Search results for "Abilities"

showing 10 items of 538 documents

NF1 microdeletion syndrome: case report of two new patients

2019

Abstract Background 17q11.2 microdeletions, which include the neurofibromatosis type 1 (NF1) gene region, are responsible for the NF1 microdeletion syndrome, observed in 4.2% of all NF1 patients. Large deletions of the NF1 gene and its flanking regions are associated with a more severe NF1 phenotype than the NF1 general population. Case presentation We hereby describe the clinical and molecular features of two girls (aged 2 and 4 years, respectively), with non-mosaic atypical deletions. Patient 1 showed fifteen café-au-lait spots and axillary freckling, as well as a Lisch nodule in the left eye, strabismus, high-arched palate, malocclusion, severe kyphoscoliosis, bilateral calcaneovalgus fo…

0301 basic medicinePathologymedicine.medical_specialtycongenital hereditary and neonatal diseases and abnormalitiesGenotype-phenotype correlationNeurofibromatosesLisch noduleContiguous gene syndromePopulationCase ReportContiguous gene syndromeChromosomesCraniofacial Abnormalities03 medical and health sciences0302 clinical medicineAtypical deletionIntellectual DisabilitymedicineHumansMultiplex ligation-dependent probe amplificationNeurofibromatosiseducationChildPreschoolNeurofibromatoseseducation.field_of_studybusiness.industryLearning DisabilitiesPair 17lcsh:RJ1-570Axillary frecklinglcsh:Pediatricsmedicine.diseaseeye diseasesMLPA030104 developmental biologyNF1 geneChild PreschoolFemalemedicine.symptomChromosome DeletionbusinessAtypical deletion; Contiguous gene syndrome; Genotype-phenotype correlation; MLPA; NF1 gene; Child Preschool; Chromosome Deletion; Chromosomes Human Pair 17; Craniofacial Abnormalities; Female; Humans; Intellectual Disability; Learning Disabilities; Neurofibromatoses030217 neurology & neurosurgeryChromosomes Human Pair 17Comparative genomic hybridizationHumanItalian Journal of Pediatrics
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7q31.32 partial duplication: First report of a child with dysmorphism, autistic spectrum disorder, moderate intellectual disability and, epilepsy. Li…

2019

Abstract Introduction Duplication of long arm of chromosome 7(q) is uncommon. It may occur as “pure”, isolated anomaly or in association with other mutations involving the same or other chromosomes. “Pure” chromosome 7q duplication has recently been classified by segment involved: the interstitial, proximal, or distal segment of the arm. Attempts to correlate genotype with phenotype in each group has yielded questionable results even though intellective disability and minor dysmorphic features of variable types are typically seen. Material and Methods In a young boy showing minor facial dysmorphism, language delay, autistic spectrum disorder, epileptic seizures, behavioral disturbances and …

0301 basic medicineProbandPediatricsmedicine.medical_specialtyAutism Spectrum DisorderLanguage delayDevelopmental DisabilitiesIrritabilityChromosomes03 medical and health sciencesEpilepsy0302 clinical medicineIntellectual DisabilityGene duplicationIntellectual disabilityHumansMedicineGenetic Association StudiesChromosome 7 (human)Epilepsybusiness.industrymedicine.disease7q31.32 duplicationDysmorphism030104 developmental biologyAutistic spectrum disorderNeurologyAutism spectrum disorderPair 7Neurology (clinical)medicine.symptombusinessChromosomes Human Pair 7030217 neurology & neurosurgeryHumanEpilepsy Research
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Collective Cell Migration in a Fibrous Environment: A Hybrid Multiscale Modelling Approach

2021

International audience; The specific structure of the extracellular matrix (ECM), and in particular the density and orientation of collagen fibres, plays an important role in the evolution of solid cancers. While many experimental studies discussed the role of ECM in individual and collective cell migration, there are still unanswered questions about the impact of nonlocal cell sensing of other cells on the overall shape of tumour aggregation and its migration type. There are also unanswered questions about the migration and spread of tumour that arises at the boundary between different tissues with different collagen fibre orientations. To address these questions, in this study we develop …

0301 basic medicineStatistics and Probabilitymulti-scale hybrid mathematical modelMaterials sciencecell migration[SDV.CAN]Life Sciences [q-bio]/Cancercontinuous cell-extracellular matrix interactionsQA273-280Articlenumerical simulationsExtracellular matrix03 medical and health sciences0302 clinical medicineCollagen fibres[SDV.BC.IC]Life Sciences [q-bio]/Cellular Biology/Cell Behavior [q-bio.CB][NLIN]Nonlinear Sciences [physics][MATH]Mathematics [math]T57-57.97Applied mathematics. Quantitative methodsApplied MathematicsCollective cell migrationCell migrationTumour invasionCollagen fibre030104 developmental biologyorientation of extracellular matrix fibresagent based discrete cell-cell interactionsContinuous fieldBiological systemProbabilities. Mathematical statistics030217 neurology & neurosurgeryFrontiers in Applied Mathematics and Statistics
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Fractional hereditariness of lipid membranes: Instabilities and linearized evolution

2016

In this work lipid ordering phase changes arising in planar membrane bilayers is investigated both accounting for elas- ticity alone and for effective viscoelastic response of such assemblies. The mechanical response of such membranes is studied by minimizing the Gibbs free energy which penalizes perturbations of the changes of areal stretch and their gradients only [1]. As material instabilities arise whenever areal stretches characterizing homogeneous configurations lie inside the spinoidal zone of the free energy density, bifurcations from such configurations are shown to occur as oscillatory perturbations of the in-plane displacement. Experimental observations [2] show a power-law in-pl…

0301 basic medicineViscoelastic lipid membranePhase transitionMembrane Fluidity0206 medical engineeringLipid BilayersBiomedical EngineeringSeparation of variablesFOS: Physical sciences02 engineering and technologyviscoelastic lipid membranesCondensed Matter - Soft Condensed Matterfractional hereditary lipid membranesViscoelasticityFractional hereditary lipid membraneMaterial instabilitieBiomaterials03 medical and health sciencessymbols.namesakeFractional hereditary lipid membranes; Material instabilities; Phase transitions; Viscoelastic lipid membranes; Biomaterials; Biomedical Engineering; Mechanics of MaterialsVariational principleElasticity (economics)Phase transitionMembranesChemistryOscillationTime evolutionBiomaterial020601 biomedical engineeringElasticityGibbs free energyphase transitions030104 developmental biologyClassical mechanicsModels ChemicalMechanics of MaterialssymbolsSoft Condensed Matter (cond-mat.soft)material instabilitiesSettore ICAR/08 - Scienza Delle Costruzionifractional hereditary lipid membranes viscoelastic lipid membranes phase transitions material instabilities
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Cohen Syndrome-Associated Cataract Is Explained by VPS13B Functions in Lens Homeostasis and Is Modified by Additional Genetic Factors

2020

International audience; Purpose: Cohen syndrome (CS) is a rare genetic disorder caused by variants of the VPS13B gene. CS patients are affected with a severe form of retinal dystrophy, and in several cases cataracts also develop. The purpose of this study was to investigate the mechanisms and risk factors for cataract in CS, as well as to report on cataract surgeries in CS patients.Methods: To understand how VPS13B is associated with visual impairments in CS, we generated the Vps13b∆Ex3/∆Ex3 mouse model. Mice from 1 to 3 months of age were followed by ophthalmoscopy and slit-lamp examinations. Phenotypes were investigated by histology, immunohistochemistry, and western blot. Literature anal…

0301 basic medicinegenetic structuresDevelopmental DisabilitiesVesicular Transport Proteins030105 genetics & hereditysurgerygenetic backgroundchemistry.chemical_compoundLensMyopiaHomeostasisMice KnockoutCohen syndrome[SDV.MHEP] Life Sciences [q-bio]/Human health and pathologymedicine.diagnostic_testRetinal DegenerationGenetic disorderinflamma- tionVPS13BcataractKnockout mouseMicrocephalyMuscle Hypotoniamedicine.medical_specialtymouse modelBlotting WesternRetinitisFingersOphthalmoscopy03 medical and health sciencesCataractsIntellectual DisabilityOphthalmologyVPS13BLens CrystallinemedicineAnimalsObesityCohen syndromebusiness.industryfibrosisRetinalgenetic modifiersmedicine.diseaseeye diseasesMice Inbred C57BLDisease Models Animalophthalmology030104 developmental biologyGene Expression RegulationchemistryinflammationRNAsense organsbusiness[SDV.MHEP]Life Sciences [q-bio]/Human health and pathologyInvestigative Ophthalmology & Visual Science
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La inclusión laboral de las personas con discapacidad desde la administración pública responsable en la Comunidad Valenciana

2020

En la presente coyuntura socioeconómica, la posibilidad de inclusión a través del empleo de las personas con discapacidad es importante no solo por las posibilidades de autonomía económica, sino también por las oportunidades de socialización. Sobre esta base, y considerando que las empresas socialmente responsables asumen la inclusión de estas personas en sus plantillas como una posibilidad de crecimiento y de diferenciación, en el texto se analiza, de manera descriptiva, el papel que las administraciones públicas (AA. PP.) autonómicas, con competencias en la materia, pueden, deben y están haciendo para fomentar la responsabilidad social corporativa (RSC) y la gestión de la inclusión labora…

030506 rehabilitationDiscapacitatsdiscapacidadSocial PsychologyPromoción de empleoCalidad de vidalcsh:BF1-990Españaresponsabilidad social corporativaSolidaridadEducationPolítica social03 medical and health sciencesCoping with disabilitySector públicolcsh:HD7255-72560501 psychology and cognitive scienceslcsh:Social sciences (General)AdministraciónHealth and Family05 social scienceslcsh:Vocational rehabilitation. Employment of people with disabilitiesinclusiónHacer frente a la discapacidadFamilia y SaludPsychiatry and Mental healthempleoinserciónlcsh:PsychologyInclusión laboralTrabajoDiscapacidadlcsh:H1-99ValenciaPshychiatric Mental Health0305 other medical science050104 developmental & child psychologyEmpresa
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Do typically and atypically developing children learn and generalize novel names similarly: The role of conceptual distance during learning and at te…

2020

International audience; There is a large body of evidence showing that comparison of multiple stimuli leads to better conceptualization and generalization of novel names than no-comparison settings in typically developing (TD) children. By contrast, the evidence regarding this issue remains scarce in children with intellectual disabilities (ID). Children with intellectual disabilities (ID) and TD children matched on mental age with the Raven's coloured progressive matrices were tested in several novel name learning comparison conditions, with familiar objects. We manipulated the conceptual distance between the learning stimuli in the learning phase and between the learning and generalizatio…

030506 rehabilitationIntellectual disabilities03 medical and health sciencesRaven's Progressive MatricesCognitionComparisonsGeneralization (learning)Intellectual DisabilityIntellectual disabilityDevelopmental and Educational PsychologymedicineHumansLearningNames0501 psychology and cognitive sciencesCognitive skill10. No inequalityChildMental ageIntelligence TestsConceptualization4. Education05 social sciencesmedicine.diseaseObject and relational categoriesClinical PsychologyLanguage developmentCategorization[SCCO.PSYC]Cognitive science/Psychology0305 other medical sciencePsychologyConceptual distance050104 developmental & child psychologyCognitive psychologyResearch in developmental disabilities
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Ensuring Diverse User Experiences and Accessibility While Developing the TeSLA e-Assessment System

2019

The TeSLA project, with its new, innovative approaches for e-assessment, offers a great possibility for increasing the educational equality and making higher education studies available for all. It has been estimated that 10–15% of students in higher education institutions have some disabilities or special educational needs. At online universities or in online programmes, the number is even higher. These numbers emphasise the importance of the universal design for learning as a leading principle while developing the digital learning environments and e-assessment procedures. In this chapter, we describe the key elements of ensuring the accessibility of the TeSLA e-assessment system during th…

030506 rehabilitationKnowledge managementHigher educationkoulutusteknologiaoppiminenComputer sciencearviointimenetelmätstudent with special educational needs and disabilities (SEND student)luottamuksellisuus03 medical and health sciencesTeSLA ProjectvammaisuusUser experience designerityisopetusverkko-opiskeluuser experienceetäopetusComputingMilieux_COMPUTERSANDEDUCATIONerityiskasvatusverkkoarviointiTeSLA-projektiDigital learninge-assessmentetäopiskeluspecial educational needskäytettävyysbusiness.industry4. Education05 social sciences050301 educationUniversal Design for LearningUsabilityaccessibilityusabilityE-assessmente-arviointidisabilitysaavutettavuusKey (cryptography)Special educational needskäyttäjäkokemus0305 other medical sciencebusiness0503 educationarviointi
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Acceptability of the e-authentication in higher education studies: views of students with special educational needs and disabilities

2021

AbstractTrust-based e-assessment systems are increasingly important in the digital age for both academic institutions and students, including students with special educational needs and disabilities (SEND). Recent literature indicates a growing number of studies about e-authentication and authorship verification for quality assurance with more flexible modes of assessment. Yet understanding the acceptability of e-authentication systems among SEND students is underexplored. This study examines SEND students’ views about the use of e-authentication systems, including perceived advantages and disadvantages of new technology-enhanced assessment. This study aims to shed light on this area by exa…

030506 rehabilitationPersonal dataeducación superiorSpecial educationaccesibilidadAcceptabilitymedia_commonlcsh:LC8-6691lcsh:T58.5-58.64lcsh:Information technology4. Education05 social scienceseducació superiordades personals050301 educatione-AuthenticationAccessibilityAuthentication (law)acceptabilitatComputer Science ApplicationssaavutettavuusStudents with special educational needs and disabilitiesaccessibilitat0305 other medical sciencePsychologyResearch Articleautenticación electrónicaEducation HigherHigher educationmedia_common.quotation_subjectCheatingInternet privacyEducationerityisopiskelijat03 medical and health sciencesOriginalityaceptabilidadEnsenyament universitariHigher educationsähköinen tunnistaminendatos personaleslcsh:Special aspects of educationbusiness.industryEducational technologyestudiantes con necesidades educativas especiales y discapacidadesautenticació electrònicahenkilötiedotkorkeakouluopiskeluKey (cryptography)Enseñanza universitariaComputerized adaptive testingbusiness0503 educationestudiants amb necessitats educatives especials i discapacitats
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Developing E-Authentication for E-Assessment : Diversity of Students Testing the System in Higher Education

2020

Sähköinen tunnistautuminen on yksi keskeisistä teemoista verkko-opetuksessa, -opiskelussa ja - arvioinnissa. Tämän tutkimuksen tarkoituksena oli tutkia opiskeluunsa erityistä tukea tarvitsevien yliopisto-opiskelijoiden käyttökokemuksia kehitteillä olevasta sähköisestä tunnistautumisjärjestelmästä. Erityistä huomiota kehittämistyössä kiinnitettiin tunnistautumisen saavutettavuuteen. Kaikkiaan 15 opiskelijaa testasi TeSLA-projektin osana kehitettyä tunnistautumisjärjestelmää, johon kuului kasvojentunnistus, äänentunnistus, näppäilyntunnistus, tekstityylianalyysi ja plagioinnin tunnistus. Opiskelijat täyttivät esi- ja jälkikyselylomakkeet sekä osallistuivat henkilökohtaisiin haastatteluihin. T…

030506 rehabilitationerilaiset oppijatHigher educationmedia_common.quotation_subjectInternet privacykorkeakouluopetus03 medical and health sciencesspecial educational needs and disabilities (SEND)ComputingMilieux_COMPUTERSANDEDUCATIONsähköinen tunnistaminenverkko-opetuse-assessmentmedia_commonbusiness.industry4. Education05 social sciences050301 educationTeSLA systemverkko-oppiminenAuthentication (law)accessibilityE-assessmentsaavutettavuuse-authenticationhigher educationkäyttäjäkokemus0305 other medical sciencePsychologybusiness0503 educationDiversity (politics)
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