Search results for "Neon"

showing 10 items of 760 documents

Q289P mutation in the FGFR2 gene: first report in a patient with type 1 Pfeiffer syndrome.

2008

When normal development and growth of the calvarial sutures is disrupted, craniosynostosis (premature calvarial suture fusion) may result. Classical craniosynostosis syndromes are autosomal dominant traits and include Apert, Pfeiffer, Crouzon, Jackson-Weiss, and Saethre-Chotzen syndromes. In these conditions, there is premature fusion of skull bones leading to an abnormal head shape, ocular hypertelorism with proptosis, and midface hypoplasia. It is known that mutations in the fibroblast growth factor receptors 1, 2, and 3 cause craniosynostosis. We report on a child with a clinically diagnosed Pfeiffer syndrome that shows the missense point mutation Q289P in exon 8 of the FGFR2 gene. This …

Malemusculoskeletal diseasescongenital hereditary and neonatal diseases and abnormalitiesPathologymedicine.medical_specialtyCraniosynostosisSettore MED/38 - Pediatria Generale E SpecialisticaHumansPoint MutationMedicineMissense mutationReceptor Fibroblast Growth Factor Type 2HypertelorismGeneticsFibrous jointbusiness.industryFibroblast growth factor receptor 2Craniofacial DysostosisInfantDysostosisExonsAcrocephalosyndactyliamedicine.diseaseSkullPhenotypemedicine.anatomical_structurePfeiffer - Crouzon - Apert - Craniosynostosis - Finger and toes abnormalities - Fibroblast growth factor receptorPediatrics Perinatology and Child HealthPfeiffer syndromeFemalemedicine.symptombusiness
researchProduct

Duchenne muscular dystrophy and idiopathic hyperCKemia segregating in a family

1995

A 7-month-old boy with gross motor delay and failure to thrive presented with rhabdomyolysis following an acute asthmatic episode. During hospitalization an electrocardiographic conversion to a Wolff-Parkinson-White type 1 (WPW) pattern took place. Duchenne muscular dystrophy (DMD) was suspected based on elevated creatine kinase (CK) serum levels, muscle biopsy, and family history. The diagnosis was confirmed by molecular analysis, which documented a deletion corresponding to cDNA probe 1-2a in the dystrophin gene, in the propositus and in an affected male cousin of his mother. "Idiopathic" hyperCKemia was found in the propositus, his father, and 5 of his relatives. We suggest that the unus…

Malemusculoskeletal diseasescongenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtyDuchenne muscular dystrophyMolecular Sequence DataGene mutationPolymerase Chain ReactionMuscular DystrophiesGenomic ImprintingPrenatal DiagnosisInternal medicinemedicineHumansFamily historyCreatine KinaseGenetics (clinical)X-linked recessive inheritanceDNA PrimersGenes DominantMuscle biopsyBase Sequencebiologymedicine.diagnostic_testGenetic Carrier ScreeningInfantExonsmedicine.diseasePedigreeEndocrinologyMutationFailure to thrivebiology.proteinFemaleCreatine kinasemedicine.symptomDystrophinMetabolism Inborn ErrorsAmerican Journal of Medical Genetics
researchProduct

Oculoectodermal syndrome: Report of a new case with a broad clinical spectrum

2014

Oculoectodermal syndrome (OMIM 600268) is rare and characterized by aplasia cutis congenita, epibulbar dermoids, and other abnormalities. We report herein on a newly recognized patient with oculoectodermal syndrome, which is the 19th reported patient with OES. The boy aged six years demonstrated a broad clinical spectrum of this condition, including aplasia cutis congenita, epibulbar dermoids, hyperkeratotic papule, mildly enlarged cisterna magna, and an enlarged fluid space in the quadrigeminal cistern, suggesting a cyst. He also manifested anomalies not reported associated with this disorder, including systematized epidermal nevus following Blaschko's lines, hypopigmented skin lesions, an…

Malemusculoskeletal diseasescongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyAplasia cutis congenitaHypopigmented skin lesionsEctodermal DysplasiaOculoectodermal syndromeGeneticsmedicineHumansCystGenetics (clinical)Dermoid CystSkinHyperkeratotic papuleHypopigmentationSystematized epidermal nevusbusiness.industryEpibulbar dermoidsBrainmedicine.diseaseMagnetic Resonance ImagingDermatologyEnlarged cisterna magnaPhenotypeChild Preschoolmedicine.symptombusinessAmerican Journal of Medical Genetics Part A
researchProduct

Viral Infections in Neonates with Suspected Late-Onset Bacterial Sepsis—A Prospective Cohort Study

2016

Objective The aim of our study was to evaluate the occurrence of viral infections in infants with suspected late-onset bacterial sepsis in a neonatal intensive care unit. Methods In a prospective study, infants with suspected late-onset bacterial sepsis underwent viral testing alongside routine blood culture sampling. Using a multiplex reverse transcription-polymerase chain reaction enzyme-linked immunosorbent assay, nasopharyngeal aspirates were analyzed for adenovirus, respiratory syncytial virus (RSV), influenza virus A and B, H1N1 virus, parainfluenza virus 1 to 4, metapneumovirus, coronavirus, and picornavirus. Stools were examined for adenovirus, rotavirus, norovirus, and enterovirus.…

MalevirusesBacteremiamedicine.disease_causeLate Onset DisordersAdenovirus Infections HumanCohort StudiesFeces0302 clinical medicineRotavirusGermanyNasopharynxBlood culture030212 general & internal medicineProspective StudiesCoronavirusCaliciviridae InfectionsParamyxoviridae InfectionsNeonatal sepsismedicine.diagnostic_testReverse Transcriptase Polymerase Chain ReactionObstetrics and Gynecologyvirus diseasesVirus DiseasesOriginal ArticleFemaleNeonatal SepsisCoronavirus InfectionsEnzyme-Linked Immunosorbent AssayvirusRespiratory Syncytial Virus InfectionsRotavirus InfectionsSepsis03 medical and health sciences030225 pediatricsIntensive Care Units NeonatalInfluenza HumanmedicineEnterovirus InfectionsHumanslate-onset bacterial sepsisPicornaviridae Infectionsbusiness.industryInfant Newbornmedicine.diseaseVirologyneonatal intensive care unitinfectionBlood CultureBacteremiaPediatrics Perinatology and Child HealthImmunologyNorovirusEnterovirusbusinessMultiplex Polymerase Chain ReactionAmerican Journal of Perinatology
researchProduct

Human newborns match tongue protrusion of disembodied human and robotic mouths

2011

International audience; No evidence had been provided so far of newborns' capacity to give a matching response to 2D stimuli. We report evidence from 18 newborns who were presented with three types of stimuli on a 2D screen. The stimuli were video-recorded displays of tongue protrusion shown by: (a) a human face, (b) a human tongue from a disembodied mouth, and (c) an artificial tongue from a robotic mouth. Compared to a baseline condition, neonates increased significantly their tongue protrusion when seeing disembodied human and artificial tongue movements, but not when seeing a 2D full-face protruding tongue. This result was interpreted as revealing the exploration of top-heavy patterns o…

Maleyoung infant[ SDV.AEN ] Life Sciences [q-bio]/Food and NutritionperceptionexplorationimitationTongueneonatal imitation[SDV.IDA]Life Sciences [q-bio]/Food engineeringHumans[SPI.GPROC]Engineering Sciences [physics]/Chemical and Process EngineeringpreferenceMouthGesturesmatchingnéonatalInfant NewbornRoboticsautomatic imitationNewbornImitative Behavior[SDV.AEN] Life Sciences [q-bio]/Food and NutritionFaceVisual PerceptiongestureFemalemovementartificiel[SDV.AEN]Life Sciences [q-bio]/Food and NutritionNouveau né humainmimicry
researchProduct

High-accuracy mass measurements on neutron deficient neon isotopes

2005

International audience; The atomic masses of the short-lived nuclides 17Ne and 19Ne have been measured with the triple-trap mass spectrometer ISOLTRAP at ISOLDE/CERN. The obtained mass excess for both nuclides deviates significantly from the literature value, in the case of 17Ne about 40 keV. The mass value of 17Ne can be applied for a test of the isobaric multiplet mass equation with respect to an isospin T = 3/2 quartet. In addition, both masses can contribute to the data analysis of collinear laser-spectroscopy experiments where mean-square nuclear-charge radii are determined.

Mass excessNuclear Theorychemistry.chemical_element[PHYS.NEXP]Physics [physics]/Nuclear Experiment [nucl-ex]01 natural sciences7. Clean energyISOLTRAPNuclear physicsNeonnuclei with mass number 6 to 190103 physical sciencesNuclideneon010306 general physicsNuclear ExperimentPhysicsmass spectrometers010308 nuclear & particles physicsneutronsAtomic massMass formulaMass21.10.Dr 27.20.+n 29.30.-hIsotopes of neonchemistrynuclear massAtomic physics
researchProduct

Vibrational spectroscopy of trans and cis deuterated formic acid (HCOOD): Anharmonic calculations and experiments in argon and neon matrices

2010

The absorption spectra of trans and cis conformers of deuterated formic acid (HCOOD) isolated in argon and neon matrices are analyzed in the mid-infrared and near-infrared spectral regions (7900–450 cm � 1 ). Vibrational excitation by narrow-band IR radiation is used to convert the lower-energy trans conformer to the higher-energy cis form. A large number of overtone and combination bands are identified. The results of anharmonic vibrational calculations (CC-VSCF) for both conformers are reported and compared to the experimental spectra.

Materials scienceAbsorption spectroscopyFormic acidOvertoneeducation116 Chemical sciencesInfrared spectroscopychemistry.chemical_element010402 general chemistry01 natural scienceschemistry.chemical_compoundNeon0103 physical sciencesPhysics::Atomic and Molecular ClustersPhysics::Chemical PhysicsPhysical and Theoretical ChemistryConformational isomerismAstrophysics::Galaxy AstrophysicsSpectroscopyQuantitative Biology::BiomoleculesArgon010304 chemical physicsMatrix isolationAtomic and Molecular Physics and Optics0104 chemical scienceschemistryPhysical chemistryJournal of Molecular Spectroscopy
researchProduct

Essential features of optical processes in neon-buffered submicron-thin Rb vapor cell

2010

A new submicron thin cell (STC) filled with Rb and neon gas is developed and comparison of resonant absorption with STC containing pure Rb is provided. The effect of collapse and revival of Dicke-type narrowing is still observable for the thickness L = lambda /2 and L = lambda , where lambda is a resonant laser wavelength 794 nm (D(1) line). For an ordinary Rb cm-size cell with addition of buffer gas, the velocity selective optical pumping/saturation (VSOP) resonances in saturated absorption spectra are fully suppressed if neon pressure0.5 Torr. A spectacular difference is that for L = lambda , VSOP resonances are still observable even when neon pressure isor = 6 Torr. Narrow fluorescence s…

Materials scienceAbsorption spectroscopy[ PHYS.QPHY ] Physics [physics]/Quantum Physics [quant-ph]Buffer gaschemistry.chemical_element01 natural sciencesOCIS : 300.6210 ; OCIS : 020.1670law.invention010309 opticsOptical pumpingNeonOptics[PHYS.QPHY]Physics [physics]/Quantum Physics [quant-ph]law0103 physical sciencesBuffer GasAtomic and molecular physics010306 general physicsSpectroscopySpectroscopybusiness.industryLaserRubidiumAtomic and Molecular Physics and OpticsWavelengthchemistryTorrNanocellAtomic physicsbusiness
researchProduct

The SMILETRAP facility

1995

The SMILETRAP experimental set-up, a Penning trap mass spectrometer for highly charged ions, is described. Capture and observation of cyclotron frequencies of externally produced highly charged ions, rapid interchange of investigated and reference ions and measurements of the rotational kinetic energies are demonstrated. Mass measurements utilizing different charge states and species to verify the consistency of the measurements are presented. A relative uncertainty of about 10−9 is attained in comparisons between highly charged carbon, nitrogen, oxygen, neon and the singly charged hydrogen molecule.

Materials scienceSpectrometerCyclotronchemistry.chemical_elementCondensed Matter PhysicsKinetic energyPenning trapMass spectrometryAtomic and Molecular Physics and OpticsIonlaw.inventionNeonchemistrylawMeasuring instrumentAtomic physicsMathematical PhysicsPhysica Scripta
researchProduct

A comparison of three different micro-tomography systems for accurate determination of microvascular parameters

2008

The investigation of micro-vessel dimensions in 3D is currently problematic due to their complex structures and fine scale. Quantification of vascular parameters is important in several fields of biomedicine; including embryogenesis, wound healing, diseases characterized by uncontrolled angiogenesis (e.g. tumor growth and metastasis) and the development of implantable bio-materials where a functional vascular supply is critical to their successful integration into host tissue. However, techniques that can resolve the micron-scaled features of these capillary beds, such as scanning electron and confocal microscopy, do not allow for total image reconstitution in 3 D in thick tissue samples [1…

Materials sciencemedicine.diagnostic_testbusiness.industryResolution (electron density)Neonatal mouseMicro tomographyHost tissuelaw.inventionOpticsConfocal microscopylawmedicineTumor growthOptical tomographybusinessCorrosion CastingBiomedical engineeringDevelopments in X-Ray Tomography VI
researchProduct