nitrosylated proteins in cysteine residues in relation to antioxidant defense, apoptosis, and cellular signal transduction in placental tissue from control (n=8) and GDM (n=8) pregnancies. S-Nitrosylation was measured using the biotin-switch …

0301 basic medicineAgingendocrine system diseasesPlacentaNitric Oxide Synthase Type IIExpressionApoptosisBiochemistryBody Mass Index0302 clinical medicineNitric-oxidePregnancyMitogen-Activated Protein Kinase 1Mitogen-Activated Protein Kinase 3biologyCaspase 3lcsh:CytologyNitrosylationP38General MedicineCatalaseCaspase 9TrophoblastsGestational diabetesmedicine.anatomical_structureCatalase030220 oncology & carcinogenesisFemaleResearch ArticleAdultmedicine.medical_specialtyArticle SubjectNitrosationNitric OxidePathophysiology03 medical and health sciencesErk1/2Internal medicinePlacentamedicineHumanslcsh:QH573-671Protein kinase BPregnancyFetusNitratesS-NitrosothiolsCesarean SectionCell BiologyPeroxiredoxinsmedicine.diseaseProtein s-nitrosylationDiabetes Gestational030104 developmental biologyEndocrinologyOxidative stressCase-Control Studiesbiology.proteinPeroxiredoxinProto-Oncogene Proteins c-aktOxidative Medicine and Cellular Longevity
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