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showing 10 items of 760 documents

International BEAT-PCD Consensus Statement for Infection Prevention and Control for Primary Ciliary Dyskinesia in collaboration with ERN-LUNG PCD Cor…

2021

Introduction In primary ciliary dyskinesia (PCD) impaired mucociliary clearance leads to recurrent airway infections and progressive lung destruction, and concern over chronic airway infection and patient-to-patient transmission is considerable. So far, there has been no defined consensus on how to control infection across centres caring for patients with PCD. Within the BEAT-PCD network, COST Action and ERS CRC together with the ERN-Lung PCD core a first initiative has now been taken towards creating such a consensus statement. Methods A multidisciplinary international PCD expert panel was set up to create a consensus statement for infection prevention and control (IP&C) for PCD, covering …

:Otros calificadores::Otros calificadores::/prevención & control [Otros calificadores]Infeccions respiratòries en els infantsPrimary Ciliary DyskinesiaStatement (logic)Respiratory SystemMULTICENTERRECOMMENDATIONS0302 clinical medicineOriginal Research ArticlesPandemicSTENOTROPHOMONAS-MALTOPHILIAInfection control:Pathological Conditions Signs and Symptoms::Pathologic Processes::Disease Attributes::Chronic Disease [DISEASES]030212 general & internal medicinePrimary ciliary dyskinesiaMalalties transmissibles - Transmissió:Environmental Health::Health::Environmental Illness::Communicable Diseases::Communicable Disease Control [PUBLIC HEALTH]FOUNDATIONbiologyTransmission (medicine)R:Congenital Hereditary and Neonatal Diseases and Abnormalities::Congenital Abnormalities::Abnormalities Multiple::Ciliopathies::Congenital Hereditary and Neonatal Diseases and Abnormalities::Ciliary Motility Disorders [DISEASES]:afecciones patológicas signos y síntomas::procesos patológicos::atributos de la enfermedad::enfermedad crónica [ENFERMEDADES]:salud ambiental::salud::enfermedad ambiental::enfermedades transmisibles::control de enfermedades transmisibles [SALUD PÚBLICA]medicine.anatomical_structureMedicinePatient representativesLife Sciences & BiomedicinePulmonary and Respiratory Medicinemedicine.medical_specialty3610 Medicine & health:enfermedades y anomalías neonatales congénitas y hereditarias::anomalías congénitas::anomalías múltiples::ciliopatías::enfermedades y anomalías neonatales congénitas y hereditarias::trastornos de la motilidad ciliar [ENFERMEDADES]:Other subheadings::Other subheadings::/prevention & control [Other subheadings]03 medical and health sciencesotorhinolaryngologic diseasesmedicineIntensive care medicineScience & TechnologyCYSTIC-FIBROSISLungbusiness.industryPSEUDOMONAS-AERUGINOSAMalalties cròniques - PrevencióCAREEFFICACYmedicine.diseasebiology.organism_classificationrespiratory tract diseases030228 respiratory systemNontuberculous mycobacteria610 Medizin und GesundheitbusinessERJ Open Research
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Possible role of ABO system in age-related diseases and longevity: a narrative review

2014

ABO blood group antigens are expressed either on the surface of red blood cells either on a variety of other cells. Based on the available knowledge of the genes involved in their biosynthesis and their tissue distribution, their polymorphism has been suggested to provide intraspecies diversity allowing to cope with diverse and rapidly evolving pathogens. Accordingly, the different prevalence of ABO group genotypes among the populations has been demonstrated to be driven by malaria selection. In the similar manner, a particular ABO blood group may contribute to favour life-extension via biological mechanisms important for surviving or eluding serious disease. In this review, we will suggest…

ABOAgingcongenital hereditary and neonatal diseases and abnormalitiesmedia_common.quotation_subjectLongevityImmunologyReviewDiseaseAntigenPolymorphism (computer science)ABO blood group systemhemic and lymphatic diseasesGenotypeparasitic diseasesmedicineCancermedia_commonInflammationSettore MED/04 - Patologia GeneralebiologyLongevitymedicine.diseaseAgeingCardiovascular diseasesImmunologybiology.proteinAntibodyABO Cancer Cardiovascular diseases Inflammation LongevityMalaria
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Acute Promyelocytic Leukemia during Pregnancy: A Systematic Review of the Literature

2020

The management of pregnant women with acute promyelocytic leukemia (APL) is a challenging situation where limited evidence-based information is available. We performed a systematic literature review to analyze the outcomes reported for both mother and fetus when APL is diagnosed during pregnancy. PubMed, Scopus and Web of Science databases were systematically searched to identify studies reporting cases of APL during pregnancy. Sixty-six articles met the eligibility criteria (53 single case reports). Ninety-two patients were eligible for induction therapy, with most them being treated with all-trans retinoic acid alone (32%) or combined with chemotherapy (43%), while the remaining patients …

Acute promyelocytic leukemiaCancer Researchmedicine.medical_specialtyAbortionchemotherapylcsh:RC254-282Article03 medical and health sciences0302 clinical medicineObstetrics and gynaecologymedicineNeonatologyPregnancyFetal viabilityRespiratory distressObstetricsbusiness.industryGestational ageacute promyelocytic leukemialcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogensmedicine.diseaseall-trans retinoic acidarsenic trioxideOncology030220 oncology & carcinogenesispregnancybusiness030215 immunologyCancers
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Incident colorectal cancer in Lynch syndrome is usually not preceded by compromised quality of colonoscopy

2019

AbstractBackground: Lifetime incidence of colorectal cancer (CRC) especially in carriers of MLH1 and MSH2 pathogenic germline variants in mismatch repair genes is high despite ongoing colonoscopy s...

AdenomaAdultMaleOncologycongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyHereditary non-polyposis colorectal cancerCOLONOSCOPYColorectal cancersurveillance colonoscopyeducationColonoscopycolorectal cancerMLH1Germline03 medical and health sciences0302 clinical medicineInternal medicinemedicineHumansRegistriesneoplasmsFinlandAgedNeoplasm StagingRetrospective Studiesmedicine.diagnostic_testbusiness.industryIncidenceIncidence (epidemiology)LYNCH SYNDROMEGastroenterologynutritional and metabolic diseasesMiddle Agedmedicine.diseaseColorectal Neoplasms Hereditary Nonpolyposisdigestive system diseasesLynch syndrome3. Good healthMSH2Population Surveillance030220 oncology & carcinogenesis3121 General medicine internal medicine and other clinical medicineFemale030211 gastroenterology & hepatologyDNA mismatch repairColorectal Neoplasmsbusiness
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Chromosomal changes in renal oncocytomas Evidence that t(5;11)(q35;q13) may characterize a second subgroup of oncocytomas

1995

Many of the reported oncocytomas have different chromosome abnormalities, indicating that they comprise a cytogenetically heterogenous group of tumors consisting of potentially cytogenetic subgroups. We have performed cytogenetic studies on nine renal oncocytomas. Clonal abnormalities were present in eight tumors. The findings most observed were the loss of the Y chromosome, and abnormalities of chromosomes 1 and 22. We also observed telomeric associations (tas) in two tumors and structural aberrations of chromosomes 9p and 19q, as well as monosomy 10. In two cases we found a similar reciprocal t(5;11)(q35;q13) in two cases. Review of the literature disclosed one other oncocytoma with a t(5…

AdenomaMalecongenital hereditary and neonatal diseases and abnormalitiesCancer ResearchMonosomyPathologymedicine.medical_specialtyChromosomal translocationBiologyurologic and male genital diseasesY chromosomeTranslocation GeneticGeneticsmedicineHumansOncocytomaMolecular BiologyAgedChromosome AberrationsGeneticsChromosome 7 (human)KidneyChromosomes Human Pair 11ChromosomeCancerMiddle Agedmedicine.diseaseKidney Neoplasmsmedicine.anatomical_structureKaryotypingChromosomes Human Pair 5FemaleCancer Genetics and Cytogenetics
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Different types of intestinal atresia in identical twins

2008

The authors present a previously unreported association of different types of intestinal atresia in identical low-birth-weight twins. Both babies were affected by duodenal atresia, associated in the first case with a complete mucosal duodenal membrane and in the second one with an "apple-peel" jejunal atresia. These occurrences may suggest that they were either the consequence of linkage of 2 genes or a pleiotropic expression of a single gene responsible for such rare conditions.

AdultAbortion Habitualcongenital hereditary and neonatal diseases and abnormalitiesIntestinal AtresiaPhysiologySingle geneInfant Premature DiseasesAnastomosisModels BiologicalDuodenal atresiaDuodenal atresia intestinal atresiamonozygotic twins newbornnewbornPregnancyDiseases in TwinsmedicineHumansInfant Very Low Birth Weightintestinal malformationGeneLaparotomybusiness.industrySettore MED/20 - Chirurgia Pediatrica E InfantileAnastomosis SurgicalIntestinal atresiaInfant NewborntwinsJejunal DiseasesTwins MonozygoticGeneral Medicinemedicine.diseaseJejunumJejunal atresiaPediatrics Perinatology and Child HealthFemaleParenteral Nutrition TotalSurgeryDuodenal ObstructionIdentical twinsbusinessInfant PrematureIntestinal VolvulusJournal of Pediatric Surgery
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COVID-19 in Infants Less than 3 Months: Severe or Not Severe Disease?

2022

Abstract: Compared to adults, severe or fatal COVID-19 disease is much less common in children. However, a higher risk for progression has been reported in infants. Different pediatric COVID-19 severity scores are reported in the literature. Methods: Subjects under 90 days of age admitted to 35 Italian institutions for COVID-19 were included. The severity of COVID-19 was scored as mild/moderate or severe/critical following the classification reported in the literature by Venturini, Dong, Kanburoglu, and Gale. To assess the diagnostic accuracy of each classification system, we stratified all enrolled patients developing a posteriori severity score based on clinical presentation and outcomes…

AdultFeverCOVID-19.SARS-CoV-2infantsSARS-CoV-2; COVID-19; neonates; infantsCOVID-19; SARS-CoV-2; infants; neonatesInfant NewbornCOVID-19infantneonatesInfectious DiseasesCoughVirologyHumansneonateChildHumanViruses; Volume 14; Issue 10; Pages: 2256
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A Study of Maternal Competence in Preterm Birth Condition, during the Transition from Hospital to Home: An Early Intervention Program’s Proposal

2021

The study was conducted with 104 mothers (average age 32.5 years, SD 6.1) of preterm infants (very and moderately preterm but still healthy) to monitor the perceived maternal role competence from the time of hospitalisation to post-discharge, in order to define an intervention program to support mothers during this transition. A targeted Q-Sort tool (Maternal Competence Q-Sort in preterm birth) was applied at two different times as a self-observation tool for parenting competence in neonatology. A tendency towards dysregulation of the maternal role competence was detected, mainly in terms of low self-assessment and was found to worsen during post-discharge, particularly with regard to careg…

AdultGerontologymedicine.medical_specialtyNeonatal intensive care unitHealth Toxicology and MutagenesisAftercareMothersPsychoeducational interventionArticleCompetence (law)Settore M-PSI/04 - Psicologia Dello Sviluppo E Psicologia Dell'EducazioneMaternal rolePregnancyEarly Medical InterventionIntensive Care Units NeonatalHumansMedicineNeonatologyIntervention programbusiness.industryparental competenceInfant NewbornPublic Health Environmental and Occupational HealthRInfantpreterm birthHospitalsPatient DischargePremature BirthMedicineFemalebusinessearly intervention programInfant PrematureInternational Journal of Environmental Research and Public Health
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The Fontan-Operation: From Intra- to Extracardiac Procedure

2003

Purpose For treatment of univentricular heart, the Fontan operation has been established as the definitive palliation. The current controversy is mainly based on the high incidence of arrhythmias after an intra-atrial lateral tunnel Fontan operation. Methods From January 1995 until April 2002. 46 children underwent a Fontan-type operation with or without a small fenestration. In 33 patients (group I) an Intracardiac tunnel and in 13 patients (group II) an extracardiac conduit procedure was performed. Principal findings There was no perioperative mortality. All patients showed postoperative a significant increase of arterial oxygen saturation, from 76 to 86% after surgery with fenestration, …

AdultHeart Defects CongenitalMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyAdolescentmedicine.medical_treatmentGroup iiExtracardiac conduitFontan ProcedureIntracardiac injectionFontan procedureIntraoperative PeriodmedicineHumansRadiology Nuclear Medicine and imagingcardiovascular diseasesChildCardiopulmonary Bypassbusiness.industryInfantPerioperativeSurgical InjuryUniventricular heartSurgeryOxygensurgical procedures operativeChild Preschoolcardiovascular systemFemaleSurgeryHigh incidencebusinessCardiology and Cardiovascular MedicineFollow-Up StudiesCardiovascular Surgery
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Genotype and phenotype analysis of Friedreich's ataxia compound heterozygous patients

2000

Friedreich's ataxia is caused by mutations in the FRDA gene that encodes frataxin, a nuclear-encoded mitochondrial protein. Most patients are homozygous for the expansion of a GAA triplet repeat within the FRDA gene, but a few patients show compound heterozygosity for a point mutation and the GAA-repeat expansion. We analyzed DNA samples from a cohort of 241 patients with autosomal recessive or isolated spinocerebellar ataxia for the GAA triplet expansion. Patients heterozygous for the GAA expansion were screened for point mutations within the FRDA coding region. Molecular analyses included the single-strand conformation polymorphism analysis, direct sequencing, and linkage analysis with FR…

AdultHeterozygotecongenital hereditary and neonatal diseases and abnormalitiesAtaxiaGenotypeGenetic LinkageDNA Mutational AnalysisGenes RecessiveCompound heterozygosityLoss of heterozygosityTrinucleotide RepeatsIron-Binding ProteinsGenotypeGeneticsmedicineHumansPoint MutationAge of OnsetAlleleChildAllelesPolymorphism Single-Stranded ConformationalGenetics (clinical)Family HealthGeneticsbiologynutritional and metabolic diseasesmedicine.diseasePedigreePhosphotransferases (Alcohol Group Acceptor)PhenotypeFriedreich AtaxiaChild PreschoolFrataxinbiology.proteinSpinocerebellar ataxiamedicine.symptomTrinucleotide Repeat ExpansionTrinucleotide repeat expansionMicrosatellite Repeats
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