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showing 10 items of 4837 documents

Measuring Orthorexia Nervosa: Psychometric Limitations of the ORTO-15.

2017

AbstractOrthorexia nervosa has recently been defined as excessive preoccupation with healthy eating, causing significant nutritional deficiencies and social and personal impairments. The ORTO-15 is the most widely used instrument to evaluate orthorexia nervosa, although previous studies obtained inconsistent results about its psychometric properties, and there are no data on the Spanish version. Thus, the main objective of the present study was to analyze the psychometric properties of the Spanish adaptation of the ORTO-15. In order to cross-validate the results, two independent samples were used (Sample 1: n = 807, 74.1% women; Sample 2: n = 242, 63.2% women). The results did not support t…

0301 basic medicineAdultMale050103 clinical psychologyLinguistics and LanguagePsychometricsHealth BehaviorHealthy eatingLanguage and LinguisticsDevelopmental psychologyFeeding and Eating Disorders03 medical and health sciencesYoung AdultInternal consistencyIndependent samplesmedicineHumans0501 psychology and cognitive sciencesGeneral PsychologyOrthorexia nervosaPsychiatric Status Rating Scales030109 nutrition & dietetics05 social sciencesReproducibility of ResultsSpanish versionmedicine.diseaseSpainFemalePsychologyPsychopathologyThe Spanish journal of psychology
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Genome-wide association meta-analysis highlights light-induced signaling as a driver for refractive error

2018

Skin affections after sulfur mustard (SM) exposure include erythema, blister formation and severe inflammation. An antidote or specific therapy does not exist. Anti-inflammatory compounds as well as substances counteracting SM-induced cell death are under investigation. In this study, we investigated the benzylisoquinoline alkaloide berberine (BER), a metabolite in plants like berberis vulgaris, which is used as herbal pharmaceutical in Asian countries, against SM toxicity using a well-established in vitro approach. Keratinocyte (HaCaT) mono-cultures (MoC) or HaCaT/THP-1 co-cultures (CoC) were challenged with 100, 200 or 300 mM SM for 1 h. Post-exposure, both MoC and CoC were treated with 1…

0301 basic medicineAdultMaleCell typeResearchInstitutes_Networks_Beacons/MICRAIn silicotaittovirheetGenome-wide association studyRetinal Pigment EpitheliumBiologyBlindnessPolymorphism Single NucleotideSensory disorders Donders Center for Medical Neuroscience [Radboudumc 12]ArticleRetinaWhite People03 medical and health sciencesHIGH-GRADE MYOPIA ; RETINAL-PIGMENT EPITHELIUM ; SEROTONIN PATHWAY GENES ; FORM-DEPRIVATION MYOPIA ; COMMON VARIANTS ; OCULAR GROWTH ; RETINITIS-PIGMENTOSA ; GENOTYPE IMPUTATION ; MISSENSE MUTATIONS ; DOPAMINE-RECEPTORSAsian Peoplerefractive errorsRetinitis pigmentosaGeneticsmedicineMyopiaJournal ArticleHumansGenetic Predisposition to Disease610 Medicine & healthRegulation of gene expressionRetinaRetinal pigment epitheliummedicine.diseaseRefractive Errors030104 developmental biologymedicine.anatomical_structureManchester Institute for Collaborative Research on AgeingGene Expression Regulationgenetic factorsEye disorderFemalesense organsgeneettiset tekijätNeuroscienceGenome-Wide Association StudySignal Transduction
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Molecular diagnosis of PIK3CA-related overgrowth spectrum (PROS) in 162 patients and recommendations for genetic testing.

2017

Postzygotic activating mutations of PIK3CA cause a wide range of mosaic disorders collectively referred to as PIK3CA-related overgrowth spectrum (PROS). We describe the diagnostic yield and characteristics of PIK3CA sequencing in PROS. We performed ultradeep next-generation sequencing (NGS) of PIK3CA in various tissues from 162 patients referred to our clinical laboratory and assessed diagnostic yield by phenotype and tissue tested. We identified disease-causing mutations in 66.7% (108/162) of patients, with mutant allele levels as low as 1%. The diagnostic rate was higher (74%) in syndromic than in isolated cases (35.5%; P = 9.03 × 10−5). We identified 40 different mutations and found stro…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyAdolescentGenotypeClass I Phosphatidylinositol 3-KinasesPrenatal diagnosisBioinformaticsmedicine.disease_causeDNA sequencing03 medical and health sciencesYoung Adult0302 clinical medicinePrenatal DiagnosisGenotypemedicineHumansGenetic Predisposition to DiseaseGenetic TestingAlleleChildGenetics (clinical)AllelesGenetic Association StudiesGrowth DisordersGenetic testingMutationmedicine.diagnostic_testbusiness.industryMosaicismInfant NewbornDisease ManagementHigh-Throughput Nucleotide SequencingInfantSequence Analysis DNAPhenotype030104 developmental biologyPhenotypeAmino Acid SubstitutionChild PreschoolMutationAllelic heterogeneityFemalebusiness030217 neurology & neurosurgeryGenetics in medicine : official journal of the American College of Medical Genetics
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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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Cortical network fingerprints predict deep brain stimulation outcome in dystonia.

2018

AbstractBackgroundDeep brain stimulation (DBS) is an effective evidence-based therapy for dystonia. However, no unequivocal predictors of therapy responses exist. We investigate whether patients optimally responding to DBS present distinct brain network organization and structural patterns.MethodsBased on a German multicentre cohort of eighty-two dystonia patients with segmental and generalized dystonia, who received DBS implantation in the globus pallidus internus patients were classified based on the clinical response 36 months after DBS, as superior-outcome group or moderate-outcome group, as above or below 70% motor improvement, respectively. Fifty-one patients met MRI-quality and treat…

0301 basic medicineAdultMaleTreatment responsemedicine.medical_specialtyDeep brain stimulationMovement disordersmedicine.medical_treatmentDeep Brain Stimulation610 MedizinStimulationGrey matterGlobus PallidusSeverity of Illness IndexCohort Studies03 medical and health sciences0302 clinical medicineText miningPhysical medicine and rehabilitationAtrophy610 Medical sciencesmedicineHumansddc:610Dystoniabusiness.industryStructural integrityMiddle Agedmedicine.diseasenervous system diseasesDystoniamedicine.anatomical_structure030104 developmental biologyNeurologyCortical networkDystonic DisordersCohortFemaleNeurology (clinical)medicine.symptombusiness030217 neurology & neurosurgeryMovement disorders : official journal of the Movement Disorder SocietyReferences
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When do myopia genes have their effect? Comparison of genetic risks between children and adults

2016

Item does not contain fulltext Previous studies have identified many genetic loci for refractive error and myopia. We aimed to investigate the effect of these loci on ocular biometry as a function of age in children, adolescents, and adults. The study population consisted of three age groups identified from the international CREAM consortium: 5,490 individuals aged 25 years. All participants had undergone standard ophthalmic examination including measurements of axial length (AL) and corneal radius (CR). We examined the lead SNP at all 39 currently known genetic loci for refractive error identified from genome-wide association studies (GWAS), as well as a combined genetic risk score (GRS). …

0301 basic medicineAdultMalemedicine.medical_specialtyBiometryAdolescentGenotypeEpidemiologySingle-nucleotide polymorphismGenome-wide association studyBiologyPolymorphism Single NucleotideConnexinsSensory disorders Donders Center for Medical Neuroscience [Radboudumc 12]03 medical and health sciencesYoung Adult0302 clinical medicineRisk FactorsInternal medicineGenotypemedicineMyopiaSNPHumansAlleleYoung adult610 Medicine & healthChildGenetics (clinical)AllelesGenetic associationGenetics030104 developmental biologyGenetic Loci030221 ophthalmology & optometryPopulation studyFemaleRELamininGenome-Wide Association Study
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Neuropeptide Y (NPY) in cerebrospinal fluid from patients with Huntington's Disease: increased NPY levels and differential degradation of the NPY1-30…

2016

Huntington's disease (HD) is an inherited and fatal polyglutamine neurodegenerative disorder caused by an expansion of the CAG triplet repeat coding region within the HD gene. Progressive dysfunction and loss of striatal GABAergic medium spiny neurons (MSNs) may account for some of the characteristic symptoms in HD patients. Interestingly, in HD, MSNs expressing neuropeptide Y (NPY) are spared and their numbers is even up-regulated in HD patients. Consistent with this, we report here on increased immuno-linked NPY (IL-NPY) levels in human cerebrospinal fluid (hCSF) from HD patients (Control n = 10; early HD n = 9; mid HD n = 11). As this antibody-based detection of NPY may provide false pos…

0301 basic medicineAdultMalemedicine.medical_specialtyCathepsin DDynorphinMedium spiny neuronBiochemistry03 medical and health sciencesCellular and Molecular NeuroscienceMice0302 clinical medicineCerebrospinal fluidHuntington's diseaseInternal medicinemental disordersmedicineAnimalsHumansNeuropeptide YNeprilysinAgedThimet oligopeptidaseChemistryMiddle Agedmedicine.diseaseNeuropeptide Y receptorPeptide FragmentsRats030104 developmental biologyEndocrinologyHEK293 CellsHuntington DiseaseProteolysisFemale030217 neurology & neurosurgeryBiomarkersJournal of neurochemistry
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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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New approach to generating insights for aging research based on literature mining and knowledge integration.

2017

The proportion of the elderly population in most countries worldwide is increasing dramatically. Therefore, social interest in the fields of health, longevity, and anti-aging has been increasing as well. However, the basic research results obtained from a reductionist approach in biology and a bioinformatic approach in genome science have limited usefulness for generating insights on future health, longevity, and anti-aging-related research on a case by case basis. We propose a new approach that uses our literature mining technique and bioinformatics, which lead to a better perspective on research trends by providing an expanded knowledge base to work from. We demonstrate that our approach …

0301 basic medicineAgingAging and Cancerlcsh:MedicineOtologyDeafnessBioinformaticsBiochemistryField (computer science)Database and Informatics MethodsOxidative DamageKnowledge integrationBasic researchDrug DiscoveryMedicine and Health SciencesData MiningPost-Translational Modificationlcsh:ScienceHearing DisordersEnergy-Producing OrganellesGlycationReductionismMultidisciplinaryCancer Risk FactorsMitochondriaKnowledgeOncologyKnowledge baseSocial interestCellular Structures and OrganellesInformation TechnologyResearch ArticleComputer and Information SciencesDrug Research and DevelopmentBioinformaticsBioenergeticsBiologyResearch and Analysis Methods03 medical and health sciencesLead (geology)Research basedHumansPharmacologybusiness.industrylcsh:RBiology and Life SciencesProteinsCell BiologyData science030104 developmental biologyOtorhinolaryngologylcsh:QReactive Oxygen SpeciesbusinessPLoS ONE
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Aging-Related Disorders and Mitochondrial Dysfunction: A Critical Review for Prospect Mitoprotective Strategies Based on Mitochondrial Nutrient Mixtu…

2020

A number of aging-related disorders (ARD) have been related to oxidative stress (OS) and mitochondrial dysfunction (MDF) in a well-established body of literature. Most studies focused on cardiovascular disorders (CVD), type 2 diabetes (T2D), and neurodegenerative disorders. Counteracting OS and MDF has been envisaged to improve the clinical management of ARD, and major roles have been assigned to three mitochondrial cofactors, also termed mitochondrial nutrients (MNs), i.e., alpha-lipoic acid (ALA), Coenzyme Q10 (CoQ10), and carnitine (CARN). These cofactors exert essential-and distinct-roles in mitochondrial machineries, along with strong antioxidant properties. Clinical trials have mostly…

0301 basic medicineAgingAntioxidantUbiquinonemedicine.medical_treatmentmitochondrial nutrientsReviewoptic neuropathiesType 2 diabetesPharmacologyMitochondrionmedicine.disease_causeAntioxidantslcsh:Chemistrychemistry.chemical_compound0302 clinical medicineCardiovascular Diseaseoxidative stressaging-related disorderslcsh:QH301-705.5SpectroscopyThioctic AcidMitochondrial nutrientNeurodegenerative DiseasesGeneral MedicineComputer Science ApplicationsMitochondriaCardiovascular DiseasesAntioxidantmedicine.drugHumanCatalysisAging-related disorderCell LineInorganic Chemistry03 medical and health sciencesCarnitinemedicineAnimalsHumansMicrobiomeCarnitinePhysical and Theoretical ChemistryMolecular BiologyCoenzyme Q10business.industryAnimalOrganic ChemistryOxidative Stremedicine.diseaseClinical trial030104 developmental biologylcsh:Biology (General)lcsh:QD1-999chemistryDiabetes Mellitus Type 2MicrobiomeOptic neuropathiebusinessMitochondrial dysfunction030217 neurology & neurosurgeryOxidative stress
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