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showing 10 items of 23890 documents

Analysis of cold activation of the contact system in hereditary angioedema with normal C1 inhibitor.

2021

Hereditary angioedema (HAE) attacks are caused by excessive activation of the contact system. Understanding how the contact system is activated in HAE, especially in patients with normal C1 inhibitor (HAEnCI), is essential to effectively treat this disease. Contact system activation involves the cleavage of several proteins including Factor XII (FXII), high molecular weight kininogen (HK), prekallikrein, sgp120 (ITIH4) and C1 inhibitor (C1-INH) before the subsequent generation of bradykinin that mediates HAE. In this study, we evaluated the fragmentation and enzymatic activity of contact system proteins in HAEnCI plasma samples before and after contact system activation induced by incubatio…

0301 basic medicineAdultMalemedicine.medical_specialtyHigh-molecular-weight kininogenImmunologyProteinase Inhibitory Proteins SecretoryBradykininBradykininC1-inhibitorHereditary Angioedema Type III03 medical and health scienceschemistry.chemical_compoundYoung Adult0302 clinical medicineInternal medicinemedicineHumansFragmentation (cell biology)Molecular BiologyBlood CoagulationFactor XIIbiologyKininogensPrekallikreinPrekallikreinEstrogensPlasminogenKallikreinMiddle Agedmedicine.diseaseCold Temperature030104 developmental biologyEndocrinologychemistryHereditary angioedemaFactor XIIbiology.proteinFemaleKallikreinsComplement C1 Inhibitor Protein030215 immunologyMolecular immunology
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Vitamin D receptor polymorphisms and 25-hydroxyvitamin D in a group of Sicilian multiple sclerosis patients

2016

Multiple sclerosis (MS) is an auto-immune disease whose etiology remains controversial. Both genetic and environmental factors are thought to be involved in the risk of developing the disease. The purpose of our study was to assess the association of Vitamin D receptor (VDR) polymorphisms with MS and to investigate the interaction of these polymorphisms with vitamin D levels. A total of 179 Sicilian subjects, including 104 MS patients and 75 healthy controls, were studied. The most common VDR polymorphisms (Fok-I, Bsm-I, Taq-I and Apa-I) were genotyped by polymerase chain reaction (PCR) followed by restriction fragment length polymorphism (RFLP) analyses in both groups and serum 25-hydroxyv…

0301 basic medicineAdultMalemedicine.medical_specialtyMultiple SclerosisGenotypeVDR polymorphismsDermatologyCalcitriol receptor25(OH)D; Multiple sclerosis; VDR polymorphisms; Vitamin D; Adult; Female; Gene Frequency; Genotype; Humans; Male; Middle Aged; Multiple Sclerosis; Receptors Calcitriol; Sicily; Vitamin D; Polymorphism Restriction Fragment Length03 medical and health sciences0302 clinical medicineGene FrequencyCalcitriolInternal medicineGenotypeReceptorsmedicineGenetic predispositionVitamin D and neurologyHumansMultiple sclerosiAlleleVitamin DPolymorphismAllele frequencySicilyVDR25(OH)Dbusiness.industryMultiple sclerosisGeneral MedicineMiddle Agedmedicine.diseaseVitamin D 25(OH)DPsychiatry and Mental healthSettore BIO/12 - Biochimica Clinica E Biologia Molecolare Clinica030104 developmental biologyEndocrinologyRestriction Fragment LengthImmunologyReceptors CalcitriolSettore MED/26 - NeurologiaFemaleNeurology (clinical)Restriction fragment length polymorphismbusiness030217 neurology & neurosurgeryPolymorphism Restriction Fragment Length
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Incidental findings in a series of 2500 gene panel tests for a genetic predisposition to cancer: Results and impact on patients.

2020

Abstract With next generation sequencing, physicians are faced with more complex and uncertain data, particularly incidental findings (IF). Guidelines for the return of IF have been published by learned societies. However, little is known about how patients are affected by these results in a context of oncogenetic testing. Over 4 years, 2500 patients with an indication for genetic testing underwent a gene cancer panel. If an IF was detected, patients were contacted by a physician/genetic counsellor and invited to take part in a semi-structured interview to assess their understanding of the result, the change in medical care, the psychological impact, and the transmission of results to the f…

0301 basic medicineAdultMalemedicine.medical_specialtyPatientsContext (language use)030105 genetics & heredity03 medical and health sciencesNeoplasmsGeneticsmedicineGenetic predispositionHumansGenetic Predisposition to DiseaseGenetic TestingCHEK2Genetics (clinical)Genetic testingAgedIncidental Findingsmedicine.diagnostic_testbusiness.industryCancerRegretGeneral MedicineMiddle Agedmedicine.disease030104 developmental biologyAttitudeFamily medicineAnxietyFemalemedicine.symptomReturn of resultsbusinessEuropean journal of medical genetics
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Preliminary evidence of reductive stress in human cytotoxic T cells following exercise.

2018

This study investigated immunophenotypic differences in intracellular thiol redox state of peripheral blood mononuclear cells (PBMCs) isolated from trained [ n = 9, means ± SD: age 28 ± 5 yr; (body mass index) BMI 23.2 ± 2.6 kg/m2; V̇o2max (maximal oxygen intake)56.9 ± 6.1 ml·kg−1·min−1] and recreationally active (RA, n = 11, means ± SD: age 27 ± 6 yr; BMI 24.2 ± 3.7 kg/m2; V̇o2max 45.1 ± 6.4 ml·kg−1·min−1) participants before and after a maximal aerobic exercise tolerance test. Blood samples were taken before (Pre), during (sample acquired at 70% maximum heart rate), immediately after (Post + 0), and 15 min postexercise (Post + 15). PBMCs were isolated, and reduced thiol analysis [fluores…

0301 basic medicineAdultMalemedicine.medical_specialtyPhysiologyLymphocytePopulationPeripheral blood mononuclear cellRC120003 medical and health sciencesLeukocyte Count0302 clinical medicineOxygen ConsumptionStress PhysiologicalPhysiology (medical)Internal medicinemedicineLeukocytesAerobic exerciseCytotoxic T cellHumanseducationExercisechemistry.chemical_classificationeducation.field_of_studyChemistryFlow Cytometry030104 developmental biologyEndocrinologymedicine.anatomical_structureQR180ThiolExercise TestLeukocytes MononuclearFemaleThiol redox030217 neurology & neurosurgeryCD8T-Lymphocytes CytotoxicJournal of applied physiology (Bethesda, Md. : 1985)
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Clinical implications of serum neurofilament in newly diagnosed MS patients: a longitudinal multicentre cohort study

2020

Abstract Background We aim to evaluate serum neurofilament light chain (sNfL), indicating neuroaxonal damage, as a biomarker at diagnosis in a large cohort of early multiple sclerosis (MS) patients. Methods In a multicentre prospective longitudinal observational cohort, patients with newly diagnosed relapsing-remitting MS (RRMS) or clinically isolated syndrome (CIS) were recruited between August 2010 and November 2015 in 22 centers. Clinical parameters, MRI, and sNfL levels (measured by single molecule array) were assessed at baseline and up to four-year follow-up. Findings Of 814 patients, 54.7% (445) were diagnosed with RRMS and 45.3% (369) with CIS when applying 2010 McDonald criteria (R…

0301 basic medicineAdultMalemedicine.medical_specialtyResearch paperClinical Decision-MakingIntermediate Filamentslcsh:Medicine610 Medicine & healthNewly diagnosedGeneral Biochemistry Genetics and Molecular BiologyMultiple sclerosis03 medical and health sciences0302 clinical medicineAtrophyMultiple Sclerosis Relapsing-RemittingNeurofilament ProteinsInternal medicineGermanymedicineHumansLongitudinal StudiesProspective Studiesddc:610610 Medicine & healthNeurofilament light chainlcsh:R5-920Clinically isolated syndromebusiness.industryMultiple sclerosislcsh:RMcDonald criteriaGeneral MedicineBiomarkermedicine.diseasesNfL030104 developmental biology030220 oncology & carcinogenesisCohortDisease ProgressionCommentaryBiomarker (medicine)Femalelcsh:Medicine (General)businessPredictionFunction and Dysfunction of the Nervous SystemBiomarkersCohort study
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Molecular and clinical studies in five index cases with novel mutations in the GLA gene

2016

Fabry disease is a metabolic and lysosomal storage disorder caused by the functional defect of the α-galactosidase A enzyme; this defect is due to mutations in the GLA gene, that is composed of seven exons and is located on the long arm of the X-chromosome (Xq21–22). The enzymatic deficit is responsible for the accumulation of glycosphingolipids in lysosomes of different cellular types, mainly in those ones of vascular endothelium. It consequently causes a cellular and microvascular dysfunction. In this paper, we described five novel mutations in the GLA gene, related to absent enzymatic activity and typical manifestations of Fabry disease. We identified three mutations (c.846_847delTC, p.E…

0301 basic medicineAdultMalep.R227Pnovel moutationAdolescentc.639 + 5G > TMutation MissenseBiologyLeft ventricular hypertrophy03 medical and health sciencesExonYoung Adult0302 clinical medicineSettore BIO/13 - Biologia ApplicataGeneticsmedicinefabry; novel moutationMissense mutationAlpha-galactosidase AHumansPoint MutationCornea verticillataGenetic Predisposition to DiseaseChildfabryGLA genec.846_847delTCGeneticsAlpha-galactosidasePoint mutationFabry disease; Alpha-galactosidase A; c.846_847delTC; p.E341X; p.C382X; p.R227P; c.639 + 5G > Tp.E341XGeneral MedicineMiddle Agedmedicine.diseaseMolecular biologyFabry diseaseStop codon030104 developmental biologyp.C382Xalpha-Galactosidasebiology.proteinFabry DiseaseFemalemedicine.symptom030217 neurology & neurosurgery
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Gene-environment interactions between education and body mass: Evidence from the UK and Finland

2017

More education is associated with a lower body mass index (BMI) and likelihood of being overweight. However, since a large proportion of the variation in body mass is due to genetic makeup, it has been hypothesized that education may moderate the genetic risk. We estimate main associations between (i) education, (ii) genetic risk, and (iii) interactions between education and genetic risk on BMI and the probability of being overweight in the UK and Finland. The estimates show that education is negatively associated with BMI and overweightness, and genetic risk is positively associated. However, the interactions between education and genetic risk are small and statistically insignificant. pee…

0301 basic medicineAdultMaleta520obesityHealth (social science)OverweightBiologyBody Mass Index03 medical and health sciences0302 clinical medicineLower bodyHistory and Philosophy of SciencekoulutustasoNegatively associatedmedicineHumansMass indexGenetic Predisposition to Diseaseta516030212 general & internal medicineLongitudinal StudiesGene–environment interactionGeneFinlandAged2. Zero hungerAged 80 and overeducationta511nutritional and metabolic diseasesylipainogene-environment interactionsta3142Middle AgedOverweightmedicine.diseaseObesityUnited Kingdom030104 developmental biologykoulutusEducational StatuslihavuusFemaleGene-Environment Interactionmedicine.symptomgeneettiset tekijätBody mass indexDemographySocial Science and Medicine
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Human limbal fibroblast-like stem cells induce immune-tolerance in autoreactive T lymphocytes from female patients with Hashimoto’s thyroiditis

2017

Background Due to their “natural immune privilege” and immunoregulatory properties human fibroblast-like limbal stem cells (f-LSCs) have acquired great interest as a potential tool for achieving immunotolerance. Hashimoto’s thyroiditis (HT) is the most common thyroid autoimmune disease and cause of hypothyroidism. To date, conventional hormone replacement therapy and unspecific immunosuppressive regimens cannot provide a definitive cure for HT subjects. We explored the immunosuppressant potential of human f-LSCs on circulating lymphomonocytes (PBMCs) collected from healthy donors and female HT patients. Methods We assessed the immunophenotyping of f-LSCs, both untreated and after 48 h of pr…

0301 basic medicineAdultMedicine (miscellaneous)Hashimoto DiseaseCD8-Positive T-LymphocytesInflammatory diseasesMajor histocompatibility complexBiochemistry Genetics and Molecular Biology (miscellaneous)Settore MED/13 - EndocrinologiaProinflammatory cytokineImmune tolerancelcsh:Biochemistry03 medical and health scienceschemistry.chemical_compoundHuman limbal stem cells Hashimoto’s thyroiditis Immunoregulation Tolerance induction Inflammatory diseasesImmune privilegeImmune ToleranceMedicineHumanslcsh:QD415-436Tolerance inductionCells CulturedAgedlcsh:R5-920biologybusiness.industryResearchStem CellsInterleukinImmunoregulationCarboxyfluorescein succinimidyl esterCell BiologyHashimoto’s thyroiditisFibroblastsMiddle AgedTh1 Cells030104 developmental biologychemistryImmunologybiology.proteinHuman limbal stem cellsMolecular MedicineCytokinesFemaleStem cellbusinesslcsh:Medicine (General)CD8Stem Cell Research & Therapy
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In Vivo Functional Evaluation of Tissue-Engineered Vascular Grafts Fabricated Using Human Adipose-Derived Stem Cells from High Cardiovascular Risk Po…

2016

Many preclinical evaluations of autologous small-diameter tissue-engineered vascular grafts (TEVGs) utilize cells from healthy humans or animals. However, these models hold minimal relevance for clinical translation, as the main targeted demographic is patients at high cardiovascular risk such as individuals with diabetes mellitus or the elderly. Stem cells such as adipose-derived mesenchymal stem cells (AD-MSCs) represent a clinically ideal cell type for TEVGs, as these can be easily and plentifully harvested and offer regenerative potential. To understand whether AD-MSCs sourced from diabetic and elderly donors are as effective as those from young nondiabetics (i.e., healthy) in the conte…

0301 basic medicineAdultPathologymedicine.medical_specialtyCell typeBiomedical EngineeringAdipose tissueContext (language use)Bioengineering030204 cardiovascular system & hematologyBiochemistryBiomaterials03 medical and health sciences0302 clinical medicineTissue engineeringBlood vessel prosthesisRisk FactorsDiabetes mellitusmedicineAnimalsHumansAgedBioprosthesisTissue Engineeringbusiness.industryMesenchymal stem cellMesenchymal Stem CellsOriginal ArticlesMiddle Agedmedicine.diseaseBiomaterialBlood Vessel ProsthesisRats030104 developmental biologyAdipose TissueCardiovascular DiseasesRats Inbred LewFemaleStem cellbusiness
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Viral encephalitis in Parry-Romberg syndrome

2019

A 28-year-old woman was admitted to our clinic for acute onset of headache, aphasia and confusion, followed by a first episode of generalised tonic–clonic seizures. She had a history of left-sided migraine, accompanied by visual aura and right arm dysesthesia since the age of 20 years and

0301 basic medicineAdultPediatricsmedicine.medical_specialtyImages In…030105 genetics & heredityinfection (neurology)03 medical and health sciences0302 clinical medicineSeizuresAphasiaFacial HemiatrophymedicineHumansEncephalitis ViralFirst episodeDysesthesianeuroimagingbusiness.industryViral encephalitisneurologymeningitisParry–Romberg syndromeGeneral Medicinemedicine.diseasenervous system diseasesMigraineFemaleSettore MED/26 - Neurologiamedicine.symptombusinessMeningitis030217 neurology & neurosurgeryEncephalitis
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