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showing 10 items of 7948 documents

Antibiotic treatment of infections caused by carbapenem-resistant Gram-negative bacilli: an international ESCMID cross-sectional survey among infecti…

2018

ESGAP, ESGBIS, ESGIE and the CRGNB treatment survey study group.

0301 basic medicineAcinetobacter baumanniiCarbapenemAntibioticsDrug ResistanceDrug resistanceTigecyclineAcinetobacter baumannii; Carbapenem; Carbapenem-resistant Gram-negative bacilli; Combination therapy; Enterobacteriaceae; Polymyxin; Pseudomonas aeruginosa; SurveyPolymyxin0302 clinical medicineSurveys and Questionnairespolycyclic compounds030212 general & internal medicineAcinetobacter baumannii; Carbapenem; Carbapenem-resistant Gram-negative bacilli; Combination therapy; Enterobacteriaceae; Polymyxin; Pseudomonas aeruginosa; Survey; Anti-Bacterial Agents; Carbapenems; Cross Infection; Cross-Sectional Studies; Drug Resistance Bacterial; Gram-Negative Bacteria; Gram-Negative Bacterial Infections; Hospitals; Humans; Microbial Sensitivity Tests; Surveys and QuestionnairesSurveyCarbapenemAcinetobacter baumannii; Carbapenem; Carbapenem-resistant Gram-negative bacilli; Combination therapy; Enterobacteriaceae; Polymyxin; Pseudomonas aeruginosa; Survey; Anti-Bacterial Agents; Carbapenems; Cross Infection; Cross-Sectional Studies; Drug Resistance Bacterial; Gram-Negative Bacteria; Gram-Negative Bacterial Infections; Hospitals; Humans; Microbial Sensitivity Tests; Surveys and Questionnaires; Microbiology (medical); Infectious DiseasesCross InfectionbiologyMicrobial Sensitivity TestCarbapenem-resistant Gram-negative bacilliBacterialantibiotic management carbapenem-resistant Gram-negative bacteriaGeneral MedicineHospitals3. Good healthAcinetobacter baumanniiAnti-Bacterial AgentsInfectious DiseasesPseudomonas aeruginosamedicine.drugHumanMicrobiology (medical)medicine.medical_specialtymedicine.drug_class030106 microbiologyMicrobial Sensitivity TestsFosfomycincarbapenem-resistant Gram-negative bacteria03 medical and health sciencesHospitalEnterobacteriaceaeInternal medicineAnti-Bacterial AgentDrug Resistance BacterialGram-Negative BacteriamedicineGram-Negative Bacterial InfectionHumansCombination therapyCross-Sectional Studiebusiness.industrybiochemical phenomena metabolism and nutritionbiology.organism_classificationbacterial infections and mycosesCross-Sectional StudiesCarbapenemsInfectious disease (medical specialty)Carbapenem-resistant gram-negative bacilli[SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologieantibiotic managementbusinessGram-Negative Bacterial InfectionsRifampicin
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Type of chromosome abnormality affects embryo morphology dynamics.

2016

Objective To study the differences in the cleavage time between types of embryo chromosomal abnormalities and elaborate algorithm to exclude aneuploid embryos according to the likelihood to be euploid. Design Retrospective cohort study. Setting University affiliated private center. Patient(s) Preimplantational genetic screening patients (n = 112) including cases of advanced maternal age, repeated implantation failure, and recurrent miscarriage. A total of 485 embryos were analyzed. Intervention(s) None. Main Outcome Measure(s) All biopsied embryos were cultured in an incubator with time-lapse technology, cleavage timing from insemination to day 3 and all kinetic parameters that have been de…

0301 basic medicineAdultBiopsyAneuploidyEmbryonic DevelopmentChromosome DisordersFertilization in VitroBiologyTime-Lapse ImagingAndrology03 medical and health sciences0302 clinical medicinePredictive Value of TestsPregnancyRisk FactorsRecurrent miscarriagemedicineOdds RatioChromosomes HumanHumansAdvanced maternal ageGenetic TestingPreimplantation DiagnosisRetrospective StudiesGeneticsChromosome AberrationsComparative Genomic Hybridization030219 obstetrics & reproductive medicineMicroscopy VideoObstetrics and GynecologyRetrospective cohort studyEmbryoOdds ratiomedicine.diseaseAneuploidyConfidence intervalKinetics030104 developmental biologyBlastocystLogistic ModelsReproductive Medicineembryonic structuresChromosome abnormalityFemaleFertility and sterility
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What is the impact of a novel MED12 variant on syndromic conotruncal heart defects? Analysis of case report on two male sibs

2020

Abstract Background Syndromic congenital heart disease accounts for 30% of cases and can be determined by genetic, environmental or multifactorial causes. In many cases the etiology remains uncertain. Many known genes are responsible for specific morphopathogenetic mechanisms during the development of the heart whose alteration can determine specific phenotypes of cardiac malformations. Case presentation We report on two cases of association of conotruncal heart defect with facial dysmorphisms in sibs. In both cases the malformations’ identification occurred by ultrasound in the prenatal period. It was followed by prenatal invasive diagnosis. The genetic analysis revealed no rearrangements …

0301 basic medicineAdultHeart Defects CongenitalMaleHeart diseaseFacial dysmorphismCase ReportGenetic analysisFacial dysmorphismsCongenital heart diseases030218 nuclear medicine & medical imagingConotruncal heart defectsMED1203 medical and health sciences0302 clinical medicinePregnancyNext generation sequencingPrenatal DiagnosismedicineHumansGenetic TestingGeneX chromosomeConotruncal heart defectsCongenital heart diseaseGeneticsMediator Complexbusiness.industrylcsh:RJ1-570lcsh:Pediatricsmedicine.diseasePhenotypeMED12Fetal Diseases030104 developmental biologyConotruncal heart defectEchocardiographyEtiologyFemalebusinessItalian Journal of Pediatrics
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Real-Life Study for the Diagnosis of House Dust Mite Allergy - The Value of Recombinant Allergen-Based IgE Serology.

2016

<b><i>Background:</i></b><i>Dermatophagoides pteronyssinus</i> is one of the most important perennial allergen sources worldwide. Molecular diagnostics using the commercially available major allergens (Der p 1 and Der p 2) in combination with Der p 10 do not detect house dust mite (HDM) sensitization in a number of cases when used alone. The objective was to evaluate the IgE reactivity profiles of these patients using an experimental immunoassay biochip. <b><i>Methods: </i></b>Sera of HDM-allergic patients (positive skin prick test, CAP class ≥1 for allergen extract, and positive intranasal provocation) were tested for IgE antibodi…

0301 basic medicineAdultMaleAdolescentImmunologymedicine.disease_causeImmunoglobulin ESerologylaw.invention03 medical and health sciencesYoung Adult0302 clinical medicineAllergenlawmedicineHypersensitivityImmunology and AllergyAnimalsHumansSerologic TestsAntigens DermatophagoidesChildAgedbiologybusiness.industryHouse dust mite allergyPyroglyphidaeGeneral MedicineAllergensImmunoglobulin EMiddle Aged030104 developmental biology030228 respiratory systemImmunologybiology.proteinRecombinant DNAFemalebusinessLife studyInternational archives of allergy and immunology
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Implicitly and explicitly assessed anxiety: No relationships with recognition of and brain response to facial emotions.

2019

Abstract Trait anxiety, the disposition to experience anxiety, is known to facilitate perception of threats. Trait anxious individuals seem to identify threatening stimuli such as fearful facial expressions more accurately, especially when presented under temporal constraints. In past studies on anxiety and emotion face recognition, only self-report or explicit measures of anxiety have been administered. Implicit measures represent indirect tests allowing to circumvent problems associated with self-report. In our study, we made use of implicit in addition to explicit measures to investigate the relationships of trait anxiety with recognition of and brain response to emotional faces. 75 heal…

0301 basic medicineAdultMaleBeck Anxiety InventoryEmotionsAnxietyAffect (psychology)Facial recognition systemAssociation03 medical and health sciencesYoung Adult0302 clinical medicinemedicineHumansFacial expressionmedicine.diagnostic_testGeneral NeuroscienceImplicit-association testBrainMagnetic Resonance ImagingFacial Expression030104 developmental biologyVisual PerceptionAnxietyFemalemedicine.symptomFunctional magnetic resonance imagingPsychologyFacial Recognition030217 neurology & neurosurgeryState-Trait Anxiety InventoryCognitive psychologyNeuroscience
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Early effects of fluoro-edenite: correlation between IL-18 serum levels and pleural and parenchymal abnormalities.

2016

Fluoro-edenite (FE) is a natural mineral asbestos-like fibrous species first isolated in Biancavilla, Sicily. In order to clarify potential involvement of IL-18 in the pathogenesis of FE-induced chest abnormalities, we analyzed IL-18 serum levels in FE-exposed workers (FEEW) and correlated them with pleural and parenchymal abnormalities. A total of 21 FEEWs, residing in Biancavilla for >30 years, with a working seniority of 17 ± 6.1 years were examined. High-resolution computed tomography scans revealed low grade of fibrosis in 8 (38%) FEEWs, and pleural plaques (PPs) in 13 (62%) FEEWs. The mean IL-18 level was 203.13 ± 90.43 pg/ml. Pearson correlation showed a significant association (…

0301 basic medicineAdultMaleCancer ResearchPathologymedicine.medical_specialtyFluoro-edeniteComputed tomographyNatural mineralPathogenesis03 medical and health sciences0302 clinical medicineFibrosisRisk FactorsOccupational ExposureParenchymamedicineHumansIL-18; asbestos; natural carcinogenic fibermedicine.diagnostic_testbusiness.industryAsbestos AmphiboleInterleukin-18General MedicineMiddle AgedPleural Diseasesmedicine.diseaseRespiratory Function Tests030104 developmental biologyOncology030220 oncology & carcinogenesisInterleukin 18FemalebusinessLung Diseases InterstitialTomography Spiral ComputedIL-18
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Reducing diagnostic turnaround times of exome sequencing for families requiring timely diagnoses

2017

IF 2.137; International audience; BACKGROUND AND OBJECTIVE:Whole-exome sequencing (WES) has now entered medical practice with powerful applications in the diagnosis of rare Mendelian disorders. Although the usefulness and cost-effectiveness of WES have been widely demonstrated, it is essential to reduce the diagnostic turnaround time to make WES a first-line procedure. Since 2011, the automation of laboratory procedures and advances in sequencing chemistry have made it possible to carry out diagnostic whole genome sequencing from the blood sample to molecular diagnosis of suspected genetic disorders within 50 h. Taking advantage of these advances, the main objective of the study was to impr…

0301 basic medicineAdultMaleExome sequencingmedicine.medical_specialtyTime FactorsAdolescentGenetic counselingBioinformaticsTurnaround timeSensitivity and SpecificityUndiagnosed genetic conditions03 medical and health sciencesGeneticsmedicineHumansExomeGenetic TestingMedical diagnosisIntensive care medicineChildExomeGenetics (clinical)Exome sequencingGenetic testingWhole genome sequencing[SDV.GEN]Life Sciences [q-bio]/Geneticsmedicine.diagnostic_testbusiness.industryInfant NewbornInfantGeneral MedicineSequence Analysis DNADiagnostic turnaround time3. Good healthClinical trial030104 developmental biologyEarly DiagnosisChild PreschoolFemalebusiness[ SDV.GEN ] Life Sciences [q-bio]/Genetics
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Genetics of Wilson disease and Wilson-like phenotype in a clinical series from eastern Spain.

2019

Wilson's disease (WD) is an autosomal recessive disorder caused by ATP7B mutations. Subjects with only one mutation may show clinical signs and individuals with biallelic changes may remain asymptomatic. We aimed to achieve a conclusive genetic diagnosis for 34 patients clinically diagnosed of WD. Genetic analysis comprised from analysis of exons to WES (whole exome sequencing), including promoter, introns, UTRs (untranslated regions), besides of study of large deletions/duplications by MLPA (multiplex ligation-dependent probe amplification). Biallelic ATP7B mutations were identified in 30 patients, so that four patients were analyzed using WES. Two affected siblings resulted to be compound…

0301 basic medicineAdultMaleNerve Tissue Proteins030105 genetics & heredityBiologymedicine.disease_causeCompound heterozygosityGenetic analysis03 medical and health sciencesExonHepatolenticular DegenerationExome SequencingGeneticsmedicineHumansGenetic Predisposition to DiseaseMultiplex ligation-dependent probe amplificationGenetic TestingGenetics (clinical)Exome sequencingGeneticsMutationExonsmedicine.diseaseWilson's disease030104 developmental biologyPhenotypeCopper-Transporting ATPasesSpainMutationFemaleCongenital disorder of glycosylationClinical geneticsREFERENCES
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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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The largest caucasian kindred with dentatorubral-pallidoluysian atrophy: A founder mutation in italy

2019

BACKGROUND: Dentatorubral-pallidoluysian atrophy is a hereditary neurodegenerative disease prevalently reported in Japan but rare in Caucasians. The objective of this study was to reconstruct the pedigree of Italian dentatorubral-pallidoluysian atrophy familial cases describing their clinical features. METHODS: We investigated 6 apparently unrelated dentatorubral-pallidoluysian atrophy families comprising a total of 51 affected individuals: 13 patients were clinically examined, and for 38 patients clinical data were collected from clinical sources. The dentatorubral-pallidoluysian atrophy diagnosis was genetically confirmed in 18 patients. Genealogical data from historical archives were ana…

0301 basic medicineAdultMalePediatricsmedicine.medical_specialtyAdolescentDiseaseNeuropsychological TestsWhite People03 medical and health sciencesEpilepsyYoung Adult0302 clinical medicineAtrophyTrinucleotide Repeatsdentatorubral-pallidoluysian atrophymedicineHumansFamilyATN1 geneChildFounder mutationAgedDentatorubral-pallidoluysian atrophyEpilepsybusiness.industrygenealogical methodMiddle Agedmedicine.diseaseMyoclonic Epilepsies ProgressivePedigree030104 developmental biologyfounder effectNeurologyCerebellar cognitive affective syndromeItalycerebellar cognitive-affective syndromeMutationFemaleNeurology (clinical)business030217 neurology & neurosurgeryFounder effect
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