Search results for "Cystic Fibrosis"

showing 10 items of 113 documents

A NEW MODEL OF CF DISEASE: NO F508, NO KINASE?

2008

Pulmonary and Respiratory Medicine0303 health sciencesKinasebusiness.industryDiseasemedicine.diseaseCystic fibrosis03 medical and health sciences0302 clinical medicine030228 respiratory systemPediatrics Perinatology and Child HealthmedicineCancer researchPediatrics Perinatology and Child Healthbusinessskin and connective tissue diseases030304 developmental biologyJournal of Cystic Fibrosis
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Cardiorespiratory Fitness and Physical Activity following Lung Transplantation: A National Cohort Study

2020

<b><i>Background:</i></b> Low cardiorespiratory fitness and inactivity are common after lung transplantation (LTx). The causes of exercise intolerance are incompletely understood. <b><i>Objectives:</i></b> The aim of this study was to objectively assess cardiorespiratory fitness and physical activity, evaluate causes of exercise intolerance, and explore clinical factors associated with cardiorespiratory fitness after bilateral LTx (BLTx). <b><i>Materials and Methods:</i></b> Peak oxygen uptake (V<b>∙</b>O<sub>2peak</sub>) and exercise-limiting factors were evaluated by a treadmill cardiopulmonary…

Pulmonary and Respiratory MedicineAdultMalemedicine.medical_specialtyCystic Fibrosismedicine.medical_treatmentPhysical activityNational cohortCohort Studies03 medical and health sciencesHemoglobinsPulmonary Disease Chronic ObstructiveYoung Adult0302 clinical medicineOxygen ConsumptionForced Expiratory VolumemedicineLung transplantationHumans030212 general & internal medicineLicenseExerciseAgedExercise ToleranceCardiovascular Deconditioningbusiness.industryNorwayPulmonary Gas ExchangeCardiorespiratory fitnessCardiopulmonary exercise testingCreative commonsMiddle AgedVDP::Medisinske Fag: 700::Idrettsmedisinske fag: 850030228 respiratory systemCardiorespiratory FitnessFamily medicineExercise TestFemalebusinessLung Diseases InterstitialVDP::Samfunnsvitenskap: 200::Samfunnsvitenskapelige idrettsfag: 330Lung Transplantation
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Coil embolisation for massive haemoptysis in cystic fibrosis.

2021

IntroductionMassive haemoptysis is a life-threatening event in advanced cystic fibrosis (CF) lung disease with bronchial artery embolisation (BAE) as standard of care treatment. The aim of our study was to scrutinise short-term and long-term outcomes of patients with CF and haemoptysis after BAE using coils.MethodsWe carried out a retrospective cohort study of 34 adult patients treated for massive haemoptysis with super selective bronchial artery coil embolisation (ssBACE) between January 2008 and February 2015. Embolisation protocol was restricted to the culprit vessel(s) and three lobes maximum. Demographic data, functional end-expiratory volume in 1 s in % predicted (FEV1% pred.) and bod…

Pulmonary and Respiratory MedicineAdultmassive haemoptysismedicine.medical_specialtyHemoptysisCystic FibrosisMedizinBronchial ArteriesCulpritCystic fibrosisDiseases of the respiratory systemmedicine.arterymedicineHumansIn patient22181506Coil embolizationRetrospective StudiesRC705-779business.industryRRetrospective cohort studymedicine.diseaseEmbolization TherapeuticSurgeryMedicineSputummedicine.symptomBronchial arterybusinessBody mass indexBMJ open respiratory research
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ISOLATION PROCEDURE, SPECIES IDENTIFICATION AND CLINICAL SIGNIFICANCE OF ASPERGILLUS SPP., SCEDOSPORIUM SPP., AND OTHER FILAMENTOUS FUNGI IN CYSTIC F…

2008

Pulmonary and Respiratory MedicineAspergillusbiologybusiness.industrymedicine.diseaseIsolation (microbiology)biology.organism_classificationCystic fibrosisMicrobiologyScedosporiumPediatrics Perinatology and Child HealthmedicineSpecies identificationClinical significancePediatrics Perinatology and Child Healthbusinessskin and connective tissue diseasesJournal of Cystic Fibrosis
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Physicochemical compatibility of mixtures of dornase alfa and tobramycin containing nebulizer solutions

2008

Patients suffering from cystic fibrosis (CF) often need to inhale multiple doses of different nebulizable drugs per day. Patients attempt to shorten the time consuming administration procedure by mixing drug solutions/suspensions for simultaneous inhalation. The objective of this experimental study was to determine whether mixtures of the nebulizer solution dornase alfa (Pulmozyme) with tobramycin nebulizer solutions (TOBI and GERNEBCIN 80 mg) are physico-chemically compatible. Drug combinations were prepared by mixing the content of one respule Pulmozyme with either one respule TOBI or one ampoule GERNEBCIN 80 mg. Test solutions were stored at room temperature and exposed to light. Dornase…

Pulmonary and Respiratory MedicineChemical PhenomenaCystic FibrosisExcipientAmpouleDrug Incompatibilitychemistry.chemical_compoundAdministration InhalationTobramycinDeoxyribonuclease IHumansMedicinePotencyChromatographyInhalationbusiness.industryNebulizers and VaporizersDornase alfaSodium metabisulfiteAnti-Bacterial AgentsDrug CombinationsPharmaceutical SolutionsNebulizerchemistryAnesthesiaPediatrics Perinatology and Child HealthTobramycinbusinessmedicine.drugPediatric Pulmonology
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Nanocomplexes for gene therapy of respiratory diseases: Targeting and overcoming the mucus barrier

2015

Gene therapy, i.e. the delivery and expression of therapeutic genes, holds great promise for congenital and acquired respiratory diseases. Non-viral vectors are less toxic and immunogenic than viral vectors, although they are characterized by lower efficiency. However, they have to overcome many barriers, including inflammatory and immune mediators and cells. The respiratory and airway epithelial cells, the main target of these vectors, are coated with a layer of mucus, which hampers the effective reaching of gene therapy vectors carrying either plasmid DNA or small interfering RNA. This barrier is thicker in many lung diseases, such as cystic fibrosis. This review summarizes the most impor…

Pulmonary and Respiratory MedicineCystic FibrosisGenetic enhancementContext (language use)Gene deliveryVectors in gene therapyPolyethylene GlycolsViral vectorPolyethyleinimine Poly-L-lysine Ethylene glycol Chitosan PAMAM G0 dendrimer N-(1-(23-Dioleyloxy)propyl)-NNNtrimethylammonium chloride 12-Dioleoylphosphatidylethanolamine N-acetylcystein 12-Dioctadecanoyl-sn-glycero-3-phosphoethanolaminemedicineHumansTechnology PharmaceuticalPharmacology (medical)RNA Small InterferingLungExpectorantsInflammationLungbusiness.industryBiochemistry (medical)Gene Transfer TechniquesGenetic TherapyMucusMucusmedicine.anatomical_structureSettore CHIM/09 - Farmaceutico Tecnologico ApplicativoImmunologyNanoparticlesInflammation MediatorsbusinessPlasmidsRespiratory tract
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Development and electronic validation of the revised Cystic Fibrosis Questionnaire (CFQ-R Teen/Adult)

2018

Abstract Background The Cystic Fibrosis Questionnaire-Revised (CFQ-R+14) is a disease-specific, health-related quality of life instrument for cystic fibrosis (CF) patients ≥14years. We have developed a Spanish electronic version of the CFQ-R (e-CFQ-R+14 Spain). Our aim was to compare the paper and electronic versions and to validate the electronic version. Methods Fifty CF patients completed the study. All answered the paper and electronic versions on day 1 and repeated the e-CFQR version 15days later. Results Concordance between the electronic and paper copy versions was high, with correlations above 0.9 in all domains. Test-retest reliability of the e-CFQ-R results was strong, with coeffi…

Pulmonary and Respiratory MedicineHealth related quality of lifemedicine.medical_specialtybusiness.industryConcordancemedicine.diseaseCystic fibrosis03 medical and health sciences0302 clinical medicine030228 respiratory systemQuality of lifePediatrics Perinatology and Child HealthPhysical therapyMedicine030212 general & internal medicinebusinessPsychosocialReliability (statistics)Clinical psychologyJournal of Cystic Fibrosis
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IMPROVEMENT OF MOLECULAR TECHNOLOGIES AND PRENATAL DIAGNOSIS OF CYSTIC FIBROSIS

2008

Pulmonary and Respiratory MedicinePediatricsmedicine.medical_specialtybusiness.industryPediatrics Perinatology and Child HealthMedicinePrenatal diagnosisPediatrics Perinatology and Child Healthbusinessmedicine.diseaseCystic fibrosisJournal of Cystic Fibrosis
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Molecular analysis of genes encoding CFTR interactors of SLC26 family in CF patients: preliminary results

2008

Pulmonary and Respiratory Medicinebusiness.industryPediatrics Perinatology and Child HealthMedicineEncoding (semiotics)Computational biologyPediatrics Perinatology and Child Healthbusinessmedicine.diseaseBioinformaticsGeneCystic fibrosisMolecular analysis
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Bronchial to subclavian shunt in a CF patient. A potential pitfall for embolization

2003

Bronchial artery embolization is a well accepted and widely used technique in the management of massive haemoptysis in cystic fibrosis (CF). It can be a complex procedure requiring a deep knowledge of the bronchial artery anatomy including the possible bronchial anastomoses. We report a case of complex vascular anatomy of the left bronchial artery with multiple anastomoses with the ipsilateral subclavian artery as cause of non-attempted embolization. © 2003 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Pulmonary and Respiratory Medicinemedicine.medical_specialtyHemoptysisAdolescentCystic FibrosisVascular anatomymedicine.medical_treatmentSubclavian ArteryBronchial ArteriesAnastomosisCystic fibrosisRisk AssessmentCatheterizationEmbolizationArterio-Arterial Fistulamedicine.arterymedicineLeft bronchial arteryHumansEmbolizationTreatment FailurePediatrics Perinatology and Child HealthSubclavian arteryBronchial arterybusiness.industryrespiratory systemmedicine.diseaseEmbolization Therapeuticrespiratory tract diseasesSurgeryShunt (medical)RadiographyPediatrics Perinatology and Child HealthFemaleRadiologyBronchial arterybusinessFollow-Up StudiesJournal of Cystic Fibrosis
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