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showing 10 items of 20942 documents

Resting Energy Expenditure and Substrate Oxidation in Malnourished Patients With Type 1 Glycogenosis.

2019

Abstract Context Type 1a and 1b glycogenosis [glycogen storage disorder (GSD)1a, GSD1b] are rare diseases generally associated with malnutrition. Although abnormal substrate oxidation rates and elevated energy expenditures might contribute to malnutrition, this issue has not been investigated. Objective To investigate whether abnormal resting energy expenditure (REE) and substrate oxidation rate characterize patients with GSD1. Design Cross-sectional study Setting Outpatient referral center for rare diseases and laboratory of clinical nutrition at the University Hospital of Palermo Patients Five consecutive patients with GSD1 (4 type a, 1 type b; 3 men, 2 women; age range, 19 to 49 years) M…

0301 basic medicineAdultMalemedicine.medical_specialtyEndocrinology Diabetes and MetabolismClinical Biochemistry030209 endocrinology & metabolismClinical nutritionmalnoutritionGlycogen Storage Disease Type IProtein oxidationBiochemistryGastroenterology03 medical and health sciencesBasal (phylogenetics)chemistry.chemical_compoundYoung Adult0302 clinical medicineEndocrinologyOxygen ConsumptionLipid oxidationsubstrate oxidationInternal medicineMedicineHumansResting energy expenditureSettore MED/49 - Scienze Tecniche Dietetiche ApplicateResting energy expenditureGlycogenbusiness.industryBiochemistry (medical)MalnutritionCalorimetry IndirectCarbohydratetype 1 glycogenosis.Middle Agedmedicine.diseaseMalnutrition030104 developmental biologyEndocrinologyCross-Sectional StudieschemistryBody CompositionFemalebusinessEnergy MetabolismOxidation-ReductionThe Journal of clinical endocrinology and metabolism
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Sex Alters the MHC Class I HLA-A Association With Polyglandular Autoimmunity.

2018

Abstract Context The major histocompatibility complex (MHC) strongly contributes to the development of polyglandular autoimmunity (PGA). Objective To evaluate the impact of sex on human leukocyte antigen (HLA) association with PGA for the first time. Design Cross-sectional immunogenetic study. Setting Academic tertiary referral Orphan Disease Center for PGA (ORPHA 282196) and immunogenetics laboratory. Subjects Patients (158) with coexistent type 1 diabetes and autoimmune thyroid disease (adult type 3 PGA, ORPHA 227982) and 479 unrelated healthy controls. Interventions All 637 white subjects were typed for HLA-A, -B, -DRB1, -DQA1, and -DQB1 alleles at a two-field level. Main Outcome Measure…

0301 basic medicineAdultMalemedicine.medical_specialtyEndocrinology Diabetes and MetabolismClinical Biochemistry030209 endocrinology & metabolismContext (language use)Human leukocyte antigenMajor histocompatibility complexmedicine.disease_causeBiochemistryAutoimmunity03 medical and health sciences0302 clinical medicineEndocrinologySex FactorsGene FrequencyInternal medicineMHC class ImedicineHumansGenetic Predisposition to DiseasePolyendocrinopathies AutoimmuneType 1 diabetesMHC class IIbiologyHLA-A Antigensbusiness.industryHistocompatibility TestingBiochemistry (medical)Histocompatibility Antigens Class IMiddle Agedmedicine.diseasePrognosisHLA-A030104 developmental biologyEndocrinologyCross-Sectional StudiesDiabetes Mellitus Type 1HaplotypesCase-Control Studiesbiology.proteinFemalebusinessBiomarkersFollow-Up StudiesThe Journal of clinical endocrinology and metabolism
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Liver Fat Content in People with Pituitary Diseases: Influence of Serum IGF1 Levels

2017

AbstractNon-alcoholic fatty liver disease (NAFLD) is commonly associated with obesity, metabolic syndrome, and type 2 diabetes. NAFLD is also seen in patients with endocrinopathies. However, the relationship between endocrine diseases and the development of NAFLD is not well known. In this study, we set out to determine whether liver fat content (LFC) was associated with IGF1 levels in people with pituitary diseases (PD). Eighty-nine patients with pituitary diseases and 74 healthy controls were included in this study. LFC was measured using MRI. Hepatic steatosis was defined as LFC>5.5%. Patients with PD were older, and had a higher BMI than healthy controls. LFC was significantly higher…

0301 basic medicineAdultMalemedicine.medical_specialtyEndocrinology Diabetes and MetabolismClinical Biochemistry030209 endocrinology & metabolismDiseaseType 2 diabetesIntra-Abdominal FatBiochemistryBody Mass Index03 medical and health sciences0302 clinical medicineEndocrinologyInternal medicine[ SDV.MHEP ] Life Sciences [q-bio]/Human health and pathologyLiver fatmedicinesteatosisEndocrine systemHumansProspective StudiesInsulin-Like Growth Factor Ibusiness.industryBiochemistry (medical)Fatty liverIGF1pituitary diseasesnon-alcoholic fatty liver diseaseGeneral MedicineMiddle Agedmedicine.diseaseObesity030104 developmental biologyEndocrinologyCase-Control StudiesFemaleSteatosisMetabolic syndromebusinessBiomarkers
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Obesity impairs leukocyte‐endothelium cell interactions and oxidative stress in humans

2018

Background:To evaluate the relationship between leukocyte‐endothelial cellinteractions and oxidative stress parameters in non‐diabetic patients with differentgrades of obesity.Material and methods:For this cross‐sectional study, 225 subjects wererecruited from January 1, 2014 to December 31, 2016 and divided into groupsaccording to BMI (40 kg/m²). We determined clin-ical parameters, systemic inflammatory markers, soluble cellular adhesion mole-cules, leukocyte‐endothelium cell interactions—rolling flux, velocity and adhesion—, oxidative stress parameters—total ROS, total superoxide, glutathione—andmitochondrial membrane potential in leukocytes.Results:We verified that HOMA‐IR and hsCRP incr…

0301 basic medicineAdultMalemedicine.medical_specialtyEndotheliumAdolescentClinical BiochemistryCell Communication030204 cardiovascular system & hematologyMitochondrionmedicine.disease_causeBiochemistryProinflammatory cytokineBody Mass Index03 medical and health sciencesYoung Adult0302 clinical medicineInternal medicinemedicineCell AdhesionLeukocytesHumansObesityEndothelial dysfunctionCell adhesionAgedchemistry.chemical_classificationMembrane Potential MitochondrialReactive oxygen speciesChemistryCell adhesion moleculeEndothelial CellsGeneral MedicineMiddle AgedAtherogenesismedicine.diseaseIntercellular Adhesion Molecule-1Oxidative Stress030104 developmental biologyEndocrinologymedicine.anatomical_structureCross-Sectional StudiesCytokinesReactive oxygen specieFemaleMitochondrial membrane potentialReactive Oxygen SpeciesOxidative stress
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Effects of the flavonol quercetin and α-linolenic acid on n-3 PUFA status in metabolically healthy men and women: a randomised, double-blinded, place…

2017

AbstractIncreased dietary intake and tissue status of the long-chainn-3 PUFA, EPA and DHA, is associated with cardiovascular benefits. Epidemiological and animal studies suggest that concomitant nutritive intake of flavonoids may increase the conversion ofα-linolenic acid (ALA) to longer-chainn-3 fatty acids EPA and DHA. We investigated the effects of increased ALA intake on fatty acid composition of serum phospholipids and erythrocytes in metabolically healthy men and women and whether fatty acid profiles and ALA conversion were affected by regular quercetin intake or sex. Subjects (n74) were randomised to receive at least 3·3 g/d ALA with either 190 mg/d quercetin (ALA+quercetin) or place…

0301 basic medicineAdultMalemedicine.medical_specialtyErythrocytesDocosahexaenoic AcidsMedicine (miscellaneous)PlaceboPlacebos03 medical and health scienceschemistry.chemical_compoundDouble-Blind MethodInternal medicineFatty Acids Omega-3MedicineHumansN 3 pufaPhospholipidsα-linolenic acidchemistry.chemical_classification030109 nutrition & dieteticsNutrition and DieteticsCross-Over Studiesbusiness.industryFatty AcidsFatty acidalpha-Linolenic AcidCrossover studyDietEndocrinologychemistryBiochemistryEicosapentaenoic AcidDietary SupplementsBody Compositionlipids (amino acids peptides and proteins)FemaleQuercetinAnimal studiesbusinessQuercetinPolyunsaturated fatty acidThe British journal of nutrition
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Lacosamide in patients with temporal lobe epilepsy: An observational multicentric open-label study.

2016

Abstract Purpose The aim of this study was to evaluate the efficacy and tolerability of lacosamide (LCM) both as add-on therapy and monotherapy in patients with temporal lobe epilepsy (TLE) based on an observational, prospective, multicenter study. Methods We enrolled 100 patients (mean age: 43.4 ± 12.53 years, 57 females) with nonlesional TLE and TLE with hippocampal sclerosis (HS) that did not respond to the first drug and who were referred to epilepsy centers of the University of Catanzaro, University of Palermo, IRCSS Neuromed of Pozzilli, and Otto-von-Guericke University of Magdeburg. In this open-label, multicenter trial, patients were initiated on oral LCM as add-on therapy to first …

0301 basic medicineAdultMalemedicine.medical_specialtyLacosamideCohort Studies03 medical and health sciencesBehavioral NeuroscienceEpilepsyYoung Adult0302 clinical medicineLacosamideMulticenter trialInternal medicineAcetamidesMedicineHumansProspective StudiesAdverse effectProspective cohort studyHippocampal sclerosisbusiness.industryMiddle Agedmedicine.diseaseMagnetic Resonance Imaging030104 developmental biologyNeurologyTolerabilityEpilepsy Temporal LobeAnesthesiaConcomitantAnticonvulsantsDrug Therapy CombinationFemaleNeurology (clinical)business030217 neurology & neurosurgerymedicine.drugFollow-Up StudiesEpilepsybehavior : EB
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Elevated circulating levels of succinate in human obesity are linked to specific gut microbiota

2018

Gut microbiota-related metabolites are potential clinical biomarkers for cardiovascular disease (CVD). Circulating succinate, a metabolite produced by both microbiota and the host, is increased in hypertension, ischemic heart disease, and type 2 diabetes. We aimed to analyze systemic levels of succinate in obesity, a major risk factor for CVD, and its relationship with gut microbiome. We explored the association of circulating succinate with specific metagenomic signatures in cross-sectional and prospective cohorts of Caucasian Spanish subjects. Obesity was associated with elevated levels of circulating succinate concomitant with impaired glucose metabolism. This increase was associated wit…

0301 basic medicineAdultMalemedicine.medical_specialtyMetaboliteSuccinic AcidMicrobiota intestinalType 2 diabetesGut floraPrevotellaceaeCarbohydrate metabolismMicrobiologyArticle03 medical and health scienceschemistry.chemical_compoundDiabetes mellitusInternal medicinemedicineHumansMicrobiomeProspective StudiesObesityGastrointestinal microbiomeEcology Evolution Behavior and SystematicsPhylogenyAgedbiologyBacteriaSuccinate dehydrogenaseMiddle Agedmedicine.diseasebiology.organism_classificationGastrointestinal MicrobiomeDiet030104 developmental biologyEndocrinologyCross-Sectional StudieschemistryDiabetes Mellitus Type 2biology.proteinObesitatFemaleDietaBiomarkers
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CYP27A1, CYP24A1, and RXR-α Polymorphisms, Vitamin D, and Multiple Sclerosis: a Pilot Study.

2018

Multiple sclerosis (MS) is a neurodegenerative autoimmune disease resulting from a complex interaction of genetic and environmental factors. Hypovitaminosis D seems to contribute to MS susceptibility as both an environmental and a genetic risk factor. The aim of our study was to investigate the association of SNPs in CYP27A1, CYP24A1, and RXR- α genes, vitamin D status, and MS risk. We performed a nested case-control study on patients with multiple sclerosis and healthy controls. Serum 25(OH)D3 levels and genotyping of CYP27A1, CYP24A1, and RXR-α -SNPs were investigated both in MS patients and in healthy controls. Serum 25(OH)D3 levels were measured by a high-performance liquid chromatograp…

0301 basic medicineAdultMalemedicine.medical_specialtyMultiple SclerosisRXRSingle-nucleotide polymorphismPilot ProjectsPolymorphism Single Nucleotide03 medical and health sciencesCellular and Molecular Neuroscience0302 clinical medicineInternal medicineGenotypemedicineVitamin D and neurologyHumansVitamin DAlleleVitamin D3 24-HydroxylaseGenotypingAutoimmune disease25-Hydroxyvitamin D 2Retinoid X Receptor alphabusiness.industryMultiple sclerosisGeneral MedicineMiddle Agedmedicine.diseaseMinor allele frequencyCYP24A1030104 developmental biologyEndocrinologyCase-Control StudiesCYP27A1Cholestanetriol 26-MonooxygenaseFemalebusiness030217 neurology & neurosurgeryJournal of molecular neuroscience : MN
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New insights in the neurological phenotype of aceruloplasminemia in Caucasian patients

2017

Abstract Introduction The diagnosis aceruloplasminemia is usually made in patients with advanced neurological manifestations of the disease. In these patients prognosis is poor, disabilities are severe and patients often die young. The aim of our study was to facilitate recognition of aceruloplasminemia at a disease stage at which treatment can positively influence outcome. Currently, the neurological phenotype of aceruloplasminemia has been mainly described in Japanese patients. This ‘classical’ phenotype consists of cerebellar ataxia, hyperkinetic movement disorders and cognitive decline. In this study we describe the spectrum of neurological disease in Caucasian patients. Methods Data on…

0301 basic medicineAdultMalemedicine.medical_specialtyPediatricsAtaxiaMovement disordersBiologyWhite People03 medical and health sciencesNeurological manifestation0302 clinical medicinePhenotypic variabilitymedicineAceruloplasminemiaHumansCognitive declineAceruloplasminemiaPsychiatryDystoniaCerebellar ataxiaParkinsonismCeruloplasminChoreaNeurodegenerative DiseasesMiddle Agedmedicine.diseaseIron Metabolism DisordersPedigree030104 developmental biologyPsychiatric changesPhenotypeNeurologyFemaleNeurology (clinical)Geriatrics and Gerontologymedicine.symptomNervous System DiseasesSettore M-EDF/01 - Metodi E Didattiche Delle Attivita' Motorie030217 neurology & neurosurgeryFollow-Up Studies
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Primary prophylaxis of invasive fungal infections with posaconazole or itraconazole in patients with acute myeloid leukaemia or high-risk myelodyspla…

2017

This is an observational-retrospective study comparing the real-world outcomes associated with posaconazole vs. itraconazole as prophylaxis treatments. Two hundred and ninety-three patient admissions attributable to 174 patients were included in the study. Patients were treated with itraconazole (n = 114 admissions; 39%) or posaconazole (n = 179; 61%). Antifungal prophylaxis failure (APF) due to treatment-related adverse events (in 34 out of 293 patient admissions; 11.6%) was more frequent in the posaconazole group (6.1% vs. 15.1%; P = 0.024). There were 9 patient admissions for episodes of APF due to probable/proven breakthrough fungal infection (primary endpoint): 6 and 3 in the itraconaz…

0301 basic medicineAdultMalemedicine.medical_specialtyPosaconazoleAntifungal AgentsDrug-Related Side Effects and Adverse ReactionsItraconazole030106 microbiologyDermatologyNeutropenia03 medical and health sciencesInternal medicinemedicineClinical endpointHumansIn patientTreatment FailureAdverse effectAgedRetrospective StudiesInvasive Pulmonary Aspergillosisbusiness.industryMyelodysplastic syndromesGeneral MedicineMiddle AgedTriazolesmedicine.diseaseSurgeryClinical trialLeukemia Myeloid AcuteInfectious DiseasesMyelodysplastic SyndromesFemalePre-Exposure ProphylaxisItraconazolebusinessInvasive Fungal Infectionsmedicine.drugMycoses
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