Search results for "Normal"

showing 10 items of 2571 documents

Outcome of enzyme replacement therapy in patients with Gaucher disease type I. The Romanian experience

2007

This study reports the first evaluation of therapeutic response in Romanian patients with Gaucher disease type I, after therapy with Cerezyme recently became available in our country.24 patients (11-50 years) received Cerezyme 20-60 U/kg every two weeks for at least 18 months. Haemoglobin, platelet count, volume of the liver and spleen, plasma chitotriosidase and the severity score were assessed every 6 months; skeletal radiography and osteodensitometry were also monitored.Eleven patients were splenectomized before start of therapy. Eight patients had anaemia (mean haemoglobin 9.4 g/dl) and 14 patients, of whom 13 were without splenectomy, had thrombocytopenia (mean 65,692/mm3). Haemoglobin…

AdultMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyPediatricsTime FactorsAdolescentBone diseasemedicine.medical_treatmentDiseaseWeight GainSeverity of Illness IndexHemoglobinsGeneticsmedicineHumansIn patientChildGenetics (clinical)ChemotherapyGaucher DiseasePlatelet CountRomaniabusiness.industryLiver DiseasesRomaniannutritional and metabolic diseasesAnemiaEnzyme replacement therapyMiddle Agedmedicine.diseaseCombined Modality TherapyThrombocytopeniaRecombinant Proteinslanguage.human_languageSurgeryHexosaminidasesTreatment OutcomeSplenomegalyQuality of LifeSplenectomylanguageGlucosylceramidaseFemaleBone DiseasesbusinessFollow-Up StudiesJournal of Inherited Metabolic Disease
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Prothrombotic State Induced by Middle-Distance Endurance Exercise in Middle-Aged Athletes

2018

AbstractSince the impact of possible prothrombotic factors on blood coagulation resulting from exercise remains elusive, this study investigated the acute effects of middle-distance endurance running on blood coagulation parameters in middle-aged athletes. The study population consisted of 33 male endurance runners who were engaged in a 21.1 km run under competitive conditions. Blood samples were collected before the run, immediately after the run, and 3 hours after run completion. Samples were assessed for activated partial thromboplastin time (APTT), prothrombin time (PT), fibrinogen, D-dimer, factor VIII (FVIII), von Willebrand factor antigen (VWF:Ag), endogenous thrombin potential (area…

AdultMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyphysical activity030204 cardiovascular system & hematologyFibrinogenRunningblood coagulation03 medical and health sciences0302 clinical medicineVon Willebrand factorEndurance traininghemic and lymphatic diseasesInternal medicineABO blood group systemvon Willebrand FactormedicineHumansExerciseblood coagulation; hemostasis; physical activity; thrombin generation; Adult; Blood Coagulation; Exercise; Factor VIII; Fibrinogen; Humans; Male; Middle Aged; Partial Thromboplastin Time; Physical Endurance; Running; Thrombin; Thrombosis; von Willebrand Factor; AthletesProthrombin timeFactor VIIImedicine.diagnostic_testbiologyChemistryThrombinFibrinogenThrombosisHematologyMiddle Agedprothrombotic factors blood coagulation sportprothrombotic factorsEndocrinologyCoagulationAthletesthrombin generationHemostasishemostasisPhysical Endurancebiology.proteinPartial Thromboplastin TimeCardiology and Cardiovascular Medicinesportcirculatory and respiratory physiology030215 immunologyPartial thromboplastin timemedicine.drug
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Analysis of chromosomal abnormalities in testicular and epididymal spermatozoa from azoospermic ICSI patients by fluorescence in-situ hybridization

2003

BACKGROUND: An increased incidence of numerical chromosomal abnormalities has been reported in the ejaculated spermatozoa of infertile patients. However, there are few cytogenetic studies of testicular and epididymal spermatozoa, and their results are still controversial. METHODS: Fluorescence in-situ hybridization (FISH) analysis of chromosomes 13, 18, 21, X and Y was performed on seven testicular samples and two epididymal samples from patients with obstructive azoospermia (OA), and on 13 testicular samples from patients with non-obstructive azoospermia (NOA). Five ejaculated sperm samples from normozoospermic fertile donors were evaluated as a control group. RESULTS: Both epididymal sper…

AdultMaleendocrine systemNumerical Chromosomal AbnormalityObstructive azoospermiaTesticleBiologyAndrologyTestismedicineHumansSperm Injections IntracytoplasmicIn Situ Hybridization FluorescenceChromosome AberrationsEpididymisAzoospermiaSex Chromosomesmedicine.diagnostic_testurogenital systemRehabilitationObstetrics and GynecologyOligospermiamedicine.diseaseEpididymisSpermatozoamedicine.anatomical_structureReproductive MedicineCase-Control StudiesChromosome abnormalityPloidyFluorescence in situ hybridizationHuman Reproduction
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Gender differences on oxidative stress markers and complement component C3 plasma values after an oral unsaturated fat load test

2020

Abstract Objective Post-prandial lipaemia (PL), oxidative stress (OS), and complement component C3 (C3) values are related to the atherosclerosis process. The post-prandial response of C3 after an oral fat load test (OFLT) using unsaturated fat is poorly addressed. The aim of this study was to analyze and compare the post-prandial response of OS markers and C3 values in men and women after an OFLT using unsaturated fat. Methods The study included a total of 22 healthy subjects with normal lipids and normal blood glucose (11 men and 11 pre-menopausal women). An oral unsaturated fat load test (OFLT: 50 g fat per m2 body surface) was performed using a commercial liquid preparation of long chai…

AdultMalemedicine.medical_specialty030209 endocrinology & metabolismmedicine.disease_cause03 medical and health scienceschemistry.chemical_compoundSex Factors0302 clinical medicineInternal medicinemedicineHumansPharmacology (medical)TriglyceridesFasting stateGeneral Environmental Science030219 obstetrics & reproductive medicineGlutathione Disulfidebusiness.industryUnsaturated fatHealthy subjectsGeneral EngineeringComplement C3FastingGlutathioneMiddle AgedPostprandial PeriodGlutathioneLipidsFats UnsaturatedOxidized GlutathioneOxidative StressEndocrinologychemistryGeneral Earth and Planetary SciencesFemaleCardiology and Cardiovascular MedicineNormal blood glucosebusinessLong chainBiomarkersOxidative stressClínica e Investigación en Arteriosclerosis (English Edition)
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Multichannel functional testing in normal subjects, glaucoma suspects, and glaucoma patients

2012

Purpose.: To evaluate visual function with a novel multichannel functional test named the ATD Multichannel Functional Test. Methods.: This multicenter study had a prospective and cross-sectional design. A total of 186 eyes were included: 42 with glaucoma, 14 glaucoma suspects due to optic nerve characteristics, 25 ocular hypertensives, and 105 normal eyes. All patients performed standard visual fields (Humphrey 24-2) and ATD with eight stimuli configurations: four achromatic (A), two red-green (T), and two blue-yellow (D). To derive main outcome measures, mean sensitivity, mean defect (MD), and pattern standard deviation (PSD) were calculated and compared among groups and types of stimuli w…

AdultMalemedicine.medical_specialty61genetic structuresFunctional testingGlaucomaAudiologyCycles per degreeContrast SensitivityPattern standard deviationOjos--EnfermedadesReference ValuesOphthalmologyOptic Nerve Diseases617MedicineHumansProspective StudiesIntraocular PressureAgedbusiness.industryVision TestsReproducibility of ResultsGlaucomaMiddle Agedmedicine.diseaseNormal limiteye diseasesOphthalmologymedicine.anatomical_structureCross-Sectional StudiesMulticenter studyOftalmologiaEye--DiseasesOptic nerveOftalmologíaFemaleOcular Hypertensionsense organsVisual FieldsbusinessUlls--MalaltiesColor PerceptionOptic disc
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Central nervous system involvement in late-onset Pompe disease: clues from neuroimaging and neuropsychological analysis

2018

Background and purpose Late-onset Pompe disease (LOPD) is a rare, multisystem disorder that is well established to mainly impair skeletal muscle function. Systematic studies exploring brain functions in LOPD are lacking. The aim of this study was to detect morphological and functional brain alterations as well as neuropsychological impairment in LOPD. Methods We studied 21 patients (10 male, mean age 49 ± 18.4 years) with defined diagnosis of LOPD, divided into two groups: one with pre-symptomatic hyperCKemia with no muscle weakness and the second with limb-girdle muscle weakness. All patients underwent 3T magnetic resonance imaging (MRI) to obtain morphological/angiographic evaluation as w…

AdultMalemedicine.medical_specialtyAdolescentSmoker scoreNeuropsychological Testscerebrovascular abnormalitieslate-onset Pompe diseaseYoung Adult03 medical and health sciences0302 clinical medicineAtrophyNeuroimagingInternal medicineConnectomemedicinecerebrovascular abnormalities Fazekas score functional magnetic resonance imaging late-onset Pompe disease Pompe disease Smoker score Neurology Neurology (clinical)HumansCognitive Dysfunction030212 general & internal medicineNeuropsychological assessmentAge of OnsetGray MatterAgedmedicine.diagnostic_testGlycogen Storage Disease Type IIbusiness.industryMuscle weaknessPompe diseaseMagnetic resonance imagingMiddle Agedmedicine.diseaseMagnetic Resonance Imagingfunctional magnetic resonance imagingHyperintensityFazekas scoreSuperior frontal gyrusNeurologyBrain sizeCardiologyFemaleNeurology (clinical)medicine.symptombusiness030217 neurology & neurosurgery
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Relationship between video head impulse test (vHIT) and caloric test in patients with vestibular neuritis.

2016

Abstract Introduction and objectives The caloric test is the gold standard for the loss of vestibular function diagnosis. The video head impulse test (vHIT) assesses the same reflex by using a video-assisted examination of the impulsive manoeuvre. We intend to compare the variation of results of the vHIT and the caloric test in patients with vestibular neuritis with respect to their initial condition at two different moments of their evolution and to check the level of correlation between them and with that of the DHI test. Methods We explored 20 patients with neuritis by using both vHIT and the caloric test on the same day. We assessed the correlation between these two tests and with the D…

AdultMalemedicine.medical_specialtyAdolescentVideo RecordingCaloric testAudiology03 medical and health sciencesYoung Adult0302 clinical medicineVertigoCaloric TestsSaccadesMedicineVideonystagmographyHumansProspective Studies030223 otorhinolaryngologyHead Impulse TestVestibular NeuronitisAgedVestibular systembiologymedicine.diagnostic_testReflex Abnormalbusiness.industryHead impulse testGeneral MedicineGold standard (test)Reflex Vestibulo-OcularMiddle Agedbiology.organism_classificationhumanitiesTest (assessment)ReflexRegression AnalysisFemalebusiness030217 neurology & neurosurgeryFollow-Up StudiesActa otorrinolaringologica espanola
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Cross-cultural adaptation of the orthognathic quality of life questionnaire (OQLQ) in a Brazilian sample of patients with dentofacial deformities

2010

Objectives: The aim of this study is to translate into Portuguese-Brazilian language and adapt cross-culturally to the Brazilian population the Orthognathic Quality of Life Questionnaire (OQLQ). Study Design: The cross-cultural adaptation process followed six stages which are; (I) initial translation, (II) synthesis of the translation, (III) back translation, (IV) expert committee and (V) test of the prefinal version. For validation process, the OQLQ results were compared with Oral Health Impact Profile Questionnaire (OHIP-49), with the generic SF-36 Quality of Life Questionnaire and a visual analogue scale. A convenience sample of 25 patients was selected in two Southern Brazilian states o…

AdultMalemedicine.medical_specialtyAdolescentVisual analogue scaleSample (statistics)Young AdultQuality of lifeSurveys and QuestionnairesmedicineCross-culturalHumansAdaptation (computer science)ChildGeneral DentistryReliability (statistics)LanguageCultural CharacteristicsTooth AbnormalitiesConstruct validityMiddle Aged:CIENCIAS MÉDICAS [UNESCO]Test (assessment)Maxillofacial AbnormalitiesOtorhinolaryngologyUNESCO::CIENCIAS MÉDICASPhysical therapyQuality of LifeSurgeryFemalePsychologySocial psychologyBrazil
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Intragenic KANSL1 mutations and chromosome 17q21.31 deletions: broadening the clinical spectrum and genotype-phenotype correlations in a large cohort…

2015

Background The 17q21.31 deletion syndrome phenotype can be caused by either chromosome deletions or point mutations in the KANSL1 gene. To date, about 60 subjects with chromosome deletion and 4 subjects with point mutation in KANSL1 have been reported. Prevalence of chromosome deletions compared with point mutations, genotype–phenotype correlations and phenotypic variability have yet to be fully clarified. Methods We report genotype–phenotype correlations in 27 novel subjects with 17q21.31 deletion and in 5 subjects with KANSL1 point mutation , 3 of whom were not previously reported. Results The prevalence of chromosome deletion and KANSL1 mutation was 83% and 17%, respectively. All patient…

AdultMalemedicine.medical_specialtyAdolescentgenotype-phenotype correlationsKoolen De Vries syndromeKANSL1 mutationHaploinsufficiencyBiologySettore MED/03 - GENETICA MEDICASeverity of Illness IndexCraniofacial AbnormalitiesYoung AdultSeizuresMolecular geneticsGeneticsmedicineHumansAbnormalities MultipleLanguage Development DisordersChildGenetics (clinical)Genetic Association StudiesGeneticsOptic nerve hypoplasiaFetal Growth RetardationPoint mutationMacrocephalyInfantNuclear ProteinsSyndromeclinical heterogeneitySmith–Magenis syndromemedicine.diseaseChild PreschoolSpeech delayFemalemedicine.symptomChromosome DeletionSmith-Magenis SyndromeHaploinsufficiencyChromosomes Human Pair 1717q21.31 deletion
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Benign oral vascular lesions treated by sclerotherapy with ethanolamine oleate : a retrospective study of 43 patients

2017

Background Although sclerotherapy is a common treatment for benign oral vascular lesions, there is no well-standardized protocol for this purpose. The aim of the present study was to describe the clinical characteristics of patients treated by sclerotherapy with ethanolamine oleate (EO), in order to contribute to a better understanding of this technique. Material and Methods Medical records and images of 90 patients treated by the same sclerotherapy protocol were retrieved and analysed. Thus, 43 cases with complete information were selected and described. Results The most affected age group was 41–70 years, with a female predominance and 86% of patients being Caucasian. Lips were the most a…

AdultMalemedicine.medical_specialtyAdolescentmedicine.medical_treatmentOleic AcidsGastroenterologyCongenital AbnormalitiesLesion03 medical and health sciencesYoung Adult0302 clinical medicineTongueInternal medicineSclerotherapymedicineSclerotherapyHumansYoung adultEthanolamine OleateChildGeneral DentistryAgedRetrospective StudiesMouthOral Medicine and Pathologybusiness.industryMedical recordResearchClinical appearanceRetrospective cohort study030206 dentistryMiddle Aged:CIENCIAS MÉDICAS [UNESCO]Sclerosing Solutionsmedicine.anatomical_structureOtorhinolaryngology030220 oncology & carcinogenesisUNESCO::CIENCIAS MÉDICASBlood VesselsSurgeryFemaleMouth Neoplasmsmedicine.symptombusinessHemangioma
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