Cystic Fibrosis Concept

subject area of

• Pseudomonas aeruginosaLasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator–IL-6–antimicrobial–repair pathway  Journal Articles
• ¹⁹F MRI of the Lungs Using Inert Fluorinated Gases: Challenges and New Developments  Journal Articles
• A divergent Pseudomonas aeruginosa palmitoyltransferase essential for cystic fibrosis‐specific lipid A  Journal Articles
• A novel lung disease phenotype adjusted for mortality attrition for cystic fibrosis Genetic modifier studies  Journal Articles
• A polymicrobial perspective of pulmonary infections exposes an enigmatic pathogen in cystic fibrosis patients  Journal Articles
• A randomized controlled trial of a 3-year home exercise program in cystic fibrosis  Journal Articles
• A study of the salivary glycoprotein in cystic fibrosis patients and controls: fucose incorporation and protein pattern  Journal Articles
• Abnormalities in the Pulmonary Innate Immune System in Cystic Fibrosis  Journal Articles
• Age and height dependence of lung clearance index and functional residual capacity  Journal Articles
• Alendronate Once Weekly for the Prevention and Treatment of Bone Loss in Canadian Adult Cystic Fibrosis Patients (CFOS Trial)  Journal Articles
• Alternative outcomes for the multiple breath washout in children with CF  Journal Articles
• 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  Journal Articles
• An international, multicentre evaluation and description of Burkholderia pseudomallei infection in cystic fibrosis  Journal Articles
• Analysis of factors limiting maximal exercise performance in cystic fibrosis  Journal Articles
• Antibiotic susceptibility and molecular mechanisms of macrolide resistance in streptococci isolated from adult cystic fibrosis patients  Journal Articles
• Assessment of body composition in pediatric patients with cystic fibrosis  Journal Articles
• Assessment of the Microbial Constituents of the Home Environment of Individuals with Cystic Fibrosis (CF) and Their Association with Lower Airways Infections  Journal Articles
• Attitudes of high school students toward carrier screening and prenatal diagnosis of cystic fibrosis  Journal Articles
• Azithromycin and the microbiota of cystic fibrosis sputum  Journal Articles
• Bisphosphonates for osteoporosis in people with cystic fibrosis  Journal Articles
• Bullying and mental health amongst Australian children and young people with cystic fibrosis.  Journal Articles
• CFTR2: How will it help care?  Journal Articles
• Cardiac Output Determination during Progressive Exercise in Cystic Fibrosis  Journal Articles
• Changes in multiple breath washout measures after raised volume rapid thoracoabdominal compression maneuvers in infants.  Conferences
• Characterization ofStreptococcus milleriGroup Isolates from Expectorated Sputum of Adult Patients with Cystic Fibrosis  Journal Articles
• Chest Physical Therapy Management of Patients with Cystic Fibrosis: A Meta-analysis  Journal Articles
• Clinical Insights into Pulmonary Exacerbations in Cystic Fibrosis from the Microbiome. What Are We Missing?  Journal Articles
• Clinical epidemiology and impact of Haemophilus influenzae airway infections in adults with cystic fibrosis.  Journal Articles
• Clinical implications and characterization of Group A Streptoccoccus infections in adults with cystic fibrosis  Journal Articles
• Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria: a randomised, double-blind, controlled clinical trial  Journal Articles
• Common content between quality of life questionnaires for children with cystic fibrosis and the International Classification of Functionality, Disability and Health.  Journal Articles
• Comparing the American and European diagnostic guidelines for cystic fibrosis: same disease, different language?  Journal Articles
• Comparison of three techniques for body composition analysis in cystic fibrosis  Journal Articles
• Complex Pseudomonas Population Structure in Cystic Fibrosis Airway Infections  Journal Articles
• Complex two-gene modulation of lung disease severity in children with cystic fibrosis  Journal Articles
• Correlation of Lung Clearance Index with Hyperpolarized ¹²⁹Xe Magnetic Resonance Imaging in Pediatric Subjects with Cystic Fibrosis  Journal Articles
• Culture Enriched Molecular Profiling of the Cystic Fibrosis Airway Microbiome  Journal Articles
• Culture-enriched metagenomic sequencing enables in-depth profiling of the cystic fibrosis lung microbiota  Journal Articles
• Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis  Journal Articles
• Cystic Fibrosis Patients Infected With Epidemic Pseudomonas aeruginosa Strains Have Unique Microbial Communities  Journal Articles
• Cystic Fibrosis: A Polymicrobial Infectious Disease  Journal Articles
• Cystic fibrosis and CFTR  Journal Articles
• Cystic fibrosis modifier genes related to Pseudomonas aeruginosa infection  Journal Articles
• Cystic fibrosis survival  Journal Articles
• Cystic fibrosis: past, present, future. Dr. Andreas Freitag in an interview with Dr. Roman Jaeschke  Journal Articles
• Cystic fibrosis–related diabetes onset can be predicted using biomarkers measured at birth  Journal Articles
• Cystic fibrosis—related inhibition of mucociliaryclearance in vivo in man  Journal Articles
• Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene  Journal Articles
• Determination of 4‐hydroxyproline‐2‐epimerase activity by capillary electrophoresis: A stereoselective platform for inhibitor screening of amino acid isomerases  Journal Articles
• Development and Validation of a PCR Assay To Detect the Prairie Epidemic Strain of Pseudomonas aeruginosa from Patients with Cystic Fibrosis  Journal Articles
• 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  Journal Articles
• Differences in hyperpolarized ³He ventilation imaging after 4 years in adults with cystic fibrosis  Journal Articles
• Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease  Journal Articles
• Distribution of CFTR mutations in Saguenay– Lac-Saint-Jean: proposal of a panel of mutations for population screening  Journal Articles
• Do common in silico tools predict the clinical consequences of amino‐acid substitutions in the CFTR gene?  Journal Articles
• Does Integration of Various Ion Channel Measurements Improve Diagnostic Performance in Cystic Fibrosis?  Journal Articles
• Does extensive genotyping and nasal potential difference testing clarify the diagnosis of cystic fibrosis among patients with single-organ manifestations of cystic fibrosis?  Journal Articles
• Does prophylaxis with palivizumab reduce hospitalisation rates for respiratory‐syncytial‐virus‐related infection in cystic fibrosis children less than 2 years of age?  Journal Articles
• Dry Powder versus Intravenous and Nebulized Gentamicin in Cystic Fibrosis and Bronchiectasis  Journal Articles
• Dry powder versus intravenous and nebulized gentamicin in cystic fibrosis and bronchiectasis - A pilot study  Journal Articles
• Early life nasal microbiota in infants with cystic fibrosis  Journal Articles
• Effect of freezing sputum on Pseudomonas aeruginosa population heterogeneity  Journal Articles
• Effects of inspiratory muscle training in cystic fibrosis: a systematic review  Journal Articles
• Epidemic Pseudomonas aeruginosa infection in patients with cystic fibrosis is not a risk factor for poor clinical Outcomes following lung transplantation  Journal Articles
• Epidemiology and impact of methicillin-sensitive Staphylococcus aureus with β-lactam antibiotic inoculum effects in adults with cystic fibrosis  Journal Articles
• Epidemiology of Clonal Pseudomonas aeruginosa Infection in a Canadian Cystic Fibrosis Population  Journal Articles
• Evaluation of the Disease Liability of CFTR Variants  Journal Articles
• Exacerbation frequency and clinical outcomes in adult patients with cystic fibrosis  Journal Articles
• Exercise and physical activity in children with cystic fibrosis  Journal Articles
• Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies  Journal Articles
• Experiences of adults with cystic fibrosis in adhering to medication regimens  Journal Articles
• Expert consensus on palivizumab use for respiratory syncytial virus in developed countries  Journal Articles
• Exploring the Cystic Fibrosis Lung Microbiome: Making the Most of a Sticky Situation  Journal Articles
• Factors Influencing Family Participation in a Longitutdinal Study: Comparison of Pediatric and Healthy Samples  Journal Articles
• Factors associated with response to treatment of pulmonary exacerbations in cystic fibrosis patients  Journal Articles
• Gender differences in habitual activity in children with cystic fibrosis  Journal Articles
• Gene Therapy for Pulmonary Diseases  Journal Articles
• Genetic Dissection of Diabetes: Facing the Giant  Journal Articles
• Genetic Modifiers of Liver Disease in Cystic Fibrosis  Journal Articles
• Genetic Modifiers of Lung Disease in Cystic Fibrosis  Journal Articles
• Genetic signature of bacterial pathogen adaptation during chronic pulmonary infections  Journal Articles
• Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2  Journal Articles
• Hyperpolarized 3He Magnetic Resonance Functional Imaging Semiautomated Segmentation  Journal Articles
• Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function  Journal Articles
• Impact of CFTR modulation with Ivacaftor on Gut Microbiota and Intestinal Inflammation  Journal Articles
• Impairment of mucociliary transport in cystic fibrosis.  Journal Articles
• Implementation of a cystic fibrosis lung transplant referral patient decision aid in routine clinical practice: an observational study  Journal Articles
• Increased Serum Vitamin A and E Levels After Lung Transplantation  Journal Articles
• Increased detection of interleukin-;5 in sputum by addition of protease inhibitors  Journal Articles
• Infant spirometry as a predictor of lung function at early childhood in cystic fibrosis patients  Journal Articles
• Infection With Transmissible Strains of Pseudomonas aeruginosa and Clinical Outcomes in Adults With Cystic Fibrosis  Journal Articles
• Infection control knowledge, beliefs and behaviours amongst cystic fibrosis patients with epidemic Pseudomonas aeruginosa  Journal Articles
• Inflammatory and growth factor response to continuous and intermittent exercise in youth with cystic fibrosis  Journal Articles
• Intussusception in Adults with Cystic Fibrosis: A Case Series with Review of the Literature  Journal Articles
• Lentiviral vectors pseudotyped with minimal filovirus envelopes increased gene transfer in murine lung  Journal Articles
• Limitations of electronic compensation for measuring plethysmographic airway resistance in infants  Journal Articles
• Living with cystic fibrosis: patient, family, and physician realities.  Journal Articles
• Long-term clinical outcomes of ‘Prairie Epidemic Strain’Pseudomonas aeruginosainfection in adults with cystic fibrosis  Journal Articles
• Longitudinal analysis of vertebral fracture and BMD in a Canadian cohort of adult cystic fibrosis patients  Journal Articles
• Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis  Journal Articles
• Longitudinal sampling of the lung microbiota in individuals with cystic fibrosis  Journal Articles
• Lung Clearance Index as an Outcome Measure for Clinical Trials in Young Children with Cystic Fibrosis. A Pilot Study Using Inhaled Hypertonic Saline  Journal Articles
• Lung clearance index in cystic fibrosis subjects treated for pulmonary exacerbations  Journal Articles
• Macrolide and Clindamycin Resistance inStreptococcus milleriGroup Isolates from the Airways of Cystic Fibrosis Patients  Journal Articles
• Maximal short-term exercise performance and ion regulation in cystic fibrosis  Journal Articles
• McKay agar enables routine quantification of the ‘Streptococcus milleri’ group in cystic fibrosis patients  Journal Articles
• Metabolic Signatures of Cystic Fibrosis Identified in Dried Blood Spots For Newborn Screening Without Carrier Identification  Journal Articles
• Modifier Gene Studies to Identify New Therapeutic Targets in Cystic Fibrosis  Journal Articles
• Modifier gene study of meconium ileus in cystic fibrosis: statistical considerations and gene mapping results  Journal Articles
• Modulation of Pseudomonas aeruginosa gene expression by host microflora through interspecies communication  Journal Articles
• Modulation of cAMP metabolism for CFTR potentiation in human airway epithelial cells  Journal Articles
• Modulatory effect of the SLC9A3 gene on susceptibility to infections and pulmonary function in children with cystic fibrosis  Journal Articles
• Mucociliary clearance.  Journal Articles
• Mucus-penetrating solid lipid nanoparticles for the treatment of cystic fibrosis: Proof of concept, challenges and pitfalls  Journal Articles
• Multiple Breath Nitrogen Washout: A Feasible Alternative to Mass Spectrometry  Journal Articles
• Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis  Journal Articles
• Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report  Journal Articles
• Multi‐centre ethics and research governance review can impede non‐interventional clinical research  Journal Articles
• Muscle Function and Resting Energy Expenditure in Female Athletes with Cystic Fibrosis  Journal Articles
• Nasal cilia in normal man, primary ciliary dyskinesia and other respiratory diseases: analysis of motility and ultrastructure.  Journal Articles
• No Evidence for Direct Activation of the Cystic Fibrosis Transmembrane Conductance Regulator by 8-Cyclopentyl-1,3-Dipropylxanthine  Journal Articles
• Off-label use of inhaled tobramycin in Ontario, Canada  Journal Articles
• Omalizumab for asthma and allergic bronchopulmonary aspergillosis in adults with cystic fibrosis  Journal Articles
• Osteoporosis in Canadian adult cystic fibrosis patients: A descriptive study  Journal Articles
• Palivizumab prophylaxis for respiratory syncytial virus in infants with cystic fibrosis: is there a need?  Journal Articles
• Periodic health examination, 1991 update: 4. Screening for cystic fibrosis. Canadian Task Force on the Periodic Health Examination.  Journal Articles
• Phenotypic Heterogeneity of Pseudomonas aeruginosa Populations in a Cystic Fibrosis Patient  Journal Articles
• Physiological correlates of pulmonary function in children with cystic fibrosis  Journal Articles
• Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis  Journal Articles
• Positron Emission Tomography and Computed Tomography versus Positron Emission Tomography Computed Tomography: Tools for Imaging the Lung  Journal Articles
• Potential Contributions of Anaerobes in Cystic Fibrosis Airways  Journal Articles
• Predictors of pulmonary exacerbations in patients with cystic fibrosis infected with multi-resistant bacteria  Journal Articles
• Prenatal Diagnosis of Genetic Disorders: Trials and Tribulations  Journal Articles
• Prevalence and Outcomes of Achromobacter Species Infections in Adults with Cystic Fibrosis: a North American Cohort Study  Journal Articles
• Prevalence of Low Bone Mass and Deficiencies of Vitamins D and K in Pediatric Patients With Cystic Fibrosis From 3 Canadian Centers  Journal Articles
• Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene  Journal Articles
• Progression of Lung Disease in Preschool Patients with Cystic Fibrosis  Journal Articles
• Psychologic Adjustment of the Family With a Member Who Has Cystic Fibrosis  Journal Articles
• Pulmonary Mucociliary Clearance in Cystic Fibrosis  Journal Articles
• Pulmonary rehabilitation programs in Canada: national survey.  Journal Articles
• Quality of Life in Cystic Fibrosis  Journal Articles
• Quantitative Evaluation of Hyperpolarized Helium-3 Magnetic Resonance Imaging of Lung Function Variability in Cystic Fibrosis  Journal Articles
• Randomized Trial of a Decision Aid for Patients with Cystic Fibrosis Considering Lung Transplantation  Journal Articles
• Randomized controlled study of in‐hospital exercise training programs in children with cystic fibrosis  Journal Articles
• Randomized controlled trial of biofilm antimicrobial susceptibility testing in cystic fibrosis patients  Journal Articles
• Rapid chloride and bicarbonate determination by capillary electrophoresis for confirmatory testing of cystic fibrosis infants with volume-limited sweat specimens  Journal Articles
• Redefining Disease? The Nosologic Implications of Molecular Genetic Knowledge  Journal Articles
• Reduced fat oxidation rates during submaximal exercise in boys with cystic fibrosis  Journal Articles
• Reduction in Pseudomonas aeruginosa sputum density during a cystic fibrosis pulmonary exacerbation does not predict clinical response  Journal Articles
• Reemergence of Lower-Airway Microbiota in Lung Transplant Patients with Cystic Fibrosis  Journal Articles
• Relationships among nutritional status and skeletal and respiratory muscle function in cystic fibrosis: does early dietary supplementation make a difference?  Journal Articles
• Reliability and validity of the habitual activity estimation scale (HAES) in patients with cystic fibrosis  Journal Articles
• Respiratory Syncytial Virus Immunoprophylaxis with Palivizumab: 12-Year Observational Study of Usage and Outcomes in Canada  Journal Articles
• Respiratory and Peripheral Muscle Function in Cystic Fibrosis  Journal Articles
• Respiratory syncytial virus prophylaxis in cystic fibrosis: the Canadian registry of palivizumab data (2005–2016)  Journal Articles
• Semiautomated Segmentation and Analysis of Airway Lumen in Pediatric Patients Using Ultra Short Echo Time MRI  Journal Articles
• Serum alpha - fetoprotein levels in patients with cystic fibrosis and their parents and siblings.  Journal Articles
• Significant differences in type IV pilin allele distribution among Pseudomonas aeruginosa isolates from cystic fibrosis (CF) versus non-CF patients  Conferences
• Skeletal Muscle Metabolism in Cystic Fibrosis and Primary Ciliary Dyskinesia  Journal Articles
• Sputum microbiota in adults with CF associates with response to inhaled tobramycin  Journal Articles
• Sputum microbiota is predictive of long-term clinical outcomes in young adults with cystic fibrosis  Journal Articles
• Staphylococcus aureus Protein A Mediates Interspecies Interactions at the Cell Surface of Pseudomonas aeruginosa  Journal Articles
• Subclinical anaemia of chronic disease in adult patients with cystic fibrosis  Journal Articles
• Sweat collection for testing in Canadian Cystic Fibrosis Centers, is it optimal?  Journal Articles
• Sweat‐testing: A review of current technical requirements  Journal Articles
• Synergistic Effects of cAMP- and Calcium-Mediated Amylase Secretion in Isolated Pancreatic Acini from Cystic Fibrosis Mice  Journal Articles
• Systematic Review of Postural Assessment in Individuals With Obstructive Respiratory Conditions  Journal Articles
• TLR5 as an Anti-Inflammatory Target and Modifier Gene in Cystic Fibrosis  Journal Articles
• The Streptococcus milleri Population of a Cystic Fibrosis Clinic Reveals Patient Specificity and Intraspecies Diversity  Journal Articles
• The Streptococcus milleri group—An unrecognized cause of disease in cystic fibrosis: A case series and literature review  Journal Articles
• The Cystic Fibrosis Gene: A Molecular Genetic Perspective  Journal Articles
• The Cystic Fibrosis Lung Microbiome  Journal Articles
• The Efficiency of Sputum Cell Counts in Cystic Fibrosis  Journal Articles
• The Evolving Cystic Fibrosis Microbiome: A Comparative Cohort Study Spanning 16 Years  Journal Articles
• The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus  Journal Articles
• 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)  Journal Articles
• The Relationship between Genotype and Exercise Tolerance in Children with Cystic Fibrosis  Journal Articles
• The cystic fibrosis airway microbiome  Journal Articles
• The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis  Journal Articles
• The effect of early Pseudomonas aeruginosa treatment on lung function in pediatric cystic fibrosis  Journal Articles
• The effects of acute and chronic exercise on inflammatory markers in children and adults with a chronic inflammatory disease: a systematic review.  Journal Articles
• The effects of cycled inhaled aztreonam on the cystic fibrosis (CF) lung microbiome  Journal Articles
• The effects of inhaled aztreonam on the cystic fibrosis lung microbiome  Journal Articles
• The genus Prevotella in cystic fibrosis airways  Journal Articles
• The musculoskeletal manifestations of cystic fibrosis  Journal Articles
• The natural history and genetic diversity of Haemophilus influenzae infecting the airways of adults with cystic fibrosis  Journal Articles
• 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.  Journal Articles
• The relevance of the polymicrobial nature of airway infection in the acute and chronic management of patients with cystic fibrosis.  Journal Articles
• Treatment of Aspergillus fumigatus in Patients with Cystic Fibrosis: A Randomized, Placebo-Controlled Pilot Study  Journal Articles
• Triaging Access to Critical Care Resources in Patients With Chronic Respiratory Diseases in the Event of a Major COVID-19 Surge  Journal Articles
• Twenty-Five-Year Outbreak of Pseudomonas aeruginosa Infecting Individuals with Cystic Fibrosis: Identification of the Prairie Epidemic Strain  Journal Articles
• Type of CFTR Mutation Determines Risk of Pancreatitis in Patients With Cystic Fibrosis  Journal Articles
• Understanding the natural history and clinical implications of airways infections in CF — Moving beyond the classics  Journal Articles
• Understanding the population structure of North American patients with cystic fibrosis  Journal Articles
• Uptake of¹⁸fluorodeoxyglucose in the cystic fibrosis lung: a measure of lung inflammation?  Journal Articles
• Using photo-elicitation to explore perceptions of physical activity among young people with cystic fibrosis  Journal Articles
• Validation of shuttle tests in children with cystic fibrosis  Journal Articles
• Validity of the Stage of Exercise Scale in Children with Rheumatologic Conditions  Journal Articles
• Voluntary dehydration and heat intolerance in cystic fibrosis  Journal Articles
• Voriconazole-Induced Periostitis Deformans: A Mimicker of Hypertrophic Pulmonary Osteoarthropathy  Journal Articles
• What to Expect When You’re Expectorating: Cystic Fibrosis Exacerbations and Microbiota  Journal Articles
• α₁-Antitrypsin Deficiency Alleles in Cystic Fibrosis Lung Disease  Journal Articles