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showing 10 items of 1287 documents

Clinical characteristics of adults with slowly progressing mucopolysaccharidosis VI: a case series.

2012

To assess clinical features and general health status of adult patients with mucopolysaccharidosis (MPS) VI. This report includes the clinical history of patients older than 18 years with slowly progressing MPS VI and the retrospective analysis of the outcomes of available data collected between September 2003 and October 2008 at the Center of Pediatric and Adolescent Medicine, University Medical Center, Johannes Gutenberg-University of Mainz, Germany. Variables included were urinary glycosaminoglycan (uGAG) level, mutation analysis, body height, forced vital capacity (FVC), 6-minute walk test, echocardiographic findings, the need for craniocervical decompression surgery, orthopaedic findin…

AdultMalemedicine.medical_specialtyN-Acetylgalactosamine-4-SulfataseMucopolysaccharidosismedicine.medical_treatmentCarpal tunnel surgeryFEV1/FVC ratioAdolescent medicineYoung AdultValve replacementGermanyGeneticsmedicineHumansAge of OnsetCarpal tunnel syndromeGenetics (clinical)GlycosaminoglycansMucopolysaccharidosis VIbusiness.industryMucopolysaccharidosis VIEnzyme replacement therapymedicine.diseaseSurgeryPhenotypeDisease ProgressionFemalebusinessFollow-Up StudiesJournal of inherited metabolic disease
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Detection of possible factors favouring the evolution of migraine without aura into chronic migraine

2012

In a minority of cases, the natural history of migraine without aura (MO) is characterised over time by its evolution into a form of chronic migraine (CM). In order to detect the possible factors predicting this negative evolution of MO, we searched in our Headache Centre files for all clinical records that met the following criteria: (a) first visit between 1976 and 1998; (b) diagnosis of MO or of common migraine at the first observation, with or without association with other primary headache types; (c) <15 days per month of migraine at the first observation; and (d) at least one follow-up visit at least 10 years after the first visit. The patients thus identified were then divided into t…

AdultMalemedicine.medical_specialtyNeurologyTime FactorsAuraMigraine DisordersDermatologyYoung AdultChronic MigraineMigraine Migraine without aura Chronic migraine Chronic daily headache Chronic headacheInternal medicinemedicineHumansYoung adultDepression (differential diagnoses)business.industryDepressionGeneral MedicineMiddle Agedmedicine.diseaseComorbidityNatural historyPsychiatry and Mental healthMigraineChronic DiseaseHypertensionPhysical therapyDisease ProgressionFemaleSettore MED/26 - NeurologiaNeurology (clinical)business
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The differing roles of the frontal cortex in fluency tests

2012

Fluency tasks have been widely used to tap the voluntary generation of responses. The anatomical correlates of fluency tasks and their sensitivity and specificity have been hotly debated. However, investigation of the cognitive processes involved in voluntary generation of responses and whether generation is supported by a common, general process (e.g. fluid intelligence) or specific cognitive processes underpinned by particular frontal regions has rarely been addressed. This study investigates a range of verbal and non-verbal fluency tasks in patients with unselected focal frontal ( n  = 47) and posterior ( n  = 20) lesions. Patients and controls ( n  = 35) matched for education, age and s…

AdultMalemedicine.medical_specialtyNeuropsychological TestsAudiologybehavioral disciplines and activitiesBrain mappingFunctional LateralityFluencyCognitionRaven's Progressive MatricesmedicineHumansSet (psychology)Brain MappingBrain NeoplasmsVerbal BehaviorCognitionOriginal ArticlesMiddle AgedMagnetic Resonance ImagingFrontal LobeCognitive testStrokeFrontal lobeCase-Control StudiesFemaleNeurology (clinical)PsychologyCognitive psychologyGestureBrain
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Addition of rituximab to fludarabine and cyclophosphamide in patients with chronic lymphocytic leukaemia: a randomised, open-label, phase 3 trial.

2010

On the basis of promising results that were reported in several phase 2 trials, we investigated whether the addition of the monoclonal antibody rituximab to first-line chemotherapy with fludarabine and cyclophosphamide would improve the outcome of patients with chronic lymphocytic leukaemia.Treatment-naive, physically fit patients (aged 30-81 years) with CD20-positive chronic lymphocytic leukaemia were randomly assigned in a one-to-one ratio to receive six courses of intravenous fludarabine (25 mg/m(2) per day) and cyclophosphamide (250 mg/m(2) per day) for the first 3 days of each 28-day treatment course with or without rituximab (375 mg/m(2) on day 0 of first course, and 500 mg/m(2) on da…

AdultMalemedicine.medical_specialtyNeutropeniaFCR RegimenKaplan-Meier EstimateOfatumumabSeverity of Illness IndexGastroenterologyDisease-Free SurvivalDrug Administration ScheduleAntibodies Monoclonal Murine-Derivedchemistry.chemical_compoundChemoimmunotherapyObinutuzumabInternal medicineAntineoplastic Combined Chemotherapy ProtocolsHumansImmunologic FactorsMedicineCyclophosphamideAgedAged 80 and overbusiness.industryIncidenceAntibodies MonoclonalLeukopeniaGeneral MedicineMiddle AgedLeukemia Lymphocytic Chronic B-CellSurgeryFludarabineTreatment OutcomechemistryDisease ProgressionFemaleRituximabRefractory Chronic Lymphocytic LeukemiaRituximabbusinessVidarabineUntreated Chronic Lymphocytic Leukemiamedicine.drug
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Functional correlates of vertical gaze palsy and other ocular motor deficits in PSP: An FDG-PET study

2014

Abstract Objective To determine the functional correlates of vertical gaze palsy and other ocular motor deficits in patients with progressive supranuclear palsy (PSP) using [ 18 F]fluorodeoxyglucose (FDG-)PET. Methods Twenty-six patients with PSP underwent clinical examination of vertical gaze combined with FDG-PET scans to assess regional cerebral glucose metabolism as a marker of neuronal activity. Of these, eighteen PSP patients were also investigated by electrical nystagmography to determine horizontal ocular motor deficits. Statistical parametric mapping analyses were performed to correlate regional neuronal activity with ocular motor functions. Results In categorical comparisons, pati…

AdultMalemedicine.medical_specialtyNystagmographygenetic structuresSmooth pursuitOcular Motility DisordersLingual gyrusOcular Motility DisordersFluorodeoxyglucose F18OphthalmologymedicineHumansPremovement neuronal activityAgedAged 80 and overPalsyOptokinetic reflexMiddle AgedGazeeye diseasesNeurologyPositron-Emission TomographyFemaleSupranuclear Palsy ProgressiveNeurology (clinical)RadiopharmaceuticalsGeriatrics and GerontologyPsychologyNeuroscienceParkinsonism &amp; Related Disorders
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Risk for periodontal disease in patients with longstanding rheumatoid arthritis.

1998

Objective. To quantify periodontal disease in rheumatoid arthritis (RA) patients and controls, and to correlate the degree of destruction from periodontal disease and from RA Methods. Fifty RA patients were matched for age, sex, smoking status, and oral hygiene with 101 controls. Correlations between indices of chronic destruction in periodontal disease (gingival attachment loss) and in RA (Larsen radiographic score) were determined. Results. Patients with longstanding active RA (mean ± SD 13 ± 8 years) who were receiving treatment with disease-modifying antirheumatic drugs (n = 46), corticosteroids (n = 38), or nonsteroidal antiinflammatory drugs (n = 43) had a higher rate of gingival blee…

AdultMalemedicine.medical_specialtyOral Hygiene IndexImmunologyGingivaArthritisDentistryOral hygieneGastroenterologyArthritis RheumatoidRheumatologyRisk FactorsInternal medicineImmunopathologyOral and maxillofacial pathologymedicineImmunology and AllergyHumansPharmacology (medical)Risk factorArthrographyAgedAutoimmune diseasebusiness.industryMiddle Agedmedicine.diseaseOral HygieneClinical attachment lossRheumatoid arthritisGingival DiseasesDisease ProgressionFemaleJointsbusinessArthritis and rheumatism
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End-of-life situations in cardiology: a qualitative study of physicians' and nurses' experience in a large university hospital.

2018

IF 2.335 (2-year Impact Factor); International audience; Background: rofessional societies call for integration of end-of-life discussions early in the trajectory of heart failure, yet it remains unclear where current practices stand in relation to these recommendations. We sought to describe the perceptions and attitudes of caregivers in cardiology regarding end-of-life situations.Methods: e performed a qualitative study using semi-directive interviews in the cardiology department of a university teaching hospital in France. Physicians, nurses and nurses’ aides working full-time in the department at the time of the study were eligible. Participants were asked to describe how they experienc…

AdultMalemedicine.medical_specialtyPalliative careAttitude of Health Personnellcsh:Special situations and conditionsClinical Decision-MakingPsychological interventionCardiologyContext (language use)Legislation030204 cardiovascular system & hematologyNursing Staff Hospital03 medical and health sciences0302 clinical medicine[SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular systemProfessional-Family RelationsInternal medicinemedicineHumans030212 general & internal medicineQualitative ResearchHeart FailureTerminal Carelcsh:RC952-1245Palliative CareGeneral MedicineProfessional-Patient RelationsMiddle Aged[SDV.ETH] Life Sciences [q-bio]/Ethics[SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system[SDV.ETH]Life Sciences [q-bio]/Ethics3. Good healthDistressHospitalistsCardiologyDisease ProgressionProfessional associationFemaleInterdisciplinary CommunicationCardiology Service HospitalFranceThematic analysisPsychologyAdvance DirectivesQualitative researchResearch ArticleEnd-of-lifeBMC palliative care
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Lysosomal aspartic and cysteine proteinases serum levels in patients with pancreatic cancer or pancreatitis

1997

Lysosomal cathepsins D (CD), B (CB), and L (CL) serum levels were determined by immunoassays in patients with chronic (CHP) or acute (AP) pancreatitis and in patients with ductal pancreatic carcinoma (DPC) and correlated with some biological and clinical parameters of this tumor. CB serum concentrations significantly higher than those measured in healthy subjects (NS) were observed in CHP, AP, and DPC patients (p &lt; 0.01). However, no significant difference was noted among these groups. Increased CL serum levels were evident only in cancer patients compared to NS, AP, or CHP groups (p &lt; 0.05), while no difference was observed among these groups. Elevated CD serum values were observed i…

AdultMalemedicine.medical_specialtyPancreatic diseaseCA-19-9 AntigenEndocrinology Diabetes and MetabolismCathepsin LLysosomal proteinaseCathepsin DTumor markers.Cathepsin BEndocrinologyPancreatic cancerInternal medicineEndopeptidasesInternal MedicinemedicineCarcinomaAspartic Acid EndopeptidasesHumansAntigens Tumor-Associated CarbohydrateAgedAged 80 and overVHepatologybusiness.industryCarcinoma Ductal BreastCancerPancreatic cancerMiddle Agedmedicine.diseaseCathepsinsPancreatic NeoplasmsCysteine EndopeptidasesEndocrinologymedicine.anatomical_structurePancreatitisTumor progressionAdenocarcinomaPancreatitisFemalePancreasbusinessLysosomes
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Colony-Stimulating Factor-1

2015

A noninvasive means to predict the onset and recurrence of lupus nephritis (LN) before overt renal injury is needed to optimize and individualize treatment. Colony-stimulating factor-1 (CSF-1) is expressed by kidney tubules at the onset of LN, increases with disease progression, and spills into the circulation in lupus-prone mice. We tested the hypothesis that amplified expression of CSF-1 detected in the serum or urine correlates with intrarenal CSF-1 expression and histopathology (increased macrophage accumulation, activity indices) and clinical kidney disease activity and predicts the onset and recurrence of nephritis in patients with systemic lupus erythematosus (SLE). We found increase…

AdultMalemedicine.medical_specialtyPathologyAdolescentBiopsyKidney GlomerulusLupus nephritisSeverity of Illness IndexGastroenterologyYoung AdultPredictive Value of TestsInternal medicineBiopsymedicineHumansLongitudinal StudiesAgedRetrospective StudiesKidneymedicine.diagnostic_testbusiness.industryMacrophage Colony-Stimulating FactorReproducibility of ResultsGlomerulonephritisGeneral MedicineMiddle Agedmedicine.diseaseLupus NephritisBasic ResearchKidney Tubulesmedicine.anatomical_structureNephrologyCase-Control StudiesDisease ProgressionFemaleHistopathologybusinessSerositisNephritisBiomarkersKidney diseaseJournal of the American Society of Nephrology
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Changing associations between cognitive impairment and imaging in multiple sclerosis as the disease progresses.

2013

The authors explored cross-sectional associations between MRI parameters (lesion metrics, brain volumes, magnetization transfer ratio histograms, and metabolite concentrations) and cognition in 61 patients who experienced clinically-isolated syndromes (CIS) 7 years earlier. IQ decline and poorer overall cognition were associated with T2 white-matter lesions, and slow information-processing with both T2 lesions and gray-matter atrophy. In a previous study of the same cohort, gray-matter atrophy measured shortly after CIS failed to predict development of cognitive impairment years later. Our findings suggest that gray-matter pathology, reflected by atrophy measurements, becomes increasingly i…

AdultMalemedicine.medical_specialtyPathologyIntelligenceStatistics as TopicDiseaseAudiologyNeuropsychological Testsmultiple sclerosisLesionDisability EvaluationExecutive FunctionAtrophyMemorymedicineHumansOptic neuritisAttentionCognitive impairmentbusiness.industryMultiple sclerosisBrainCognitionMiddle AgedVerbal Learningmedicine.diseaseMagnetic Resonance ImagingPsychiatry and Mental healthCross-Sectional StudiesCohortDisease ProgressionFemaleNeurology (clinical)medicine.symptombusinessCognition DisordersFollow-Up Studies
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