Search results for "Neonatal screening"

showing 2 items of 22 documents

Diagnostic efficacy of the fluorometric determination of enzyme activity for Pompe disease from dried blood specimens compared with lymphocytes-possi…

2009

Pompe disease is a rare, autosomal-recessive disorder which results from a defect in the lysosomal enzyme acid alpha-glucosidase (GAA). The onset of this disease is highly variable, with infantile types being the most severe. Traditionally, lymphocytes, fibroblasts or muscle biopsies were necessary for enzyme activity measurement, because these materials do not express maltase-glucoamylase (MGA) that interferes with the assay. Recently, acarbose was found to inhibit MGA activity selectively, so that dried blood became accessible for GAA assessment.To evaluate the diagnostic efficacy of GAA measurement in dried blood specimens (DBSs) in comparison with lymphocytes. If DBSs provided reliable …

medicine.medical_specialtyTime FactorsLymphocyteBiopsyNeonatal ScreeningInternal medicineBiopsyGeneticsmedicineHumansFalse Positive ReactionsFluorometryLymphocytesGenetics (clinical)Acarbosechemistry.chemical_classificationNewborn screeningmedicine.diagnostic_testbiologybusiness.industryGlycogen Storage Disease Type IIMusclesInfant NewbornReproducibility of Resultsalpha-GlucosidasesEnzyme replacement therapyFibroblastsHydrogen-Ion ConcentrationEnzyme assaymedicine.anatomical_structureEndocrinologyEnzymechemistryCarbohydrate Metabolism Disorderbiology.proteinFeasibility Studiesbusinessmedicine.drugJournal of inherited metabolic disease
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Newborn hearing-screening project using transient evoked otoacoustic emissions: western sicily experience

2006

Summary Objective To study the incidence of congenital sensorineural hearing loss in all newborns introducing a screen test with a protocol no expensive, with a good “screen sensitivity” that could let an earlier identification of hearing impairment beginning early intervention by 2 months of age and increasing the probability of having language development within the normal range of development. Methods The study was conducted in Sciacca hospital from the beginning of 2003 to our days and was carried out with transient evoked otoacoustic emission using the criteria for PASS or RETEST and considering eventual prenatal and perinatal risk factors. All the newborns were divided into four group…

medicine.medical_specialtymedicine.medical_treatmentHearing Loss SensorineuralOtoacoustic Emissions SpontaneousOtoacoustic emissionNewborn Hearing screeningAudiologyScreen testHearing screeningConsanguinityNeonatal Screeningotorhinolaryngologic diseasesmedicineHumansNormal rangeRehabilitationbusiness.industryIncidence (epidemiology)Hearing TestsIncidenceInfant NewbornGeneral Medicinemedicine.diseaseSettore MED/32 - AudiologiaSettore MED/31 - OtorinolaringoiatriaOtorhinolaryngologySpeech developmentItalyPediatrics Perinatology and Child HealthSensorineural hearing lossbusiness
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