Cystic Fibrosis Concept

subject area of

• A divergentPseudomonas aeruginosapalmitoyltransferase essential for cystic fibrosis-specific lipid A  Academic Article
• A novel lung disease phenotype adjusted for mortality attrition for cystic fibrosis Genetic modifier studies  Academic Article
• A polymicrobial perspective of pulmonary infections exposes an enigmatic pathogen in cystic fibrosis patients  Academic Article
• A randomized controlled trial of a 3-year home exercise program in cystic fibrosis  Academic Article
• A study of the salivary glycoprotein in cystic fibrosis patients and controls: fucose incorporation and protein pattern  Academic Article
• Abnormalities in the Pulmonary Innate Immune System in Cystic Fibrosis  Academic Article
• Absence of typical unfolded protein response in primary cultured cystic fibrosis airway epithelial cells  Academic Article
• Age and height dependence of lung clearance index and functional residual capacity  Academic Article
• Aggregates of mutant CFTR fragments in airway epithelial cells of CF lungs: New pathologic observations  Academic Article
• Alendronate Once Weekly for the Prevention and Treatment of Bone Loss in Canadian Adult Cystic Fibrosis Patients (CFOS Trial)  Academic Article
• Alternative outcomes for the multiple breath washout in children with CF  Academic Article
• An Official American Thoracic Society Workshop Report: Optimal Lung Function Tests for Monitoring Cystic Fibrosis, Bronchopulmonary Dysplasia, and Recurrent Wheezing in Children Less Than 6 Years of Age  Academic Article
• An international, multicentre evaluation and description of Burkholderia pseudomallei infection in cystic fibrosis  Academic Article
• Analysis of factors limiting maximal exercise performance in cystic fibrosis  Academic Article
• Analysis of repeated events  Academic Article
• Analysis of the Cystic Fibrosis Lung Microbiota via Serial Illumina Sequencing of Bacterial 16S rRNA Hypervariable Regions  Academic Article
• Antibiotic susceptibility and molecular mechanisms of macrolide resistance in streptococci isolated from adult cystic fibrosis patients  Academic Article
• Aspergillus fumigatusenhances elastase production inPseudomonas aeruginosaco-cultures  Academic Article
• Assessment of body composition in pediatric patients with cystic fibrosis  Academic Article
• Assessment of the Microbial Constituents of the Home Environment of Individuals with Cystic Fibrosis (CF) and Their Association with Lower Airways Infections  Academic Article
• Attitudes of high school students toward carrier screening and prenatal diagnosis of cystic fibrosis  Academic Article
• Bisphosphonates for osteoporosis in people with cystic fibrosis  Academic Article
• Bone marrow-derived progenitor cells in end-stage lung disease patients  Academic Article
• Cardiac Output Determination during Progressive Exercise in Cystic Fibrosis  Academic Article
• Changes in multiple breath washout measures after raised volume rapid thoracoabdominal compression maneuvers in infants.  Conference Paper
• Characterization of Streptococcus milleri Group Isolates from Expectorated Sputum of Adult Patients with Cystic Fibrosis  Academic Article
• Chest Physical Therapy Management of Patients with Cystic Fibrosis: A Meta-analysis  Academic Article
• Chronic childhood illnesses: what aspects of caregiving do parents value?  Academic Article
• Clinical Insights into Pulmonary Exacerbations in Cystic Fibrosis from the Microbiome. What Are We Missing?  Academic Article
• Clinical implications and characterization of Group A Streptoccoccus infections in adults with cystic fibrosis  Academic Article
• Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria: a randomised, double-blind, controlled clinical trial  Academic Article
• Comparing the American and European diagnostic guidelines for cystic fibrosis: same disease, different language?  Academic Article
• Comparison of three techniques for body composition analysis in cystic fibrosis  Academic Article
• Complex two-gene modulation of lung disease severity in children with cystic fibrosis  Academic Article
• ComplexPseudomonasPopulation Structure in Cystic Fibrosis Airway Infections  Academic Article
• Correlation of Lung Clearance Index with Hyperpolarized 129Xe Magnetic Resonance Imaging in Pediatric Subjects with Cystic Fibrosis  Academic Article
• Culture Enriched Molecular Profiling of the Cystic Fibrosis Airway Microbiome  Academic Article
• Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis  Academic Article
• Cystic fibrosis and CFTR  Academic Article
• Cystic fibrosis modifier genes related to Pseudomonas aeruginosa infection  Academic Article
• Cystic fibrosis patients bearing both the common missense mutation Gly----Asp at codon 551 and the delta F508 mutation are clinically indistinguishable from delta F508 homozygotes, except for decreased risk of meconium ileus.  Academic Article
• Cystic fibrosis: a polymicrobial infectious disease  Academic Article
• Cystic fibrosis: past, present, future. Dr. Andreas Freitag in an interview with Dr. Roman Jaeschke  Academic Article
• Cystic fibrosis—related inhibition of mucociliaryclearance in vivo in man  Academic Article
• Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene  Academic Article
• Determination of 4-hydroxyproline-2-epimerase activity by capillary electrophoresis: A stereoselective platform for inhibitor screening of amino acid isomerases  Academic Article
• Development and Validation of a PCR Assay To Detect the Prairie Epidemic Strain of Pseudomonas aeruginosa from Patients with Cystic Fibrosis  Academic Article
• Development of Real-Time PCR Assays for Detection of the Streptococcus milleri Group from Cystic Fibrosis Clinical Specimens by Targeting the cpn60 and 16S rRNA Genes  Academic Article
• Directed evolution of adeno-associated virus to an infectious respiratory virus  Academic Article
• Distribution of CFTR mutations in Saguenay– Lac-Saint-Jean: proposal of a panel of mutations for population screening  Academic Article
• Do commonin silicotools predict the clinical consequences of amino-acid substitutions in the CFTR gene?  Academic Article
• Does Integration of Various Ion Channel Measurements Improve Diagnostic Performance in Cystic Fibrosis?  Academic Article
• Does extensive genotyping and nasal potential difference testing clarify the diagnosis of cystic fibrosis among patients with single-organ manifestations of cystic fibrosis?  Academic Article
• Does prophylaxis with palivizumab reduce hospitalisation rates for respiratory-syncytial-virus-related infection in cystic fibrosis children less than 2 years of age?  Academic Article
• Dry Powder versus Intravenous and Nebulized Gentamicin in Cystic Fibrosis and Bronchiectasis  Academic Article
• Early life nasal microbiota in infants with cystic fibrosis  Academic Article
• Effect of chest physiotherapy on the removal of mucus in patients with cystic fibrosis.  Academic Article
• Effect of freezing sputum on Pseudomonas aeruginosa population heterogeneity  Academic Article
• Effect of infection with transmissible strains of Pseudomonas aeruginosa on lung transplantation outcomes in patients with cystic fibrosis  Academic Article
• Effects of Recipient Age and Diagnosis on Health-related Quality-of-Life Benefit of Lung Transplantation  Academic Article
• Effects of inspiratory muscle training in cystic fibrosis: a systematic review  Academic Article
• Epidemic Pseudomonas aeruginosa infection in patients with cystic fibrosis is not a risk factor for poor clinical Outcomes following lung transplantation  Academic Article
• Evaluation of the Disease Liability of CFTR Variants  Academic Article
• Exacerbation frequency and clinical outcomes in adult patients with cystic fibrosis  Academic Article
• Exercise and physical activity in children with cystic fibrosis  Academic Article
• Experiences of adults with cystic fibrosis in adhering to medication regimens  Academic Article
• Factors Influencing Family Participation in a Longitutdinal Study: Comparison of Pediatric and Healthy Samples  Academic Article
• Factors associated with response to treatment of pulmonary exacerbations in cystic fibrosis patients  Academic Article
• Feline immunodeficiency virus vectors persistently transduce nondividing airway epithelia and correct the cystic fibrosis defect  Academic Article
• GI Complications After Lung Transplantation in Patients With Cystic Fibrosis  Academic Article
• Gender differences in habitual activity in children with cystic fibrosis  Academic Article
• Gene Therapy for Pulmonary Diseases  Academic Article
• Genetic Dissection of Diabetes: Facing the Giant  Academic Article
• Genetic Modifiers of Liver Disease in Cystic Fibrosis  Academic Article
• Genetic Modifiers of Lung Disease in Cystic Fibrosis  Academic Article
• Genetic signature of bacterial pathogen adaptation during chronic pulmonary infections  Academic Article
• Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2  Academic Article
• Home nebulizers in patients with cystic fibrosis  Academic Article
• Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance  Academic Article
• Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function  Academic Article
• Impairment of mucociliary transport in cystic fibrosis.  Academic Article
• Implementation of a cystic fibrosis lung transplant referral patient decision aid in routine clinical practice: an observational study  Academic Article
• In Vivo Fluorescence Imaging of Bacteriogenic Cyanide in the Lungs of Live Mice Infected with Cystic Fibrosis Pathogens  Academic Article
• Incidence and prevalence of diabetes mellitus in patients with cystic fibrosis undergoing lung transplantation before and after lung transplantation*  Academic Article
• Increased Serum Vitamin A and E Levels After Lung Transplantation  Academic Article
• Increased detection of interleukin-5 in sputum by addition of protease inhibitors  Academic Article
• Infection With Transmissible Strains of Pseudomonas aeruginosa and Clinical Outcomes in Adults With Cystic Fibrosis  Academic Article
• Infection control knowledge, beliefs and behaviours amongst cystic fibrosis patients with epidemic Pseudomonas aeruginosa  Academic Article
• Infection withBurkholderia cepaciain Cystic Fibrosis  Academic Article
• Inflammatory and growth factor response to continuous and intermittent exercise in youth with cystic fibrosis  Academic Article
• Intermediate-term Outcome in Lung Transplantation From a Donor With Glioblastoma Multiforme  Academic Article
• International Guidelines for the Selection of Lung Transplant Candidates: 2006 Update—A Consensus Report From the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation  Academic Article
• Limitations of electronic compensation for measuring plethysmographic airway resistance in infants  Academic Article
• Long-term clinical outcomes of ‘Prairie Epidemic Strain’ Pseudomonas aeruginosa infection in adults with cystic fibrosis  Academic Article
• Longitudinal analysis of vertebral fracture and BMD in a Canadian cohort of adult cystic fibrosis patients  Academic Article
• Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis  Academic Article
• Longitudinal sampling of the lung microbiota in individuals with cystic fibrosis  Academic Article
• Lung Clearance Index as an Outcome Measure for Clinical Trials in Young Children with Cystic Fibrosis. A Pilot Study Using Inhaled Hypertonic Saline  Academic Article
• Lung Defense Mechanisms  Academic Article
• Lung clearance index in cystic fibrosis subjects treated for pulmonary exacerbations  Academic Article
• Lung transplantation for cystic fibrosis: an update  Academic Article
• Macrolide and Clindamycin Resistance in Streptococcus milleri Group Isolates from the Airways of Cystic Fibrosis Patients  Academic Article
• Maximal short-term exercise performance and ion regulation in cystic fibrosis  Academic Article
• McKay agar enables routine quantification of the 'Streptococcus milleri' group in cystic fibrosis patients  Academic Article
• Modifier Gene Studies to Identify New Therapeutic Targets in Cystic Fibrosis  Academic Article
• Modifier gene study of meconium ileus in cystic fibrosis: statistical considerations and gene mapping results  Academic Article
• Modulation of Pseudomonas aeruginosa gene expression by host microflora through interspecies communication  Academic Article
• Modulatory effect of the SLC9A3 gene on susceptibility to infections and pulmonary function in children with cystic fibrosis  Academic Article
• Multiple Breath Nitrogen Washout: A Feasible Alternative to Mass Spectrometry  Academic Article
• Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis  Academic Article
• Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report  Academic Article
• Muscle Function and Resting Energy Expenditure in Female Athletes with Cystic Fibrosis  Academic Article
• Nasal cilia in normal man, primary ciliary dyskinesia and other respiratory diseases: analysis of motility and ultrastructure.  Academic Article
• No Evidence for Direct Activation of the Cystic Fibrosis Transmembrane Conductance Regulator by 8-Cyclopentyl-1,3-Dipropylxanthine  Academic Article
• Off-label use of inhaled tobramycin in Ontario, Canada  Academic Article
• Osteoporosis in Canadian adult cystic fibrosis patients: A descriptive study  Academic Article
• Outcomes of patients with cystic fibrosis undergoing lung transplantation with and without cystic fibrosis-associated liver cirrhosis*  Academic Article
• Palivizumab prophylaxis for respiratory syncytial virus in infants with cystic fibrosis: is there a need?  Academic Article
• Phenotypic Heterogeneity of Pseudomonas aeruginosa Populations in a Cystic Fibrosis Patient  Academic Article
• Phenotypic diversity within a Pseudomonas aeruginosa population infecting an adult with cystic fibrosis  Academic Article
• Physiological correlates of pulmonary function in children with cystic fibrosis  Academic Article
• Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis  Academic Article
• Positron Emission Tomography and Computed Tomography versus Positron Emission Tomography Computed Tomography: Tools for Imaging the Lung  Academic Article
• Predictors of pulmonary exacerbations in patients with cystic fibrosis infected with multi-resistant bacteria  Academic Article
• Prenatal diagnosis of genetic disorders: trials and tribulations.  Academic Article
• Pretransplant Aspergillus Colonization of Cystic Fibrosis Patients and the Incidence of Post–Lung Transplant Invasive Aspergillosis  Academic Article
• Prevalence and Outcomes of Achromobacter Species Infections in Adults with Cystic Fibrosis: a North American Cohort Study  Academic Article
• Prevalence of Low Bone Mass and Deficiencies of Vitamins D and K in Pediatric Patients With Cystic Fibrosis From 3 Canadian Centers  Academic Article
• Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene  Academic Article
• Primary ciliary dyskinesia: what has it taught us about pulmonary disease?  Academic Article
• Progression of Lung Disease in Preschool Patients with Cystic Fibrosis  Academic Article
• Pseudomonas aeruginosaLasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator–IL-6–antimicrobial–repair pathway  Academic Article
• Pulmonary rehabilitation programs in Canada: national survey.  Academic Article
• Quality of Life in Cystic Fibrosis  Academic Article
• Randomized Trial of a Decision Aid for Patients with Cystic Fibrosis Considering Lung Transplantation  Academic Article
• Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis  Academic Article
• Randomized controlled trial of biofilm antimicrobial susceptibility testing in cystic fibrosis patients  Academic Article
• Redefining Disease? The Nosologic Implications of Molecular Genetic Knowledge  Academic Article
• Reduced fat oxidation rates during submaximal exercise in boys with cystic fibrosis  Academic Article
• Reduction in Pseudomonas aeruginosa sputum density during a cystic fibrosis pulmonary exacerbation does not predict clinical response  Academic Article
• Reemergence of Lower-Airway Microbiota in Lung Transplant Patients with Cystic Fibrosis  Academic Article
• Relationships among nutritional status and skeletal and respiratory muscle function in cystic fibrosis: does early dietary supplementation make a difference?  Academic Article
• Reliability and validity of the habitual activity estimation scale (HAES) in patients with cystic fibrosis  Academic Article
• Respiratory and Peripheral Muscle Function in Cystic Fibrosis  Academic Article
• Respiratory syncytial virus prophylaxis in cystic fibrosis: the Canadian registry of palivizumab data (2005–2016)  Academic Article
• Responses of Well-Differentiated Airway Epithelial Cell Cultures from Healthy Donors and Patients with Cystic Fibrosis to Burkholderia cenocepacia Infection  Academic Article
• Serum alpha - fetoprotein levels in patients with cystic fibrosis and their parents and siblings.  Academic Article
• Significant differences in type IV pilin allele distribution among Pseudomonas aeruginosa isolates from cystic fibrosis (CF) versus non-CF patients  Conference Paper
• Skeletal Muscle Metabolism in Cystic Fibrosis and Primary Ciliary Dyskinesia  Academic Article
• Subclinical anaemia of chronic disease in adult patients with cystic fibrosis  Academic Article
• Survival of Burkholderia cepacia sepsis following lung transplantation in recipients with cystic fibrosis  Academic Article
• Survival of Lung Transplant Patients With Cystic Fibrosis Harboring Panresistant Bacteria Other Than Burkholderia cepacia, Compared With Patients Harboring Sensitive Bacteria  Academic Article
• Sweat collection for testing in Canadian Cystic Fibrosis Centers, is it optimal?  Academic Article
• Sweat-testing: A review of current technical requirements  Academic Article
• Systematic Review of Postural Assessment in Individuals With Obstructive Respiratory Conditions  Academic Article
• TLR5 as an Anti-Inflammatory Target and Modifier Gene in Cystic Fibrosis  Academic Article
• The Cystic Fibrosis Gene: A Molecular Genetic Perspective  Academic Article
• The Cystic Fibrosis Lung Microbiome  Academic Article
• The Evolving Cystic Fibrosis Microbiome: A Comparative Cohort Study Spanning 16 Years  Academic Article
• The Internet is valid and reliable for child-report: An example using the Activities Scale for Kids (ASK) and the Pediatric Quality of Life Inventory (PedsQL)  Academic Article
• The Relationship between Genotype and Exercise Tolerance in Children with Cystic Fibrosis  Academic Article
• The Streptococcus milleri Population of a Cystic Fibrosis Clinic Reveals Patient Specificity and Intraspecies Diversity  Academic Article
• The cystic fibrosis airway microbiome  Academic Article
• The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis  Academic Article
• The effect of early Pseudomonas aeruginosa treatment on lung function in pediatric cystic fibrosis  Academic Article
• The effects of acute and chronic exercise on inflammatory markers in children and adults with a chronic inflammatory disease: a systematic review.  Academic Article
• The effects of inhaled aztreonam on the cystic fibrosis lung microbiome  Academic Article
• The genus Prevotella in cystic fibrosis airways  Academic Article
• The polymicrobial nature of airway infections in cystic fibrosis: Cangene Gold Medal LectureThis article is based on a presentation by Dr. Christopher Sibley at the 60th Annual Meeting of the Canadian Society of Microbiologists in Hamilton, Ontario, on 15 June 2010. Dr. Sibley was the recipient of the 2010 Cangene Gold Medal as the Canadian Graduate Student Microbiologist of the Year, an annual award sponsored by Cangene Corporation intended to recognize excellence in graduate research.  Academic Article
• The relevance of the polymicrobial nature of airway infection in the acute and chronic management of patients with cystic fibrosis.  Academic Article
• TheStreptococcus milleri group—An unrecognized cause of disease in cystic fibrosis: A case series and literature review  Academic Article
• Treatment of Aspergillus fumigatus in Patients with Cystic Fibrosis: A Randomized, Placebo-Controlled Pilot Study  Academic Article
• Twenty-Five-Year Outbreak of Pseudomonas aeruginosa Infecting Individuals with Cystic Fibrosis: Identification of the Prairie Epidemic Strain  Academic Article
• Twenty-year experience of lung transplantation at a single center: influence of recipient diagnosis on long-term survival  Academic Article
• Type of CFTR Mutation Determines Risk of Pancreatitis in Patients With Cystic Fibrosis  Academic Article
• Understanding the natural history and clinical implications of airways infections in CF — Moving beyond the classics  Academic Article
• Understanding the population structure of North American patients with cystic fibrosis  Academic Article
• Uptake of18fluorodeoxyglucose in the cystic fibrosis lung: a measure of lung inflammation?  Academic Article
• Validation of shuttle tests in children with cystic fibrosis  Academic Article
• Validity of the Stage of Exercise Scale in Children with Rheumatologic Conditions  Academic Article
• Voluntary dehydration and heat intolerance in cystic fibrosis  Academic Article
• What to Expect When You’re Expectorating: Cystic Fibrosis Exacerbations and Microbiota  Academic Article
• α1-Antitrypsin Deficiency Alleles in Cystic Fibrosis Lung Disease  Academic Article