Search results for "eps"

showing 10 items of 1777 documents

NDST1 missense mutations in autosomal recessive intellectual disability.

2014

NDST1 was recently proposed as a candidate gene for autosomal recessive intellectual disability in two families. It encodes a bifunctional GlcNAc N-deacetylase/N-sulfotransferase with important functions in heparan sulfate biosynthesis. In mice, Ndst1 is crucial for embryonic development and homozygous null mutations are perinatally lethal. We now report on two additional unrelated families with homozygous missense NDST1 mutations. All mutations described to date predict the substitution of conserved amino acids in the sulfotransferase domain, and mutation modeling predicts drastic alterations in the local protein conformation. Comparing the four families, we noticed significant overlap in …

AdultMaleModels MolecularCandidate geneAdolescentGenotypeProtein ConformationDNA Mutational AnalysisMutation MissenseGenes RecessiveBiologyBioinformaticsPolymorphism Single NucleotideAnimals Genetically ModifiedEpilepsyConsanguinityYoung AdultProtein structureIntellectual DisabilityIntellectual disabilityGeneticsmedicineMissense mutationAnimalsHumansChildGenetics (clinical)GeneticsGene knockdownMuscular hypotoniaBehavior AnimalComputational BiologyFaciesHigh-Throughput Nucleotide Sequencingmedicine.diseasePhenotypePedigreePhenotypeChild PreschoolGene Knockdown TechniquesDrosophilaFemaleSulfotransferasesGenome-Wide Association StudyAmerican journal of medical genetics. Part A
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Complications in third molar removal: A retrospective study of 588 patients

2010

Objectives: Surgical removal of third molars is a regular surgical procedure, which like all operations, may have complications. The purpose of the study was to analyze the incidence of complications and their relationship with the surgical difficulty in a group of 588 patients treated by the same oral and maxillofacial surgeon. Study design: This retrospective cohort study consisted of 1699 third molars (M3) removed between 2005 and 2008. The teeth were grouped into a 6-class scale of surgical difficulty rated according to the surgical procedure description in the patient?s file: I: upper M3 requiring forceps only; II: upper M3 requiring osteotomy; III: upper M3 requiring osteotomy and too…

AdultMaleMolarmedicine.medical_specialtyAdolescentmedicine.medical_treatmentForcepsDentistryOsteotomyCohort StudiesYoung AdultPostoperative Complicationsstomatognathic systemmedicineHumansGeneral DentistryRetrospective Studiesbusiness.industryIncidenceIncidence (epidemiology)Retrospective cohort studyMiddle Aged:CIENCIAS MÉDICAS [UNESCO]medicine.diseaseSurgeryTemporomandibular jointmedicine.anatomical_structureOtorhinolaryngologyTooth ExtractionUNESCO::CIENCIAS MÉDICASFemaleMolar ThirdSurgeryOsteitisbusinessCohort studyMedicina Oral Patología Oral y Cirugia Bucal
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A comparison of the triceps surae and residual muscle moments at the ankle during cycling.

1991

The rigid linked system model and principles of inverse dynamics have been widely used to calculate residual muscle moments during various activities. EMG driven models and optimization algorithms have also been presented in the literature in efforts to estimate skeletal muscle forces and evaluate their possible contribution to the residual muscle moment. Additionally, skeletal muscle-tendon forces have been measured, directly, in both animals and humans. The purpose of this investigation was to calculate the moment produced by the triceps surae muscles and compare it to the residual muscle moment at the ankle during cycling at three power outputs (90, 180 and 270 W). Inferences were made r…

AdultMaleMovementTransducersBiomedical EngineeringBiophysicsElectromyographyAchilles TendonModels BiologicalInverse dynamicsTriceps surae musclemedicineHumansOrthopedics and Sports MedicinePhysicsAchilles tendonmedicine.diagnostic_testElectromyographyMusclesRehabilitationBiomechanicsAnatomyProstheses and ImplantsTendonBicyclingMoment (mathematics)medicine.anatomical_structureStress MechanicalAnkleAnkleAnkle JointBiomedical engineeringJournal of biomechanics
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Fatal sepsis due to mycobacterium tuberculosis after allogeneic bone marrow transplantation.

2001

Mycobacterium tuberculosis is a serious, but rare infectious complication after allogeneic bone marrow transplantation. We describe a case of fatal sepsis due to Mycobacterium tuberculosis after allogeneic bone marrow transplantation for Philadelphia chromosome-positive ALL. The diagnosis was made after BAL. Although broad-spectrum antituberculous therapy was started immediately after diagnosis, blood cultures became positive for Mycobacterium tuberculosis. The patient developed severe pyrexias and finally died of multi-organ failure. Rapid progression of mycobacterial infection should be considered in patients post BMT with unexplained fever, particularly in patients from endemic areas.

AdultMaleMycobacterium tuberculosisSepsisFatal OutcomeAcute lymphocytic leukemiamedicineHumansTransplantation HomologousTuberculosisIn patientAutogenous boneBone Marrow TransplantationTransplantationbiologybusiness.industryMarrow transplantationHematologyMycobacterium tuberculosisPrecursor Cell Lymphoblastic Leukemia-Lymphomamedicine.diseasebiology.organism_classificationmedicine.anatomical_structureImmunologyBone marrowbusinessComplicationBone marrow transplantation
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Poor prognosis despite successful treatment of postanoxic generalized myoclonus.

2010

Generalized myoclonus (GM) after cardiopulmonary resuscitation (CPR) implies a poor prognosis.1 Postanoxic GM is usually classified as one type of convulsive status epilepticus,2 which is also reflected by terms like myoclonic status,3 myoclonic status epilepticus,4 or postanoxic status epilepticus.5 Antiepileptic drugs commonly used in the treatment of status epilepticus such as phenytoin or valproate, however, are ineffective in the majority of these patients.3,4,6 Reports of single patients7 and our own observations6 indicated that propofol may control GM. This prompted us to use propofol as standard treatment of postanoxic GM in 60 consecutive comatose survivors of CPR. ### Level of evi…

AdultMaleMyoclonusmedicine.medical_treatmentMyoclonic JerkStatus epilepticusEpilepsyIntensive caremedicineHumansCardiopulmonary resuscitationComaHypoxia BrainPropofolAgedAged 80 and overbusiness.industryStandard treatmentElectroencephalographyMiddle Agedmedicine.diseasePrognosisTreatment OutcomeAnesthesiaFemaleNeurology (clinical)medicine.symptomPropofolbusinessMyoclonusmedicine.drugNeurology
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Quality of life in drug-resistant epilepsy: relationships with negative affectivity, memory, somatic symptoms and social support

2018

Objective: To investigate the relative contribution of factors non-directly related to seizures such as negative affectivity, social support, somatic symptoms and memory performance on quality of life (QOL) in patients with drug-resistant epilepsy. Methods: This is a cross-sectional study. Seventy patients with drug-resistant epilepsy were consecutively recruited from the inpatient Epilepsy Unit, Hospital Universitario y Politecnico La Fe, between April 2015 and October 2017. Medical history provided demographic characteristics of the patients (sex, age, and educational level), and clinical data (age at epilepsy onset, duration of epilepsy in years, frequency of seizures per month, type of …

AdultMaleNegative affectivity03 medical and health sciencesSocial supportEpilepsy0302 clinical medicineQuality of lifeMemoryHumansMedicineMedical history030212 general & internal medicineNeuropsychological assessmentEpilepsymedicine.diagnostic_testbusiness.industrySocial SupportDrug Resistant Epilepsymedicine.diseasePsychiatry and Mental healthClinical PsychologyCross-Sectional StudiesMedically Unexplained SymptomsQuality of LifeFemaleVerbal memorybusiness030217 neurology & neurosurgeryClinical psychologyJournal of Psychosomatic Research
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3T Double Inversion Recovery Magnetic Resonance Imaging: diagnostic advantages in the evaluation of cortical development anomalies

2016

Abstract Purpose The aim of this work was to investigate the diagnostic value of the DIR sequence at 3T MR imaging operating in the evaluation of cortical development anomalies. Methods We studied 40 patients, with a previous diagnosis of cortical dysplasia, by FLAIR-3D, DIR, FSE T2 and MPR-GE T1 sequences at 3T MRI. Two independent observers evaluated, for each sequence and lesion, some semiological aspects (cortical thickness, cortical signal intensity, white-gray matter blurring, subcortical white matter intensity). We made also a quantitative evaluation of the cortical signal intensity in lesion site, drawing a ROI on each MRI sequences and comparing them to the correspondent normal con…

AdultMalePathologymedicine.medical_specialty030218 nuclear medicine & medical imagingWhite matter03 medical and health sciences0302 clinical medicineNuclear magnetic resonancePolimicrogyriamedicineHumansRadiology Nuclear Medicine and imagingProspective StudiesCortical dysplasia; DIR; Epilepsy; Polimicrogyria; Taylor; Tuberous sclerosisCerebral CortexDIRCortical dysplasiaEpilepsymedicine.diagnostic_testbusiness.industryTuberous sclerosisSignificant differenceReproducibility of ResultsMagnetic resonance imagingGeneral MedicineMiddle AgedCortical dysplasiamedicine.diseaseMagnetic Resonance ImagingIntensity (physics)DIR Epilepsy Cortical dysplasia Taylor Tuberous sclerosis PolimicrogyriaMalformations of Cortical DevelopmentTaylormedicine.anatomical_structureWhite matter hyperintensityFemaleDouble inversion recoverybusiness030217 neurology & neurosurgeryLesion siteEuropean Journal of Radiology
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Subregional Pathology of the Amygdala Complex and Entorhinal Region in Surgical Specimens From Patients With Pharmacoresistant Temporal Lobe Epilepsy

2000

The hippocampus, amygdala complex, and entorhinal region represent anatomically linked limbic structures of the mesiotemporal lobe. Chronic seizures and mnestic deficits in patients with pharmacoresistant mesial temporal lobe epilepsy (TLE) appear to correlate with distinct patterns of histopathological alterations in these areas. The complex anatomical organization of the amygdala and entorhinal region, however, render a detailed neuropathological evaluation of surgical specimens difficult. In this study, we present a combined cytoarchitectonical, pigmentarchitectonical, myelinarchitectonical, and immunohistochemical reconstruction of the amygdala, entorhinal region, and hippocampus from s…

AdultMalePathologymedicine.medical_specialtyAdolescentDrug ResistanceHippocampusAutopsyAmygdalaPathology and Forensic MedicineTemporal lobeCentral nervous system diseaseCellular and Molecular NeuroscienceEpilepsyBasal (phylogenetics)medicineEntorhinal CortexHumansGliosisSclerosisGeneral MedicineMiddle AgedAmygdalamedicine.diseaseTemporal LobeLobemedicine.anatomical_structureEpilepsy Temporal Lobenervous systemNeurologyChild PreschoolAnticonvulsantsFemaleNeurology (clinical)Psychologypsychological phenomena and processesJournal of Neuropathology & Experimental Neurology
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Detection of gastric atrophy by circulating pepsinogens: A comparison of three assays.

2017

Background Circulating levels of pepsinogens have been used in high gastric cancer-risk Asian and European populations to triage endoscopic evaluation for more severe pathology. There are different analytic methods with uncertain correlations. We therefore compared diagnostic performance of three commonly used pepsinogen assays to detect histologically confirmed gastric atrophy. Methods We tested plasma samples from adult patients with (n=50) and without (n=755) moderate or severe gastric corpus atrophy, as determined histologically by consensus of three expert pathologists. A single laboratory measured pepsinogens I (PgI) and II (PgII) using commercially available assays: two ELISA assays …

AdultMalePathologymedicine.medical_specialtyAdolescentStomach DiseasesEnzyme-Linked Immunosorbent AssayGastroenterologyArticle03 medical and health sciencesYoung Adult0302 clinical medicineAtrophyPepsinInternal medicineMedicineHumansAgedAged 80 and overAdult patientsPlasma samplesReceiver operating characteristicbiologyPepsinogensbusiness.industryDiagnostic Tests RoutineHistocytochemistryGastric AtrophyStomachGastroenterologyGeneral MedicineMiddle Agedmedicine.diseasedigestive system diseasesInfectious Diseasesmedicine.anatomical_structureROC CurveGastric Mucosa030220 oncology & carcinogenesisbiology.protein030211 gastroenterology & hepatologyTest performanceFemaleAtrophybusinessLatex Fixation TestsHelicobacter
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Expression of Matrix-Degrading Cysteine Proteinase Cathepsin K in Cholesteatoma

2001

Cholesteatoma is a nonneoplastic lesion of the middle ear space or mastoid that is histologically characterized by a progressive bone erosion of the ossicles and surrounding bone. Several matrix-degrading enzymes have been implicated as mediators of this bone erosion. Because the novel cysteine proteinase cathepsin K has been shown to play a central role in bone resorption, we examined the expression of this enzyme in tissue specimens of cholesteatoma. Tissue specimens of 9 patients with cholesteatoma were obtained during middle-ear surgery. Expression of cathepsin K mRNA was determined by RT-PCR using specific primers. Immunohistochemical analysis of cathepsin K protein expression in tissu…

AdultMalePathologymedicine.medical_specialtyCathepsin KOsteoclastsMatrix (biology)Giant CellsBone resorptionPathology and Forensic MedicineImmunoenzyme Techniquesotorhinolaryngologic diseasesCathepsin KmedicineHumansRNA MessengerBone ResorptionChildAgedCathepsin SCathepsinCholesteatoma Middle EarReverse Transcriptase Polymerase Chain ReactionChemistryCholesteatomaMiddle Agedmedicine.diseaseCathepsinsEpitheliummedicine.anatomical_structureGiant cellFemaleModern Pathology
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