Pending

Genome features and antibiotic resistance of Pseudomonas aeruginosa strains isolated in patients with cystic fibrosis in the Russian Federation
The prevalence of CFTR mutations in patients with chronic rhinosinusitis: A systematic review and meta-analysis
Cystic Fibrosis Increases Long-Term Revision Rates of Endoscopic Sinus Surgery in Patients With Comorbid Chronic Rhinosinusitis
Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis
Myriad Faces of Active Tuberculosis: Intrapulmonary Bronchial Artery Pseudoaneurysm
Evaluation of different phenotypic methods to detect methicillin resistance in Staphylococcus aureus isolates recovered from cystic fibrosis patients
VX-809 mitigates disease in a mouse model of autosomal dominant polycystic kidney disease bearing the R3277C human mutation
Zebrafish Embryo Infection Model to Investigate Pseudomonas aeruginosa Interaction With Innate Immunity and Validate New Therapeutics
Following the Trace of HVS II Mitochondrial Region Within the Nine Iranian Ethnic Groups Based on Genetic Population Analysis
Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis
Gastrointestinal manifestations of cystic fibrosis in children
Corrigendum to: “Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance”
The Role of Nurses in the Management of Respiratory Disorders in Children
The tyrosine-kinase inhibitor Nintedanib ameliorates autosomal-dominant polycystic kidney disease
Prevalence, variability, and predictors of sinus surgery in pediatric patients with cystic fibrosis
Impact of the SARS-CoV-2 pandemic on cystic fibrosis centres and care: survey results from US centres
Assessment of the microbial load of airway clearance devices used by a cohort of children with cystic fibrosis
Health Disparity in CF: Perspectives from a Lived Experience
Bacteria in cystic fibrosis: United they stand – Recent advances in microbiology research through the eyes of ENS students
The molecular evolution of function in the CFTR chloride channel
The horizontal transfer of Pseudomonas aeruginosa PA14 ICE PAPI-1 is controlled by a transcriptional triad between TprA, NdpA2 and MvaT
A small molecule CFTR potentiator restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR
A Phase 3 Study of VX-121 Combination Therapy in Participants With Cystic Fibrosis (CF) Who Are Homozygous for F508del, Heterozygous for F508del and a Gating (F/G) or Residual Function (F/RF) Mutation, or Have At Least 1 Other Triple Combination Responsive (TCR) CFTR Mutation and No F508del Mutation
Parasite Survival and Disease Persistence in Cystic Fibrosis, Schistosomiasis and Pathogenic Bacterial Diseases: A Role for Universal Stress Proteins?
SARS-CoV-2 driving rapid change in adult cystic fibrosis services: the role of the clinical nurse specialist
Immunometabolic factors in adolescent chronic disease are associated with Th1 skewing of invariant Natural Killer T cells
Renal plasticity revealed through reversal of polycystic kidney disease in mice
Prevalence and factors associated with urinary incontinence in females with cystic fibrosis: an Italian single-centre cross-sectional analysis
Increased Plasma YKL-40 Level and Chitotriosidase Activity in Cystic Fibrosis Patients
P. aeruginosa Induced Lipid Peroxidation Causes Ferroptotic Cell Death in Airways
Efficacy of epetraborole against Mycobacterium abscessus is increased with norvaline
Peripherally Inserted Central Catheters: 100% success providing 13 day antibiotic therapy in cystic fibrosis patients aged 2 to 18 years
A systematic Cochrane Review of the timing of pancreatic enzyme replacement therapy (PERT) in cystic fibrosis
The feasibility of home monitoring of young people with cystic fibrosis: Results from CLIMB-CF
Isolation of Total RNA from Pseudomonas aeruginosa within Biofilms for Measuring Gene Expression
Preconception risk assessment for thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease
Sheep models of F508del and G542X cystic fibrosis mutations show cellular responses to human therapeutics
Epidemiology of Mycobacterium abscessus in England: an observational study
Risk factors for Pseudomonas aeruginosa airway infection and lung function decline in children with cystic fibrosis
Outcomes of inpatient cholecystectomy among adults with cystic fibrosis in the United States
Cysteamine Inhibits Glycine Utilisation and Disrupts Virulence in Pseudomonas aeruginosa
Regional brain tissue changes in patients with cystic fibrosis
Growth medium and nitric oxide alter Mycobacterium abscessus morphotype and virulence
Impact of timing of PERT on gastrointestinal symptoms in Danish children and adolescents with CF
Spray-freeze-dried inhalable composite microparticles containing nanoparticles of combinational drugs for potential treatment of lung infections caused by Pseudomonas aeruginosa
Combined use of rheology and portable low-field NMR in cystic fibrosis patients
COVID-19 outcomes in people with cystic fibrosis
Granulocytic Myeloid-Derived Suppressor Cells in Cystic Fibrosis
Prospectively predicting Pseudomonas aeruginosa infection/s using routine data from the UK cystic fibrosis register
Evaluation of anthropometry as an alternative to DXA as predictor of low bone mineral density in children and adolescents with cystic fibrosis
Sweat testing in the modern era: A national survey of sweat testing practice in the Republic of Ireland
The Role of Telehealth Services in Children with Cystic Fibrosis During Coronavirus Disease 2019 Outbreak
The favorable prognosis of cystic biliary atresia may be related to early surgery and mild liver pathological changes
Screening strategy for gastrointestinal and hepatopancreatobiliary cancers in cystic fibrosis
Severe Spontaneous Pneumomediastinum in a Girl with Cystic Fibrosis
Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment
Culture with apically applied healthy or disease sputum alters the airway surface liquid proteome and ion transport across human bronchial epithelial cells
Divergence of bacterial communities in the lower airways of CF patients in early childhood
Study to Evaluate Symptoms of Exocrine Pancreatic Insufficiency in Adult Participants With Cystic Fibrosis or Chronic Pancreatitis Treated With Creon
Characteristics of electrolyte imbalance and pseudo-bartter syndrome in hospitalized cystic fibrosis children and adolescents
Cystic Fibrosis Foundation Nebulizer and Compressor Accessibility Survey
cAMP triggers Na(+) absorption by distal airway surface epithelium in cystic fibrosis swine
beta-Catenin-NFkB-CFTR interactions in cholangiocytes regulate inflammation and fibrosis during ductular reaction
CFTR Rescue in Intestinal Organoids with GLPG/ABBV-2737, ABBV/GLPG-2222 and ABBV/GLPG-2451 Triple Therapy
A natural language processing pipeline to synthesize patient-generated notes toward improving remote care and chronic disease management: a cystic fibrosis case study
Improvement of anti-biofilm activities via co-delivery of curcumin and gentamicin in lipid-polymer hybrid nanoparticle
Tigecycline-associated acute pancreatitis in a child with pulmonary cystic fibrosis: A case report and literature review
BACH1, the master regulator of oxidative stress, has a dual effect on CFTR expression
Retracing changes in cystic fibrosis understanding and management over the past twenty years
Demographic characteristics, clinical and laboratory features, and the distribution of pathogenic variants in the CFTR gene in the Cypriot cystic fibrosis (CF) population demonstrate the utility of a national CF patient registry
Comparison of the readability of lay summaries and scientific abstracts published in CF Research News and the Journal of Cystic Fibrosis: Recommendations for writing lay summaries
Effects of environmental air pollutants on CFTR expression and function in human airway epithelial cells
Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy
Improved survival albeit with persistent disparities in prognosis for people with cystic fibrosis in European countries
Neutrophil Elastase Triggers the Release of Macrophage Extracellular Traps: Relevance to CF
The Effect of COVID-19 on Anxiety Levels of Children with CF and Healthy Peers
Pregnancy rates and outcomes amongst women with cystic fibrosis in the UK: comparisons with the general population before and after the introduction of disease modifying treatment, 2003-17
A Rapid and Sensitive Detection Method for Pseudomonas aeruginosa Using Visualized Recombinase Polymerase Amplification and Lateral Flow Strip Technology
2-Alkyl-4-quinolone quorum sensing molecules are biomarkers for culture-independent Pseudomonas aeruginosa burden in adults with cystic fibrosis
A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis
Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging
Cystic Fibrosis Transmembrane Conductance Regulator Modulators During Pregnancy: A Case Series
Administration of pilocarpine by microneedle patch as a novel method for cystic fibrosis sweat testing
Duration of intravenous antibiotic treatment for acute exacerbations of cystic fibrosis: A systematic review: Duration of treatment for acute exacerbations of cystic fibrosis
Rothia mucilaginosa is an anti-inflammatory bacterium in the respiratory tract of patients with chronic lung disease
Mislocalization of CFTR expression in acute pancreatitis and the beneficial effect of VX-661 + VX-770 treatment on disease severity
Interplay of OpdP Porin and Chromosomal Carbapenemases in the Determination of Carbapenem Resistance/Susceptibility in Pseudomonas aeruginosa
Assessing Psychosocial Distress in Cystic Fibrosis: Validation of the ‘Distress in Cystic Fibrosis Scale’
Elexacaftor/tezacaftor/ivacaftor for CFTR variants giving rise to diagnostic uncertainty: Personalised medicine or over-medicalisation?
Clearance of 25-hydroxyvitamin D in Cystic Fibrosis
A rare case of pancytopenia in a child with cystic fibrosis: Can copper cure it all?
Risk factors leading to pulmonary exacerbation in patients with cystic fibrosis: A systematic review
‘You’re on mute!’ Does paediatric CF home spirometry require physiologist supervision?
Palliative Care in Pediatric Pulmonology
Spns2 Transporter Contributes to the Accumulation of S1P in Cystic Fibrosis Human Bronchial Epithelial Cells
Anti-Inflammatory Effects of RTD-1 in a Murine Model of Chronic Pseudomonas aeruginosa Lung Infection: Inhibition of NF-κB, Inflammasome Gene Expression, and Pro-IL-1β Biosynthesis
Increased Inflammatory Markers Detected in Nasal Lavage Correlate with Paranasal Sinus Abnormalities at MRI in Adolescent Patients with Cystic Fibrosis
Increased Prevalence of Celiac Disease in Patients with Cystic Fibrosis: A Systematic Review and Meta-Analysis
Planktonic and Biofilm-Associated Pseudomonas aeruginosa and Staphylococcus epidermidis Elicit Differential Human Peripheral Blood Cell Responses
Current and Emerging Therapies to Combat Cystic Fibrosis Lung Infections

Preclinical Development of a Bacteriophage Cocktail for Treating Multidrug Resistant Pseudomonas aeruginosa Infections
State of the Art on Approved Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators and Triple-Combination Therapy
Staphylococcus aureus and Cystic Fibrosis-A Close Relationship. What Can We Learn from Sequencing Studies?
The Impact of Highly Effective CFTR Modulators on Growth and Nutrition Status
Being Underweight Increases the Risk of Non-Cystic Fibrosis Bronchiectasis in the Young Population: A Nationwide Population-Based Study
Breastfeeding in Cystic Fibrosis: A Systematic Review on Prevalence and Potential Benefits
The Burkholderia cenocepacia Type VI Secretion System Effector TecA Is a Virulence Factor in Mouse Models of Lung Infection
Model-based iterative reconstruction in CT of paranasal sinuses in cystic fibrosis
The impact of modulator therapy in cystic fibrosis on patterns of hospitalization related to age. Results from an analysis of US inpatient episodes 2006-2016
Criteria and definitions for the radiological and clinical diagnosis of bronchiectasis in adults for use in clinical trials: international consensus recommendations
Draft Genome Sequence of the Environmental Fungus Scedosporium dehoogii
Colocolic intussusception mimicking distal intestinal obstruction syndrome in a young adult with cystic fibrosis
Characterising the allergic profile of children with cystic fibrosis
Optimizing Sexual and Reproductive Health Across the Lifespan in People with Cystic Fibrosis
Digital healthcare in cystic fibrosis. Learning from the pandemic to innovate future care
Using a learning health system to understand the mismatch between medicines supply and actual medicines use among adults with cystic fibrosis
Type-4 Phosphodiesterase (PDE4) Blockade Reduces NETosis in Cystic Fibrosis
Mechanisms of Post-Pancreatitis Diabetes Mellitus and Cystic Fibrosis-Related Diabetes: A Review of Preclinical Studies
Is it Worth Adding Systemic Antibiotics to Inhalational Tobramycin Therapy to Treat Pseudomonas Infections in Cystic Fibrosis?
Genomic analysis of Burkholderia cenocepacia isolated from a liver abscess in a patient with cystic fibrosis
An in vitro model for the cultivation of polymicrobial biofilms under continuous-flow conditions
A review of cystic fibrosis: Basic and clinical aspects
Description of wylie-stanley agar for the recovery of mycobacterium abscessus
Self-management intervention to reduce pulmonary exacerbations by supporting treatment adherence in adults with cystic fibrosis: a randomised controlled trial
Exploring the challenges of accessing medication for patients with cystic fibrosis
Projecting the impact of triple CFTR modulator therapy on intravenous antibiotic requirements in cystic fibrosis using patient registry data combined with treatment effects from randomised trials
COVID-19 pandemic restrictions continuously impact on physical activity in adults with cystic fibrosis
Combined agonists act synergistically to increase mucociliary clearance in a cystic fibrosis airway model
Anti-IgE therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
The AWESCORE, a patient-reported outcome measure: development, feasibility, reliability, validity and responsiveness for adults with cystic fibrosis
Outcomes of early repeat sweat testing in infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome (CRMS)/CF screen-positive, inconclusive diagnosis (CFSPID)
Clinical Considerations for Routine Auditory and Vestibular Monitoring in Patients With Cystic Fibrosis
Should the physiotherapy outcomes airway clearance, physical activity and fitness be recorded on the Australian Cystic Fibrosis Data Registry? A consensus approach
A Case Report of Cystic Fibrosis Plus Tuberous Sclerosis: A Cautionary Tale Regarding Lung Transplantation
Lung transplantation disparities based on diagnosis for patients bridging to transplant on extracorporeal membrane oxygenation
Comparison of microbial composition of cough swabs and sputum for pathogen detection in patients with cystic fibrosis
Inactivation of Nitrite-Dependent Nitric Oxide Biosynthesis Is Responsible for Overlapped Antibiotic Resistance between Naturally and Artificially Evolved Pseudomonas aeruginosa
Genetic diversification of persistent Mycobacterium abscessus within cystic fibrosis patients
Dissemination of Mycobacterium abscessus via global transmission networks
Model Systems to Study the Chronic, Polymicrobial Infections in Cystic Fibrosis: Current Approaches and Exploring Future Directions
Two Steps Forward: Improving the Management of Cystic Fibrosis Pulmonary Exacerbations
Chronic Joint Pain in a Young Adult With Cystic Fibrosis
Magnesium implantation or supplementation ameliorates bone disorder in CFTR-mutant mice through an ATF4-dependent Wnt/β-catenin signaling
Outcomes of artery embolisation for cystic fibrosis patients with haemoptysis: a 20-year experience at a major Australian tertiary centre
The cystic fibrosis lung microenvironment alters antibiotic activity: causes and effects
Colistin neurotoxicity mimicking Guillain-Barre syndrome in a patient with cystic fibrosis: case report and review
Inflammatory epithelial cytokines after in vitro respiratory syncytial viral infection are associated with reduced lung function
Elevated Alkaline Phosphatase in a 10-Year-Old With Cystic Fibrosis
Role of inflammation and oxidative stress in tissue damage associated with cystic fibrosis: CAPE as a future therapeutic strategy
Mycobacteriophage-antibiotic therapy promotes enhanced clearance of drug-resistant Mycobacterium abscessus
The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis
Indoor air pollution exposure is associated with greater morbidity in cystic fibrosis
Filamentous fungi in the airway of patients with cystic fibrosis: Just spectators?
Cancer in Cystic Fibrosis: A Narrative Review of Prevalence, Risk Factors, Screening and Treatment Challenges
Bronchoscopic airway clearance therapy for acute exacerbations of bronchiectasis
Full The Relationship between Continuous Glucose Monitoring and OGTT in Youth and Young Adults with Cystic Fibrosis
CFTR modulator use in post lung transplant recipients
Empire-CF study: A phase 2 clinical trial of leukotriene A4 hydrolase inhibitor acebilustat in adult subjects with cystic fibrosis
Development of Tinnitus and Hyperacusis in a Mouse Model of Tobramycin Cochleotoxicity
Cystic Fibrosis – Insulin Deficiency, Early Action
Infants with cystic fibrosis have altered fecal functional capacities with potential clinical and metabolic consequences
Evaluation of antimicrobial susceptibility testing methods for Burkholderia cenocepacia and Burkholderia multivorans isolates from cystic fibrosis patients
CFTR gene mutation spectrum among 735 Iranian patients with cystic fibrosis: A comprehensive systematic review
Coughing and diaphragmatic breathing exercise: What is the repercussion on respiratory mechanics of children and adolescents with cystic fibrosis?
Ascending Cholangitis Caused by Methicillin-Resistant Staphylococcus aureus Species in a Patient With Cystic Fibrosis
Barriers implementing infection prevention and control experienced by healthcare workers, people with CF and parents
Telehealth after the pandemic: Will the inverse care law apply?
Outcomes of SARS-CoV-2 infection in patients with cystic fibrosis: A multicenter retrospective research network study
Social support and social isolation in adults with cystic fibrosis: An integrative review
Correction to “Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis”
Gender differences in non-cystic fibrosis bronchiectasis severity and bacterial load: the potential role of hormones
Functional correction of CFTR mutations in human airway epithelial cells using adenine base editors
Assessment of hepatic involvement by two-dimensional shear wave elastography in paediatric patients with cystic fibrosis
Novel detection of specific bacterial quorum sensing molecules in saliva: Potential non-invasive biomarkers for pulmonary Pseudomonas aeruginosa in cystic fibrosis
Traditional Chinese Medicine is an Alternative Therapeutic Option for Treatment of Pseudomonas aeruginosa Infections
ABCC7/CFTR Expression Is Associated with the Clinical Course of Ulcerative Colitis Patients
Cardiovascular System Involvement in Cystic Fibrosis
An Innovative Protocol for Metaproteomic Analyses of Microbial Pathogens in Cystic Fibrosis Sputum
Antimicrobial activity of ceftazidime/avibactam, ceftolozane/tazobactam and comparator agents against Pseudomonas aeruginosa from cystic fibrosis patients
Mechanistic analysis and significance of sphingomyelinase-mediated decreases in transepithelial CFTR currents in nHBEs
Association of Quality of Life Measures and Otolaryngologic Care in Cystic Fibrosis Patients
Diagnosing constipation in patients with cystic fibrosis applying ESPGHAN criteria
Restoration of exocrine pancreatic function in child with lumacaftor/ivacaftor therapy in cystic fibrosis
Serological biomarkers for the diagnosis of Mycobacterium abscessus infections in cystic fibrosis patients
Hypoplasia of Extrahepatic Biliary Tree and Intrahepatic Cholangiolopathy in Cystic Fibrosis Imperfectly Mimic Biliary Atresia in 4 Infants With Cystic Fibrosis and Kasai Portoenterostomy
Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease?
Quality of life and exercise tolerance tools in children/adolescents with cystic fibrosis: Systematic review
TeleHealth or TeleWealth? Equity challenges for the future of cystic fibrosis care – commentary
Phosphopantetheinyl transferase binding and inhibition by amidino-urea and hydroxypyrimidinethione compounds
CFTR corrector C17 is effective in muscular dystrophy, in vivo proof of concept in LGMDR3
Commentary on: “Evaluating barriers and promotors of telehealth during the COVID-19 pandemic at cystic fibrosis programs to inform new models of CF care”
Kinetic and structural investigations of novel inhibitors of human epithelial 15-lipoxygenase-2
Draft Genome Sequence of the Multidrug-Resistant Strain Pseudomonas aeruginosa PA291, Isolated from Cystic Fibrosis Sputum
Zinc Status and Growth in Infants and Young Children with Cystic Fibrosis
Development of the A-STEP: a new incremental maximal exercise capacity step test in cystic fibrosis
Role of topical antibacterial therapy in patients with cystic fibrosis
Functional endoscopic sinus surgery in a patient with cystic fibrosis after lung transplantation
Early insurance coverage of elexacaftor/tezacaftor/ivacaftor for cystic fibrosis
Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?
Therapeutic pipeline for individuals with cystic fibrosis with mutations nonresponsive to current cystic fibrosis transmembrane conductance regulator modulators

Use of recombinant human deoxyribonuclease in pediatric intensive care unit – a single-center experience
Association of sleep disorders with heart rate variability in children and adolescents with cystic fibrosis
Mind the gap!: Need for improved harmonization of antibiotic susceptibility testing of the nontuberculous mycobacteria between cystic fibrosis clinical guidelines and Mycobacterium antibiotic susceptibility testing laboratories
Rethinking physical exercise training in the modern era of cystic fibrosis: A step towards optimising short-term efficacy and long-term engagement
Cathepsin C inhibitors as anti-inflammatory drug discovery: Challenges and opportunities
Nanoparticle-Based Drug Delivery System: The Magic Bullet for the Treatment of Chronic Pulmonary Diseases
Improving outcomes for Colorado’s IRT-IRT-DNA cystic fibrosis newborn screening algorithm by implementing floating cutoffs
Needle-free iontophoresis-driven β-adrenergic sweat rate test
Clinical presentation and basic defect of the CFTR genotype p.Phe508del / p.Arg117His in a mother and her monozygous twin daughters
Chronic Obstructive Pulmonary Disease Is Associated With Decreased Quality of Life in Bronchiectasis Patients: Findings From the KMBARC Registry
Elexacaftor/tezacaftor/ivacaftor as rescue therapy in a patient with the cystic fibrosis genotype F508DEL/G1244E
Pericardial constriction with calcified cystic mass compressing the right ventricle and right coronary artery
Spectrum Of Pulmonary Exacerbation In Children And Adolescent With Cystic Fibrosis And Physicians’ Practices: Experience From Tertiary Care Center From Lower-Middle Income Country
Pandoraea apista Bacteremia in a COVID-Positive Man: A Rare Coinfection Case Report from North India
The evolving role of radiological imaging in cystic fibrosis
Emerging clinical perspectives in cystic fibrosis liver disease
Clinical Profile of Children With Cystic Fibrosis Surviving Through Adolescence and Beyond
Impact of the vancomycin and amikacin therapeutic monitoring in the optimization of antimicrobial dose in pediatric patients
Correction to: 2-O, 3-O desulfated heparin (ODSH) increases bacterial clearance and attenuates lung injury in cystic fibrosis by restoring HMGB1-compromised macrophage function
Assessment of hospitalization rates, factors associated with hospitalization and in-patient mortality in pediatric patients with cystic fibrosis
Survival in cystic fibrosis after acute respiratory failure supported by extracorporeal membrane oxygenation and/or invasive mechanical ventilation
Administration and monitoring of clofazimine for NTM infections in children with and without cystic fibrosis
Highly efficient photothermal nanoparticles for the rapid eradication of bacterial biofilms
Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection
A comparison of clinic and home spirometry as longtudinal outcomes in cystic fibrosis
The chloride channel CFTR is not required for cyst growth in an ADPKD mouse model
Recent Advances in the Early Treatment of Cystic Fibrosis: Bridging the Gap to Highly Effective Modulator Therapy
Cation complexation by mucoid Pseudomonas aeruginosa extracellular polysaccharide
Efficacy of Problem-Solving Intervention to Improve Adherence in Adolescents and Adults with Cystic Fibrosis
A Phase 3 Study of VX-121 Combination Therapy in Participants With Cystic Fibrosis (CF) Heterozygous for F508del and a Minimal Function Mutation (F/MF)
Relationship between peripheral muscle strength, exercise capacity and body composition in children and adolescents with cystic fibrosis
The impact of cystic fibrosis on the working life of patients: A systematic review
A Randomized Trial of Antimicrobial Duration for Cystic Fibrosis Pulmonary Exacerbation Treatment
Understanding the Intersection between Gender Transition and Health Outcomes in Cystic Fibrosis
Outcomes of infants born during the first 9 years of CF newborn screening in the United States: a retrospective Cystic Fibrosis Foundation Patient Registry cohort study
A future focus on cystic fibrosis in an era of uncertainty despite opportunity
Cystic Fibrosis-related Diabetes is Associated with Worse Lung Function Trajectory Despite Ivacaftor Use
Aspergillus fumigatus In-Host HOG Pathway Mutation for Cystic Fibrosis Lung Microenvironment Persistence
Validation of the French 3-year prognostic score for death or lung transplant in the United States cystic fibrosis population
Genomic Diversity and Antimicrobial Resistance of Haemophilus Colonizing the Airways of Young Children with Cystic Fibrosis
Characterization of clinical Ralstonia strains and their taxonomic position
Gene-specific MicroRNA antagonism protects against HIV Tat and TGF-β-mediated suppression of CFTR mRNA and function
Nutritional status and intake in patients with non-cystic fibrosis bronchiectasis (NCFB) – a cross sectional study
Anti-LPS IgA and IgG can inhibit serum killing of Pseudomonas aeruginosa in patients with cystic fibrosis
Clinical pharmacokinetics and dose recommendations for posaconazole gastroresistant tablets in children with cystic fibrosis
Effectiveness of the Hippotherapy Simulator in Children With Cystic Fibrosis
Steps Ahead: optimising physical activity in adults with cystic fibrosis: Study Protocol for a pilot randomised trial using wearable technology
Acute Liver Failure Triggered by Therapeutic Dose of Acetaminophen in a Patient with Cystic Fibrosis
Longitudinal changes and regional variation of incident infection rates at cystic fibrosis centers, United States 2010-2016
Pregnancy in cystic fibrosis: Review of the literature and expert recommendations
Adenylate Cyclase 1 Links Calcium Signaling to CFTR-Dependent Cytosolic Chloride Elevations in Chick Amacrine Cells
Lentiviral vectors transduce lung stem cells without disrupting plasticity
Protecting clinical trials in cystic fibrosis during the SARS-CoV-2 pandemic: risks and mitigation measures
Impact of guideline-recommended dietitian assessments on weight gain in infants with cystic fibrosis
Lumacaftor-ivacaftor effects on cystic fibrosis-related liver involvement in adolescents with homozygous F508 del-CFTR
Pseudomonas aeruginosa Consumption of Airway Metabolites Promotes Lung Infection
Adaptive Interactions of Achromobacter spp. with Pseudomonas aeruginosa in Cystic Fibrosis Chronic Lung Co-Infection
Cystic Fibrosis Clinical Isolates of Aspergillus fumigatus Induce Similar Muco-inflammatory Responses in Primary Airway Epithelial Cells
Advantages and Disadvantages of Using Magnetic Nanoparticles for the Treatment of Complicated Ocular Disorders
Cystic Fibrosis Related Gastrointestinal Manifestations – Moving Forward
Impulse oscillometry indices to detect an abnormal lung clearance index in childhood cystic fibrosis
Pharmacology Focus: Cystic Fibrosis in the Era of Highly Effective Modulatory Therapy
Global phylogenomic analyses of Mycobacterium abscessus provide context for non cystic fibrosis infections and the evolution of antibiotic resistance
Two Years of Newborn Screening for Cystic Fibrosis in North Macedonia: First Experience
The Intestinal Microbiome and Cystic Fibrosis Transmembrane Conductance Regulator Modulators: Emerging Themes in the Management of Gastrointestinal Manifestations of Cystic Fibrosis
Therapeutic Approach of Chronic Pseudomonas Infection in Cystic Fibrosis-A Network Meta-Analysis
Anti-Virulence Activity of the Cell-Free Supernatant of the Antarctic Bacterium Psychrobacter sp. TAE2020 against Pseudomonas aeruginosa Clinical Isolates from Cystic Fibrosis Patients
Antimicrobial Synergy Testing: Comparing the Tobramycin and Ceftazidime Gradient Diffusion Methodology Used in Assessing Synergy in Cystic Fibrosis-Derived Multidrug-Resistant Pseudomonas aeruginosa
Neutrophil Elastase and Chronic Lung Disease
Structural and Functional Insights into the Biofilm-Associated BceF Tyrosine Kinase Domain from Burkholderia cepacia
Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor Therapy in Three Subjects with the Cystic Fibrosis Genotype Phe508del/Unknown and Advanced Lung Disease
Anti-Inflammatory and Anti-Oxidant Effect of Dimethyl Fumarate in Cystic Fibrosis Bronchial Epithelial Cells
Etiological Work-Up for Adults with Bronchiectasis: A Predictive Diagnostic Score for Primary Ciliary Dyskinesia and Cystic Fibrosis
New Insights in Microbial Species Predicting Lung Function Decline in CF: Lessons from the MucoFong Project
Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators
Management of Individual Patient Expectations When Starting with Highly Effective CFTR Modulators
Decontamination Strategies Used for AFB Culture Significantly Reduce the Viability of Mycobacterium abscessus Complex in Sputum Samples from Patients with Cystic Fibrosis
Phenotypic Clustering in Non-Cystic Fibrosis Bronchiectasis Patients: The Role of Eosinophils in Disease Severity
Specific and Global RNA Regulators in Pseudomonas aeruginosa
Cellular Therapy for the Treatment of Paediatric Respiratory Disease
Zebrafish Model as a Screen to Prevent Cyst Inflation in Autosomal Dominant Polycystic Kidney Disease
Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?
Comparison of Intravenous and Non-Intravenous Antibiotic Regimens in Eradication of P.aeruginosa and MRSA in Cystic Fibrosis
Impact of CFTR Therapy on Chronic Rhinosinusitis and Health Status: Deep Learning CT Analysis and Patient Reported Outcomes
Updates on the Management of Cystic Fibrosis: Development of Modulators and Advancement of Antibiotic Therapies
Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes
Effect of Respiratory Tract Disease on Particle Deposition
Home spirometry utilisation in telemedicine clinic for cystic fibrosis care during COVID-19 pandemic: a quality improvement process
Assessing Shared Decision-Making in Cystic Fibrosis Care Using collaboRATE: A Cross-Sectional Study of 159 Programs
Telehealth and virtual health monitoring in cystic fibrosis
Nontuberculous mycobacteria in cystic fibrosis
Twelve years of progressive Mycobacterium abscessus lung disease in CF-Response to Trikafta
Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis
Structural Lung Disease following Allergic Bronchopulmonary Aspergillosis Complicating Paediatric Cystic Fibrosis
β-eudesmol but not atractylodin exerts an inhibitory effect on CFTR-mediated chloride transport in human intestinal epithelial cells
Design and Development of a Model to Study the Effect of Supplemental Oxygen on the Cystic Fibrosis Airway Microbiome
Recombinant growth hormone therapy for cystic fibrosis in children and young adults
Cystic fibrosis: A changing landscape
Viable but non-cultivable state: a strategy for Staphylococcus aureus survivable in dual-species biofilms with Pseudomonas aeruginosa?
Impact of COVID-19 on social determinants of health for adults with cystic fibrosis

TLR5 signalling is hyper-responsive in porcine cystic fibrosis airways epithelium
Elexacaftor/tezacaftor/ivacaftor for cystic fibrosis
Nontuberculous Mycobacterial Infection Predisposing to Chronic Cavitary Pulmonary Aspergillosis
Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis
Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world – opportunities and challenges
Very early glucose tolerance abnormalities in children with cystic fibrosis
Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in PKP3 in lung epithelial cells
Associations Between Glucose Tolerance, Insulin Secretion, Muscle and Fat Mass in Cystic Fibrosis
Assessing the impact of exposome on the course of chronic obstructive pulmonary disease and cystc fibrosis: The REMEDIA European Project Approach
Preclinical Development of a HIV-Based Gene Therapeutic for Cystic Fibrosis Pulmonary Disease
Feasibility and implementation of a personalized, web-based exercise intervention for people with cystic fibrosis for 1 year
Findings from a feasibility study of estradiol for hypogonadal women with cystic fibrosis-related bone disease
Combined Heart-Lung-Liver Transplantation for Patients With Cystic Fibrosis: The Australian Experience
Theratyping cystic fibrosis in vitro in ALI-culture and organoid models generated from patient-derived nasal epithelial Conditionally Reprogrammed Stem Cells
Apples to apples? Comparative analyses of national CF registries
An outer-pore gate modulates the pharmacology of the TMEM16A channel
Meconium Ileus
Polycystic Kidney Disease Of Childhood
Functional stability of CFTR depends on tight binding of ATP at its degenerate ATP-binding site
Insights Into Patient Variability During Ivacaftor-Lumacaftor Therapy in Cystic Fibrosis
Use of Home-Based Connected Devices in Patients With Cystic Fibrosis for the Early Detection and Treatment of Pulmonary Exacerbations: Protocol for a Qualitative Study
Clinical outcomes of cystic fibrosis patients with hemoptysis treated with bronchial artery embolization
Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance
CFTR Modulator Therapy and Its Impact on Lung Transplantation in Cystic Fibrosis
Cystic Fibrosis Respiratory Microbiology Monitoring during a Global Pandemic: Lessons Learned from a Shift to Telehealth
Review of CFTR modulators 2020
Family-building and parenting considerations for people with cystic fibrosis
SFPQ rescues F508del-CFTR expression and function in cystic fibrosis bronchial epithelial cells
Adenoviral Transduction of Dickkopf-1 Alleviates Silica-induced Silicosis Development in Lungs of Mice
Nebulized Bacteriophage Therapy in Cystic Fibrosis Patients With Chronic Pseudomonas Aeruginosa Pulmonary Infection
Identifying specific needs in adult cystic fibrosis patients: a pilot study using a custom questionnaire
Case report: Three adult brothers with cystic fibrosis (delF508-delF508) maintain unusually preserved clinical profile in the absence of standard CF care
Here’s the rub: A case of constrictive pericarditis in an adult with cystic fibrosis
Trajectories of oral glucose tolerance testing in cystic fibrosis
Defining the role of pharmacists in medication-related genetic counseling
The Effect of a Pre-meal Snack and/or Exercise on Post-prandial Glycemic Excursions in Adults With Cystic Fibrosis
Safety and effectiveness of a risk-stratified venous thromboembolism prophylaxis algorithm in young people with cystic fibrosis
Impact of artificial sputum media formulation on Pseudomonas aeruginosa secondary metabolite production
Ion concentrations in nasal airway surface liquid: a prediction model for the identification of cystic fibrosis carriers
Comparing encounter-based and annualized chronic pseudomonas infection definitions in cystic fibrosis
Parent-Child Dyadic Coping and Quality of Life in Chronically Diseased Children
The Potential Causes of Cystic Fibrosis-Related Diabetes
Correction to: Cystic fibrosis-related diabetes onset can be predicted using biomarkers measured at birth
Evaluation of Children and Adolescent with Cystic Fibrosis by Pancreatic Elastography
A new perspective on opportunistic pathogens of the genus Bordetella in cystic fibrosis
Assessment of Lentiviral Vector Mediated CFTR Correction in Mice Using an Improved Rapid in vivo Nasal Potential Difference Measurement Protocol
Non-invasive Diagnostic Tests in Cystic Fibrosis-Related Liver Disease: A Diagnostic Test Accuracy Network Meta-Analysis
Management of chronic Pseudomonas aeruginosa infection with inhaled levofloxacin in people with cystic fibrosis
Outcome and prognostic factors after lung transplantation for bronchiectasis other than cystic fibrosis
Use of elexacaftor/tezacaftor/ivacaftor in liver transplant patients with cystic fibrosis
Improving lung function in adolescents with CF by tracking annual rate of lung function decline
Coil embolisation for massive haemoptysis in cystic fibrosis
Enhanced pyrazolopyrimidinones cytotoxicity against glioblastoma cells activated by ROS-Generating cold atmospheric plasma
Efficacy and safety of inhaled ENaC inhibitor BI 1265162 in patients with cystic fibrosis: BALANCE-CF 1 – a randomised, Phase II study
Familial cluster of Inquilinus limosus infection among three brothers with cystic fibrosis
Imaging in non-cystic fibrosis bronchiectasis and current limitations
Sphingomyelinase decreases transepithelial anion secretion in airway epithelial cells in part by inhibiting CFTR-mediated apical conductance
The impact of liver disease on mortality in cystic fibrosis-A systematic review
Elexacaftor/Tezacaftor/Ivacaftor Improved Clinical Outcomes in an N1303K-CFTR Patient Based on in vitro Experimental Evidence
Pharmacokinetic modelling to predict risk of ototoxicity with intravenous tobramycin treatment in cystic fibrosis
Liver transplant in children and adults with cystic fibrosis: Impact of growth failure and nutritional status
Posaconazole-induced hypertension in children with cystic fibrosis
First report of the cystic fibrosis transmembrane conductance regulator mutation c.1521_1523delCTT (p. Phe508del) in two Qatari patients with cystic fibrosis
Evolution towards Virulence in a Burkholderia Two-Component System
O-Specific Antigen-Dependent Surface Hydrophobicity Mediates Aggregate Assembly Type in Pseudomonas aeruginosa
Evaluating CRISPR-based prime editing for cancer modeling and CFTR repair in organoids
Structural insights into the gating mechanism of human SLC26A9 mediated by its C-terminal sequence
IL-6-elafin genetically modified macrophages as a lung immunotherapeutic strategy against Pseudomonas aeruginosa infections
Activity of imipenem-relebactam against multi-drug and extensively-drug resistant Burkholderia cepacia complex and Burkholderia gladioli
Miltefosine Against Scedosporium and Lomentospora Species: Antifungal Activity and Its Effects on Fungal Cells
Accuracy of the ActivPAL and Fitbit Charge 2 in measuring step count in Cystic Fibrosis
Manuka Honey Sinus Rinse Study
Impact of bone-active drugs and underlying disease on bone health after lung transplantation: A longitudinal study
Pilot RCT of a telehealth intervention to reduce symptoms of depression and anxiety in adults with cystic fibrosis
Continuous glucose monitoring in cystic fibrosis-Benefits, limitations, and opportunities
The Rough Inhalable Ciprofloxacin Hydrochloride Microparticles Based on Silk Fibroin for Non-Cystic Fibrosis Bronchiectasis Therapy with Good Biocompatibility
The Association between IgG and Disease Severity Parameters in CF Patients
Cucumis sativus extract elicits chloride secretion by stimulation of the intestinal TMEM16A ion channel
Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice
Polymicrobial Interactions in the Cystic Fibrosis Airway Microbiome Impact the Antimicrobial Susceptibility of Pseudomonas aeruginosa
Elexacaftor-Tezacaftor-Ivacaftor Therapy for Cystic Fibrosis Patients with The F508del/Unknown Genotype
Role of RND Efflux Pumps in Drug Resistance of Cystic Fibrosis Pathogens
The Glycosylphosphatidylinositol-Anchored Superoxide Dismutase of Scedosporium apiospermum Protects the Conidia from Oxidative Stress
A Precision Medicine Approach to Optimize Modulator Therapy for Rare CFTR Folding Mutants
DISEASE CHARACTERISATION OF PEOPLE WITH CYSTIC FIBROSIS AND A MINIMAL FUNCTION MUTATION: DATA FROM THE ITALIAN REGISTRY
Challenges Faced by Women with Cystic Fibrosis
Interleukin-13: A pivotal target against influenza-induced exacerbation of chronic lung diseases
Correction of sensor crosstalk error in Exhalyzer D multiple-breath washout device significantly impacts outcomes in children with cystic fibrosis
Cystic fibrosis in South Africa: spectrum of disease and determinants of outcome
Functional respiratory imaging in relation to classical outcome measures in cystic fibrosis: a cross-sectional study
Response to elexacaftor/tezacaftor/ivacaftor in intestinal organoids derived from people with cystic fibrosis
Incidence and risk factors of cancer in individuals with cystic fibrosis in the UK; a case-control study
The role of Psl in the failure to eradicate Pseudomonas aeruginosa biofilms in children with cystic fibrosis
Rapid detection by MALDI-TOF MS of isolates from cystic fibrosis patients belonging to the epidemic clones Achromobacter xylosoxidans ST137 or Achromobacter ruhlandii DES
The Indispensable role of pharmacy services and medication therapy management in cystic fibrosis
Impact of non-cystic fibrosis bronchiectasis on critically ill patients in Korea: a retrospective observational study
Islet Function in the Pathogenesis of Cystic Fibrosis-Related Diabetes Mellitus
High-Frequency Percussive Ventilation in Cystic Fibrosis Patients With Acute Respiratory Failure: A Case Series
The Cost-effectiveness of Genotyping versus Sequencing for Prenatal Cystic Fibrosis Carrier Screening
Early Antibiotics in Cystic Fibrosis: Lessons from the CF Pig Model

A topological switch in CFTR modulates channel activity and sensitivity to unfolding
Heterogeneity of non-cystic-fibrosis bronchiectasis in multiethnic Singapore: A prospective cohort study at a tertiary pulmonology centre
Timing of pancreatic enzyme replacement therapy (PERT) in cystic fibrosis
Structure-function characterization of Streptococcus intermedius surface antigen Pas
The Gut-Lung Axis in Cystic Fibrosis
Engineering mechanobiology through organoids-on-chip:A strategy to boost therapeutics
Defect distribution index: A novel metric for functional lung MRI in cystic fibrosis
Management of non-cystic fibrosis bronchiectasis
Health-Related and Motor Performance-Related Fitness and Physical Activity Among Youth With Cystic Fibrosis
CFTR gene variations and phenotypes in seven children
Sense of Stress and Posttraumatic Growth in Mothers of Children with Cystic Fibrosis-The Moderating Role of Resilience
Increased Virulence of Outer Membrane Porin Mutants of Mycobacterium abscessus
The Role of Neutrophil Extracellular Traps in Central Nervous System Diseases and Prospects for Clinical Application
Health Disparities among adults cared for at an urban cystic fibrosis program
Clinician variability in the diagnosis and treatment of aspergillus fumigatus-related conditions in cystic fibrosis: An international survey
A novel ITGB2 variant with long survival in patients with leukocyte adhesion defect type-I
Urinary tract infections in cystic fibrosis patients
Pancytopenia in a child with cystic fibrosis and severe copper deficiency: Insight from bone marrow evaluation
Postnatal Ozone Exposure Disrupts Alveolar Development, Exaggerates Mucoinflammatory Responses, and Suppresses Bacterial Clearance in Developing Scnn1b -Tg+ Mice Lungs
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis
Therapeutic potential for CFTR Correctors in Autosomal Recessive Polycystic Kidney Disease
Molecular analysis of Pseudomonas aeruginosa strains isolated from cystic fibrosis patients
Pharmacological Modulation of Ion Channels for the Treatment of Cystic Fibrosis
A doggy tale: Risk of zoonotic infection with Bordetella bronchiseptica for cystic fibrosis (CF) patients from live licenced bacterial veterinary vaccines for cats and dogs
Year in Review 2020: Multisystemic impact of cystic fibrosis
Genomic characterization of sporadic isolates of the dominant clone of Mycobacterium abscessus subspecies massiliense
Species-Specific Interferon-Gamma Release Assay for the Diagnosis of Mycobacterium abscessus Complex Infection
IL-8 correlates with reduced baseline femoral neck bone mineral density in adults with cystic fibrosis: a single center retrospective study
Immediate Effect Of Inhalation Therapy Combined With Oscillatory Positive Expiratory Pressure On The Respiratory System Of Children With Cystic Fibrosis
Long-term coexistence of Pseudomonas aeruginosa and Staphylococcus aureus using an in vitro cystic fibrosis model
Emergence of Thymidine-Dependent Staphylococcus aureus Small-Colony Variants in Cystic Fibrosis Patients in Southern Brazil
Emerging Fungal Threats in Cystic Fibrosis
Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients
Pulmonary fibrosis distal airway epithelia are dynamically and structurally dysfunctional
Peroxiredoxin AhpC1 protects Pseudomonas aeruginosa against the inflammatory oxidative burst and confers virulence
Year in review 2020: Nutrition and gastrointestinal disease in cystic fibrosis
Continuous Glucose Monitoring as a Valuable Tool in the Early Detection of Diabetes Related to Cystic Fibrosis
Global Burden of Anxiety and Depression among Cystic Fibrosis Patient: Systematic Review and Meta-Analysis
Difference in Performance of EPI Pigs Fed Either Lipase-Predigested or Creon®-Supplemented Semielemental Diet
High-Throughput Virtual Screening for a New Class of Antagonist Targeting LasR of Pseudomonas aeruginosa
Serum bile acids in cystic fibrosis patients-glycodeoxycholic acid as a potential marker of liver disease
Utilization of electronic patient-reported outcome measures in cystic fibrosis research: Application to the GALAXY study
Novel mutation c.1210-3C > G in cis with a poly-T tract of 5T affects CFTR mRNA splicing in a Chinese patient with cystic fibrosis
CFTR Lifecycle Map-A Systems Medicine Model of CFTR Maturation to Predict Possible Active Compound Combinations
Anti-Inflammatory Influences of Cystic Fibrosis Transmembrane Conductance Regulator Drugs on Lung Inflammation in Cystic Fibrosis
Lung Auscultation Using the Smartphone-Feasibility Study in Real-World Clinical Practice
Potential of helper-dependent Adenoviral vectors in CRISPR-cas9-mediated lung gene therapy
Longitudinal assessment of lung clearance index to monitor disease progression in children and adults with cystic fibrosis
It’s not all about inhaled treatment: challenges with oral therapy in paediatric respiratory medicine
The treatment burden of cystic fibrosis: a day-to-day experience with treatment as someone with cystic fibrosis
Case Report: Analysis of Inflammatory Cytokines IL-6, CCL2/MCP1, CCL5/RANTES, CXCL9/MIG, and CXCL10/IP10 in a Cystic Fibrosis Patient Cohort During the First Wave of the COVID-19 Pandemic
Chronic rhinosinusitis in cystic fibrosis: a review of therapeutic options
Short midline catheters: High success rates for antibiotic therapy in children with cystic fibrosis
Genomic characterization of Achromobacter species isolates from chronic and occasional lung infection in cystic fibrosis patients
Cytokine storm syndrome in a young patient with cystic fibrosis
Reduced neutrophil elastase inhibitor elafin and elevated transforming growth factor-beta(1) are linked to inflammatory response in sputum of cystic fibrosis patients with Pseudomonas aeruginosa
Improving Outcome Measures For Adult CF ACT Trials
Disease burden in people with cystic fibrosis heterozygous for F508del and a minimal function mutation
Association of lung clearance index with survival in individuals with cystic fibrosis
Aquagenic wrinkling of the palms and cystic fibrosis diagnosis
Early intrahepatic duct defects in a cystic fibrosis porcine model
Cystic Fibrosis Blood Neutrophils
Screening for autosomal recessive and X-linked conditions during pregnancy and preconception: a practice resource of the American College of Medical Genetics and Genomics (ACMG)
“Fertility and Pregnancy in Cystic fibrosis”
Anti-biofilm activity of murepavadin against cystic fibrosis Pseudomonas aeruginosa isolates
Magnetic Resonance Imaging Detects Progression of Lung Disease and Impact of Newborn Screening in Preschool Children with Cystic Fibrosis
Acceptability of patient reported outcome measures (PROMs) in a cystic fibrosis data registry
A Study to Evaluate ELX/TEZ/IVA on Cough and Physical Activity in Subjects With Cystic Fibrosis (CF)
Population pharmacokinetic modeling and dosing simulations of tobramycin in pediatric patients with cystic fibrosis
Endurance cycle ergometry tests performed at a sub-maximal work rate elicit peak physiological and symptom responses in adults with cystic fibrosis
A New Posaconazole Dosing Regimen for Paediatric Patients With Cystic Fibrosis and Aspergillus Infection
A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: A pilot study
Beneath the surface: assessing cystic fibrosis pulmonary infections in the era of highly effective modulator therapy
Whole-Exome Sequencing Identified CFTR Variants in Two Consanguineous Families in China
Peripheral blood mononuclear cell response to YKL-40 and Galectin-3 in cystic fibrosis
A shifting landscape: Practice patterns and outcomes of cystic fibrosis and non-cystic fibrosis pediatric lung transplantation
Geographical Barriers to Timely Diagnosis of Cystic Fibrosis and Anxiety Level of Parents During Newborn Screening in Turkey
2-O, 3-O desulfated heparin (ODSH) increases bacterial clearance and attenuates lung injury in cystic fibrosis by restoring HMGB1-compromised macrophage function
Aquagenic (pseudo) keratoderma (aquagenic palmoplantar keratoderma, aquagenic wrinkling of palms)
A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion
Emergence of GES-19-producing Pseudomonas aeruginosa exoU+ belonging to the global high-risk clone ST235 in cystic fibrosis infection
A Review of Trikafta: Triple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulator Therapy
Impact of Anaerobic Antibacterial Spectrum on Cystic Fibrosis Airway Microbiome Diversity and Pulmonary Function
Care of people with cystic fibrosis: What is the role of specialists in Endocrinology and Nutrition?
Variation in treatment preferences of pulmonary exacerbations among Australian and New Zealand cystic fibrosis physicians
The rescue of F508del-CFTR by elexacaftor/tezacaftor/ivacaftor (Trikafta) in human airway epithelial cells is underestimated due to the presence of ivacaftor
Artificial intelligence in CT for quantifying lung changes in the era of CFTR modulators
Whole-Genome Sequences of Mycobacterium abscessus subsp. massiliense Isolates from Brazil
Change in CF care during COVID-19 pandemic: Single-center experience in a middle-income setting
Secondary spontaneous pneumothorax: a time to re-evaluate management
Erratum to: A retrospective ‘real-world’ cohort study of azole therapeutic drug monitoring and evolution of antifungal resistance in cystic fibrosis
Neutrophil Elastase-Regulated Macrophage Sheddome/ Secretome and Phagocytic Failure
Smoking accelerates renal cystic disease and worsens cardiac phenotype in Pkd1-deficient mice
Aspergillus-Associated Endophenotypes in Bronchiectasis
Pseudomonas aeruginosa in Bronchiectasis
Genome Sequences of Two Pseudomonas aeruginosa Isolates with Defects in Type III Secretion System Gene Expression from a Chronic Ankle Wound Infection
Multidimensional Clinical Surveillance of Pseudomonas aeruginosa Reveals Complex Relationships between Isolate Source, Morphology, and Antimicrobial Resistance
Liver X Receptor beta regulates bile volume and the expression of Aquaporins and Cystic Fibrosis Transmembrane Conductance Regulator in the gallbladder
Correction to: Real-World Long-Term Ivacaftor for Cystic Fibrosis in France: Clinical Effectiveness and Healthcare Resource Utilization
Low Prevalence of Severe Underlying Pathology in Children With Recurrent Respiratory Tract Infections

Vitamin D status and pulmonary exacerbations in children and adolescents with cystic fibrosis: Experience from a tertiary care center
Asthma-like symptom or “cystic fibrosis asthma”?
Satisfaction and effectiveness of opioid pain management among adults with cystic fibrosis: A mixed methods study
In vitro and ex vivo models in inhalation biopharmaceutical research – advances, challenges and future perspectives
Infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis and acute recurrent pancreatitis: what definition?
Rheological effects of hypertonic saline and sodium bicarbonate solutions on cystic fibrosis sputum in vitro
Preclinical atherosclerosis in cystic fibrosis: Two distinct presentations are related to pancreatic status
Pharmacokinetics of Polymyxin B in Hospitalized Adults with Cystic Fibrosis
Pediatric population with cystic fibrosis in the centre of Portugal: Candidates for new therapies
Cystás fibrosisban szenvedő nők várandóssága
Quantitative mapping of mRNA 3′ ends in Pseudomonas aeruginosa reveals a pervasive role for premature 3′ end formation in response to azithromycin
Cystic fibrosis with focal biliary cirrhosis and portal hypertension in Japan: a case report
Epithelial Barrier Dysfunction in Chronic Respiratory Diseases
NtrBC Selectively Regulates Host-Pathogen Interactions, Virulence, and Ciprofloxacin Susceptibility of Pseudomonas aeruginosa
Reversible tremor in an infant with vitamin E deficiency and cystic fibrosis
Immunoglobulin GM and KM allotypes are associated with antibody responses to Pseudomonas aeruginosa antigens in chronically infected cystic fibrosis patients
Improved correction of F508del-CFTR biogenesis with a folding facilitator and an inhibitor of protein ubiquitination
Human molecular genetics and the long road to treating cystic fibrosis
NUTRITIONAL STATUS BETWEEN 5 – 10 YEARS IS ASSOCIATED WITH CYSTIC FIBROSIS-RELATED DIABETES IN ADOLESCENCE
Sensitive markers to detect progression of lung disease in children with cystic fibrosis
Comparison of the predictive value of area under the curve versus maximum serum concentration of intravenous tobramycin in cystic fibrosis patients treated for an acute pulmonary exacerbation
Peptide inhibition of neutrophil-mediated injury after in vivo challenge with supernatant of Pseudomonas aeruginosa and immune-complexes
Aerobic fitness is associated with extracellular DNA levels in the sputum of patients with cystic fibrosis
Mild Cystic Fibrosis Lung Disease Is Associated with Bacterial Community Stability
Achromobacter spp. genetic adaptation in cystic fibrosis
Perceptions of Social Media Use to Augment Health Care: A Survey of Adolescents and Young Adults with Cystic Fibrosis
A Clinician Survey of Use Assessment, Documentation, and Education about Cannabis in Persons with Cystic Fibrosis
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation
New Perspectives in Underlying Molecular Defects Based Cystic Fibrosis Therapeutics
The Bactericidal Tandem Drug, AB569: How to Eradicate Antibiotic-Resistant Biofilm Pseudomonas aeruginosa in Multiple Disease Settings Including Cystic Fibrosis, Burns/Wounds and Urinary Tract Infections
Ethnic Differences in the Frequency of CFTR Gene Mutations in Populations of the European and North Caucasian Part of the Russian Federation
Sputum and Plasma Neutrophil Elastase in Stable Adult Patients With Cystic Fibrosis in Relation to Chronic Pseudomonas Aeruginosa Colonization
A retrospective ‘real-world’ cohort study of azole therapeutic drug monitoring and evolution of antifungal resistance in cystic fibrosis
Antibiotic Susceptibility and Biofilm Formation of Bacterial Isolates Derived from Pediatric Patients with Cystic Fibrosis from Tehran, Iran
MRSA Strains with Distinct Accessory Genes Predominate at Different Ages in Cystic Fibrosis
Nontuberculous mycobacterial infection and environmental molybdenum in persons with cystic fibrosis: a case-control study in Colorado
Identification of infiltrating immune cell subsets and heterogeneous macrophages in the lesion microenvironment of hepatic cystic echinococcosis patients with different cyst viability
Resolution of severe hepatosteatosis in a cystic fibrosis patient with multifactorial choline deficiency: A case report
Optimization of antibiotics for cystic fibrosis pulmonary exacerbations due to highly resistant nonlactose fermenting Gram negative bacilli: Meropenem-vaborbactam and cefiderocol
Telemedicine and cystic fibrosis: Do we still need face-to-face clinics?
A new era for people with cystic fibrosis
Role of Protein Kinase A-Mediated Phosphorylation in CFTR Channel Activity Regulation
DETERMINATION OF AMOXICILLIN AND COTRIMOXAZOLE CONCENTRATIONS IN SPUTUM OF PATIENTS WITH CYSTIC FIBROSIS
Cystic Fibrosis Sputum Impairs the Ability of Neutrophils to Kill Staphylococcus aureus
Bicarbonate Evokes Reciprocal Changes in Intracellular Cyclic di-GMP and Cyclic AMP Levels in Pseudomonas aeruginosa
New Therapies to Correct the Cystic Fibrosis Basic Defect
Cystic Fibrosis Human Organs-on-a-Chip
Targeted Drug Delivery Technologies Potentiate the Overall Therapeutic Efficacy of an Indole Derivative in a Mouse Cystic Fibrosis Setting
Systems Biology and Bile Acid Signalling in Microbiome-Host Interactions in the Cystic Fibrosis Lung
Severe COVID-19 in Hospitalized Carriers of Single CFTR Pathogenic Variants
The Fitter the Better? Cardiopulmonary Exercise Testing Can Predict Pulmonary Exacerbations in Cystic Fibrosis
Metaproteomics to Decipher CF Host-Microbiota Interactions: Overview, Challenges and Future Perspectives
Prevotella melaninogenica, a Sentinel Species of Antibiotic Resistance in Cystic Fibrosis Respiratory Niche?
The Effects of the COVID-19 Pandemic on Pediatric Cystic Fibrosis Patients
Serratus Anterior Plane Block as a Primary Anesthetic Technique for Video-Assisted Thoracic Surgery in a Child
Automatic referrals within a cystic fibrosis multidisciplinary clinic improve patient evaluation and management
Effect of malnutrition in infants with cystic fibrosis in India: An underestimated danger
Assessment of a novel genetic counselling intervention to inform assisted reproductive technology treatments and other family-building options in adults with cystic fibrosis
Elucidating clinical phenotypic variability associated with the polyT tract and TG repeats in CFTR
Real World Clinical Outcomes With Novel Modulator Therapy Combinations in People With CF (RECOVER)
Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung
Isolation of and risk factors for airway infection with Pseudomonas aeruginosa in patients with non-cystic fibrosis bronchiectasis
Impact of a Pharmacy Technician and Pharmacist on Time to Inhaled Tobramycin Therapy in a Pediatric Cystic Fibrosis Clinic
Development of an Advance Care Planning Protocol in a Cystic Fibrosis Outpatient Clinic
A narrative review of in utero gene therapy: advances, challenges, and future considerations
CFTR-mediated anion secretion in parathyroid hormone-treated Caco-2 cells is associated with PKA and PI3K phosphorylation but not intracellular pH changes or Na+ /K+ -ATPase abundance
Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment
The bacterial toxin ExoU requires a host trafficking chaperone for transportation and to induce necrosis
Measuring Knowledge of Fertility Preservation in Women With Cystic Fibrosis: Instrument Development and Psychometric Analysis
Real-world adherence among adults with cystic fibrosis is low – a retrospective analysis of the CFHealthHub digital learning health system
Designing multi-epitope vaccine candidates against functional amyloids in Pseudomonas aeruginosa through immunoinformatic and structural bioinformatics approach
Perceptions of Diabetes Technology Use in Cystic Fibrosis Related Diabetes Management
Genomic and Phenotypic Evolution of Achromobacter xylosoxidans during Chronic Airway Infections of Patients with Cystic Fibrosis
The association between body composition, leptin levels and glucose dysregulation in youth with cystic fibrosis
Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis
Cystic fibrosis: From gene discovery to precision medicine
CFTR and cystic fibrosis, a 50-year long history
Pycnogenol-Centellicum supplementation improves lung fibrosis and Post-Covid-19 lung healing
An Organ System-Based Synopsis of Pseudomonas aeruginosa Virulence
Functional Contribution and Targeted Migration of Group-2 Innate Lymphoid Cells in Inflammatory Lung Diseases: Being at the Right Place at the Right Time
Decreased Wait Time and Increased Satisfaction With Bedside Pancreatic Enzyme Dosing for the Inpatient Adolescent With Cystic Fibrosis: A Quality Improvement Project Comparing Enzyme Self-Administration to Nurse Administration
Lingering Identity as Chronically Ill and the Unanticipated Effects of Life-Changing Precision Medicine in Cystic Fibrosis: A Case Report
Impact of COVID-19 Lockdown on Pulmonary and Nutritional Status in Children and Young Adults With Cystic Fibrosis, in Greece
Partnership Enhancement Program: Piloting a Communication Training Program for Cystic Fibrosis Care Teams
Association of vitamin A status with lung function in children and adolescents with cystic fibrosis
Rewriting CFTR to cure cystic fibrosis
Breast development in a 7 year old girl with CF treated with ivacaftor: An indication for personalized dosing?
Association between insurance variability and early lung function in children with cystic fibrosis
Otitis media prone children with cystic fibrosis: A new normal
Formal vs. informal transition in adolescents with cystic fibrosis: A retrospective comparison of outcomes
Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration
Early Lung Disease Exhibits Bacterial-Dependent and -Independent Abnormalities in Cystic Fibrosis Pigs
Primary ciliogenesis is a crucial step for multiciliated cell determinism in the respiratory epithelium
Systemic pseudohypoaldosteronism-1 with episodic dyslipidemia in a Sudanese child
Inflammatory cytokines TNFalpha and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators
Notch-Hes1 signaling activation in Caroli disease and polycystic liver disease
Factors Influencing Lung Function in Patients with Cystic Fibrosis in Western Romania
Airway Clearance Therapy in Cystic Fibrosis Patients Insights from a Clinician Providing Cystic Fibrosis Care
Alteration of Membrane Cholesterol Content Plays a Key Role in Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Channel Activity
Ketone Supplementation in Cystic Fibrosis

Combined Liver-Lung-Kidney Transplant in a Patient with Cystic Fibrosis
Short-term effect of elexacaftor-tezacaftor-ivacaftor on lung function and transplant planning in cystic fibrosis patients with advanced lung disease
Association of Diverse Staphylococcus aureus Populations with Pseudomonas aeruginosa Coinfection and Inflammation in Cystic Fibrosis Airway Infection
Both Pseudomonas aeruginosa and Candida albicans Accumulate Greater Biomass in Dual-Species Biofilms under Flow
CFTR Modulators to the Rescue of Individuals with Cystic Fibrosis and Advanced Lung Disease
Genome-wide association study of signature genetic alterations among pseudomonas aeruginosa cystic fibrosis isolates
Single versus combination intravenous anti-pseudomonal antibiotic therapy for people with cystic fibrosis
Lung Inflammation and Simulated Airway Resistance in Infants with Cystic Fibrosis
Pharmacokinetics and pharmacodynamics of antibiotics in cystic fibrosis: a narrative review
Untargeted Metabolomics Reveals Species-Specific Metabolite Production and Shared Nutrient Consumption by Pseudomonas aeruginosa and Staphylococcus aureus
Filamentous Bacteriophages and the Competitive Interaction between Pseudomonas aeruginosa Strains under Antibiotic Treatment: a Modeling Study
Coping with Cystic Fibrosis: An analysis from the sibling’s point of view
Paranasal mucoceles in children with cystic fibrosis: Management of a not so rare clinical condition
Multidisciplinary Management of Non-cystic-fibrosis Bronchiectasis – State of the Art
TWEAK Signaling Pathway Blockade Slows Cyst Growth and Disease Progression in Autosomal Dominant Polycystic Kidney Disease
Burkholderia cepacia infection in a non-cystic fibrosis patient: an arcane presentation
Review of Gastrointestinal Motility in Cystic Fibrosis
Pulmonary Complications in Cystic Fibrosis: past, present and future
PepBiotics, novel cathelicidin-inspired antimicrobials to fight pulmonary bacterial infections
Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection
Cystic fibrosis transmembrane conductance regulator-related male infertility: Relevance of genetic testing & counselling in Indian population
Critical roles of SMYD2 lysine methyltransferase in mediating renal fibroblast activation and kidney fibrosis
Hyperinflation is Associated with Increased Respiratory Rate and is a More Sensitive Measure of Cystic Fibrosis Lung Disease During Infancy Compared to Forced Expiratory Measures
Pancreas in Hereditary Syndromes: Cross-sectional Imaging Spectrum
Novel CFTR Modulators for Treating Cystic Fibrosis
Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator
Physiotherapy service provision in a specialist adult cystic fibrosis service: A pre-post design study with the inclusion of an allied health assistant
Cardiovascular complications in cystic fibrosis: A review of the literature
Non-invasive ventilation is associated with long-term improvements in lung function and gas exchange in cystic fibrosis adults with hypercapnic respiratory failure
Neonatal cystic fibrosis screening: Analysis and differences in immunoreactive trypsin levels in newborns with a positive screen
The impact of the high emergency lung transplantation program in cystic fibrosis in France: insight from a comparison with Canada
Serum hepatocyte growth factor as a predictor of disease severity and future exacerbations in patients with non-cystic fibrosis bronchiectasis
Potential role for the use of gliptins in cystic fibrosis-related diabetes
Aggressive vasculitis after lung transplantation for cystic fibrosis
Healthcare Resource Utilization and Costs Among Children With Cystic Fibrosis in the United States
COVID-19: Impact, experiences and support needs of children and young adults with Cystic Fibrosis and parents
System-Level Variation in Multiple Sclerosis Care Outcomes: Initial Findings from the Multiple Sclerosis Continuous Quality Improvement Research Collaborative
Cystic fibrosis-related diabetes (CFRD) and cognitive function in adults with cystic fibrosis
Pyoverdine Inhibitors and Gallium Nitrate Synergistically Affect Pseudomonas aeruginosa
Combined indeterminate and impaired glucose tolerance is a novel group at high risk of Cystic Fibrosis-related Diabetes
Combined indeterminate and impaired glucose tolerance is a novel group at high risk of Cystic Fibrosis-related Diabetes
Safety, Tolerability and Efficacy of S-1226 in Cystic Fibrosis and Non CF Bronchiectasis
USE OF COMPUTED TOMOGRAPHY (CT) TO DETERMINE THE SENSITIVITY OF CLINICAL SIGNS AS A DIAGNOSTIC TOOL FOR RESPIRATORY DISEASE IN BORNEAN ORANGUTANS (PONGO PYGMAEUS )
Ivacaftor-elexacaftor-tezacaftor and tacrolimus combination in cystic fibrosis
Clinical findings of patients with cystic fibrosis according to newborn screening results
Italian Cystic Fibrosis Registry (ICFR). Report 2017-2018
Colonic Transit Time and Fecal Impaction in Children and Adolescents with Cystic Fibrosis-Associated Constipation
Primary care providers’ role in newborn screening result notification for cystic fibrosis
Newborn screening for cystic fibrosis: Role of primary care providers in caring for infants with positive screening results
Diffuse cystic lung disease in sickle cell anaemia: a series of 22 cases and a case-control study
A First in Human Study to Evaluate the Safety, Tolerability and Pharmacokinetics of Single and Multiple Ascending Doses of Inhaled ETD001 in Healthy Subjects
Aquagenic Keratoma: Treatment Update
A Report of Candida blankii Fungemia and Possible Endocarditis in an Immunocompetent Individual and the Review of Literature
Predictive factors for premature birth and respiratory exacerbation in pregnancies of women with cystic fibrosis
Disruption of biofilms and killing of Burkholderia cenocepacia from cystic fibrosis lung using an antioxidant-antibiotic combination therapy
Microbial interaction: Prevotella spp. reduce P. aeruginosa induced inflammation in cystic fibrosis bronchial epithelial cells
Pragmatic Implementation Trial of a CF Primary Palliative Care Intervention
Cystic Fibrosis and Urinary Incontinence
Bright Light Therapy for Depression Symptoms in Adults With Cystic Fibrosis (CF)
Long-term bone mineral density changes and fractures in lung transplant recipients with cystic fibrosis
Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung
Citizen-science based study of the oral microbiome in Cystic fibrosis and matched controls reveals major differences in diversity and abundance of bacterial and fungal species
Cystic fibrosis foundation consensus statements for the care of cystic fibrosis lung transplant recipients
Comparison of Cas9 and Cas12a CRISPR editing methods to correct the W1282X-CFTR mutation
Tezacaftor-ivacaftor use in routine care of adults with cystic fibrosis: a medicine use evaluation
Phenotypic characteristics of incident and chronic MRSA isolates in cystic fibrosis
Airway Administration of Flagellin Regulates the Inflammatory Response to Pseudomonas aeruginosa
Association Between Number of Intravenous Antipseudomonal Antibiotics and Clinical Outcomes of Pediatric Cystic Fibrosis Pulmonary Exacerbations
Real-World Long-Term Ivacaftor for Cystic Fibrosis in France: Clinical Effectiveness and Healthcare Resource Utilization
Effectiveness of enteral nutrition by percutaneous endoscopic gastrostomy in malnourished patients with cystic fibrosis: Does the gender gap play a role?
Inhaled mannitol (Bronchitol) for cystic fibrosis
Airway Microbiology in Children with Cystic Fibrosis: A Prospective Cohort Study from Northern India
Clinical impact of levofloxacin inhalation solution in cystic fibrosis patients in a real-world setting
Treatment-resistant hypertension in a post-transplant patient with cystic fibrosis: a rare case of phaeochromocytoma
Biofilm inhibitory effect of alginate lyases on mucoid P. aeruginosa from a cystic fibrosis patient
An Animated Functional Data Analysis Interface to Cluster Rapid Lung Function Decline and Enhance Center-Level Care in Cystic Fibrosis
A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?
Cystic fibrosis
Sex differences in treatment patterns in cystic fibrosis pulmonary exacerbations
HOMA indices as screening tests for cystic fibrosis-related diabetes
Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease
Survival Benefits Following Liver Transplantation – A Matched-Pair Analysis in Pediatric Patients with Cystic Fibrosis
Pulmonary Biofilm-Based Chronic Infections and Inhaled Treatment Strategies
Respiratory symptoms do not reflect functional impairment in early CF lung disease
Tensin 1 (TNS1) is a modifier gene for low body mass index (BMI) in homozygous [F508del]CFTR patients
Correction to: Cystic Fibrosis Revisited – A Review Study
Association of the CFTR gene with asthma and airway mucus hypersecretion
Single-Dose Lentiviral Mediated Gene Therapy Recovers CFTR Function in Cystic Fibrosis Knockout Rats
A chromobacter xylosoxidans airway infection is associated with lung disease severity in children with cystic fibrosis
Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study
Beyond the Guidelines: Treatment of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis
Diabetes outbreak during COVID19 lock-down in a prediabetic patient with cystic fibrosis long treated with glargine
Ivacaftor and Airway Inflammation in Preschool Children with Cystic Fibrosis
Does Cefiderocol Have a Potential Role in Cystic Fibrosis Pulmonary Exacerbation Management?
Best Practices: Imaging Strategies for Reduced-Dose Chest CT in the Management of Cystic Fibrosis-Related Lung Disease
Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era
Airway Epithelial Nucleotide Release Contributes to Mucociliary Clearance
Proteases, Mucus, and Mucosal Immunity in Chronic Lung Disease
Personalized Medicine Based on Nasal Epithelial Cells: Comparative Studies with Rectal Biopsies and Intestinal Organoids
Potential of Intestinal Current Measurement for Personalized Treatment of Patients with Cystic Fibrosis

Computed Tomographic Changes in Patients with Cystic Fibrosis Treated by Combination Therapy with Lumacaftor and Ivacaftor
The Equitable Implementation of Cystic Fibrosis Personalized Medicines in Canada
Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment
Increased Incidence and Associated Risk Factors of Aspergillosis in Patients with Bronchiectasis
Fungal Infection and Inflammation in Cystic Fibrosis
Plasmonic Sensing Studies of a Gas-Phase Cystic Fibrosis Marker in Moisture Laden Air
Comparison of Physical Activity and Sedentary Behaviour between Schoolchildren with Cystic Fibrosis and Healthy Controls: A Gender Analysis
Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis
Health Characteristics of Patients with Cystic Fibrosis whose Genotype Includes a Variant of the Nucleotide Sequence c.3140-16T>A and Functional Analysis of this Variant
Cystic Fibrosis, New Frontier: Exploring the Functional Connectivity of the Brain Default Mode Network. Comment on Elce et al. Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis. Diagnostics 2020, 10, 489
Reply to Gambazza et al. Cystic Fibrosis, New Frontier: Exploring the Functional Connectivity of the Brain Default Mode Network. Comment on “Elce et al. Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis. Diagnostics 2020, 10, 489”
Change in Nutrient and Dietary Intake in European Children with Cystic Fibrosis after a 6-Month Intervention with a Self-Management mHealth Tool
CFTR Modulators: Does One Dose Fit All?
Pulmonary Mycobacterium abscessus Infection in an 11-Year-Old Child, Successfully Treated with Inhaled/Parenteral Amikacin: A Case Report and Review of Literature
Papilledema and hypervitaminosis A after elexacaftor/tezacaftor/ivacaftor for cystic fibrosis
Enhancing Cystic Fibrosis Immune Regulation
Enhanced Expression of Human Epididymis Protein 4 (HE4) Reflecting Pro-Inflammatory Status Is Regulated by CFTR in Cystic Fibrosis Bronchial Epithelial Cells
Rating of Perceived Exertion in Three-Minute Step Test in Children with Cystic Fibrosis
A rare case of idiopathic intrauterine intestinal volvulus complicated with intestinal perforation: a case report from Syria
Renal Ciliopathies: Sorting Out Therapeutic Approaches for Nephronophthisis
Biomarkers in Cystic Fibrosis Lung Disease – A Review
Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study
The immune response to airway mycosis
Automated antimicrobial susceptibility testing of slow-growing Pseudomonas aeruginosa strains in the presence of tetrazolium salt WST-1
Epigenome editing of the CFTR-locus for treatment of cystic fibrosis
Enabling Antibiotic Allergy Evaluations and Reintroduction of First-Line Antibiotics for Cystic Fibrosis Patients
Compensatory evolution of Pseudomonas aeruginosa’s slow growth phenotype suggests mechanisms of adaptation in cystic fibrosis
Ivacaftor Inhibits Glioblastoma Stem Cell Maintenance and Tumor Progression
Would it have happened without Christmas? Thoracic emphysema and allergic bronchopulmonary aspergillosis in a juvenile cystic fibrosis patient with the G551D mutation receiving ivacaftor
Testing of aerosolized ciprofloxacin nanocarriers on cystic fibrosis airway cells infected with P. aeruginosa biofilms
Allergic Broncho-Pulmonary Aspergillosis
Erratum: Explaining the Sex Effect on Survival in Cystic Fibrosis: A Joint Modeling Study of UK Registry Data
Orthopedic Manifestations of Cystic Fibrosis
Actigraphic and patient and family reported sleep outcomes in children and youth with cystic fibrosis: A systematic review
Use of the NINJA (Nephrotoxic Injury Negated by Just-in-Time Action) Program to Identify Nephrotoxicity in Pediatric Patients with Cystic Fibrosis
Association of Cystic Fibrosis Transmembrane Conductance Regulator with Epithelial Sodium Channel Subunits Carrying Liddle’s Syndrome Mutations
Tackling the Misconception That Cystic Fibrosis Is a “White People’s Disease”
VIP reduction in the pancreas of F508del homozygous CF mice and early signs of Cystic Fibrosis Related Diabetes (CFRD)
Risk factors for cystic fibrosis arthropathy: Data from the German cystic fibrosis registry
The Current Practice of Noninvasive Ventilation in Patients With Cystic Fibrosis
Correlation of computed tomography findings and lung function in children and adolescents with cystic fibrosis
DRESS syndrome in a patient with cystic fibrosis: a pediatric case report
Gain-of-Function Polymorphisms in Human Inflammasomes: Implications for Cystic Fibrosis
Cystic fibrosis year in review 2020: Section 2 pulmonary disease, infections, and inflammation
Phosphorus stress induces the synthesis of novel glycolipids in Pseudomonas aeruginosa that confer protection against a last-resort antibiotic
Monovalent: Divalent Anion Selectivity in the CFTR Channel Pore
Chronic bacterial pulmonary infections in advanced cystic fibrosis differently affect the level of sputum neutrophil elastase, IL-8 and IL-6
Pancreas transplantation for Cystic Fibrosis: A Frequently Missed Opportunity
The long-term safety of chronic azithromycin use in adult patients with cystic fibrosis, evaluating biomarkers for renal function, hepatic function and electrical properties of the heart
Pseudomonas aeruginosa infection, but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis
Pharmacokinetics of oral antimycobacterials and dosing guidance for Mycobacterium avium complex treatment in cystic fibrosis
Cerebral paradoxical embolisation in a patient with cystic fibrosis with patent foramen ovale: a comparative review of literature
Survival models to support shared decision-making about advance care planning for people with advanced stage cystic fibrosis
The role of suboptimal concentrations of nebulized tobramycin in driving antimicrobial resistance in Pseudomonas aeruginosa isolates in cystic fibrosis – an in vitro study
The Impact of Resistant Bacterial Pathogens including Pseudomonas aeruginosa and Burkholderia on Lung Transplant Outcomes
Ivacaftor Reduces Inflammatory Mediators in Upper Airway Lining Fluid From Cystic Fibrosis Patients With a G551D Mutation: Serial Non-Invasive Home-Based Collection of Upper Airway Lining Fluid
Novel reaction to new cystic fibrosis medication Trikafta
Honeycomb-like structures in sarcoidosis pathologically showing granulomas in walls of clustered bronchioles
Bone Cells Differentiation: How CFTR Mutations May Rule the Game of Stem Cells Commitment?
The Human Microbiome, an Emerging Key-Player in the Sex Gap in Respiratory Diseases
Cystic fibrosis in the kidney: new lessons from impaired renal HCO3- excretion
Perinatal outcomes in women with cystic fibrosis: data from the Italian Cystic Fibrosis Registry
Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease
Two positively charged amino acid side-chains in the inner vestibule of the CFTR channel pore play analogous roles in controlling anion binding and anion conductance
Laryngeal Injury Due to Amikacin Inhalation for Refractory Mycobacterium avium Complex Infection
Randomized, Controlled Trial Of Sitagliptin And Islet Function In Cystic Fibrosis With Abnormal Glucose Tolerance
Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors
Importance of beta-lactam pharmacokinetics and pharmacodynamics on the recovery of microbial diversity in the airway of persons with cystic fibrosis
Diabetes and Prediabetes in Children With Cystic Fibrosis: A Systematic Review of the Literature and Recommendations of the Italian Society for Pediatric Endocrinology and Diabetes (ISPED)
Sweat Testing and Recent Advances
The Correlation of Health-Related Quality of Life with Cystic Fibrosis Severity Markers in Chest CT Scan and 6-Minute Walk Test: A Cross-Sectional Study
Systematic review: cystic fibrosis in the SARS-CoV-2/COVID-19 pandemic
Targeted exhaled breath analysis for detection of Pseudomonas aeruginosa in cystic fibrosis patients
Lack of CFTR alters the ferret pancreatic ductal epithelial secretome and cellular proteome: Implications for exocrine/endocrine signaling
Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020
Aquagenic wrinkling in children under two years of age: Could this be a potential clinical referral tool for cystic fibrosis among non-screened populations?
Management of an unusual cause of life-threatening haemoptysis: a multidisciplinary approach
Complete Genome Sequence of Burkholderia gladioli Myophage Mana
Gastrointestinal Factors Associated With Hospitalization in Infants With Cystic Fibrosis: Results from the BONUS Study
Topic of the Month: Transient Elastography in Pediatric Liver Disease
National Trends of Hospitalizations in Cystic Fibrosis Highlight a Need for Pediatric to Adult Transition Clinics
Cystic fibrosis: A changing landscape
Cystic fibrosis: A changing landscape
Evaluating the Impact of Education on Pharmacist Tobramycin Dose Recommendations for Cystic Fibrosis and a Review of Perceptions on Pharmacist-Led Charting
VEGF receptor 2 (KDR) protects airways from mucus metaplasia through a Sox9-dependent pathway
CFTR Function and Clinical Response to Modulators Parallel Nasal Epithelial Organoid Swelling
Value Assessment in Cystic Fibrosis: ICER’s Rejection of the Axioms of Fundamental Measurement
Aquagenic Wrinkling of the Palm: A Rare Diagnostic Clue of Cystic Fibrosis and the Response to CFTR-Modulating Therapy
Atypical haemolytic uraemic syndrome in a child with cystic fibrosis
Pharmacokinetic interactions between ivacaftor and cytochrome P450 3A4 inhibitors in people with cystic fibrosis and healthy controls
Sleep disturbances in children with cystic fibrosis, primary ciliary dyskinesia and typically developing children during COVID-19 pandemic
The associations between pediatric weight status and cystic fibrosis-related diabetes status and health-related quality of life among children and young adults with cystic fibrosis: A systematic review
Cystic fibrosis being a polyendocrine disease (Review)
Neonatal screening in Europe revisited: An ISNS-perspective on the current state and developments since 2010
Newborn screening for cystic fibrosis in France
Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Advanced Cystic Fibrosis Lung Disease
Safety and Feasibility of High-intensity Interval Training Program in CF Patients
Aquagenic wrinkling of the palms is a rare dermatological disease associated with cystic fibrosis
Vibrio parahaemolyticus induces inflammation-associated fluid accumulation via activation of the cystic fibrosis transmembrane conductance regulator
Defining the role of CFTR channel blocker and ClC-2 activator in DNBS induced gastrointestinal inflammation

Fungal infection in cystic fibrosis
Towards harmonization of solutions used for cystic fibrosis diagnosis by nasal potential difference measurements: a formulation approach with CHESS software
An integrated liquid crystal sensing device assisted by the surfactant-embedded smart hydrogel
Sleep assessment in cystic fibrosis patients using a minimal-impact biomotion system
Elevated sweat chloride test: is it always cystic fibrosis?
On the Corner of Models and Cure: Gene Editing in Cystic Fibrosis
Nocturnal non invasive ventilation in normocapnic cystic fibrosis patients: a pilot study
The lived experience of Jordanian parents of children with cystic fibrosis: Qualitative study
Aminoglycoside dosing and monitoring for Pseudomonas aeruginosa during acute pulmonary exacerbations in cystic fibrosis
SARS-CoV-2 infection in cystic fibrosis: A multicentre prospective study with a control group, Italy, February-July 2020
The Evaluation of Determinants and Impacts of Co-Production in Healthcare: A Research Protocol for OPAT in Cystic Fibrosis
Bactericidal/Permeability-Increasing Protein Preeminently Mediates Clearance of Pseudomonas aeruginosa In Vivo via CD18-Dependent Phagocytosis
Clinical Pharmacokinetic and Pharmacodynamic Considerations in the Drug Treatment of Non-Tuberculous Mycobacteria in Cystic Fibrosis
A short-term evaluation of a prototype disposable Oscillating Positive Expiratory Pressure (OPEP) device in a cohort of children with cystic fibrosis
The measurement properties of tests and tools used in cystic fibrosis studies: a systematic review
Lung function in children with cystic fibrosis in the USA and UK: a comparative longitudinal analysis of national registry data
Catamenial haemoptysis in females with cystic fibrosis: a case series with review of management strategies
Tobramycin Liquid Crystal Nanoparticles Eradicate Cystic Fibrosis-Related Pseudomonas aeruginosa Biofilms
New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via Sodium/Hydrogen Exchanger inhibition
Bacille Calmette-Guerin (BCG) Vaccine for Immune Protection Against Infections
SLEEP IN CHILDREN WITH CYSTIC FIBROSIS: MORE UNDER THE COVERS
LPS decreases CFTR open probability and mucociliary transport through generation of reactive oxygen species
General Health, Attitudes, and Activity in Self-directed Exercise in Patients With Cystic Fibrosis (GAINS-CF)
Cystic fibrosis improves COVID-19 survival and provides clues for treatment of SARS-CoV-2
Gene Therapy: A Possible Alternative to CFTR Modulators?
Azithromycin Exhibits Activity Against Pseudomonas aeruginosa in Chronic Rat Lung Infection Model
Adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: analysis of a national specialty pharmacy database
Long-term safety of lumacaftor-ivacaftor in children aged 2-5 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a multicentre, phase 3, open-label, extension study
Combination CFTR modulator therapy in children and adults with cystic fibrosis
Személyre szabott, pozitív nyomású légzésterápia cystás fibrosisban
In Vitro Antimicrobial Susceptibility Testing of Biofilm-Growing Bacteria: Current and Emerging Methods
The molecular mechanism of CFTR- and secretin-dependent renal bicarbonate excretion
Rapid cystic fibrosis lung-function decline and in-vitro CFTR modulation
Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study
Chronic Pseudomonas aeruginosa Lung Infection Is IL-1R Independent, but Relies on MyD88 Signaling
SPLUNC1: a novel marker of cystic fibrosis exacerbations
Transcriptional analysis of cystic fibrosis airways at single-cell resolution reveals altered epithelial cell states and composition
Clinical Determinants of the Six-Minute Walk Test (6MWT) in Stable Non-Cystic Fibrosis Bronchiectasis Patients
Association Between Non-Cystic Fibrosis Bronchiectasis and Quality of Life: A Single-Center Cross-Sectional Study
Molecular genetics of renal ciliopathies
Cystic fibrosis mutation classes in pediatric otitis media – Fickle or faulty?
Liver-first strategy for a combined lung and liver transplant in patients with cystic fibrosis
CFTR regulates embryonic T lymphopoiesis via Wnt signaling in zebrafish
Many patients with cystic fibrosis have a better quality of life now
Cystic fibrosis and coeliac disease: a case report of an unusual association
The clinical effects of combining postural exercises with chest physiotherapy in cystic fibrosis: A single-blind, randomized-controlled trial
Transcriptional firing represses bactericidal activity in cystic fibrosis airway neutrophils
Evaluation of Two Chest Tomosynthesis Cystic Fibrosis Scoring Systems Using High-Resolution Computed Tomography Brody Scoring as Reference
Patient personalized translational tools in Cystic fibrosis to transform data from bench to bed-side and back
Cystic fibrosis in Brazil: achievements in survival
Effect of Alpha-1 Antitrypsin on CFTR Levels in Primary Human Airway Epithelial Cells Grown at the Air-Liquid-Interface
Prenatal Ultrasound Suspicion of Cystic Fibrosis in a Multiethnic Population: Is Extensive CFTR Genotyping Needed?
The effects of conditioning and lentiviral vector pseudotype on short- and long-term airway reporter gene expression in mice
Inhaled antibiotics for treatment of adults with non-cystic fibrosis bronchiectasis: A systematic review and meta-analysis
Heterogenous Susceptibility to R-Pyocins in Populations of Pseudomonas aeruginosa Sourced from Cystic Fibrosis Lungs
Development and Preclinical Evaluation of New Inhaled Lipoglycopeptides for the Treatment of Persistent Pulmonary MRSA Infections
Systematic depression and anxiety screening for patients and caregivers: implementation and process improvement in a cystic fibrosis clinic
Bayesian forecasting for intravenous tobramycin dosing in adults with cystic fibrosis using one versus two serum concentrations in a dosing interval
The cAMP-phosphodiesterase 4 (PDE4) controls β-adrenoceptor- and CFTR-dependent saliva secretion in mice
Decreasing the Cut-off Score Value of MALDI-ToF MS Increase the Identities of Burkholderia cepacia Complex Species
COVID-19 vaccine prioritisation for people with cystic fibrosis
Cystic Fibrosis Lung Disease in the Aging Population
Case Report: White Colored Stool: An Early Sign of Cystic Fibrosis in Infants
Prevalence and serotyping of S. pneumoniae in a large vaccine-naive cohort of adults with cystic fibrosis
Management of chronic pulmonary disease in the time of coronavirus disease 2019
The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations
Lung transplantation for non-cystic fibrosis bronchiectasis in Turkey: Initial institutional experience
Diagnostic tools and CFTR functional assays in cystic fibrosis: utility and availability in Switzerland
Integrative chemogenomic analysis identifies small molecules that partially rescue deltaF508-CFTR for cystic fibrosis
Characterizing medication management and the role of pharmacists in caring for people living with cystic fibrosis: A work system approach
Virtual medication tours with a pharmacist as part of a cystic fibrosis telehealth visit
Diversity of cystic fibrosis chronic rhinosinusitis microbiota correlates with different pathogen dominance
A histone-like nucleoid structuring protein regulates several virulence traits in Burkholderia multivorans
Peptide 1018 inhibits swarming and influences Anr-regulated gene expression downstream of the stringent stress response in Pseudomonas aeruginosa
A medication adherence-enhancing simulation intervention in pediatric cystic fibrosis
Burkholderia cepacia complex bacteremia outbreaks among non-cystic fibrosis patients in the pediatric unit of a university hospital
ACINETOBACTER BAUMANNII Infection in a Child with Cystic Fibrosis
Revealing the Metabolic Alterations during Biofilm Development of Burkholderia cenocepacia Based on Genome-Scale Metabolic Modeling
DNA Methylation Patterns Correlate with the Expression of SCNN1A , SCNN1B , and SCNN1G (Epithelial Sodium Channel, ENaC) Genes
Real-World Outcomes of Ivacaftor Treatment in People with Cystic Fibrosis: A Systematic Review
Evaluation of Aerosol Therapy during the Escalation of Care in a Model of Adult Cystic Fibrosis
Achromobacter xylosoxidans and Stenotrophomonas maltophilia: Emerging Pathogens Well-Armed for Life in the Cystic Fibrosis Patients’ Lung
DeltaF508 CFTR Hetero- and Homozygous Paediatric Patients with Cystic Fibrosis Do Not Differ with Regard to Nutritional Status
The Impact of an Efflux Pump Inhibitor on the Activity of Free and Liposomal Antibiotics against Pseudomonas aeruginosa
Identification of Potential Leukocyte Biomarkers Related to Drug Recovery of CFTR: Clinical Applications in Cystic Fibrosis
Biofilm Formation on Dental Implant Biomaterials by Staphylococcus aureus Strains Isolated from Patients with Cystic Fibrosis
Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM
Isorhamnetin Ameliorates Dry Eye Disease via CFTR Activation in Mice
Unknown Renal Impairment: A Rare Case of Inhaled Tobramycin Induced Acute Kidney Injury in a Cystic Fibrosis Patient
Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review
New Sequence Type ST3449 in Multidrug-Resistant Pseudomonas aeruginosa Isolates from a Cystic Fibrosis Patient
Transduction of Pig Small Airway Epithelial Cells and Distal Lung Progenitor Cells by AAV4
Nasal Epithelial Cell-Based Models for Individualized Study in Cystic Fibrosis
Virus Infection of Aspergillus fumigatus Compromises the Fungus in Intermicrobial Competition
Small Hsps as Therapeutic Targets of Cystic Fibrosis Transmembrane Conductance Regulator Protein
Cystic Fibrosis Lung Disease Modifiers and Their Relevance in the New Era of Precision Medicine
Physical Activity Regulates TNFalpha and IL-6 Expression to Counteract Inflammation in Cystic Fibrosis Patients
In-Host Emergence of Linezolid Resistance in a Complex Pattern of Toxic Shock Syndrome Toxin-1-Positive Methicillin-Resistant Staphylococcus aureus Colonization in Siblings with Cystic Fibrosis
Individual and Group Response of Treatment with Ivacaftor on Airway and Gut Microbiota in People with CF and a S1251N Mutation
Pseudomonas aeruginosa Virulence Factors Support Voriconazole Effects on Aspergillus fumigatus

Home physiotherapists assisting follow-up treatment in cystic fibrosis: a multicenter observational study
Allergic Broncho-Pulmonary Aspergillosis (ABPA) in cystic fibrosis: Mechanisms, diagnosis and therapeutic options
Glycated Hemoglobin as a First-line Screening Test for Cystic Fibrosis-Related Diabetes and Impaired Glucose Tolerance in Children With Cystic Fibrosis: A Validation Study
Stepwise pathogenic evolution of Mycobacterium abscessus
Mycobacterium abscessus in cystic fibrosis
Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension
Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis
Huff and puff of exercise for airway clearance in cystic fibrosis: how clear is the evidence?
Calprotectin-mediated zinc chelation inhibits Pseudomonas aeruginosa protease activity in cystic fibrosis sputum
Characteristics and Outcomes of Children With Cystic Fibrosis Hospitalized With Cirrhosis in the United States
Loss of PKD1 and PKD2 share common effects on intracellular Ca2+ signaling
Aquagenic Wrinkling of the Palms after Brief Immersion to Water test as a screening tool for Cystic Fibrosis diagnosis
Mycobacterium abscessus biofilms produce an extracellular matrix and have a distinct mycolic acid profile
Long-Term Impact of Ivacaftor on Healthcare Resource Utilization Among People with Cystic Fibrosis in the United States
Effects of patient room layout on viral accruement on healthcare professionals’ hands
CFTR MODULATOR DRUG DESENSITIZATION: PRESERVING THE HOPE OF LONG TERM IMPROVEMENT
Risk factors for Pseudomonas aeruginosa colonization in non-cystic fibrosis bronchiectasis and clinical implications
Ultrasound Echogenicity of Papillary Thyroid Cancer Is Affected by Tumor Growth Patterns and Tumor Fibrosis
Rapid macrolide and amikacin resistance testing for Mycobacterium abscessus in people with cystic fibrosis
The impact of plasma 25-hydroxyvitamin D on pulmonary function and exercise physiology in cystic fibrosis: a multicentre retrospective study
Could automated analysis of chest X-rays detect early bronchiectasis in children?
Systematic Review of the Prevalence of Kidney Stones in Cystic Fibrosis
Hyperinflammation and airway surface liquid dehydration in cystic fibrosis: purinergic system as therapeutic target
Drug therapies for reducing gastric acidity in people with cystic fibrosis
The anti-sigma factor MucA is required for viability in Pseudomonas aeruginosa
Psychological interventions during COVID pandemic: Telehealth for individuals with cystic fibrosis and caregivers
Sweat sticker for cystic fibrosis
Respiratory pathogens in patients with acute exacerbation of non-cystic fibrosis bronchiectasis from a developing country
The Future of Highly Effective Modulator Therapy in Cystic Fibrosis
Tools for the Real-Time Assessment of a Pseudomonas aeruginosa Infection Model
Phenotypic and Genomic Variability of Serial Peri-Lung Transplantation Pseudomonas aeruginosa Isolates From Cystic Fibrosis Patients
Full characterization of plasmids from Achromobacter ruhlandii isolates recovered from a single patient with cystic fibrosis (CF)
Lumacaftor/ivacaftor changes the lung microbiome and metabolome in cystic fibrosis patients
Cystic fibrosis airway microbiota associated with outcomes of nontuberculous mycobacterial infection
Guiding the rational design of patient-centred drug trials in Cystic Fibrosis: A Delphi study
Integrating Electronic Patient Reported Biometric Measures (ePReBMs) From Wearable Devices in Respiratory Diseases
Lives changed: A new era for people with cystic fibrosis
Crowdsourcing airway annotations in chest computed tomography images
Assessing Practices, Beliefs, and Attitudes about Palliative Care among People with Cystic Fibrosis, Their Caregivers, and Clinicians: Results of a Content Analysis
Lumacaftor and Ivacaftor
Tezacaftor and Ivacaftor
Ivacaftor
Elexacaftor, Tezacaftor and Ivacaftor
Use of hemoglobin A1c to identify dysglycemia in cystic fibrosis
A multi-centre, randomized, controlled trial on coaching and telemonitoring in patients with cystic fibrosis: conneCT CF
SARS-CoV-2 and Burkholderia cenocepacia infection in a patient with Cystic Fibrosis: An unfavourable conjunction?
A survey of the prevalence, management and outcome of infants with an inconclusive diagnosis following newborn bloodspot screening for cystic fibrosis (CRMS/CFSPID) in six Italian centres
Varied clinical significance of ATP-binding cassette C sub-family members for lung adenocarcinoma
Extensive Mycobacterium abscessus Pneumonia in an Immunocompetent Infant with No Underlying Lung Pathology
Facile antibacterial materials with turbine-like structure for P. aeruginosa infected scald wound healing
The effects of nano-curcumin as a nutritional strategy on clinical and inflammatory factors in children with cystic fibrosis: the study protocol for a randomized controlled trial
Improving and standardizing protocols for cystic fibrosis routine microbiology
Study to Evaluate Adverse Events and Change in Disease Activity With Oral Capsules of Galicaftor/Navocaftor/ABBV-119 Combination Therapy in Adult Participants With Cystic Fibrosis
Survival and lung transplant outcomes for individuals with advanced cystic fibrosis lung disease in the United States and Canada: an analysis of national registries
Antimicrobial Activity of Dalbavancin and Comparators against Staphylococcus aureus Causing Pneumonia in Patients with and without Cystic Fibrosis
A systematic cochrane review of corrector therapies (with or without potentiators) for people with cystic fibrosis with class II gene variants (most commonly F508DEL)
Optimising equity of access: how should we allocate slots to the most competitive trials in Cystic Fibrosis (CF)?
Inhibition of Pseudomonas aeruginosa Alginate Synthesis by Ebselen Oxide and Its Analogues
Antibodies Against Pseudomonas aeruginosa Alkaline Protease Directly Enhance Disruption of Neutrophil Extracellular Traps Mediated by This Enzyme
Subinhibitory Concentrations of Biogenic Silver Nanoparticles Affect Motility and Biofilm Formation in Pseudomonas aeruginosa
Early Interleukin-22 and Neutrophil Proteins Are Correlated to Future Lung Damage in Children With Cystic Fibrosis
Case Report: Maintenance of Desensitization to Nebulized Colomycin Over 10 Years
Dose Escalation Study of ALX-009 in Healthy Men and Cystic Fibrosis (CF) and Non-CF Bronchiectasis Patients
Temporal trends in healthcare resource use and associated costs of patients with cystic fibrosis
Maintaining normal lung function in children with cystic fibrosis is possible with aggressive treatment regardless of Pseudomonas aeruginosa infections
Smoothing the transition of adolescents with CF from pediatric to adult care: Pre-transfer needs
Can clinical benefits of modulators effectively ‘modulate’ adherence in people with CF?
Trichomonas vaginalis infection impairs anion secretion in vaginal epithelium
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies
Effects of a Short-Term Resistance-Training Program on Heart Rate Variability in Children With Cystic Fibrosis-A Randomized Controlled Trial
USE OF PROTON PUMP INHIBITORS IS ASSOCIATED WITH LOWER HEMOGLOBIN LEVELS IN PEOPLE WITH CYSTIC FIBROSIS
Clinical characteristics of children with cystic fibrosis infected with unusual bacteria
Caregiver burden and related factors in mothers of children and adolescents with cystic fibrosis
Comparative meta-analysis of cystic fibrosis cell models suggests partial endothelial-to-mesenchymal transition
Rectal organoid morphology analysis (ROMA) as a promising diagnostic tool in cystic fibrosis
Population Genomics of Mycobacterium abscessus from United States Cystic Fibrosis Care Centers
A Novel Cystic Fibrosis Gene Mutation C.4242+1G>C in an Omani Patient: A Case Report
Acute hyperglycaemia in cystic fibrosis pulmonary exacerbations
The c.3140-26A>G Variant of the CFTR Gene in Homozygous State Causes Mild Cystic Fibrosis – Overview of Longitudinal Clinical Data of the Patient Managed in our CF Center and Review of the Literature
Children with Cystic Fibrosis Hospitalised with COVID-19: Multicentre Experience
Cystic fibrosis in disguise – the wolf in sheep’s clothing, a case report
Development and validation of the Cystic Fibrosis Decisional Balance for Physical Activity scale (CF-DB-PA)
Developing a Decision Aid to Facilitate Informed Decision Making About Invasive Mechanical Ventilation and Lung Transplantation Among Adults With Cystic Fibrosis: Usability Testing
miR-125b/NRF2/HO-1 axis is involved in protection against oxidative stress of cystic fibrosis: A pilot study
Genetics of CFTR and male infertility
How do we counsel men with obstructive azoospermia due to CF mutations?-a review of treatment options and outcomes
Longitudinal analysis of sinus microbiota post endoscopic surgery in patients with cystic fibrosis and chronic rhinosinusitis: a pilot study
Inflammasome Genetic Variants, Macrophage Function, and Clinical Outcomes in Cystic Fibrosis
Tobramycin and Colistin display anti-inflammatory properties in CuFi-1 cystic fibrosis cell line
The Natural Alkaloid Berberine Can Reduce the Number of Pseudomonas aeruginosa Tolerant Cells
Remote support by multidisciplinary teams: a crucial means to cope with the psychological impact of SARS-COV-2 pandemic on patients with cystic fibrosis and inflammatory bowel disease in Lombardia
Detection of Bacterial Pathogens Using Home Oropharyngeal Swab Collection in Children with Cystic Fibrosis
CFTR chloride channel activity modulates the mitochondrial morphology in cultured epithelial cells
A multimodal approach to detect and monitor early lung disease in cystic fibrosis
Interferon regulatory factor 8 regulates expression of acid ceramidase and infection susceptibility in cystic fibrosis
Non-contrast pulmonary perfusion MRI in patients with cystic fibrosis
A Qualitative Exploration of Sleep Habits and Intervention Needs Among Youth with Cystic Fibrosis
Pseudomonas aeruginosa, the type of mutation, lung function, and nutritional status in adults with cystic fibrosis
Predictors of sinonasal disease onset, progression, and severity in pediatric cystic fibrosis patients
Impact of newborn screening for cystic fibrosis on clinical outcomes of pediatric patients: 10 years’ experience in Lodz Voivodship

NLR family pyrin domain containing 3 (NLRP3) and caspase 1 (CASP1) modulation by intracellular Cl- concentration
Association of Medication Regimen Complexity With Clinical Endpoints in Pediatric Patients With Cystic Fibrosis
Allergic Reactions to Serine Protease-Like Proteins of Staphylococcus aureus
Can Targeting Iron Help in Combating Chronic Pseudomonas Infection? A Systematic Review
Ophthalmologic manifestations of adult patients with cystic fibrosis
Pregnancy in women with Cystic Fibrosis in the 21(st) century
Peripheral lung effect of elexacaftor/tezacaftor/ivacaftor in adult cystic fibrosis
Vitamin D intoxication in patients with cystic fibrosis: report of a single-center cohort
Fatal Pandoraea nosoerga infection after combined liver-lung transplantation for cystic fibrosis: a recontamination by the pre-transplantation strain
Bronchodilator Responsiveness in Cystic Fibrosis Children Treated for Pulmonary Exacerbations
Use of a Portable Spirometer in Pediatric Patients With Cystic Fibrosis at the Nancy CHRU: Feasibility Study
Percussion Palm Cup: Safety and Usability in Infants and Children With Cystic Fibrosis
Fibrotic expression profile analysis reveals repurposed drugs with potential anti-fibrotic mode of action
Quinomycin A reduces cyst progression in polycystic kidney disease
Audit of sweat chloride testing reveals analytical errors
Pseudo-Bartter Syndrome and Intermediate Sweat Chloride Levels-It Could Still be Cystic Fibrosis!
Ivacaftor partially corrects airway inflammation in a humanized G551D rat
Mini-gut feelings: perspectives of people with cystic fibrosis on the ethics and governance of organoid biobanking
Discovery of CFTR Modulators for the Treatment of Cystic Fibrosis
Co-infection with Pseudomonas aeruginosa impacts virulence of Staphylococcus aureus and intensifies the severity of infection
Burkholderia cenocepacia BCAM2418-induced antibody inhibits bacterial adhesion, confers protection to infection and enables identification of host glycans as adhesin targets
ROLE OF SWEAT ION RATIOS IN DIAGNOSING CYSTIC FIBROSIS
Thoracic movement screening in adults with cystic fibrosis: reliability of the Manchester musculoskeletal screening tool
A complicated association between two different genetic rare disorders: Cystic Fibrosis and Spinal Muscular Atrophy
Trends of antimicrobial resistance and combination susceptibility testing of cystic fibrosis multidrug-resistant Pseudomonas aeruginosa: A ten-year update
Severe obstructive biliopathy mimicking biliary atresia in an infant with cystic fibrosis
Development and evaluation of an internet-based cognitive behavioral therapy intervention for anxiety and depression in adults with cystic fibrosis (eHealth CF-CBT): An international collaboration
Changes in the Cystic Fibrosis Airway Microbiome in Response to CFTR Modulator Therapy
Nasal high-flow therapy as an adjunct to exercise in patients with cystic fibrosis: A pilot feasibility trial
Optical Measurements of Sweat for in Vivo Quantification of CFTR Function in Individual Sweat Glands
A mutational approach to dissect the functional role of the putative CFTR “PTM-CODE”
Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study
Microbiota: A Missing Link in The Pathogenesis of Chronic Lung Inflammatory Diseases
Case Report of a Successful Pregnancy in a Cystic Fibrosis Patient with The c.1521_1523delCTT/c.3718-2477C>t Genotypes
CF Fungal Disease in the Age of CFTR Modulators
Simultaneous pancreas and kidney transplant after bilateral lung transplant for a recipient with Cystic Fibrosis
Pharmacokinetics of Meropenem in People with Cystic Fibrosis-A Proof of Concept Clinical Trial
Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?
Allergic Diseases Caused by Aspergillus Species in Patients with Cystic Fibrosis
Actin-Resistant DNase1L2 as a Potential Therapeutics for CF Lung Disease
IRE1alpha Is a Therapeutic Target for Cystic Fibrosis Airway Inflammation
Functional and Transcriptional Adaptations of Blood Monocytes Recruited to the Cystic Fibrosis Airway Microenvironment In Vitro
Oxidative Stress and Endoplasmic Reticulum Stress in Rare Respiratory Diseases
Pseudomonas aeruginosa : An Audacious Pathogen with an Adaptable Arsenal of Virulence Factors
Antimicrobial Resistance in Common Respiratory Pathogens of Chronic Bronchiectasis Patients: A Literature Review
Copper-Associated Oxidative Stress Contributes to Cellular Inflammatory Responses in Cystic Fibrosis
A Novel Infection Protocol in Zebrafish Embryo to Assess Pseudomonas aeruginosa Virulence and Validate Efficacy of a Quorum Sensing Inhibitor In Vivo
The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models
Lumacaftor and Matrine: Possible Therapeutic Combination to Counteract the Inflammatory Process in Cystic Fibrosis
Airway Surface Liquid pH Regulation in Airway Epithelium Current Understandings and Gaps in Knowledge
Phenotypic Presentations of Cystic Fibrosis in Children of African Descent
Screening Methods for Diagnosing Cystic Fibrosis-Related Diabetes: A Network Meta-Analysis of Diagnostic Accuracy Studies
CFTR Cooperative Cis -Regulatory Elements in Intestinal Cells
Two Siblings Homozygous for F508del-CFTR Have Varied Disease Phenotypes and Protein Biomarkers
Neonatal Screening in Europe Revisited: An ISNS Perspective on the Current State and Developments Since 2010
Bacterial Re-Colonization Occurs Early after Lung Transplantation in Cystic Fibrosis Patients
CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis
DNA Methylation at ATP11A cg11702988 Is a Biomarker of Lung Disease Severity in Cystic Fibrosis: A Longitudinal Study
Cystic Fibrosis-Related Diabetes (CFRD): Overview of Associated Genetic Factors
Cystic Fibrosis: Recent Insights into Inhaled Antibiotic Treatment and Future Perspectives
Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis
Variability in Bacteriophage and Antibiotic Sensitivity in Serial Pseudomonas aeruginosa Isolates from Cystic Fibrosis Airway Cultures over 12 Months
Factors influencing physical activity in adults with cystic fibrosis
Real-world use of ivacaftor in Canada: A retrospective analysis using the Canadian Cystic Fibrosis Registry
Treatment of Cystic Fibrosis: From Gene- to Cell-Based Therapies
Could Azithromycin Be Part of Pseudomonas aeruginosa Acute Pneumonia Treatment?
Sclerosing mucoepidermoid carcinoma of the salivary glands: report of three cases with special concern to the counterpart accompanied by eosinophilia
Insomnia in adults with cystic fibrosis: strong association with anxiety/depression and impaired quality of life
Simultaneous Double Intussusception in a Patient with Cystic Fibrosis
Targeted replacement of full-length CFTR in human airway stem cells by CRISPR/Cas9 for pan-mutation correction in the endogenous locus
Effect of transfer from a paediatric to adult cystic fibrosis centre on clinical status and hospital attendance
Managing Fungal Infections in Cystic Fibrosis Patients: Challenges in Clinical Practice
The impact of COVID-19 shielding on the wellbeing, mental health and treatment adherence of adults with cystic fibrosis
Pulmonary rehabilitation for cystic fibrosis: A narrative review of current literature
Remote Monitoring of High-Risk Patients With Chronic Cardiopulmonary Diseases
MyVoice:CF Decision Aid for Women With Cystic Fibrosis
Racially equitable diagnosis of cystic fibrosis using next-generation DNA sequencing: a case report
Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study
Soft, skin-interfaced sweat stickers for cystic fibrosis diagnosis and management
V-type ATPase Mediates Airway Surface Liquid Acidification in Pig Small Airway Epithelial Cells
Characterization of a novel mCH3 conjugated anti-PcrV scFv molecule
A Rare Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutation Associated With Typical Cystic Fibrosis in an Arab Child
Azithromycin and the microbiota of cystic fibrosis sputum
Cystic fibrosis transmembrane conductance regulator modulators for cystic fibrosis: a new dawn?
A Pseudomonas aeruginosa Antimicrobial Affects the Biogeography but Not Fitness of Staphylococcus aureus during Coculture
Ventilation Heterogeneity in Asthma and COPD: The Value of the Poorly Communicating Fraction as the Ratio of Total Lung Capacity to Alveolar Volume
Minimising the risk of cross infection between siblings with cystic fibrosis (CF) within the home: Successful domestic steam disinfection of CF bacterial and foodborne pathogens on common household cutlery and crockery utensils
Continuous glucose monitoring indices predict poor FEV(1) recovery following cystic fibrosis pulmonary exacerbations
The CFTR P67L variant reveals a key role for N-terminal lasso helices in channel folding, maturation, and pharmacologic rescue
Increased mortality in patients with non cystic fibrosis bronchiectasis with respiratory comorbidities
Immunoglobulin G: A useful outcome marker in the follow-up of cystic fibrosis patients?
Update on Calcium Signaling in Cystic Fibrosis Lung Disease
Murine Model of Sinusitis Infection for Screening Antimicrobial and Immunomodulatory Therapies
Chronic infection by nontypeable Haemophilus influenzae fuels airway inflammation
Quantification of Cystic Fibrosis Lung Disease with Radiomics-based CT Scores
CFTR modulator therapy improves cystic fibrosis-related diabetes. But how?
Impact of airway Exophiala spp. on children with cystic fibrosis
Emergence and impact of oprD mutations in Pseudomonas aeruginosa strains in cystic fibrosis
Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR
Patient and family perceptions of telehealth as part of the cystic fibrosis care model during COVID-19

Clostridioides difficile colonisation and infection in a cohort of Australian adults with cystic fibrosis
I won’t let cystic fibrosis stop me from living my best life
Co-translational folding of the first transmembrane domain of ABC-transporter CFTR is supported by assembly with the first cytosolic domain
High-fiber diets attenuate emphysema development via modulation of gut microbiota and metabolism
An Unusual Presentation of Chest Pain in a Patient With Cystic Fibrosis: A Case Report
Staphylococcus aureus Potentiates the Hemolytic Activity of Burkholderia cepacia Complex (Bcc) Bacteria
An observational study of anaerobic bacteria in cystic fibrosis lung using culture dependant and independent approaches
New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation
Increased CFTR expression and function from an optimized lentiviral vector for cystic fibrosis gene therapy
Fighting diagnostic confirmation bias: Cystic fibrosis, allergic bronchopulmonary aspergillus, or both?
Prediction and Validation of a Druggable Site on Virulence Factor of Drug Resistant Burkholderia cenocepacia
Post COVID-19 large pneumatocele: clinical and pathological perspectives
Does cystic fibrosis make susceptible to celiac disease?
Trainability of Health-Related and Motor Performance Fitness in Adults with Cystic Fibrosis within a 12-Month Partially Supervised Exercise Program
Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation
Improved detection of air trapping on expiratory computed tomography using deep learning
Luminal Gastrointestinal Manifestations of Cystic Fibrosis
Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis
A bacterial stimulation assay for bronchoalveolar lavage immune cells from young children with cystic fibrosis
Tobramycin and vestibulotoxicity: retrospective analysis of four cases
Characterizing mucociliary clearance in young children with cystic fibrosis
Isolated Liver Rejection After Lung and Combined Kidney-Liver Transplantation: A Case Report
A formalized transition program for cystic fibrosis: a ten-year retrospective analysis of 97 patients in Lyon
A rare case of a cystic renal mass with heterotopic ossification and a mini literature review
Untreated dental disease and lung transplant waitlist evaluation time for individuals with cystic fibrosis
Resolvin D1 and D2 reduce SARS-CoV-2-induced inflammatory responses in cystic fibrosis macrophages
How Bacterial Adaptation to Cystic Fibrosis Environment Shapes Interactions Between Pseudomonas aeruginosa and Staphylococcus aureus
Changes in handgrip strength in children with cystic fibrosis compared to children without cystic fibrosis
Cyproheptadine as an appetite stimulant in children with cystic fibrosis
Commentary on early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study
Rates of adverse and serious adverse events in children with cystic fibrosis
Acceptability, Feasibility and Preliminary Efficacy of CFWP to Treat Clinically Elevated Fatigue in Adults With CF
Computed tomography in cystic fibrosis lung disease: a focus on radiation exposure
Anti-quorum sensing and antibiofilm potential of 1,8-cineole derived from Musa paradisiaca against Pseudomonas aeruginosa strain PAO1
Cytokine signaling pathway in cystic fibrosis: expression of SOCS and STATs genes in different clinical phenotypes of the disease
Evaluation of bone metabolism in children with cystic fibrosis
Assisting PNA transport through cystic fibrosis human airway epithelia with biodegradable hybrid lipid-polymer nanoparticles
A Phase 3 Open-Label Study of ELX/TEZ/IVA in Children 6 Through 11 Years of Age With CF and at Least One F508del Allele
Is Impulse Oscillometry System a Useful Method for the Evaluation and Follow-Up of Patients with Cystic Fibrosis?
Impact of 1h oral glucose tolerance test on the clinical status of adult cystic fibrosis patients over a 4-year period
Dornase alfa for cystic fibrosis
Cystic fibrosis and noninvasive liver fibrosis assessment methods in children
Telerehabilitation Using Fitness Application in Patients with Severe Cystic Fibrosis Awaiting Lung Transplant: A Pilot Study
A retrospective evaluation of vitamin K for hemoptysis in adult cystic fibrosis patients
Physiotherapy via telehealth for acute respiratory exacerbations in paediatric cystic fibrosis
One versus Many: Polymicrobial Communities and the Cystic Fibrosis Airway
SPI-1005 for Prevention and Treatment of Tobramycin Induced Ototoxicity
The clinical features that contribute to poor bone health in young Australians living with cystic fibrosis: A recommendation for BMD screening
Managing Cystic Fibrosis related diabetes via telehealth during COVID-19 pandemic
Assessing the Potency of β-Lactamase Inhibitors with Diverse Inactivation Mechanisms against the PenA1 Carbapenemase from Burkholderia multivorans
Computationally designed pyocyanin demethylase acts synergistically with tobramycin to kill recalcitrant Pseudomonas aeruginosa biofilms
Molecular mechanism mediating enteric bacterial translocation after severe burn: the role of cystic fibrosis transmembrane conductance regulator
Stability Prediction for Mutations in the Cytosolic Domains of Cystic Fibrosis Transmembrane Conductance Regulator
Transmission, adaptation and geographical spread of the Pseudomonas aeruginosa Liverpool epidemic strain
Genomics of an endemic cystic fibrosis Burkholderia multivorans strain reveals low within-patient evolution but high between-patient diversity
Toward the Establishment of New Clinical Endpoints for Cystic Fibrosis: The Role of Lung Clearance Index and Cardiopulmonary Exercise Testing
Chest physiotherapy enhances detection of Pseudomonas aeruginosa in nonexpectorating children with cystic fibrosis
Geographic distribution of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Saudi Arabia
Role of Toilet Bronchoscopy in RICU
Care of people with Cystic Fibrosis: what is the role of specialists in Endocrinology and Nutrition?
Efficacy and safety of inhaled dry-powder mannitol in adults with cystic fibrosis: An international, randomized controlled study
Ex vivo model predicted in vivo efficacy of CFTR modulator therapy in a child with rare genotype
An innovative role for luteolin as a natural quorum sensing inhibitor in Pseudomonas aeruginosa
Expression and Role of CFTR in Human Esophageal Squamous Cell Carcinoma
Pseudomonas aeruginosa PA80 is a cystic fibrosis isolate deficient in RhlRI quorum sensing
Burkholderia cenocepacia transcriptome during the early contacts with giant plasma membrane vesicles derived from live bronchial epithelial cells
VJ Segment Usage of TCR-Beta Repertoire in Monozygotic Cystic Fibrosis Twins
Ceftolozane/tazobactam for Pseudomonas aeruginosa pulmonary exacerbations in cystic fibrosis adult patients: a case series
Tele-Coaching Intervention to Improve Treatment Adherence in Cystic Fibrosis
Impact of Bronchial Drainage Technique by the Medical Device Simeox® on Respiratory Function and Symptoms in Adult Patients With Cystic Fibrosis
Induction of Ciliary Orientation by Matrix Patterning and Characterization of Mucociliary Transport
The genetic background significantly impacts the severity of kidney cystic disease in the Pkd1(RC/RC) mouse model of autosomal dominant polycystic kidney disease
How abnormal is the normal? Clinical characteristics of CF patients with normal FEV1
Towards better understanding of the heparin role in NETosis: feasibility of using native mass spectrometry to monitor interactions of neutrophil elastase with heparin oligomers
Immune Responses to Pseudomonas aeruginosa Biofilm Infections
Current therapies for gastro-oesophageal reflux in the setting of chronic lung disease: state of the art review
Association of Intensity of Anti-Pseudomonal Antibiotic Therapy With Risk of Treatment-Emergent Organisms in Cystic Fibrosis Children with Newly Acquired Pseudomonas Aeruginosa
Pneumocystis jirovecii among patients with cystic fibrosis and their household members
Peak glucose during an oral glucose tolerance test is associated with future diabetes risk in adults with cystic fibrosis
LasR-deficient Pseudomonas aeruginosa variants increase airway epithelial mICAM-1 expression and enhance neutrophilic lung inflammation
Pharmacological approaches to cystic fibrosis
A non-olfactory shark adenosine receptor activates CFTR with unique pharmacology and structural features
Strain-Resolved Dynamics of the Lung Microbiome in Patients with Cystic Fibrosis
Inadequate Functioning of Nebulizer System Compressors Used by Individuals With Cystic Fibrosis
Timing of dornase alfa inhalation for cystic fibrosis
Effect of Endoscopic Sinus Surgery on Clinical Outcomes in DeltaF508 Cystic Fibrosis Patients
The effect of oral and intravenous antimicrobials on pulmonary exacerbation recovery in cystic fibrosis
Derivation of iPSC line (RCMGi002-A) from dermal fibroblasts of a cystic fibrosis female patient with homozygous F508del mutation
The correlation between 6-min walk test and respiratory parameters in children with cystic fibrosis
Proteomics insights into the Burkholderia cenocepacia phosphorus stress response
Long-term benefits of nusinersen in a child affected by cystic fibrosis and spinal muscular atrophy type 1
Quantification of Pulmonary Pathology in Cystic Fibrosis-Comparison Between Digital Chest Tomosynthesis and Computed Tomography
Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry Cohort study
Genetic Association With Pseudomonas aeruginosa Acquisition in Cystic Fibrosis: Influence of Surfactant Protein D and Mannose-Binding Lectin
Comparison of typical and atypical computed tomography patterns regarding reversibility and fibrosis in pulmonary sarcoidosis
Azole Resistance in Aspergillus fumigatus: A Five-Year Follow Up Experience in a Tertiary Hospital With a Special Focus on Cystic Fibrosis
The Frequency and Related Factors of Non-Tuberculosis Mycobacteria Infections among Patients with Cystic Fibrosis
Pancreatitis severity in mice with impaired CFTR function but pancreatic sufficiency is mediated via ductal and inflammatory cells-Not acinar cells
Cystic fibrosis prognosis in Europa : chronicle of an announced dilemma
The Impact of Environmental Factors on Monogenic Mendelian Diseases

Assessment of treatment burden and complexity in cystic fibrosis: A quality improvement project
Cystic Fibrosis Newborn Screening in Austria Using PAP and the Numeric Product of PAP and IRT Concentrations as Second-Tier Parameters
In Vitro Newly Isolated Environmental Phage Activity against Biofilms Preformed by Pseudomonas aeruginosa from Patients with Cystic Fibrosis
Dysfunctional Inflammation in Cystic Fibrosis Airways: From Mechanisms to Novel Therapeutic Approaches
Intestinal Dysbiosis in Young Cystic Fibrosis Rabbits
The Diagnosis of Cystic Fibrosis in Adult Age. Data from the Italian Registry
Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis
Oral Nutritional Supplements in Adults with Cystic Fibrosis: Effects on Intake, Levels of Fat-Soluble Vitamins, and Bone Remodeling Biomarkers
Bacterial Interactions with Aspergillus fumigatus in the Immunocompromised Lung
Approaches to Targeting Bacterial Biofilms in Cystic Fibrosis Airways
Impact of Altered Gut Microbiota and Its Metabolites in Cystic Fibrosis
Development of a simple medium for the selective isolation of bacteria belonging to the Streptococcus anginosus group
Chronic incretin-based therapy in cystic fibrosis-related diabetes: A tale of 3 patients treated with sitagliptin for over 5 years
SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR
Impairment in inflammasome signaling by the chronic Pseudomonas aeruginosa isolates from cystic fibrosis patients results in an increase in inflammatory response
Inhaled Sodium Nitrite as an Antimicrobial for Cystic Fibrosis
Cyclic nucleotide phosphodiesterase inhibitors as therapeutic interventions for cystic fibrosis
Wideband Acoustic Reflex Growth in Adults With Cystic Fibrosis
Educational Needs and Preferences for Patient-Centered Outcomes Research in the Cystic Fibrosis Community: Mixed Methods Study
SARS-CoV-2 infection in a pediatric patient with cystic fibrosis
Micro-gallbladder: A rare surgical problem
Mycobacterium abscessus biofilms have viscoelastic properties which may contribute to their recalcitrance in chronic pulmonary infections
CFTR Deficiency Affects Glucose Homeostasis via Regulating GLUT4 Plasma Membrane Transportation
Anxiety in Children with Cystic Fibrosis and Their Parents: A Systematic Review
PAR-2-activated secretion by airway gland serous cells: role for CFTR and inhibition by Pseudomonas aeruginosa
Impact of hypoxia and AMPK on CFTR-mediated bicarbonate secretion in human cholangiocyte organoids
Exercise-induced desaturation in subjects with non-cystic fibrosis bronchiectasis: laboratory-based tests versus field-based exercise tests
Cystic fibrosis-related mortality trends in Brazil for the 1999-2017 period: a multiple-cause-of-death study
Outcomes Of Non-Cystic Fibrosis Related Bronchiectasis Post Lung Transplantation
Reflex zone stimulation reduces ventilation inhomogeneity in cystic fibrosis: A randomised controlled cross-over study
State of the Art in Cystic Fibrosis Pharmacology Optimization of anti-microbials in the treatment of Cystic Fibrosis Pulmonary Exacerbations: III. Executive Summary
Disparities in cystic fibrosis survival in Mexico: Impact of socioeconomic status
The Lung Microbiome in Young Children with Cystic Fibrosis: A Prospective Cohort Study
Evaluation of Fused Pyrrolothiazole Systems as Correctors of Mutant CFTR Protein
Discrete choice experiment (DCE) to quantify the influence of trial features on the decision to participate in cystic fibrosis (CF) clinical trials
Inflammation in children with cystic fibrosis: contribution of bacterial production of long-chain fatty acids
A pilot study of cystic fibrosis exacerbation response phenotypes reveals contrasting serum and sputum iron trends
Effect of CFTR correctors on the traffic and the function of intracellularly retained ABCB4 variants
Clinical use of the modified shuttle test in children with cystic fibrosis: Is one test sufficient?
Limb muscle size and contractile function in adults with cystic fibrosis: A systematic review and meta-analysis
“Il faut continuer a poser des questions” patient reported outcome measures in cystic fibrosis: An anthropological perspective
Clinical Effort Against Smoke Exposure in Cystic Fibrosis
Primary Immun Deficiency in Patients with Non-Cystic Fibrosis Bronchiectasis and Its Relationship with Clinical Parameters
Genetic modification of cystic fibrosis with deltaF508 mutation of CFTR gene using the CRISPR system in peripheral blood mononuclear cells
Multidrug resistance protein structure of Trypanosoma evansi isolated from buffaloes in Ngawi District, Indonesia: A bioinformatics analysis
Identifying children with Cystic Fibrosis in population-scale routinely collected data in Wales: A Retrospective Review
Thriving alongside CF: Developing a Grounded Theory of Empowerment in Children and Young People with Cystic Fibrosis During Key Life Transitions
The impact of physical activity and exercise interventions for physical health in people with cystic fibrosis: protocol for a systematic review
Defining key outcomes to evaluate performance of newborn screening programmes for cystic fibrosis
Favorable Clinician Acceptability of Telehealth as Part of the Cystic Fibrosis Care Model during the COVID-19 Pandemic
Corrigendum: Stenotrophomonas maltophilia phenotypic and genotypic features through 4-year cystic fibrosis lung colonization
Reference Values Matter: Fewer Patients with Malnutrition using American Compared to more Recent German Growth Charts
Comparative evaluation of expiratory airflow limitation between patients with COPD and BE using IOS
A rabbit model of cystic fibrosis
Exhaled volatile organic compounds detect pulmonary exacerbations early in children with cystic fibrosis: results of a 1 year observational pilot study
Pseudomonas aeruginosa aggregates in cystic fibrosis sputum produce exopolysaccharides that likely impede current therapies
Cystic Fibrosis Polymorphic Variants in a Russian Population
Downregulation of epithelial sodium channel (ENaC) activity in human airway epithelia after low temperature incubation
Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs
Virulence attenuating combination therapy: a potential multi-target synergy approach to treat Pseudomonas aeruginosa infections in cystic fibrosis patients
Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis
Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
Attitudes Towards Involving Children in Decision-Making Surrounding Lung Transplantation
Frequent Pet Contact as Risk Factor for Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis
Burkholderia vietnamiensis Causing a Non-lactational Breast Abscess in a Non-cystic Fibrosis Patient in Tamil Nadu, India
Neutrophil dysfunction in cystic fibrosis
Contribution of common CFTR variants (M470V, T854, and Q1463) to cystic fibrosis in Tunisia: haplotype analysis
How Many Maneuvers Should We Do for Maximal Inspiratory and Expiratory Muscle Pressure Testing in Children: A Retrospective Review in Children with Cystic Fibrosis
Disparities in insurance coverage among hospitalized adult congenital heart disease patients before and after the Affordable Care Act
Reproductive Health Counseling and Contraceptive Use in Adolescents with Cystic Fibrosis
Feasibility study on exhaled-breath analysis by untargeted Selected-Ion Flow-Tube Mass Spectrometry in children with cystic fibrosis, asthma, and healthy controls: Comparison of data pretreatment and classification techniques
Cystic fibrosis-related diabetes and lung disease: an update
Aspergillus fumigatus Strain-Specific Conidia Lung Persistence Causes an Allergic Broncho-Pulmonary Aspergillosis-Like Disease Phenotype
Comparative genomics of ST5 and ST30 methicillin-resistant Staphylococcus aureus sequential isolates recovered from paediatric patients with cystic fibrosis
An evaluation of healthcare utilization and clinical charges in children and adults with cystic fibrosis
The Incidence of Glucose Disorders Related to Cystic Fibrosis Could Be Higher with Current Criteria (Re: Carbohydrate Metabolism Changes in Cystic Fibrosis. J Pediatr Endocrinol Metab 2007; 20: 621-663)
Rapid Improvement After Starting Elexacaftor-tezacaftor-ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease
Effects of Lumacaftor-Ivacaftor on Lung Clearance Index, Magnetic Resonance Imaging and Airway Microbiome in Phe508del Homozygous Patients with Cystic Fibrosis
Bewertung der Funktionen des rechten Herzens bei Kindern mit leichter Mukoviszidose
Virtual Screening Approach to Identifying a Novel and Tractable Series of Pseudomonas aeruginosa Elastase Inhibitors
Lung Transplantation for Cystic Fibrosis in Turkey: First Report
Failed eradication therapy of new onset Pseudomonas aeruginosa infections in cystic fibrosis children is associated with bacterial resistance to neutrophil functions
Generation of two induced pluripotent stem cell lines (RCMGi004-A and -B) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/W1282X mutations in CFTR gene
The Upd3 cytokine couples inflammation to maturation defects in Drosophila
Beyond phenotype: The genomic heterogeneity of co-infecting Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients
Outcomes of cystic fibrosis pulmonary exacerbations treated with antibiotics with activity against anaerobic bacteria
Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough
Functional relationship between CFTR and RAC3 expression for maintaining cancer cell stemness in human colorectal cancer
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells
Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort
Williams-Campbell syndrome: an unusual presentation in an adult patient
Change in circulating proteins during treatment of pulmonary exacerbation in patients with cystic fibrosis
Clinical characteristics associated with lung function decline in individuals with adult-diagnosed cystic fibrosis: Contemporary analysis of the Canadian CF Registry
Interval versus constant-load exercise training in adults with Cystic Fibrosis
PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy
Withdrawal of dornase alfa increases ventilation inhomogeneity in children with cystic fibrosis
Outcomes of cystic fibrosis pulmonary exacerbations treated with antibiotics with activity against anaerobic bacteria.
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.
Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort.
Williams-Campbell syndrome: an unusual presentation in an adult patient.

Change in circulating proteins during treatment of pulmonary exacerbation in patients with cystic fibrosis.
Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough.
Functional relationship between CFTR and RAC3 expression for maintaining cancer cell stemness in human colorectal cancer.
Clinical characteristics associated with lung function decline in individuals with adult-diagnosed cystic fibrosis: Contemporary analysis of the Canadian CF Registry.
Interval versus constant-load exercise training in adults with Cystic Fibrosis.
Beyond phenotype: The genomic heterogeneity of co-infecting Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients.
Prostaglandin E2 stimulates anion and fluid secretion triggered by lipopolysaccharide in rat vaginal epithelium.
Hospice care access inequalities: a systematic review and narrative synthesis.
Immune transcriptomes of highly exposed SARS-CoV-2 asymptomatic seropositive versus seronegative individuals from the Ischgl community.
The Upd3 cytokine couples inflammation to maturation defects in Drosophila.
Inhalable nano into micro dry powders for ivacaftor delivery: The role of mannitol and cysteamine as mucus-active agents.
Genetic markers for treatment-related pancreatitis in a cohort of Hispanic children with acute lymphoblastic leukemia.
Antibiotic use evaluation in hospitalized pediatric patients with respiratory tract infections: A retrospective chart review study.
Virtual Screening Approach to Identifying a Novel and Tractable Series of Pseudomonas aeruginosa Elastase Inhibitors.
Diaphragmatic thickness and excursion by lung ultrasound in pediatric chronic pulmonary diseases.
Lung Transplantation for Cystic Fibrosis in Turkey: First Report.
Failed eradication therapy of new onset Pseudomonas aeruginosa infections in cystic fibrosis children is associated with bacterial resistance to neutrophil functions.
Generation of two induced pluripotent stem cell lines (RCMGi004-A and -B) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/W1282X mutations in CFTR gene.
Editing Myosin VB Gene to Create Porcine Model of Microvillus Inclusion Disease, With Microvillus-Lined Inclusions and Alterations in Sodium Transporters.
Feasibility study on exhaled-breath analysis by untargeted Selected-Ion Flow-Tube Mass Spectrometry in children with cystic fibrosis, asthma, and healthy controls: Comparison of data pretreatment and classification techniques.
GRAF2, WDR44, and MICAL1 mediate Rab8/10/11-dependent export of E-cadherin, MMP14, and CFTR ΔF508.
Burkholderia vietnamiensis Causing a Non-lactational Breast Abscess in a Non-cystic Fibrosis Patient in Tamil Nadu, India.
Neutrophil dysfunction in cystic fibrosis.
Contribution of common CFTR variants (M470V, T854, and Q1463) to cystic fibrosis in Tunisia: haplotype analysis.
How Many Maneuvers Should We Do for Maximal Inspiratory and Expiratory Muscle Pressure Testing in Children: A Retrospective Review in Children with Cystic Fibrosis.
A novel highly bio-available itraconazole formulation (SUBA®-Itraconazole) for anti-fungal prophylaxis in Lung Transplant recipients.
Disparities in insurance coverage among hospitalized adult congenital heart disease patients before and after the Affordable Care Act.
Reproductive Health Counseling and Contraceptive Use in Adolescents with Cystic Fibrosis.
Longitudinal effects of ivacaftor and medicine possession ratio in people with the Gly551Asp mutation: a 5-year study.
Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
Complete Genome Sequence of Burkholderia cenocepacia Phage Mica.
Phenotyping of Rare CFTR Mutations Reveals Distinct Trafficking and Functional Defects.
Current Status of Genetic Diagnosis Laboratories and Frequency of Genetic Variants Associated with Cystic Fibrosis through a Newborn-Screening Program in Turkey.
Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.
Associations Between Religious/Spiritual Coping and Depression Among Adults with Cystic Fibrosis: A 12-Month Longitudinal Study.
Friends or enemies? The complicated relationship between Pseudomonas aeruginosa and Staphylococcus aureus.
Effect of omeprazole on symptoms of gastroesophageal reflux disease in children with cystic fibrosis. A randomized, double-blind, placebo-controlled trial.
Measurement properties of the one-minute sit-to-stand test in children and adolescents with cystic fibrosis: A multicenter randomized cross-over trial.
F1099L-CFTR (c.3297C>G) has Impaired Channel Function and Associates with Mild Disease Phenotypes in Two Pediatric Patients.
First clinical trials of the inhaled epithelial sodium channel inhibitor BI 1265162 in healthy volunteers.
Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis.
Cystic fibrosis transmembrane conductance regulator-associated ligand protects dopaminergic neurons by differentially regulating metabotropic glutamate receptor 5 in the progression of neurotoxin 6-hydroxydopamine-induced Parkinson’s disease model.
Diversity and importance of tracheobronchial glands in mammalian species.
Culture-independent multilocus sequence typing of Pseudomonas aeruginosa for cross-infection screening.
Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with open-label extension.
Human Genetic Variation Influences Enteric Fever Progression.
Contribution of Drugs Interfering with Protein and Cell Wall Synthesis to the Persistence of Pseudomonas aeruginosa Biofilms: An In Vitro Model.
Pulmonary Aspergillosis: Spectrum of Disease.
Erratum to ‘From micro to macro; joining the dots of early CF lung disease’ [Journal of Cystic Fibrosis (2020) 850-851/2102].
Determinants of COVID-19 preventive behaviours among adults with chronic diseases in the USA: an analysis of the nationally representative COVID-19 impact survey.
Percutaneous endoscopic gastrostomy in children: A single center experience in Saudi Arabia.
Differential diagnosis of perinatal Bartter, Bartter and Gitelman syndromes.
Detection of cytosine methylation in Burkholderia cenocepacia by single-molecule real-time sequencing and whole-genome bisulfite sequencing.
Leukocyte Telomere Length Is Not Reduced in Children and Adults with Cystic Fibrosis but Associates with Clinical Characteristics-A Cross-Sectional Study.
Animal models for cystic fibrosis: a systematic search and mapping review of the literature. Part 2: nongenetic models.
The Potential for Phospholipids in the Treatment of Airway Inflammation: An Unexplored Solution.
Targeting G542X CFTR nonsense alleles with ELX-02 restores CFTR function in human-derived intestinal organoids.
Effect of CFTR modulator therapy on cystic fibrosis-related diabetes.
Pediatric pulmonology 2019 year in review: rare and diffuse lung disease.
Asthma in Cystic Fibrosis: Definitions and Implications of This Overlap Syndrome.
History of Newborn Screening for Cystic Fibrosis-The Early Years.
Performance of a Three-Tier (IRT-DNA-IRT) Cystic Fibrosis Screening Algorithm in British Columbia.
It All Depends What You Count-The Importance of Definitions in Evaluation of CF Screening Performance.
Constructing a Bioethical Framework to Evaluate and Optimise Newborn Bloodspot Screening for Cystic Fibrosis.
Newborn Screening for CF across the Globe-Where Is It Worthwhile?
Inconclusive Diagnosis after Newborn Screening for Cystic Fibrosis.
The Role of Extended CFTR Gene Sequencing in Newborn Screening for Cystic Fibrosis.
Processing Newborn Bloodspot Screening Results for CF.
Psychological Impact of NBS for CF.
Pancreatitis-Associated Protein in Neonatal Screening for Cystic Fibrosis: Strengths and Weaknesses.
Production of CFTR-ΔF508 Rabbits.
Prospective Evaluation of Aspergillus fumigatus-Specific IgG in Patients With Cystic Fibrosis.
Comparative Analysis of Clinical Parameters and Sputum Biomarkers in Establishing the Relevance of Filamentous Fungi in Cystic Fibrosis.
COVID-19 and Pediatric Lung Disease: A South African Tertiary Center Experience.
Differential effects on the miRNome of the treatment of human airway epithelial Calu-3 cells with peptide-nucleic acids (PNAs) targeting microRNAs miR-101-3p and miR-145-5p: Next generation sequencing datasets.
Correction of the Gene Defect in Cystic Fibrosis: Is it too late for bone?
Antibiotic Efficacy Testing in an Ex vivo Model of Pseudomonas aeruginosa and Staphylococcus aureus Biofilms in the Cystic Fibrosis Lung.
Pulmonary Infection Secondary to Blastobotrys raffinosifermentans in a Cystic Fibrosis Patient: Review of the Literature.
Telemedicine in Pediatrics: A Systematic Review of Randomized Controlled Trials.
Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor.
First and second wave of SARS-CoV2 in Italian Cystic Fibrosis patients: Data from Italian Cystic Fibrosis Registry.
Home-Care Bronchiectasis
Airway Clearance for Pediatric and Adult Cystic Fibrosis Patients Using a Portable Intra-Pulmonary Percussion Device
Prognosis-Based Early Intervention Strategies to Resolve Exacerbation and Progressive Lung Function Decline in Cystic Fibrosis.
Formulation and In Vitro and In Silico Characterization of “Nano-in-Micro” Dry Powder Inhalers Containing Meloxicam.
Urinary stone disease prevalence and associations in cystic fibrosis.
Pseudomonas aeruginosa Resistance to Bacteriophages and Its Prevention by Strategic Therapeutic Cocktail Formulation.
Complete Genome Sequence of Burkholderia gladioli Phage Maja.
Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis.
Eradication success for non-tuberculous mycobacteria in children with cystic fibrosis.
Balkan Endemic Nephropathy: An Autopsy Case Report.
The effects of the addition of a new airway clearance device to chest physiotherapy in children with cystic fibrosis pulmonary exacerbations.
A Prospective Study of the Effects of Sex Hormones on Lung Function and Inflammation in Women with Cystic Fibrosis.
Multidisciplinary approach to patients with manifestations and pulmonary complications of cystic fibrosis.
Clinical Implications of Polymicrobial Synergism Effects on Antimicrobial Susceptibility.
The Effects of Telerehabilitation on Muscle Function, Physical Activity and Sleep in Cystic Fibrosis During Pandemic
Interspecies Metabolic Complementation in Cystic Fibrosis Pathogens via Purine Exchange.
Considerations for Phage Therapy Against Mycobacterium abscessus.
Gender-affirming hormone therapy in cystic fibrosis – A case of new Pseudomonas infection.
Intestinal TMEM16A control luminal chloride secretion in a NHERF1 dependent manner.

Improvement of Pulmonary Function in Cystic Fibrosis Patients following Endoscopic Sinus Surgery.
Telemedicine Use and Health-related Concerns of Patients with Chronic Conditions During COVID-19: A Survey of Members of Online Health Communities.
The Effects of Nebulizer Hygiene Training on the Practices of Cystic Fibrosis Patients and Caregivers.
Development and in vitro characterization of a novel pMDI diclofenac formulation as an inhalable anti-inflammatory therapy for cystic fibrosis.
A Peptide-Nucleic Acid Targeting miR-335-5p Enhances Expression of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene with the Possible Involvement of the CFTR Scaffolding Protein NHERF1.
Reflections on the Importance of Cost of Illness Analysis in Rare Diseases: A Proposal.
Primary care during the transition to adult care for adolescents involved with pediatric specialty services: a scoping review protocol.
Challenges in the use of highly effective modulator treatment for cystic fibrosis.
Mesenchymal Stem Cell exosome delivered Zinc Finger Protein activation of cystic fibrosis transmembrane conductance regulator.
Histomorphology and Immunohistochemistry of a Congenital Nephromegaly Demonstrate Concurrent Features of Heritable and Acquired Cystic Nephropathies in a Girgentana Goat (Capra falconeri).
Losartan reduces cigarette smoke-induced airway inflammation and mucus hypersecretion.
Avatar acceptability: views from the Australian Cystic Fibrosis community on the use of personalised organoid technology to guide treatment decisions.
Sex disparities in cystic fibrosis: review on the effect of female sex hormones on lung pathophysiology and outcomes.
Longitudinal non-cystic fibrosis trends of pulmonary Mycobacterium abscessus disease from 2010 to 2017: spread of the “globally successful clone” in Asia.
Large pH oscillations promote host defense against human airways infection.
DNAJB12 and Hsp70 Triage Arrested Intermediates of N1303K-CFTR for ER Associated-Autophagy.
Pilocarpine Microneedles for Sweat Induction (PMN-SI)
Microbial Biomarkers of EArly Pseudomonas Aeruginosa Colonization in CHildren With Cystic Fibrosis
Environmental Microbial Contamination during Cystic Fibrosis Group-Based Psychotherapy.
JCF Year in Review.
Sclerosing Polycystic Adenoma.
Cystic Fibrosis.
Patterns of azithromycin use in obstructive airway diseases: a real-world observational study.
From genotype to phenotype: adaptations of Pseudomonas aeruginosa to the cystic fibrosis environment.
Interactions between ABCC4/MRP4 and ABCC7/CFTR in Human Airway Epithelial Cells in Lung Health and Disease.
Within-Host Microevolution of Pseudomonas aeruginosa Urinary Isolates: A Seven-Patient Longitudinal Genomic and Phenotypic Study.
How Clinically Efficient Is Lumacaftor/Ivacaftor for Cystic Fibrosis Patients? An Updated Literature Review.
Intravital Imaging of Pulmonary Immune Response in Inflammation and Infection.
Social Inequities and Cystic Fibrosis Outcomes: We Can Do Better.
Azithromycin and Tobramycin Therapy in Cystic Fibrosis Pulmonary Exacerbations: Less Is More?
Pseudomonas aeruginosa induces p38MAP kinase-dependent IL-6 and CXCL8 release from bronchial epithelial cells via a Syk kinase pathway.
Optimization of anti-pseudomonal antibiotics for cystic fibrosis pulmonary exacerbations: II. Cephalosporins and penicillins latest update.
Modulators of CFTR. Updates on clinical development and future directions.
Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study.
The O2-independent pathway of ubiquinone biosynthesis is essential for denitrification in Pseudomonas aeruginosa.
Lung Microbiome in Cystic Fibrosis.
Serious infectious events and ibuprofen administration in pediatrics: a narrative review in the era of COVID-19 pandemic.
Exploring the Developmental Impact of Cystic Fibrosis on Lung Transplant Candidacy: Considerations for Adulthood.
Pseudomonas aeruginosa Elastase Contributes to the Establishment of Chronic Lung Colonization and Modulates the Immune Response in a Murine Model.
Personalized approaches to bronchiectasis.
Outcomes of Repeat Sweat Testing in Cystic Fibrosis Newborn Screen Positive Infants.
Pseudomonas aeruginosa colonization in cystic fibrosis: Impact on neutrophil functions and cytokine secretion capacity.
Screening of depression and anxiety in adolescents with cystic fibrosis and caregivers in Turkey by PHQ-9 and GAD-7 questionnaires.
Quantitative Evaluation of CFTR Pre-mRNA Splicing Dependent on the (TG)mTn Poly-Variant Tract.
Overcoming Challenges to Make Bacteriophage Therapy Standard Clinical Treatment Practice for Cystic Fibrosis.
Early Diagnosis and Intervention in Cystic Fibrosis: Imagining the Unimaginable.
GLP-1 Agonist Therapy in Cystic Fibrosis-Related Glucose Intolerance
Recognition of Diagnostic Gaps for Laboratory Diagnosis of Fungal Diseases: Expert Opinion from the Fungal Diagnostics Laboratories Consortium (FDLC).
CFTR modulators: transformative therapies for cystic fibrosis.
Cost-effectiveness of implementing routine hearing screening using a tablet audiometer for pediatric cystic fibrosis patients receiving high-dose IV aminoglycosides.
The effectiveness and value of novel treatments for cystic fibrosis.
[Novel therapies in respiratory management].
Feasibility of Outpatient Closed Loop Control With the iLet Bionic Pancreas in Cystic Fibrosis Related Diabetes
Cystic fibrosis-related diabetes onset can be predicted using biomarkers measured at birth.
Molecular mechanism mediating enteric bacterial translocation after severe burn: the role of cystic fibrosis transmembrane conductance regulator.
Metabotypes of Pseudomonas aeruginosa Correlate with Antibiotic Resistance, Virulence and Clinical Outcome in Cystic Fibrosis Chronic Infections.
Synthesis of Tetravalent Thio- and Selenogalactoside-Presenting Galactoclusters and Their Interactions with Bacterial Lectin PA-IL from Pseudomonas aeruginosa.
Prolonged Treatment With Inhaled Ampicillin in Children With Non-cystic Fibrosis Bronchiectasis.
The proteostatic network chaperome is downregulated in F508del homozygote cystic fibrosis.
“Test-retest reliability and minimal detectable change in TGlittre-P test in children and adolescents with cystic fibrosis”.
[Portuguese Newborn Screening Program.]
Overcoming the Limits of Reconditioning: Seventeen Hours of Ex-Vivo Lung Perfusion (EVLP) with Successful Transplantation from Uncontrolled Circulatory Death Donor.
A Mobile Health Platform for Self-Management of Pediatric Cystic Fibrosis: Qualitative Study of Adaptation to Stakeholder Needs and Integration in Clinical Settings.
Peripheral muscle strength is associated with aerobic fitness and use of antibiotics in patients with cystic fibrosis.
Animal Models and Their Role in Understanding the Pathophysiology of Cystic Fibrosis-Associated Gastrointestinal Lesions.
Comparison of Hyperpolarized 3He and 129Xe MR Imaging in Cystic Fibrosis Patients.
A narrative review of electrical impedance tomography in lung diseases with flow limitation and hyperinflation: methodologies and applications.
The fractional excretion of sodium in patients with cystic fibrosis treated with oral sodium chloride.
Preoperative evaluation for lung resection in patients with bronchiectasis: should we rely on standard lung function evaluation?
Analysis of national coverage of neonatal cystic fibrosis screening in Brazil from 2008 to 2017.
Manifesto on United Airways Diseases (UAD): An Interasma (Global Asthma Association – GAA) document.
Therapeutic peptides for the treatment of cystic fibrosis: Challenges and perspectives.
Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators.
In vitro evaluation of drug delivery behavior for inhalable amorphous nanoparticle formulations in a human lung epithelial cell model.
Pharmacokinetic and Pharmacodynamic Optimization of Antibiotic Therapy in Cystic Fibrosis Patients: Current Evidences, Gaps in Knowledge and Future Directions.
Assessing the validity and applicability of the French 3-year prognostic score in the UK cystic fibrosis population – a national cohort study.
Nanoparticles for Delivery of Agents to Fetal Lungs.
Virulence attenuating combination therapy: a potential multi-target synergy approach to treat Pseudomonas aeruginosa infections in cystic fibrosis patients.
New therapies for people with CF in the CFTR modulator world.
From micro to macro; joining the dots of early CF lung disease.
The PROSPECT Is Bright for CFTR Modulators.
Microstructured Lipid Carriers (MLC) Based on N-Acetylcysteine and Chitosan Preventing Pseudomonas aeruginosa Biofilm.
Cellular Redox State Acts as Switch to Determine the Direction of NNT-Catalyzed Reaction in Cystic Fibrosis Cells.
Neutrophil extracellular traps are present in the airways of ENaC-overexpressing mice with cystic fibrosis-like lung disease.
Abnormal glucose tolerance and lung function in children with cystic fibrosis. Comparing oral glucose tolerance test and continuous glucose monitoring.
Incidence and risk factors for respiratory tract bacterial colonization and infection in lung transplant recipients.
Pediatrician intervention impacts parental smoking in cystic fibrosis, diabetes and bronchiolitis.
EGFR activity upregulates lactate dehydrogenase A (LDHA) expression, LDH activity and lactate secretion in cultured IB3-1 cystic fibrosis lung epithelial cells.
Safety of research bronchoscopy with BAL in stable adult patients with cystic fibrosis.
Myriocin modulates the altered lipid metabolism and storage in cystic fibrosis.
Transmission and antibiotic resistance of Achromobacter in cystic fibrosis.
Novel Lytic Phages Protect Cells and Mice against Pseudomonas aeruginosa Infection.
Phenotypic and Genotypic Adaptations in Pseudomonas aeruginosa Biofilms following Long-Term Exposure to an Alginate Oligomer Therapy.
Pediatric Resident Education in Pulmonary (PREP): A Subspecialty Preparatory Boot Camp Curriculum for Pediatric Residents.
The effect of an intensive residential rehabilitation program on body composition in patients with cystic fibrosis.
Early Life Height Attainment in Cystic Fibrosis Is Associated with Pulmonary Function at Age 6 Years.
[Clinical characteristics and gene variants of patients with infantile intrahepatic cholestasis].
A Case of Phage Therapy against Pandrug-Resistant Achromobacter xylosoxidans in a 12-Year-Old Lung-Transplanted Cystic Fibrosis Patient.
Role of Collagen in Airway Mechanics.
Contemporary analysis of ETEST for antibiotic susceptibility and minimum inhibitory concentration agreement against Pseudomonas aeruginosa from patients with cystic fibrosis.

Ultra-low-dose thoracic CT with model-based iterative reconstruction (MBIR) in cystic fibrosis patients undergoing treatment with cystic fibrosis transmembrane conductance regulators (CFTR).
Positive epistasis between disease-causing missense mutations and silent polymorphism with effect on mRNA translation velocity.
Meta-analysis of Efficacy and Safety of Inhaled Ciprofloxacin in Non-cystic Fibrosis Bronchiectasis Patients.
Does Bordetella pertussis vaccine offer any cross-protection against Bordetella bronchiseptica? Implications for pet owners with cystic fibrosis.
Understanding the expanding role of pharmacy services in outpatient cystic fibrosis care.
Cystic Fibrosis Patients of Minority Race and Ethnicity Less Likely Eligible for CFTR Modulators Based on CFTR Genotype.
CF Monocyte Derived Macrophages Have an Attenuated Response to Extracellular Vesicles Secreted by Airway Epithelial Cells.
Gene Therapy for Cystic Fibrosis: Lessons Learned and Paths Forward.
In silico drug repositioning on F508del-CFTR: A proof-of-concept study on the AIFA library.
COVID-19 lockdown beneficial effects on lung function in a cohort of cystic fibrosis patients.
Changes in airway inflammation with pseudomonas eradication in early cystic fibrosis.
Neonatal screening program for five conditions in Honduras.
Sharing Patient-Controlled Real-World Data Through the Application of the Theory of Commons: Action Research Case Study.
The Risks of Complications During Endoscopic Sinus Surgery in Cystic Fibrosis Patients: An Anatomical and Endoscopic Study.
A Randomized, Double-Blind, Placebo-Controlled, Multiple Dose Escalation Study to Evaluate the Safety and Pharmacokinetics of ELX-02 in Healthy Subjects.
Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in a Pediatric Cystic Fibrosis Cohort.
Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Study Design.
Cystic fibrosis transmembrane conductance regulator in follicular fluid and cumulus cells and its relationship with age.
Preemptive non-selective bronchial artery angioembolization to reduce recurrence rate of hemoptysis.
Perioperative Management and Preemptive ECMO Cannulation of a Parturient with Cystic Fibrosis Undergoing Cesarean Delivery.
Applying Petitmengin’s Explicitation Interview Method to Elicit the Lived Experience of Breathing Upon Waking by an Individual With Cystic Fibrosis.
Hearing the Voice of a Shadow Child: Healthy Siblings Experience of Cystic Fibrosis and Other Life-Threatening Conditions.
Virtual Peer Support for People With Cystic Fibrosis and Their Family Members: A Program Evaluation.
Associations between peak oxygen uptake, lung function and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.
[Guideline for the diagnosis and treatment of patients with cystic fibrosis. Update].
Kill and cure: genomic phylogeny and bioactivity of Burkholderia gladioli bacteria capable of pathogenic and beneficial lifestyles.
diagnosis of endocrine disease: Diagnosing and classifying diabetes in diseases of the exocrine pancreas.
Lung microbiota predict chronic rejection in healthy lung transplant recipients: a prospective cohort study.
Roles of microRNAs in chronic pediatric diseases and their use as potential biomarkers: A review.
Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity.
Is hyperpolarised gas magnetic resonance imaging a valid and reliable tool to detect lung health in cystic fibrosis patients? a cosmin systematic review.
Biomarkers for the diagnosis of allergic bronchopulmonary aspergillosis in cystic fibrosis: a systematic review and meta-analysis.
The Aspergillus fumigatus Secretome Alters the Proteome of Pseudomonas aeruginosa to Stimulate Bacterial Growth: Implications for Co-infection.
Designing the GALAXY study: Partnering with the cystic fibrosis community to optimize assessment of gastrointestinal symptoms.
Signal Synthase-Type versus Catabolic Monooxygenases: Retracing 3-Hydroxylation of 2-Alkylquinolones and Their N-Oxides by Pseudomonas aeruginosa and Other Pulmonary Pathogens.
Diagnosis of cystic fibrosis in adulthood and eligibility for novel CFTR modulator therapy.
Commentary for the article: MicroRNA-1246 regulates proliferation, invasion and differentiation in human vascular smooth muscle cell by targeting cystic fibrosis transmembrane conductance regulator (CFTR).
Pancreatic Cancer-Associated Diabetes is Clinically Distinguishable From Conventional Diabetes.
Towards human translation of lentiviral airway gene delivery for cystic fibrosis: A one-month CFTR and reporter gene study in marmosets.
Complete Genome Sequence of Achromobacter xylosoxidans Myophage Mano.
Unsupervised Phenotypic Clustering for Determining Clinical Status in Children with Cystic Fibrosis.
[Bird fancier’s disease in a child: about a rare and unusual case].
Weak acids as an alternative anti-microbial therapy.
Antibiotic susceptibility of cystic fibrosis lung microbiome members in a multispecies biofilm.
Antibiotics in Adult Cystic Fibrosis Patients: A Review of Population Pharmacokinetic Analyses.
Copper nanoparticles: Biosynthesis, characterization, and protoscolicidal effects alone and combined with albendazole against hydatid cyst protoscoleces.
Cystic Fibrosis: Quality of Life and Radiological Monitoring.
Gasdermin D restricts Burkholderia cenocepacia infection in vitro and in vivo.
Modulation of cAMP metabolism for CFTR potentiation in human airway epithelial cells.
Methods and feasibility study for exome sequencing as a universal second-tier test in newborn screening.
Surgery for Ampullary Cancer in a Patient with Pancreatic Lipomatosis Caused by Cystic Fibrosis.
Influence of relevant cystic fibrosis bacteria on Scedosporium apiospermum and Scedosporium boydii growth and viability.
Exercise as a substitute for traditional airway clearance in cystic fibrosis: a systematic review.
On the relationship between anion binding and chloride conductance in the CFTR anion channel.
A multicentre analysis of Clostridium difficile in persons with Cystic Fibrosis demonstrates that carriage may be transient and highly variable with respect to strain and level: Cystic Fibrosis and Clostridium difficile.
PIPERACILLIN-TAZOBACTAM HYPERSENSITIVITY: A LARGE, MULTICENTRE ANALYSIS.
Assessing the applicability of the new Global Lung Function Initiative reference values for the diffusing capacity of the lung for carbon monoxide in a large population set.
COVID-19 rapid guideline: cystic fibrosis
Korean Red Ginseng aqueous extract improves markers of mucociliary clearance by stimulating chloride secretion.
Same Game, Different Players: Emerging Pathogens of the CF Lung.
Peptide-Conjugated Phosphorodiamidate Morpholino Oligomers Retain Activity against Multidrug-Resistant Pseudomonas aeruginosa In Vitro and In Vivo.
The GENDULF algorithm: mining transcriptomics to uncover modifier genes for monogenic diseases.
Metformin slows liver cyst formation and fibrosis in experimental model of polycystic liver disease.
The biofilm of Burkholderia cenocepacia H111 contains an exopolysaccharide composed of l-rhamnose and l-mannose: Structural characterization and molecular modelling.
Mobilome Analysis of Achromobacter spp. Isolates from Chronic and Occasional Lung Infection in Cystic Fibrosis Patients.
Molecular characterization, expression analysis of 14-3-3 beta/alpha and the effect of RNA interference on ion transporter protein Na+-K+-ATPase, Na+-H+-exchanger and CFTR in turbot (Scophthalmus maximus).
Antipseudomonal and Immunomodulatory Properties of Esc Peptides: Promising Features for Treatment of Chronic Infectious Diseases and Inflammation.
Effects of therapeutic probiotics on modulation of microRNAs.
Relationship between airway dysbiosis, inflammation and lung function in adults with cystic fibrosis.
Patient and Family Perspectives Regarding the Use of Telehealth For Cystic Fibrosis Care.
Clinical implications of fungal isolation from sputum in adult patients with cystic fibrosis.
Comparing Stable Isotope Enrichment by Gas Chromatography with Time-of-Flight, Quadrupole Time-of-Flight, and Quadrupole Mass Spectrometry.
SARS-CoV-2 may hijack GPCR signaling pathways to dysregulate lung ion and fluid transport.
A case of testicular atrophy associated with cystic fibrosis.
Reduction of pulmonary exacerbations in young children with cystic fibrosis during the COVID-19 pandemic.
Epidemiologic Study of Cystic Fibrosis: 25 years of observational research.
Bacteriophage therapy for infections in CF.
Entering the era of highly effective modulator therapies.
Assessment of Distinct Electrophysiological Parameters in Rectal Biopsies for the Choice of the Best Diagnosis/Prognosis Biomarkers for Cystic Fibrosis.
Diaphragmatic Ultrasound in Non-Cystic Fibrosis Bronchiectasis: Relationship to Clinical Parameters.
Where is the Cystic Fibrosis Transmembrane Conductance Regulator?
Colorectal Cancer Screening and Surveillance for Non-Hereditary High-Risk Groups-Is It Time for a Re-Think?
Use of a Connected Companion in Children With Cystic Fibrosis
Current and novel therapeutic strategies for the management of cystic fibrosis.
Biofilms.
Dynamic light scattering microrheology for soft and living materials.
Tyrosine supplementation for phenylketonuria.
HIGH PREVALENCE OF NONTUBERCULOUS MYCOBACTERIA IN CYSTIC FIBROSIS PATIENTS IN TROPICAL FRENCH REUNION ISLAND.
The antimicrobial peptide Gad-1 clears Pseudomonas aeruginosa biofilms under cystic fibrosis conditions.
Exhaled volatile organic compounds detect pulmonary exacerbations early in children with Cystic Fibrosis: results of a one-year observational pilot study.
Introducing the Adult Cystic Fibrosis Series: An Exciting Time of Change, But New Challenges Lie Ahead.
The modified shuttle test as a predictor of risk for hospitalization in youths with cystic fibrosis: A two-year follow-up study: Modified shuttle test as a predictor of hospitalization.
Untapped potential: Therapeutically targeting eicosanoids and endocannabinoids in the lung.
Therapeutic Potential of Antimicrobial Peptides in Polymicrobial Biofilm-Associated Infections.
MicroRNA-1246 regulates proliferation, invasion, and differentiation in human vascular smooth muscle cells by targeting cystic fibrosis transmembrane conductance regulator (CFTR).
Effect of Expiratory Muscle Training on Peak Cough Flow in Children and Adolescents with Cystic Fibrosis: A Randomized Controlled Trial.
Implementation of microbiota analysis in clinical trials for cystic fibrosis lung infection: Experience from the OligoG phase 2b clinical trials.
Looking beyond pulmonary disease in COVID-19: A lesson from patients with cystic fibrosis.
CRISPR/Cas9 Gene Editing Therapies for Cystic Fibrosis.
Clinico-microbiological profile of Burkholderia cepacia keratitis: a case series.

Significant functional differences in differentiated Conditionally Reprogrammed (CRC)- and Feeder-free Dual SMAD inhibited-expanded human nasal epithelial cells.
Ataluren and aminoglycosides stimulate read-through of nonsense codons by orthogonal mechanisms.
A dyadic approach to the delineation of diagnostic entities in clinical genomics.
Positive expiratory pressure (PEP) therapy. What pressures do we achieve in young children with cystic fibrosis? A single-centre study.
Extracellular cyclic dinucleotides induce polarized responses in barrier epithelial cells by adenosine signaling.
Tribulations and (clinical) trials in cystic fibrosis.
Impaired cholesterol metabolism in the mouse model of cystic fibrosis. A preliminary study.
Cystic fibrosis in Tuscany: evolution of newborn screening strategies over time to the present.
SARS-CoV-2 Entry Genes Expression in Relation with Interferon Response in Cystic Fibrosis Patients.
Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs.
Erratum to “Epithelial vectorial ion transport in cystic fibrosis: Dysfunction, measurement, and pharmacotherapy to target the primary deficit”.
Carbapenem-Resistant Pseudomonas aeruginosa in Chronic Lung Infection: Current Resistance Profile and Hypermutability in Patients with Cystic Fibrosis.
A Murine Model for Chronic A. fumigatus Airway Infections.
Feasibility of a Goal-based Agenda Setting Intervention
Thoracic Mobility in Cystic Fibrosis Care
Optimizations of In Vitro Mucus and Cell Culture Models to Better Predict In Vivo Gene Transfer in Pathological Lung Respiratory Airways: Cystic Fibrosis as an Example.
Synergy between Readthrough and Nonsense Mediated Decay Inhibition in a Murine Model of Cystic Fibrosis Nonsense Mutations.
Significance of kidney biopsy in autosomal dominant tubulointerstitial kidney disease-UMOD: is kidney biopsy truly nonspecific?
Comparison of respiratory pathogen colonization and antimicrobial susceptibility in people with cystic fibrosis bronchiectasis versus non-cystic fibrosis bronchiectasis: a protocol for a systematic review.
Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis.
Recurrent massive hemoptysis in a patient with cystic fibrosis: balloon assisted Onyx embolization after bronchial artery coil recanalization.
Repurposing calcium sensing receptor agonist cinacalcet for treatment of CFTR-mediated secretory diarrheas.
Pseudomonas aeruginosa associated with severity of non-cystic fibrosis bronchiectasis measured by the modified bronchiectasis severity score (BSI) and the FACED: The US bronchiectasis and NTM Research Registry (BRR) study.
New Therapeutic Approaches in Cystic Fibrosis.
Development of an intervention to increase adherence to nebuliser treatment in adults with cystic fibrosis: CFHealthHub.
A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay.
The Interplay Between Respiratory Microbiota and Innate Immunity in Flavor E-Cigarette Vaping Induced Lung Dysfunction.
Nephronophthisis due to a novel DCDC2 variant in a patient from African-Caribbean descent: A case report.
Oxygen uptake kinetics during treadmill walking in adolescents with clinically stable cystic fibrosis.
Altered Degranulation and pH of Neutrophil Phagosomes Impacts Antimicrobial Efficiency in Cystic Fibrosis.
A Case of Scedosporium aurantiacum Infection in the United States.
In vivo Evaluation of Mucociliary Clearance in Mice.
Cystic fibrosis or not? Familial occurrence of a rare mutation in the CFTR gene.
Distinctive metabolic profiles between Cystic Fibrosis mutational subclasses and lung function.
Influence of Acid Blockade on the Aerodigestive Tract Microbiome in Children With Cystic Fibrosis.
Obstructive lung diseases and allergic bronchopulmonary aspergillosis.
Erratum to: Epidemiology of Nontuberculous Mycobacteria Infection in Children and Young People With Cystic Fibrosis: Analysis of UK Cystic Fibrosis Registry.
Vitamin E supplementation in people with cystic fibrosis: Summary of a Cochrane review.
VITAMIND AND ITS THERAPEUTIC RELEVANCE IN PULMONARY DISEASES.
Abnormal Lung Clearance Index in Cystic Fibrosis Children with Normal FEV1 and Single-Breath Nitrogen Washout Test.
Predicting Substance Use from Religiosity/Spirituality in Individuals with Cystic Fibrosis.
Bridging the survival gap in cystic fibrosis: An investigation of lung transplant outcomes in Canada and the United States.
OTX2 regulates CFTR expression during endoderm differentiation and occupies 3′ cis-regulatory elements.
Psychological distress in adults with and without cystic fibrosis during the COVID-19 lockdown.
Genetic information from discordant sibling pairs points to ESRP2 as a candidate trans-acting regulator of the CF modifier gene SCNN1B.
Age at Lung Transplant Impacts Post-Transplant Survival in Cystic Fibrosis; Why?
Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation.
Identification of Predictive Epigenetic Biomarkers of Lung Disease Severity in Cystic Fibrosis
Physiometric Response to High-Flow Nasal Cannula Support in Acute Bronchiolitis.
Carbamazepine-induced sperm disorders can be associated with the altered expressions of testicular KCNJ11/miR-let-7a and spermatozoal CFTR/miR-27a.
SARS-CoV-2 related pneumonia in an adult with cystic fibrosis: natural favourable clinical course or effective therapy?
Andrological findings in infertile men with two (biallelic) CFTR mutations: results of a multicentre study in Germany and Austria comprising 71 patients.
Host genetic effects in pneumonia.
The clinical and microbiological utility of inhaled aztreonam lysine for the treatment of acute pulmonary exacerbations of cystic fibrosis: An open-label randomised crossover study (AZTEC-CF).
Long-term Follow-up in Adult Patients with Cystic Fibrosis and Deep Intronic Splicing Variants.
Identification of cell-surface glycans that mediate motility-dependent binding and internalization of Pseudomonas aeruginosa by phagocytes.
VO2max as an exercise tolerance endpoint in people with cystic fibrosis: Lessons from a lumacaftor/ivacaftor trial.
Purinergic receptors in airway hydration.
Continuous and daily oral immunotherapy for peanut allergy: results from a 2-year open-label follow-on study.
Longitudinal Course of Clinical Lung Clearance Index in Children with Cystic Fibrosis.
β-lactam combinations that exhibit synergy against Mycobacteroides abscessus clinical isolates.
Modeling of the Transport and Exchange of a Gas Species in Lungs With an Asymmetric Branching Pattern. Application to Nitric Oxide.
An open-source high-content analysis workflow for CFTR function measurements using the forskolin-induced swelling assay.
Murepavadin antimicrobial activity against and resistance development in cystic fibrosis Pseudomonas aeruginosa isolates.
Authors’ reply re: The outcome of pregnancy in women with cystic fibrosis: a UK population-based descriptive study.
Preferences for Disclosing Adverse Childhood Experiences for Children and Adults with Cystic Fibrosis.
Differentially Expressed Genes Associated with Disease Severity In Siblings with Cystic Fibrosis.
Quantifying the Effects of Antimicrobials on In vitro Biofilm Architecture using COMSTAT Software.
Correlates of distress in young people with cystic fibrosis: the role of self-efficacy and metacognitive beliefs.
Oral cysteamine as an adjunct treatment in cystic fibrosis pulmonary exacerbations: An exploratory randomized clinical trial.
Implementation of a Clinical Tool to Improve Waitlist Mortality in Patients With Cystic Fibrosis
Clinical Biofilm Ring Test® Reveals the Potential Role of β-Lactams in the Induction of Biofilm Formation by P. aeruginosa in Cystic Fibrosis Patients.
Congenital absence of the vas deferens: Cystic fibrosis transmembrane regulatory gene mutations.
Skin Metabolomics.
Appraisal of Clinical Practice Guideline: Physiotherapy for cystic fibrosis in Australia and New Zealand.
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.
Influence of pre-diabetic and pancreatic exocrine states on pulmonary and nutritional status in adults with Cystic Fibrosis.
A Pilot Study to Assess the Effect of Intermittent iNO on the Treatment of NTM Lung Infection in CF and Non-CF Patients
The Impact of Patch Rx Technologies on Adherence to Vitamin and Trikafta Therapies in Patients With Cystic Fibrosis
CYstic Fibrosis bacterioPHage Study at Yale (CYPHY)
Effects of a Telerehabilitation Approach in Children With Cystic Fibrosis
Video Telehealth Exercise Training in Cystic Fibrosis
Variant classifications, databases and genotype-phenotype correlations.
The Multifaceted Roles of MicroRNAs in Cystic Fibrosis.
Increased Fecal Calprotectin Is Associated with Worse Gastrointestinal Symptoms and Quality of Life Scores in Children with Cystic Fibrosis.
Graphene-Doped Tin Oxide Nanofibers and Nanoribbons as Gas Sensors to Detect Biomarkers of Different Diseases through the Breath.
A Transparent Approach to Calculate Detection Rate and Residual Risk for Carrier Screening.
The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network.
CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis.
Vitamin D supplementation in patients with cystic fibrosis: A systematic review and meta-analysis.
Cellulophaga algicola alginate lyase inhibits biofilm formation of a clinical Pseudomonas aeruginosa strain MCC 2081.
Caring for Adolescents with Cystic Fibrosis, in Portugal: The Nurse’s Role.
Results of Tobramycin Inhalation Therapy in Patients with Noncystic Fibrosis Bronchiectasis with Pseudomonas aeruginosa Colonization: Real Life Management.
Care of Cystic Fibrosis Children in COVID-19 Pandemic.
NKCC1: Newly Found as a Human Disease-Causing Ion Transporter.
The clinical use of lung MRI in cystic fibrosis: what, now, how?
The effect of particle size of inhaled tobramycin dry powder on the eradication of Pseudomonas aeruginosa biofilms.
A Population Pharmacokinetic Modeling Approach to Determine the Efficacy of Intravenous Amikacin in Children with Cystic Fibrosis.
Role of Tris-CaEDTA as an adjuvant with nebulised tobramycin in cystic fibrosis patients with Pseudomonas aeruginosa lung infections: A randomised controlled trial.
Ototoxicity in cystic fibrosis patients receiving intravenous tobramycin for acute pulmonary exacerbation: Ototoxicity following tobramycin treatment.

A phase 3, randomized, double-blind, parallel-group study to evaluate tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for F508del-CFTR and a gating mutation.
Impact of biofilm formation and azoles’ susceptibility in Scedosporium/Lomentospora species using an in vitro model that mimics the cystic fibrosis patients’ airway environment.
Human Papilloma Virus Vaccination Among Female Patients Attending French Paediatric Cystic Fibrosis Centres.
Can we Train the Immune System of Patients With Cystic Fibrosis?
Microbiome data enhances predictive models of lung function in people with cystic fibrosis.
[Rare diseases on the agenda for innovation in health: progress and challenges with cystic fibrosis].
Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
Respiratory muscle training for cystic fibrosis.
Glucose tolerance stages in Cystic Fibrosis are idenfied by a unique pattern of defects of Beta-cell function.
Sensitization to A.fumigatus in subjects with non-cystic fibrosis bronchiectasis.
Office-Based Sinus Surgery for Cystic Fibrosis Chronic Rhinosinusitis.
The challenge of medication acquisition in cystic fibrosis; cracking the nut with pharmacy technicians.
Adenocarcinoma arising in the multiple heterotopic submucosal glands of the intestine in a Satoyoshi syndrome patient: A case report.
Current Advances in Burkholderia Vaccines Development.
CRMS/CFSPID Subjects Carrying D1152H CFTR Variant: Can the Second Variant Be a Predictor of Disease Development?
Environmental risks of Pseudomonas aeruginosa-What to advise patients and parents.
Mechanisms and Control Strategies of Antibiotic Resistance in Pathological Biofilms.
[Evaluation and perspective of 20 years of neonatal screening in Galicia. Program results.]
[Results of the neonatal screening on Western Andalusia after a decade of experience.]
[Response of the Catalonia neonatal screening laboratory to the pandemic of SARS-CoV-2.]
[Impact of the inclusion of second-tier tests in the newborn screening program of Catalonia and in other international programs.]
[50 years of the Neonatal Screening Program in Catalonia.]
Healthcare Resource Utilization, Exacerbations, and Readmissions Among Medicare Patients with Chronic Obstructive Pulmonary Disease After Long-Acting Muscarinic Antagonist Therapy Initiation with Soft Mist versus Dry Powder Inhalers.
ELISA Test for the Serological Detection of Scedosporium/Lomentospora in Cystic Fibrosis Patients.
Cystic Fibrosis Point of Personalized Detection (CFPOPD): An Interactive Web Application.
Safety of N-Acetylcysteine at High Doses in Chronic Respiratory Diseases: A Review.
Antimicrobial Susceptibility Testing in Pseudomonas aeruginosa Biofilms: One Step Closer to a Standardized Method.
Pseudomonas aeruginosa Susceptibility Patterns and Associated Clinical Outcomes in People with Cystic Fibrosis following Approval of Aztreonam Lysine for Inhalation.
Burkholderia ubonensis high-level tetracycline resistance is due to efflux pump synergy involving a novel TetA(64) resistance determinant.
In vitro antibacterial activity and in vivo efficacy of sulbactam-durlobactam against pathogenic Burkholderia species.
Human extrahepatic and intrahepatic cholangiocyte organoids show region-specific differentiation potential and model cystic fibrosis-related bile duct disease.
The first report of Methicillin-resistant Staphylococcus aureus (MRSA) in cystic fibrosis (CF) patients in Saudi Arabia.
The human respiratory tract microbial community structures in healthy and cystic fibrosis infants.
Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia.
VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.
Development of a device to measure adherence and pressure characteristics of positive expiratory pressure therapies used by adults with cystic fibrosis.
Autosomal dominant polycystic kidney disease and pioglitazone for its therapy: a comprehensive review with an emphasis on the molecular pathogenesis and pharmacological aspects.
Wheeze and cough measurements at night in children with respiratory symptoms.
Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study.
Amphotericin B induces epithelial voltage responses in people with cystic fibrosis.
[AFU and SALF recommendations for the evaluation of male infertility].
Pseudomonas aeruginosa by Pseudomonas aeruginosa.
Processing of positive newborn screening results: a qualitative exploration of current practice in England.
‘Triple therapy’ (elexacaftor, tezacaftor, ivacaftor) skin rash in patients with cystic fibrosis.
Review of Rapid Advances in Cystic Fibrosis.
The regulatory role of vasoactive intestinal peptide in lacrimal gland ductal fluid secretion: A new piece of the puzzle in tear production.
Bronchial Artery Embolization, an Increasingly Used Method for Hemoptysis; Treatment and Avoidance.
Proximal intestinal obstruction syndrome (PIOS) in a patient with cystic fibrosis: A case report.
Parasexual recombination enables Aspergillus fumigatus to persist in cystic fibrosis.
Preclinical evaluation of the epithelial sodium channel inhibitor BI 1265162 for treatment of cystic fibrosis.
An innovative phase II trial to establish proof of efficacy and optimal dose of a new inhaled epithelial sodium channel inhibitor BI 1265162 in adults and adolescents with cystic fibrosis: BALANCE-CFTM 1.
Cefiderocol for the Treatment of Adult and Pediatric Patients with Cystic Fibrosis and Achromobacter xylosoxidans Infections.
Cyclic Peptidyl Inhibitors against CAL/CFTR Interaction for Treatment of Cystic Fibrosis.
Spellchecking for the Story of Life With CRISPR-Cas9 and Base, Prime Editors.
Optimization of Aztreonam in Combination with Ceftazidime/Avibactam in a Cystic Fibrosis Patient with Chronic Stenotrophomonas maltophilia Pneumonia Using Therapeutic Drug Monitoring: A Case Study.
Bacterial persisters in long-term infection: Emergence and fitness in a complex host environment.
Mepolizumab use in cystic fibrosis-associated allergic bronchopulmonary aspergillosis.
The impact of liver disease on mortality in cystic fibrosis – a systematic review protocol.
Organoids for personalized treatment of Cystic Fibrosis: Professional perspectives on the ethics and governance of organoid biobanking.
Targeting Cystic Fibrosis Inflammation in the Age of CFTR Modulators: Focus on Macrophages.
Rescue of multiple class II CFTR mutations by elexacaftor+ tezacaftor+ivacaftor mediated in part by the dual activities of Elexacaftor as both corrector and potentiator.
Free-breathing MRI for Monitoring Ventilation Changes following Antibiotic Treatment of Pulmonary Exacerbations in Pediatric Cystic Fibrosis.
IL-17A from innate and adaptive lymphocytes contributes to inflammation and damage in cystic fibrosis lung disease.
(R)-Roscovitine and CFTR modulators enhance killing of multi-drug resistant Burkholderia cenocepacia by cystic fibrosis macrophages.
Reciprocal Links Between Physical Health and Coping Among Adolescents With Cystic Fibrosis.
Inhaled tobramycin for chronic infection with pseudomonas aeruginosa in non-cystic fibrosis bronchiectasis: A systematic review and meta-analysis.
Airway Redox Homeostasis and Inflammation Gone Awry: From Molecular Pathogenesis to Emerging Therapeutics in Respiratory Pathology.
Comparative evaluation of the effect of different growth media on in vitro sensitivity to azithromycin in multi-drug resistant Pseudomonas aeruginosa isolated from cystic fibrosis patients.
Phages as immunomodulators and their promising use as anti-inflammatory agents in a cftr loss-of-function zebrafish model.
Lung transplant referrals for individuals with cystic fibrosis: a pediatric perspective on the Cystic Fibrosis Foundation consensus guidelines.
Cystic Fibrosis F508del Carriers and Cancer Risk: Results from the UK Biobank.
Longitudinal Assessment of Patients With Cystic Fibrosis Lung Disease With Multivolume Noncontrast MRI and Spirometry.
Growth, Body Composition, and Lung Function in Prepubertal Children with Cystic Fibrosis Diagnosed by Newborn Screening.
Trends in the Prevalence of Non-tuberculous Mycobacterial Infection in Patients with Non-Cystic Fibrosis Bronchiectasis in South Korea, 2012-2016.
Chronic rhinosinusitis and endoscopic sinus surgery in children admitted for pulmonary exacerbations of cystic fibrosis.
A Cationic Porphyrin, ZnPor, Disassembles Pseudomonas aeruginosa Biofilm Matrix, Kills Cells Directly, and Enhances Antibiotic Activity of Tobramycin.
Understanding Pseudomonas aeruginosa-Host Interactions: The Ongoing Quest for an Efficacious Vaccine.
Triage for Malnutrition Risk among Pediatric and Adolescent Outpatients with Cystic Fibrosis, Using a Disease-Specific Tool.
Essential Oils Biofilm Modulation Activity, Chemical and Machine Learning Analysis. Application on Staphylococcus aureus Isolates from Cystic Fibrosis Patients.
New Insights into the Binding Features of F508del CFTR Potentiators: A Molecular Docking, Pharmacophore Mapping and QSAR Analysis Approach.
Exogenous Nitric Oxide Improves Antibiotic Susceptibility in Resistant Bacteria.
Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251).
Comparative transcriptome analysis unveils the adaptative mechanisms of Scedosporium apiospermum to the microenvironment encountered in the lungs of patients with cystic fibrosis.
Monogenic Diabetes in a Child with Cystic Fibrosis: A Case Report and Review of the Literature.
Effectiveness of Hypertonic Saline Nebulization in Airway Clearance in Children with Non-Cystic Fibrosis Bronchiectasis: A randomized control trial.
Mental Health History and Social Barriers Impacting Caregivers of Infants with Cystic Fibrosis.
carP, encoding a Ca2+-regulated putative phytase, is evolutionarily conserved in Pseudomonas aeruginosa and has potential as a biomarker.
Clinical and molecular characterisation of the R751L-CFTR mutation.
Polyclonality, Shared Strains, and Convergent Evolution in Chronic CF S. aureus Airway Infection.
Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis.
Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19.
Development of a penem antibiotic against Mycobacteroides abscessus.
Evaluation of Rescue Oral Glucocorticoid Therapy during Inpatient Cystic Fibrosis Exacerbations.
Novel Odoribacter splanchnicus Strain and Its Outer Membrane Vesicles Exert Immunoregulatory Effects in vitro.
Novel therapeutic approaches for the management of cystic fibrosis.
Vasculitis in Cystic Fibrosis.
Steps Ahead: optimising physical activity and health in people with cystic fibrosis: Study Protocol for a pilot randomised trial.
Subacute cadmium exposure disrupts the hypothalamic-pituitary-gonadal axis, leading to polycystic ovarian syndrome and premature ovarian failure features in female rats.
A current review of the safety of cystic fibrosis transmembrane conductance regulator modulators.
siRNA delivery to macrophages using aspherical, nanostructured microparticles as delivery system for pulmonary administration.

The effect of self-efficacy, social support and quality of life on readiness for transition to adult care among adolescents with cystic fibrosis in Turkey.
ACT with CF: A telehealth and in-person feasibility study to address anxiety and depressive symptoms among people with cystic fibrosis.
Clinical evaluation of an evidence-based method based on food characteristics to adjust pancreatic enzyme supplements dose in cystic fibrosis.
CFTR plays an important role in the regulation of vascular resistance and high-fructose/salt-diet induced hypertension in mice.
Rescue from Pseudomonas aeruginosa airway infection via stem cell transplantation.
YKL-40 as a clinical biomarker in adult patients with CF: Implications of a CHI3L1 single nucleotide polymorphism in disease severity.
Can we identify cystic fibrosis (CF) from skeletal remains?: A proposed differential diagnosis.
G970R-CFTR Mutation (c.2908G>C) Results Predominantly in a Splicing Defect.
Therapeutic effect of nebulized hypertonic saline for muco-obstructive lung diseases: a systematic review and meta-analysis with trial sequential analysis.
Heme protects Pseudomonas aeruginosa and Staphylococcus aureus from calprotectin-induced iron starvation.
Lung Function Assessment by Impulse Oscillometry in Adults.
Should the dosage of propofol be higher and independent of immunosuppressive therapy in adult cystic fibrosis patients undergoing sedation during flexible video bronchoscopy?
Novel Antioxidant Therapy with the Immediate Precursor to Glutathione, γ-Glutamylcysteine (GGC), Ameliorates LPS-Induced Cellular Stress in In Vitro 3D-Differentiated Airway Model from Primary Cystic Fibrosis Human Bronchial Cells.
Early disease surveillance in young children with cystic fibrosis: A qualitative analysis of parent experiences.
Topographic heterogeneity of lung microbiota in end-stage idiopathic pulmonary fibrosis: the Microbiome in Lung Explants-2 (MiLEs-2) study.
Impaired renal HCO3 – secretion in CFTR deficient mice causes metabolic alkalosis during chronic base-loading.
Modelling experimentally measured of ciprofloxacin antibiotic diffusion in Pseudomonas aeruginosa biofilm formed in artificial sputum medium.
Extensive CFTR Gene Analysis Revealed a Higher Occurrence of Cystic Fibrosis Transmembrane Regulator-Related Disorders (CFTR-RD) among CF Carriers.
Ecological Succession of Polymicrobial Communities in the Cystic Fibrosis Airways.
Multicentre feasibility of multiple-breath washout in preschool children with cystic fibrosis and other lung diseases.
The Lung Microbiome of Three Young Brazilian Patients With Cystic Fibrosis Colonized by Fungi.
Immune defects in patients with pulmonary Mycobacterium abscessus disease without cystic fibrosis.
Non-invasive Ventilation for Children With Chronic Lung Disease.
Sleep-Disordered Breathing in Cystic Fibrosis.
COVID-19 in children with underlying chronic respiratory diseases: survey results from 174 centres.
Early COVID-19 infection after lung transplantation in a patient with cystic fibrosis.
Pseudomonas aeruginosa in the CF Airway: Does it Deserve its Reputation as a Predatory ‘Bully’?
Polysulfated Hyaluronan GM-1111 Inhibits Elastase and Improves Rheology in CF Sputum.
Heterogeneous expression of CFTR in insulin-secreting β-cells of the normal human islet.
Impact of Pseudomonas aeruginosa Infection on Patients with Chronic Inflammatory Airway Diseases.
Local and Systemic Alterations of the L-Arginine/Nitric Oxide Pathway in Sputum, Blood, and Urine of Pediatric Cystic Fibrosis Patients and Effects of Antibiotic Treatment.
Molecular Characterization of the Burkholderia cenocepacia dcw Operon and FtsZ Interactors as New Targets for Novel Antimicrobial Design.
Azithromycin potentiates avian IgY effect against Pseudomonas aeruginosa in a murine pulmonary infection model.
Right Ventricle Dysfunction in Patients With Adult Cystic Fibrosis Enlisted for Lung Transplant.
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).
IgA autoantibodies directed against self DNA are elevated in cystic fibrosis and associated with more severe lung dysfunction.
The Association of Area Deprivation and State Child Health with Respiratory Outcomes of Pediatric Patients with Cystic Fibrosis in the United States.
Stenotrophomonas maltophilia phenotypic and genotypic features through 4-year cystic fibrosis lung colonization.
The Effects of Ivacaftor on Bone Density and Microarchitecture in Children and Adults with Cystic Fibrosis.
PROBIOTICS FOR PEOPLE WITH CYSTIC FIBROSIS (REVIEW).
Repeated isolation of an antibiotic-dependent and temperature-sensitive mutant of Pseudomonas aeruginosa from a cystic fibrosis patient.
Extraction of the molecular level biomedical event trigger based on gene ontology using radial belief neural network techniques.
PEGylation of recombinant human deoxyribonuclease I deceases its transport across lung epithelial cells and uptake by macrophages.
A Scientific Rationale for Using Cystic Fibrosis Transmembrane Conductance Regulator Therapeutics in COVID-19 Patients.
Physiotherapy: At what cost? Parents experience of performing chest physiotherapy for infants with cystic fibrosis.
Development of elexacaftor – tezacaftor – ivacaftor: highly effective CFTR modulation for the majority of people with cystic fibrosis.
The cystic fibrosis gut as a potential source of multidrug resistant pathogens.
Prostaglandin E2 and phagocytosis of inhaled particulate matter by airway macrophages in cystic fibrosis.
Peptidorhamnomannans From Scedosporium and Lomentospora Species Display Microbicidal Activity Against Bacteria Commonly Present in Cystic Fibrosis Patients.
Gene therapy for sickle cell disease.
Trypsin Detection Strategies: A Review.
Effect of host-mimicking medium and biofilm growth on the ability of colistin to kill Pseudomonas aeruginosa.
Mining GWAS and eQTL data for CF lung disease modifiers by gene expression imputation.
Caring through distancing: Spatial boundaries and proximities in the cystic fibrosis clinic.
Lumacaftor/ivacaftor in people with cystic fibrosis with an A455E-CFTR mutation.
Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.
“Adenosine an old player with new possibilities in kidney diseases”: Preclinical evidences and clinical perspectives.
Effects of chlorothalonil, prochloraz and the combination on intestinal barrier function and glucolipid metabolism in the liver of mice.
Long-term azithromycin use is not associated with QT prolongation in children with cystic fibrosis.
Flow Volume Loop and Regional Ventilation Assessment Using Phase-Resolved Functional Lung (PREFUL) MRI: Comparison With 129 Xenon Ventilation MRI and Lung Function Testing.
Chlamydia pneumoniae and Mycoplasma pneumoniae in children with Cystic Fibrosis: impact on bacterial respiratory microbiota diversity.
Delayed age at menarche in chronic respiratory diseases.
Focus on gastroesophageal reflux disease in patients with cystic fibrosis.
The Changing Face of Cystic Fibrosis and Its Implications for Screening.
Why Do We Screen Newborn Infants for Cystic Fibrosis?
Newborn Screening for Cystic Fibrosis: Over the Hump, Still Need to Fine-Tune It.
Differential effects of cotreatment of the antibiotic rifampin with host-directed therapeutics in reducing intracellular Staphylococcus aureus infection.
Mycobacterium Simiae Infection in a Person With Cystic Fibrosis.
“Molecular Mechanisms for the Inflammation-Resolving Actions of Lenabasum”.
Pseudomonas Quinolone Signal-Induced Outer Membrane Vesicles Enhance Biofilm Dispersion in Pseudomonas aeruginosa.
Integrative genomic meta-analysis reveals novel molecular insights into cystic fibrosis and ΔF508-CFTR rescue.
Lung Clearance Index in Children with Sickle Cell Disease.
Aerosolized drug-loaded nanoparticles targeting migration inhibitory factors inhibit Pseudomonas aeruginosa-induced inflammation and biofilm formation.
Technology-Enabled Health Care Collaboration in Pediatric Chronic Illness: Pre-Post Interventional Study for Feasibility, Acceptability, and Clinical Impact of an Electronic Health Record-Linked Platform for Patient-Clinician Partnership.
Variations in Nutrition Practices in Cystic Fibrosis: A Survey of the DIGEST Program.
Subtle relationships between Pseudomonas aeruginosa and fungi in patients with cystic fibrosis.
Riociguat: clinical research and evolving role in therapy.
Emerging Alternatives to Conventional Clinic Visits in the Era of COVID-19: Adoption of Telehealth at VCU Adult Cystic Fibrosis Center.
Letter to the editor of JCF.
Allergic bronchopulmonary aspergillosis in children.
Physical Activity, Sedentary Time and Sleep in Cystic Fibrosis Youth: A Bi-Directional Relationship?
Phospholipase C controls chloride-dependent short-circuit current in human bronchial epithelial cells.
Inhibition of the GDP-d-Mannose Dehydrogenase from Pseudomonas aeruginosa Using Targeted Sugar Nucleotide Probes.
CRISPR-Cas9: A Preclinical and Clinical Perspective for the Treatment of Human Diseases.
Comparison Between Ultra-low-dose Computed Tomography and Lung MRI in Cystic Fibrosis
Proteomic Studies of the Biofilm Matrix including Outer Membrane Vesicles of Burkholderia multivorans C1576, a Strain of Clinical Importance for Cystic Fibrosis.
Pulmonary Manifestations of Genetic Disorders in Children.
[Bronchiectasis not related to cystic fibrosis in children: Guidelines for diagnosis, monitoring and treatment].
Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene.
Animal and Cell Culture Models for Cystic Fibrosis: Which Model is Right for Your Application?
Decoding (patho-)physiology of the lung by advanced in vitro models for developing novel anti-infectives therapies.
Effects of rehabilitation methods on lower-limb muscle function and functional performance in patients with cystic fibrosis: A systematic review.
Urban Life as Risk Factor for Aspergillosis.
Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with cystic fibrosis and meconium peritonitis in a female neonate 4 days of age – case report and review of the literature.
Re: The outcome of pregnancy in women with cystic fibrosis: a UK population-based descriptive study.
Role Of Long-Term Intermittent Use Of Oral Azithromycin On Pulmonary Exacerbations In Cystic Fibrosis Children.
Cystic Fibrosis with Non-G551D Gating Mutations in Italy: Epidemiology and clinical characteristics.
Draft Genome Sequence of Mycobacteroides sp. Strain LB1, Isolated from the Sputum of a Cystic Fibrosis Patient.
Evaluation of Inhaled Tobramycin in Early Eradication of Pseudomonas aeruginosa in Infants With Cystic Fibrosis.
Management of Cepacia Syndrome With a Combination of Intravenous and Inhaled Antimicrobials in a Non-Cystic Fibrosis Pediatric Patient.

Caroli’s Syndrome: An Early Presentation.
The pharmacokinetics of antibiotics in cystic fibrosis.
Recommendations to improve physical activity prescription for the cystic fibrosis population: an Irish perspective.
Small molecule drugs in cystic fibrosis.
Pseudomonas aeruginosa adaptation and evolution in patients with cystic fibrosis.
Prevalence of coccidioidomycosis in cystic fibrosis patients residing in Southern Arizona.
Effectiveness of Short versus Long Course Perioperative Antibiotics in Lung Transplant Recipients with Donor Positive Respiratory Cultures.
Antibacterial Effects of Bicarbonate in Media Modified to Mimic Cystic Fibrosis Sputum.
Defective immunometabolism pathways in cystic fibrosis macrophages.
Engineered mutant α-ENaC subunit mRNA delivered by lipid nanoparticles reduces amiloride currents in cystic fibrosis-based cell and mice models.
Coinfection with Pseudomonas aeruginosa and Aspergillus fumigatus in cystic fibrosis.
Repeat Dosing of AAV2.5T to Ferret Lungs Elicits an Antibody Response That Diminishes Transduction in an Age-Dependent Manner.
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Current Treatment Options for Cystic Fibrosis-Related Liver Disease.
Estimation of Proinflammatory Factors in the Saliva of Adult Patients with Cystic Fibrosis and Dental Caries.
NMR Profiling of Exhaled Breath Condensate Defines Different Metabolic Phenotypes of Non-Cystic Fibrosis Bronchiectasis.
Crystal Structure of Isoform CBd of the Basic Phospholipase A2 Subunit of Crotoxin: Description of the Structural Framework of CB for Interaction with Protein Targets.
Host-Pathogen Interactions after Lung Transplant: Are Cystic Fibrosis Patients Unique?
Age-Related Changes in the Canine Aorta.
Oxidative stress-induced inflammation in susceptible airways by anthropogenic aerosol.
Evaluation of the health-related quality of life in Turkish cystic fibrosis patients.
Non-Linear Pharmacokinetics of Oral Roscovitine (Seliciclib) in Cystic Fibrosis Patients Chronically Infected with Pseudomonas aeruginosa: A Study on Population Pharmacokinetics with Monte Carlo Simulations.
Rapidly progressive metastatic cholangiocarcinoma in a postpartum patient with cystic fibrosis: a case report.
Ultra-low-dose chest CT in adult patients with cystic fibrosis using a third-generation dual-source CT scanner.
ADIPS 2020 guideline for pre-existing diabetes and pregnancy.
[Evidence-based treatment of cystic fibrosis].
Vitamin D Deficiency Is Associated with Increased Nontuberculous Mycobacteria Risk in Cystic Fibrosis.
Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes.
How to determine the mechanism of action of CFTR modulator compounds: A gateway to theranostics.
Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis: A systematic review.
Electronic health nudges to improve reproductive health care for women with cystic fibrosis.
Evaluation of Pyocyanin induced systemic pathogenicity of Pseudomonas aeruginosa.
Utilisation, expenditure and cost-effectiveness of cystic fibrosis drugs in Ireland: a retrospective analysis of a national pharmacy claims database.
A SNARE protein Syntaxin 17 captures CFTR to potentiate autophagosomal clearance under stress.
Impact of a Gap Junction Protein Alpha 4 Variant on Clinical Disease Phenotype in F508del Homozygous Patients With Cystic Fibrosis.
Evaluation of quorum-sensing inhibitory effects of extracts of three traditional medicine plants with known antibacterial properties.
Synthesis of the Hydroxamate Siderophore N α-Methylcoprogen B in Scedosporium apiospermum Is Mediated by sidD Ortholog and Is Required for Virulence.
Current Approach in the Diagnosis and Management of Allergic Bronchopulmonary Aspergillosis in Children With Cystic Fibrosis.
The Open Challenge of in vitro Modeling Complex and Multi-Microbial Communities in Three-Dimensional Niches.
Dehydrated patient without clinically evident cause: A case report.
Cystic Fibrosis Diagnosed Using Indigenously Wrapped Sweating Technique: First Large-Scale Study Reporting Socio-Demographic, Clinical, and Laboratory Features among the Children in Bangladesh A Lower Middle Income Country.
Tackling Chronic Inflammation with Withanolide Phytochemicals-A Withaferin a Perspective.
First Wave of COVID-19 in French Patients with Cystic Fibrosis.
Evaluation of pancreatic functions in cases of primary and secondary malnutrition.
Telephone surveillance during 2019 novel coronavirus disease: Is it a helpful diagnostic tool for detecting acute pulmonary exacerbations in children with chronic lung disease?
Addressing nutritional failure in a cystic fibrosis patient using nutritional and psychological intervention.
The global impact of SARS-CoV-2 in 181 people with cystic fibrosis.
Transcriptomic analysis of CFTR-impaired endothelial cells reveals a pro-inflammatory phenotype.
Draft Genome Sequence of Pseudomonas aeruginosa Strain PA14-UM.
Distribution of Burkholderia cepacia complex species isolated from industrial processes and contaminated products in Argentina.
Molecular mechanisms of Cl- transport in fishes: New insights and their evolutionary context.
Transitioning from paediatric to adult services with cystic fibrosis or bronchiectasis: What is the impact on engagement and health outcomes?
Protease FRET Reporters Targeting Neutrophil Extracellular Traps.
An ex vivo cystic fibrosis model recapitulates key clinical aspects of chronic Staphylococcus aureus infection.
Airway epithelial stem cell chimerism in cystic fibrosis lung transplant recipients.
Osteoclastogenesis and sphingosine-1-phosphate secretion from human osteoclast precursor monocytes are modulated by the cystic fibrosis transmembrane conductance regulator.
Atypical Cystic Fibrosis: Diagnosis at the Age of 57 Years.
Multilevel Models of Therapeutic Response in the Lungs
Risk factors and clinical course of children with cystic fibrosis colonized with Staphylococcus aureus.
Insights for the Growth of Stenotrophomonas maltophilia in a Diabetic Patient with Long-Term Antibiotic Use: A Case Study.
Linaclotide, lubiprostone and tenapanor improve gut fluidity and alkalinity in CFTR-deficient and F508del mutant mice via NHE3 inhibition.
Impaired Ratio of Unsaturated to Saturated Non-Esterified Fatty Acids in Saliva from Patients with Cystic Fibrosis.
Bile Acid Signal Molecules Associate Temporally with Respiratory Inflammation and Microbiome Signatures in Clinically Stable Cystic Fibrosis Patients.
Factors influencing the acquisition and eradication of early Pseudomonas aeruginosa infection in cystic fibrosis.
Targeted Nanotherapeutics for Respiratory Diseases: Cancer, Fibrosis, and Coronavirus.
Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation.
Research advances in molecular mechanisms underlying the pathogenesis of cystic fibrosis: From technical improvement to clinical applications (Review).
Burkholderia gladioli infection in a pediatric patient with cystic fibrosis: the clinical challenges of an emergent pathogen.
P.F508del editing in cells from cystic fibrosis patients.
Mining Public Mass Spectrometry Data to Characterize the Diversity and Ubiquity of P. aeruginosa Specialized Metabolites.
Preclinical Studies of a Rare CF-Causing Mutation in the Second Nucleotide Binding Domain (c.3700A>G) Show Robust Functional Rescue in Primary Nasal Cultures by Novel CFTR Modulators.
Therapeutic potential for coxibs-nitric oxide releasing hybrids in cystic fibrosis.
Successful biodegradable stent insertion in an infant with severe bronchomalacia and cystic fibrosis.
The virtual CF clinic: Implications for sputum microbiology.
SHORT-TERM EFFECTS OF APPROPRIATE EMPIRICAL ANTIMICROBIAL TREATMENT WITH CEFTOLOZANE/TAZOBACTAM IN A SWINE MODEL OF NOSOCOMIAL PNEUMONIA.
Targeted deubiquitination rescues distinct trafficking-deficient ion channelopathies.
Longitudinal changes in exercise capacity among adult cystic fibrosis patients.
Overview of Atypical Diabetes.
The efficacy of Elexacaftor/Tezacaftor/Ivacaftor in patients with cystic fibrosis and advanced lung disease.
Burkholderia vietnamiensis causing a non-lactational breast abscess in a non-cystic fibrosis patient in Tamil Nadu, India.
Repeatability of the 6-min walk test in non-cystic fibrosis bronchiectasis.
Autologous fibrin sealant co-delivered with antibiotics is a robust method for topical antibiotic treatment after sinus surgery.
A systematic review of self-care measures for adolescents with health needs.
Fluoroquinolone resistance in Achromobacter spp.: substitutions in QRDRs of GyrA, GyrB, ParC and ParE and implication of the RND efflux system AxyEF-OprN.
Citrullination as a novel posttranslational modification of matrix metalloproteinases.
Photodynamic therapy to control microbial biofilms.
Sub-nanoliter metabolomics via mass spectrometry to characterize volume-limited samples.
“Summer hypokalemia” as an initial presentation of cystic fibrosis in a morbidly obese African American adult: case report.
Extracellular adenosine 5′-triphosphate in pulmonary disorders.
Early acquisition and conversion of Pseudomonas aeruginosa in Hispanic youth with cystic fibrosis in the United States.
Gaming Console Home-Based Exercise for Adults with Cystic Fibrosis: Study Protocol.
Hyperpolarized 129Xe MR Imaging of Lung Function in Healthy Volunteers and Subjects With Pulmonary Disease
DPI-Tobra-Kind Cyclops® in Children With Cystic Fibrosis
New Insights in Laboratory Testing for COVID-19 Patients: Looking for the Role and Predictive Value of Human epididymis secretory protein 4 (HE4) and the Innate Immunity of the Oral Cavity and Respiratory Tract.
Risk factors for respiratory Aspergillus fumigatus in German Cystic Fibrosis patients and impact on lung function.
Cell-based non-invasive prenatal diagnosis in a pregnancy at risk of cystic fibrosis.
Multi-stimuli controlled release of a transmembrane chloride ion carrier from a sulfonium-linked procarrier.
[Myelinosomes: A new pathway of protein quality control].
Lipoxin A4 promotes reduction and antibiotic efficacy against Pseudomonas aeruginosa biofilm.
In vitro activity of ceftolozane-tazobactam and ceftazidime-avibactam against Pseudomonas aeruginosa isolated from patients with cystic fibrosis.

Copper and Copper/Zinc Ratio in a Series of Cystic Fibrosis Patients.
Classical Drug Digitoxin Inhibits Influenza Cytokine Storm, With Implications for Covid-19 Therapy.
Molecular Docking and QSAR Studies as Computational Tools Exploring the Rescue Ability of F508del CFTR Correctors.
NGS-based expanded carrier screening for genetic disorders in North Indian population reveals unexpected results – a pilot study.
LC-MS detection of antibiotic agents in sputum from persons with cystic fibrosis.
Acid ceramidase rescues cystic fibrosis mice from pulmonary infections.
Sputum microbiota in adults with CF associates with response to inhaled tobramycin.
Machine learning evaluates improvement in sinus computed tomography opacification with CFTR modulator therapy.
Chest computed tomography outcomes in a randomized clinical trial in cystic fibrosis: Lessons learned from the first ataluren phase 3 study.
CF Bronchodilation
Effects of AD on VI in Patients With CF
Organoid based personalized medicine: from bench to bedside.
Factors influencing medical practitioner participation in population carrier screening for cystic fibrosis.
Cystic Fibrosis Lung Function Decline after Within-Host Evolution Increases Virulence of Infecting Pseudomonas Aeruginosa.
New strategies of physical activity assessment in cystic fibrosis: a pilot study.
Treatment of human airway epithelial Calu-3 cells with a peptide-nucleic acid (PNA) targeting the microRNA miR-101-3p is associated with increased expression of the cystic fibrosis Transmembrane Conductance Regulator () gene.
Cystic fibrosis: physiopathology and the latest pharmacological treatments.
Use of dornase alfa in the paediatric intensive care unit: current literature and a national cross-sectional survey.
Upregulation of CFTR Protects against Palmitate-Induced Endothelial Dysfunction by Enhancing Autophagic Flux.
Modulated Response of Aspergillus fumigatus and Stenotrophomonas maltophilia to Antimicrobial Agents in Polymicrobial Biofilm.
Stem Cells, Cell Therapies, and Bioengineering in Lung Biology and Disease 2019.
Inhaled dry powder alginate oligosaccharide in cystic fibrosis: a randomised, double-blind, placebo-controlled, crossover phase 2b study.
The Changing Face of Cystic Fibrosis and Its Implications for Screening.
Why Do We Screen Newborn Infants for Cystic Fibrosis?
Evaluation of Autonomic Modulation of Lung Function and Heart Rate in Children with Cystic Fibrosis.
Effects of music therapy as an adjunct to chest physiotherapy in children with cystic fibrosis: A randomized controlled trial.
Mitigating the Impact of Socioeconomic Status on Listing for Lung Transplantation in Cystic Fibrosis.
STeP IT UP CF: STimulating ImProved Health And Well-being In CysTic Fibrosis
New Antibiotics for the Treatment of Acute Bacterial Skin and Soft Tissue Infections in Pediatrics.
Pulmonary Delivery of Biological Drugs.
Potential Contributions of Anaerobes in Cystic Fibrosis Airways.
Efficacy and Safety of the CFTR Potentiator Icenticaftor (QBW251) in COPD: Results from a Phase 2 Randomized Trial.
Pseudomonas aeruginosa Type III Secretion System Virulotypes and Their Association with Clinical Features of Cystic Fibrosis Patients.
Liposomes Loaded With Phosphatidylinositol 5-Phosphate Improve the Antimicrobial Response to Pseudomonas aeruginosa in Impaired Macrophages From Cystic Fibrosis Patients and Limit Airway Inflammatory Response.
Ocular Surface Potential Difference Measured in Human Subjects to Study Ocular Surface Ion Transport.
Primary lung carcinoma in cystic fibrosis: A case report and literature review.
Pneumocystis jirovecii in Patients With Cystic Fibrosis: A Review.
The Clinicopathological and Imaging Characteristics of Primary Hepatic Carcinosarcoma and a Review of the Literature.
Newborn Screening for Cystic Fibrosis: Over the Hump, Still Need to Fine-Tune It.
EX18_21 large duplication: A novel pathogenic mutation of the CFTR gene for cystic fibrosis.
Pseudoxanthomonas winnipegensis sp. nov., derived from human clinical materials and recovered from cystic fibrosis and other patient types in Canada, and emendation of Pseudoxanthomonas spadix Young et al. 2007.
Oxygen transport and utilisation during exercise in cystic fibrosis: Contributors to exercise intolerance.
Are patients with lung cystic fibrosis at increased risk of severe and fatal COVID-19? Interleukin 6 as a predictor of COVID-19 outcomes.
Are patients with lung cystic fibrosis at increased risk of severe and fatal COVID-19? Interleukin 6 as a predictor of COVID-19 outcomes. Authors’ reply.
Treating systemic issues in families affected by cystic fibrosis: A solution-focused approach.
Streamlined Treatment of Pulmonary Exacerbations in Pediatrics (Pilot)
Use of medical marijuana in cystic fibrosis patients.
Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids.
Feasibility study for supporting medication adherence for adults with cystic fibrosis: mixed-methods process evaluation.
Gene Loss and Acquisition in Lineages of Pseudomonas aeruginosa Evolving in Cystic Fibrosis Patient Airways.
The first five-year evaluation of cystic fibrosis neonatal screening program in São Paulo State, Brazil.
Clinical impact of respiratory virus in pulmonary exacerbations of children with Cystic Fibrosis.
Getting Near to “Closing the gap” in the Pediatric Age-group for First Personalized Treatment of Cystic Fibrosis.
Strategies for cystic fibrosis transmembrane conductance regulator inhibition: from molecular mechanisms to treatment for secretory diarrhoeas.
Implementation of a Non-invasive Version of the Imaging β-adrenergic-dependent Sweat Secretion Test
New Markers of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function in Sweat
Efficacy of a New Sweat Collection Support: Impact on Neonatal Screening and Early Treatment of Cystic Fibrosis
Response of Pseudomonas aeruginosa to the innate immune system-derived oxidants hypochlorous acid and hypothiocyanous acid.
Mutant CFTR Drives TWIST1 mediated epithelial-mesenchymal transition.
How precision medicine paved the way to the first cystic fibrosis drug.
A Giant Thymic Cyst Accompanied by Acute Mediastinitis.
Learning to breathe with Tai Chi online – qualitative data from a randomized controlled feasibility study of patients with cystic fibrosis.
Sickle cell trait newborn screen results: disclosure and management.
Epidemiology, diagnosis & treatment of non-tuberculous mycobacterial diseases.
Nutrition and Markers of Disease Severity in Patients With Bronchiectasis.
Airway Mucins Inhibit Oxidative and Non-Oxidative Bacterial Killing by Human Neutrophils.
Mitochondrial Stress Responses and “Mito-Inflammation” in Cystic Fibrosis.
Temperate Bacteriophages (Prophages) in Pseudomonas aeruginosa Isolates Belonging to the International Cystic Fibrosis Clone (CC274).
Comparative Evolutionary Patterns of Burkholderia cenocepacia and B. multivorans During Chronic Co-infection of a Cystic Fibrosis Patient Lung.
Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis.
Implementing a tracking system for confirmatory diagnostic results after positive newborn screening for cystic fibrosis-implications for process quality and patient care.
Exercise Intolerance in Cystic Fibrosis: Importance of Skeletal Muscle.
Derivation of Airway Basal Stem Cells from Human Pluripotent Stem Cells.
The NHS virtual gym boosting fitness and social connections for cystic fibrosis patients.
Identification of AAV serotypes for lung gene therapy in human embryonic stem cell-derived lung organoids.
High-level antibiotic tolerance of a clinically-isolated Enterococcus faecalis strain.
Phenotypic and transcriptomic analysis of seven clinical Stenotrophomonas maltophilia isolates identify a small set of shared and commonly regulated genes involved in biofilm lifestyle.
The odorant receptor OR2W3 on airway smooth muscle evokes bronchodilation via a cooperative chemosensory tradeoff between TMEM16A and CFTR.
Managing Cystic Fibrosis in Polish Healthcare.
Pseudomonas aeruginosa isolates from cystic fibrosis patients induce neutrophil extracellular traps with different morphologies that could correlate with their disease severity.
Complete Genome Sequence of Burkholderia cenocepacia K56-2, an Opportunistic Pathogen.
Author Correction: Allele specific repair of splicing mutations in cystic fibrosis through AsCas12a genome editing.
1-BENZYLSPIRO[PIPERIDINE-4,1′-PYRIDO[3,4-b]indole] ‘co-potentiators’ for minimal function CFTR mutants.
Interdisciplinary Approach for the Medical Management of Gestational Gigantomastia.
Omics-based tracking of Pseudomonas aeruginosa persistence in “eradicated” CF patients.
Extended Screening for Cystic Fibrosis-related Liver Disease Including Elastography in Children and Adolescents.
Carbapenems drive the collateral resistance to ceftaroline in cystic fibrosis patients with MRSA.
Analysis of rearrangements of the CFTR gene in patients from Turkey with CFTR-related disorders: frequent exon 2 deletion.
TACKLING ANTIMICROBIAL RESISTANCE (AMR) – IN VITRO EFFECT OF SODIUM CHLORIDE ON ANTIBIOTIC SUSCEPTIBILITY IN CLINICAL PSEUDOMONAS AERUGINOSA ISOLATED FROM PATIENTS WITH CYSTIC FIBROSIS (CF).
In vitro assessment of CSA-131 and CSA-131 poloxamer form for the treatment of Stenotrophomonas maltophilia infections in cystic fibrosis.
Ivacaftor in People With Cystic Fibrosis and a 3849+10kb C →T or D1152H Residual Function Mutation.
Comparison between two newborn screening strategies for cystic fibrosis in Argentina: IRT/IRT vs. IRT/PAP.
miRNA repertoires of cystic fibrosis ex vivo models highlight miR-181a and miR-101 that regulate WISP1 expression.
Prevalence of CBAVD in azoospermic men carrying pathogenic CFTR mutations – evaluated in a cohort of 639 non-vasectomized azoospermic men.
Laparoscopic bile duct resection with lymph node dissection for gallbladder cancer diagnosed after laparoscopic cholecystectomy.
The German National Registry of Primary Immunodeficiencies (2012-2017).
Lipidome Alterations Induced by Cystic Fibrosis, CFTR Mutation, and Lung Function.
LocusFocus: Web-based colocalization for the annotation and functional follow-up of GWAS.
Biodistribution and elimination pathways of PEGylated recombinant human deoxyribonuclease I after pulmonary delivery in mice.
Ph 1/2 Study Evaluating Safety and Tolerability of Inhaled AP-PA02 in Subjects With Chronic Pseudomonas Aeruginosa Lung Infections and Cystic Fibrosis

A Study to Assess the Effect of ELX/TEZ/IVA on Glucose Tolerance in Participants With Cystic Fibrosis (CF)
Which score is better in association with exercise capacity and health status in non-cystic fibrosis bronchiectasis patients?
The evolution of virulence in Pseudomonas aeruginosa during chronic wound infection.
Protein kinase CK2 and ion channels (Review).
Evaluation of both exonic and intronic variants for effects on RNA splicing allows for accurate assessment of the effectiveness of precision therapies.
Glucocorticoids and serum- and glucocorticoid-inducible kinase 1 are potent regulators of CFTR in the native intestine: implications for stress-induced diarrhea.
Role of unfolded proteins in lung disease.
Long-term use of tube feeding in children with cystic fibrosis: results from two Belgian CF centers.
Utility of Mid-Upper Arm Circumference in Diagnosing Malnutrition in Children With Cystic Fibrosis.
Health and social effects of the COVID-19 pandemic on patients with cystic fibrosis.
Therapeutic Inhaled Sphingosine for Treating Lung Infection in a Mouse Model of Critical Illness.
Thymosin alpha 1 exerts beneficial extrapulmonary effects in cystic fibrosis.
Lipolytic enzymes inhibitors: A new way for antibacterial drugs discovery.
Does synbiotic supplementation affect the quality of life in children with cystic fibrosis? A pilot randomized controlled clinical trial.
Initial Evaluation of Prospective and Parallel Assessments of Cystic Fibrosis Newborn Screening Protocols in Eastern Andalusia: IRT/IRT versus IRT/PAP/IRT.
Newborn Screening: Current Status in Alberta, Canada.
History of Newborn Screening for Cystic Fibrosis-The Early Years.
Newborn Screening for CF across the Globe-Where Is It Worthwhile?
Inconclusive Diagnosis after Newborn Screening for Cystic Fibrosis.
The Role of Extended CFTR Gene Sequencing in Newborn Screening for Cystic Fibrosis.
Processing Newborn Bloodspot Screening Results for CF.
Psychological Impact of NBS for CF.
Pancreatitis-Associated Protein in Neonatal Screening for Cystic Fibrosis: Strengths and Weaknesses.
Newborn Screening for Cystic Fibrosis in Russia: A Catalyst for Improved Care.
Constructing a Bioethical Framework to Evaluate and Optimise Newborn Bloodspot Screening for Cystic Fibrosis.
Performance of a Three-Tier (IRT-DNA-IRT) Cystic Fibrosis Screening Algorithm in British Columbia.
It All Depends What You Count-The Importance of Definitions in Evaluation of CF Screening Performance.
Combination of Selective PARP3 and PARP16 Inhibitory Analogues of Latonduine A Corrects F508del-CFTR Trafficking.
The Balance between the Safety of Mother, Fetus, and Newborn Undergoing Cystic Fibrosis Transmembrane Conductance Regulator Treatments during Pregnancy.
Multiple Reaction Monitoring Mass Spectrometry for the Drug Monitoring of Ivacaftor, Tezacaftor, and Elexacaftor Treatment Response in Cystic Fibrosis: A High-Throughput Method.
Drug treatments for managing cystic fibrosis-related diabetes.
Characterization of Novel Lytic Bacteriophages of Achromobacter marplantensis Isolated from a Pneumonia Patient.
Serum-Based Proteomics Profiling in Adult Patients with Cystic Fibrosis.
Use of monoclonal antibodies for allergic bronchopulmonary aspergillosis in patients with asthma and cystic fibrosis: literature review.
Material Properties of Interfacial Films of Mucoid and Nonmucoid Pseudomonas aeruginosa Isolates.
Cystic Fibrosis: A Pocket Guide.
The Small RNA ErsA Plays a Role in the Regulatory Network of Pseudomonas aeruginosa Pathogenicity in Airway Infections.
COMPLEX ORPHAN PATHOLOGY: COMORBIDITY OF MUCOVISCIDOSIS AND CONGENITAL DYSFUNCTION OF ADRENAL GLANDS CORTEX (REFERENCES REVIEW AND OWN RESEARCH).
Biological applications of synthetic anion transporters.
Multiple breath washout quality control in the clinical setting.
[Probiotics: benefits and harms].
Nutritional Risk Index: A Predictive Metric for Mortality Following Lung Transplant.
Ambient air pollution and respiratory bacterial infections, a troubling association: epidemiology, underlying mechanisms, and future challenges.
Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry.
Early diagnosis effects the prognosis in children with atypical wheeze.
The Unique Challenges and Lessons Imparted by the Cystic Fibrosis Community in the Time of COVID-19 Pandemic.
Cystic fibrosis transmembrane conductance regulator (CFTR) regulates embryonic organizer formation during zebrafish early embryogenesis.
Patient and Family Participation in Clinical Guidelines Development: The Cystic Fibrosis Foundation Experience.
Modification of the Pseudomonas aeruginosa toxin 2-heptyl-1-hydroxyquinolin-4(1H)-one and other secondary metabolites by methyltransferases from mycobacteria.
Clinical evaluation of three chromogenic media for the isolation of Staphylococcus aureus in respiratory samples in patients with cystic fibrosis.
Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study.
Prevalence and Economic Burden of Respiratory Diseases in Central Asia and Russia: A Systematic Review.
Influence of Cystic Fibrosis-Related Diabetes on Mental Health in Adults: A Single-Center Study.
Communicating cystic fibrosis newborn screening results to parents.
Exploring the reproductive decision support needs and preferences of women with cystic fibrosis.
Impact of novel CFTR modulator on sinonasal quality of life in adult patients with cystic fibrosis.
Cystic fibrosis: On the cusp of cure?
A Phase 3 Randomized, Double-Blind Placebo-Controlled Trial to Evaluate the Efficacy and Safety of Remdesivir (GS-5734™) Treatment of COVID-19 in an Outpatient Setting
Decreased mRNA and protein stability of W1282X limits response to modulator therapy.
Taskforce recommends coordinated effort to improve clinical research conduct and find highly effective CFTR-directed treatment for rare mutations.
Theranostics by testing CFTR modulators in patient-derived materials: The current status and a proposal for subjects with rare CFTR mutations.
Important steps in the journey to highly effective CFTR modulator access for people with CF.
Transcript from Stuart Elborn’s address at the ECFC award in June 2019.
Six-Minute Walk Test: Clinical Role, Technique, Coding, and Reimbursement.
CRISPR/Cas9 genome editing tool: A Promising Tool for Therapeutic Applications on respiratory diseases.
[Neonatal cystic fibrosis screening: Analysis and differences in immunoreactive trypsin levels in newborns with a positive screen].
Extended-criteria uncontrolled DCD donor for a fragile recipient: A case report about a challenging yet successful lung transplantation.
A chronic strain of the cystic fibrosis pathogen Pandoraea pulmonicola expresses a heterogenous hypo-acylated lipid A.
Mucin secretion in cystic fibrosis – a systematic review.
Real World Experience of Pseudomonas aeruginosa Eradication at an Urban Pediatric Cystic Fibrosis Center.
A Novel Mouse Model of Aminoglycoside-Induced Hyperacusis and Tinnitus.
The current state of immunization against Gram-negative bacteria in children: a review of the literature.
Effect of apical chloride concentration on the measurement of responses to CFTR modulation in airway epithelia cultured from nasal brushings.
State of the Art in Exocrine Pancreatic Insufficiency.
Histological changes in odontogenic parakeratinized keratocysts treated with marsupialization followed by enucleation.
Glued in lipids: Lipointoxication in cystic fibrosis.
Cost-Effectiveness of Ivacaftor Therapy for Treatment of Cystic Fibrosis Patients With the G551D Gating Mutation.
Protocol for Project Fizzyo, an analytic longitudinal observational cohort study of physiotherapy for children and young people with cystic fibrosis, with interrupted time-series design.
The Antibacterial and Anti-biofilm Activity of Metal Complexes Incorporating 3,6,9-Trioxaundecanedioate and 1,10-Phenanthroline Ligands in Clinical Isolates of Pseudomonas Aeruginosa from Irish Cystic Fibrosis Patients.
Prospective longitudinal association between repeated multiple breath washout measurements and computed tomography scores in children with cystic fibrosis.
Focusing on the penultimate step: increasing early lung transplant discussion in cystic fibrosis clinic to prepare patients for referral.
Pseudomonas aeruginosa Volatilome Characteristics and Adaptations in Chronic Cystic Fibrosis Lung Infections.
The Association of Grip Strength, Body Mass Index, and Lung Function in Youth with Cystic Fibrosis.
Toll like Receptor signalling by Prevotella histicola activates alternative NF-κB signalling in Cystic Fibrosis bronchial epithelial cells compared to P. aeruginosa.
Lung Clearance Index to Track Acute Respiratory Events in School-age Children with Cystic Fibrosis.
The Enemy of my Enemy: Bacterial Competition in the Cystic Fibrosis Lung.
FREQMAX provides an alternative approach for determining high-resolution allele frequency thresholds in carrier screening.
Oral Glucose Tolerance Testing Using Candy for Cystic Fibrosis
Testing Drug Efficacy in Cystic Fibrosis Through N-of-1 Trials
Ivacaftor in Infants Aged 4 to <12 Months With Cystic Fibrosis and a Gating Mutation: Results of a 2-Part Phase 3 Clinical Trial.
Does cystic fibrosis constitute an advantage in COVID-19 infection?
Candida thrombophlebitis in children: a systematic review of the literature.
Tobacco smoke exposure limits the therapeutic benefit of tezacaftor/ivacaftor in pediatric patients with cystic fibrosis.
A Cohort Study of Sleep Quality in Adult Patients with Acute Pulmonary Exacerbations of Cystic Fibrosis.
Laparoscopic surgery in patients with cystic fibrosis: A systematic review.
Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial.
Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive CFTR genetics and sweat test.
Micro-rheological properties of lung homogenates correlate with infection severity in a mouse model of Pseudomonas aeruginosa lung infection.
The 34th Annual North American Cystic Fibrosis Conference, October 7-23, 2020.
[Therapy of cystic fibrosis – new drugs give hope].

THE PHOSPHODIESTERASE INHIBITOR ENSIFENTRINE REDUCES PRODUCTION OF PRO-INFLAMMATORY MEDIATORS IN WELL-DIFFERENTIATED BRONCHIAL EPITHELIAL CELLS BY INHIBITING PDE4.
CFTR Modulator Therapy Enhances Peripheral Blood Monocyte Contributions to Immune Responses in People With Cystic Fibrosis.
Multi-Omics Approaches: The Key to Improving Respiratory Health in People With Cystic Fibrosis?
Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis.
The Probable, Possible, and Novel Functions of ERp29.
High Occurrence of Bacterial Competition Among Clinically Documented Opportunistic Pathogens Including Achromobacter xylosoxidans in Cystic Fibrosis.
Functional Profiling of CFTR-Directed Therapeutics Using Pediatric Patient-Derived Nasal Epithelial Cell Models.
Identification of Compounds That Promote Readthrough of Premature Termination Codons in the CFTR.
Are patients with lung cystic fibrosis at increased risk for severe and fatal COVID-19? Interleukin-6 as a predictor of COVID-19 outcome.
Live imaging and quantitative analysis of Aspergillus fumigatus growth and morphology during inter-microbial interaction with Pseudomonas aeruginosa.
Combined Single Lung and Liver Transplantation in a Cystic Fibrosis Patient With Previous Contralateral Pneumonectomy: A Case Report.
Safety and efficacy of lenabasum in a phase 2 randomized, placebo-controlled trial in adults with cystic fibrosis.
Cystic Fibrosis in the PICU-More of a Zebra Than a Horse.
Prolonged infusion of beta-lactam antibiotics for Gram-negative infections: rationale and evidence base.
Are cystic fibrosis mutation carriers a potentially highly vulnerable group to COVID-19?
Impulse oscillometry and spirometry measurements relative to personal best values at the time of acute exacerbations of cystic fibrosis in adults.
Inhibitory activities of curzerenone, curdione, furanodienone, curcumol and germacrone on Ca2+-activated chloride channels.
Sit-to-stand test in children with bronchiectasis: Does it measure functional exercise capacity?
A case of reversible toxic optic neuropathy from tacrolimus (FK506).
Use of Hyaluronic Acid (HA) in Chronic Airway Diseases.
Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.
A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis.
Diagnostic Value of Sputum Cultures in Children under 2 Years of Age with Chronic Suppurative Lung Diseases.
Alleviation of depression-like behavior in a cystic fibrosis mouse model by Hdac6 depletion.
Intravenous versus oral antibiotics for eradication of Pseudomonas aeruginosa in cystic fibrosis (TORPEDO-CF): a randomised controlled trial.
SELF-BREATHE RCT for Chronic Breathlessness
Antimicrobial Peptide Exposure Selects for Resistant and Fit Stenotrophomonas maltophilia Mutants That Show Cross-Resistance to Antibiotics.
Mutations of the cystic fibrosis transmembrane conductance regulator gene in males with congenital bilateral absence of the vas deferens: Reproductive implications and genetic counseling (Review).
Membrane phospholipid composition of Pseudomonas aeruginosa grown in a cystic fibrosis mucus-mimicking medium.
Nebulizer Care and Inhalation Technique in Children with Cystic Fibrosis.
The Potential Role and Regulatory Mechanisms of MUC5AC in Chronic Obstructive Pulmonary Disease.
Clinical Presentation of the c.3844T>C (p.Trp1282Arg, W1282R) Variant in Russian Cystic Fibrosis Patients.
High-Intensity Interval Training Is Effective at Increasing Exercise Endurance Capacity and Is Well Tolerated by Adults with Cystic Fibrosis.
Sex Steroids Induce Membrane Stress Responses and Virulence Properties in Pseudomonas aeruginosa.
The first report on immunoglobulins A, E, G and M levels in cystic fibrosis patients in Saudi Arabia.
Immune transcriptomes of highly exposed SARS-CoV-2 asymptomatic seropositive versus seronegative individuals from the Ischgl community.
Stakeholder feedback informs investigations for chronic rhinosinusitis in cystic fibrosis.
Prophylactic anti-staphylococcal antibiotics for cystic fibrosis.
Factors Contributing to Vitamin D Status at Hospital Admission for Pulmonary Exacerbation in Adults With Cystic Fibrosis.
WNT-β-catenin signalling – a versatile player in kidney injury and repair.
Skin-Interfaced Microfluidic Systems that Combine Hard and Soft Materials for Demanding Applications in Sweat Capture and Analysis.
A 96-well format microvascularized human lung-on-a-chip platform for microphysiological modeling of fibrotic diseases.
Scedosporium Cell Wall: From Carbohydrate-Containing Structures to Host-Pathogen Interactions.
Bronchiectasis with secondary pulmonary infection in a child: A case report.
Abnormal glucose tolerance in a pediatric cystic fibrosis cohort: Trends in clinical outcomes and associated factors in the preceding years.
Neutrophil elastase promotes macrophage cell adhesion and cytokine production through the integrin-Src kinases pathway.
The Role of Specialized Pro-Resolving Mediators in Cystic Fibrosis Airways Disease.
Combined Bacteriophage and Antibiotic Treatment Prevents Pseudomonas aeruginosa Infection of Wild Type and cftr- Epithelial Cells.
Predicting Antibiotic-Associated Virulence of Pseudomonas aeruginosa Using an ex vivo Lung Biofilm Model.
Mycobacterium abscessus Infection after Breast Lipotransfer: A Report of 2 Cases.
A TLR2-Activating Fraction From Mycobacterium abscessus Rough Variant Demonstrates Vaccine and Diagnostic Potential.
Physical activity for cystic fibrosis: perceptions of people with cystic fibrosis, parents and healthcare professionals.
Antibacterial and antibiofilm activities of ceragenins against Achromobacter species isolated from cystic fibrosis patients.
Epithelial Barrier Properties of the 16HBE14o- Human Bronchial Epithelial Cell Culture Model.
Effect of highly effective modulator therapy on quality of life in adults with cystic fibrosis.
Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-deficient piglets.
Delayed Presentation of Meconium Ileus in an Infant With Cystic Fibrosis.
The different anion transport capability of prodiginine- and tambjamine-like molecules.
The Pseudomonas aeruginosa protease LasB directly activates IL-1β.
A Data-Driven Approach to Carrier Screening for Common Recessive Diseases.
Using next-generation sequencing of microRNAs to identify host and/or pathogen nucleic acid signatures in samples from children with biliary atresia – a pilot study.
Tracking the genome of four Pseudomonas aeruginosa isolates that have a defective Las quorum-sensing system, but are still virulent.
The first cases of genetically confirmed congenital diarrhea with chloride loss in Slovakia.
Positive orientation and posttraumatic growth in mothers of children with cystic fibrosis – mediating role of coping strategies.
Rheological analysis of sputum from patients with chronic bronchial diseases.
Consequences of CRISPR-Cas9-Mediated CFTR Knockout in Human Macrophages.
Fostering Innovation in the Treatment of Chronic Polymicrobial Cystic Fibrosis-Associated Infections Exploring Aspartic Acid and Succinic Acid as Ciprofloxacin Adjuvants.
[Management of Primary Ciliary Dyskinesia].
Phytomedicines (medicines derived from plants) for sickle cell disease.
Personalized Theratyping Trial
Choice of Differentiation Media Significantly Impacts Cell Lineage and Response to CFTR Modulators in Fully Differentiated Primary Cultures of Cystic Fibrosis Human Airway Epithelial Cells.
A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous for F508del or heterozygous for the F508del-CFTR mutation and a residual function mutation.
[Lungtransplantation in Sweden – over 1 200 patients transplanted since 1990].
Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for ≥24 Weeks in People With CF and ≥1 F508del Allele: Interim Results of an Open-Label Phase Three Clinical Trial.
Iron metabolism in Pseudomonas aeruginosa biofilm and the involved iron-targeted anti-biofilm strategies.
Oral antibiotic prescribing patterns for treatment of pulmonary exacerbations in two large pediatric CF centers.
Cystic Fibrosis Year in Review 2019: Section 2 Pulmonary disease and infections.
Colistin dry powder inhalation with the Twincer™: An effective and more patient friendly alternative to nebulization.
Discovery of novel VX-809 hybrid derivatives as F508del-CFTR correctors by molecular modeling, chemical synthesis and biological assays.
Mucosal humoral immunity in cystic fibrosis – a tangled web of failed proteostasis, infection and adaptive immunity.
Capsaicin-Loaded Chitosan Nanocapsules for wtCFTR-mRNA Delivery to a Cystic Fibrosis Cell Line.
Aquagenic keratoderma and cystic fibrosis screening.
Predictors of massive haemoptysis after a first episode of mild-to-moderate haemoptysis in patients with cystic fibrosis.
Experiences of family members of children with cystic fibrosis under the light of Callista Roy.
The Lung Life of a Cystic Fibrosis Patient: A Patient and Physician Perspective.
Long-Term Ivacaftor in People Aged 6 Years and Older with Cystic Fibrosis with Ivacaftor-Responsive Mutations.
Airway microbiota is associated with the severity of non-CF bronchiectasis.
Pulmonary exacerbations in adults with cystic fibrosis – a grown-up issue in a changing CF landscape.
Of onions and men: Report of cavitary community acquired pneumonia due to Burkholderia cepacia complex in an immunocompetent patient and review of the literature.
Oral Glutathione and Growth in Cystic Fibrosis: A Multi-Center, Randomized, Placebo-Controlled, Double-Blind Trial.
Rates of depression and anxiety in Italian patients with cystic fibrosis and parent caregivers: Implementation of the Mental Health Guidelines.
Does newborn screening improve early lung function in cystic fibrosis?
Infant spirometry as a predictor of lung function at early childhood in cystic fibrosis patients.
The coughing body: etiquettes, techniques, sonographies and spaces.
In silico prediction and validation of potential therapeutic genes in pancreatic β-cells associated with type 2 diabetes.
Review: Pathogenesis of cholestatic liver diseases.
Characterization of the genomically encoded fosfomycin resistance enzyme from Mycobacterium abscessus.
Cystic Fibrosis and COVID-19: Care Considerations.
Isolation and first draft genome sequence of a linezolid-dependent Staphylococcus aureus clinical strain.
Hypoglycaemia in cystic fibrosis during an extended oral glucose tolerance test.

Reliability of impulse oscillometry parameters in healthy children and in children with cystic fibrosis.
Physical Function Measured Prior to Lung Transplantation Is Associated With Posttransplant Patient Outcomes.
Trends in nontuberculous mycobacteria infection in children and young people with cystic fibrosis.
Low rates of macrolide-resistant Mycobacterium avium complex in cystic fibrosis despite chronic azithromycin therapy.
Targeting UBC9-mediated protein hyper-SUMOylation in cystic cholangiocytes halts polycystic liver disease in experimental models.
LncRNA and transcriptomic analysis of fetal membrane reveal potential targets involved in oligohydramnios.
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Effect of Endoscope Sinus Surgery on Pulmonary Function in Cystic Fibrosis Patients: A Meta-Analysis.
Tissue Doppler and speckle tracking echocardiography assessment of left ventricular function in children with cystic fibrosis.
Sputum versus nasopharyngeal samples for the molecular diagnosis of respiratory viral infection in cystic fibrosis: A pilot study.
Global reprogramming of virulence and antibiotic resistance in Pseudomonas aeruginosa by a single nucleotide polymorphism in elongation factor, fusA1.
Differential response to biologics in a patient with severe asthma and ABPA: a role for dupilumab?
Stability of serum precipitins to Aspergillus fumigatus for the diagnosis of allergic bronchopulmonary aspergillosis.
Scoring tools to monitor risk of disease progression in patients with cystic fibrosis.
Analysis of airway microbiota in adults from a Brazilian cystic fibrosis center.
Breathing Easier: Molecular Therapeutics for Cystic Fibrosis.
Optimization of anti-microbials in the treatment of Cystic Fibrosis Pulmonary Exacerbations:  II. Therapies for Allergic Bronchopulmonary Aspergillosis (ABPA).
Identification, Structure-Activity Relationship, and Biological Characterization of 2,3,4,5-Tetrahydro-1H-pyrido[4,3-b]indoles as a Novel Class of CFTR Potentiators.
FOXL1 Regulates Lung Fibroblast Function via Multiple Mechanisms.
Epidemiology of Burkholderia Infections in People with Cystic Fibrosis in Canada between 2000 – 2017.
Dissecting the antibacterial activity of oxadiazolone-core derivatives against Mycobacterium abscessus.
Protease inhibitors elicit anti-inflammatory effects in CF mice with Pseudomonas aeruginosa acute lung infection.
Challenging barriers to an option for improved provision of enteral nutrition.
A B cell-dependent pathway drives chronic lung allograft rejection after ischemia-reperfusion injury in mice.
Impact of KLF4 on Cell Proliferation and Epithelial Differentiation in the Context of Cystic Fibrosis.
Promise and Potential Peril with Lumacaftor for the Trafficking Defective Type 2 Long QT Syndrome-Causative Variants, p.G604S, p.N633S, and p.R685P, Using Patient-Specific Re-Engineered Cardiomyocytes.
Drug Development for Cystic Fibrosis.
Changing landscape: psychological care in the era of cystic fibrosis transmembrane conductance regulator modulators.
Sexual and reproductive health in cystic fibrosis.
The changing landscape of cystic fibrosis: new therapies, challenges and a global pandemic.
Correction: Improved residual fat malabsorption and growth in children with cystic fibrosis treated with a novel oral structured lipid supplement: A randomized controlled trial.
An Observational Long-Term Safety Surveillance of Participants from Corbus Sponsored Lenabasum Pivotal Clinical Trials
The Anti-Pseudomonal Peptide D-BMAP18 Is Active in Cystic Fibrosis Sputum and Displays Anti-Inflammatory In Vitro Activity.
Antibiotic Treatment Of Staphylococcus Aureus In Stable People With CF
Advances in Ca2+ modulation of gastrointestinal anion secretion and its dysregulation in digestive disorders (Review).
Hyaluronan is abundant in COVID-19 respiratory secretions.
Editorial: Special Issue on “Therapeutic Approaches for Cystic Fibrosis”.
Screen-Printed Sensor for Low-Cost Chloride Analysis in Sweat for Rapid Diagnosis and Monitoring of Cystic Fibrosis.
Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells.
Electrochemical Detection of Pyocyanin as a Biomarker for Pseudomonas aeruginosa: A Focused Review.
Sinister sinusitis.
Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity.
Rescue of common exon skipping mutations in Cystic Fibrosis with modified U1 snRNAs.
Carbon monoxide-releasing molecules inhibit the cystic fibrosis transmembrane conductance regulator Cl- channel.
Models of Palliative Care Delivery for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Consensus Guidelines.
Survey and Electronic Health Record-Based Medication Use Agreement in Children with Cystic Fibrosis: A Retrospective Cross-Sectional Study.
Extracellular DNA in sputum is associated with pulmonary function and hospitalization in patients with cystic fibrosis.
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.
The accuracy of four commercial broth microdilution tests in the determination of the minimum inhibitory concentration of colistin.
Delayed glucose peak and elevated 1-hour glucose on the oral glucose tolerance test identify youth with cystic fibrosis with lower oral disposition index.
Acute hyperosmolar hyperglycaemic state in cystic fibrosis-related diabetes caused by glucocorticoid and itraconazole interaction.
Qualitative evaluation of MRI features in aneurysmal bone cysts after percutaneous sclerotherapy.
Sperm retrieval and intracytoplasmic sperm injection outcomes in men with cystic fibrosis disease versus congenital bilateral absence of the vas deferens.
Kwashiorkor-like dermatosis: a rare presentation of cystic fibrosis.
Appearance of Pancreatic Sufficiency and Discontinuation of Pancreatic Enzyme Replacement Therapy (PERT) in Children with Cystic Fibrosis on Ivacaftor.
Pathways in the Pathophysiology of Coronavirus 19 Lung Disease Accessible to Prevention and Treatment.
Sars-CoV-2 infection in patients with cystic fibrosis. An overwiew.
Efficacy of Simeox Airway Clearance Therapy in Children With Cystic Fibrosis
Home Airway Clearance in CF Patients
Preliminary report of In vitro and In vivo Effectiveness of Dornase alfa on SARS-CoV-2 infection.
Ancillary Home Airway Clearance in CF Patients ( HomeCareSIMEOX )
Laying the Foundation for Cystic Fibrosis Therapy: An Interview with Michael Boyle.
Preserving Multidisciplinary Care Model and Patient Safety During Reopening of Ambulatory Cystic Fibrosis Clinic for Nonurgent Care: A Hybrid Telehealth Model.
Monitoring early stage lung disease in cystic fibrosis.
Two-Step Testing for Clostridioides Difficile is Inadequate in Differentiating Infection From Colonization in Children.
A Survey of Pediatric Competencies in Entry-Level Physical Therapy Programs in Australia.
Lung immunoglobulin A immunity dysregulation in cystic fibrosis.
Epidemiology of Pseudomonas aeruginosa in cystic fibrosis patients in Iran: A systematic review and meta-analysis.
A New Model of Chronic Mycobacterium abscessus Lung Infection in Immunocompetent Mice.
Transition to adult care in cystic fibrosis: The challenges and the structure.
A systematic cochrane review of probiotics for people with cystic fibrosis.
Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients.
Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression.
Living and dying with cystic fibrosis.
Genomics-driven discovery of a novel glutarimide antibiotic from Burkholderia gladioli reveals an unusual polyketide synthase chain release mechanism.
Exhaled volatile organic compounds analysis in clinical pediatrics: a systematic review.
Carriers of a single CFTR mutation are asymptomatic: an evolving dogma?
Infants with Congenital Disorders Identified Through Newborn Screening – United States, 2015-2017.
Neutrophil count in sputum is associated with increased sputum glucose and sputum L-lactate in cystic fibrosis.
An in vitro model for assessing drug transport in cystic fibrosis treatment: Characterisation of the CuFi-1 cell line.
Posaconazole-Induced Hypertension Masquerading as Congenital Adrenal Hyperplasia in a Child with Cystic Fibrosis.
Immune transcriptomes of highly exposed SARS-CoV-2 asymptomatic seropositive versus seronegative individuals from the Ischgl community.
MexXY RND pump of Pseudomonas aeruginosa PA7 effluxes bi-anionic β-lactams carbenicillin and sulbenicillin when it partners with the outer membrane factor OprA but not with OprM.
Incidence of nephrotoxicity with Prolonged aminoglycoside exposure in patients with cystic fibrosis.
Persistent Recovery of Pancreatic Function in Patients with Cystic Fibrosis after Ivacaftor.
Coumaronochromone as antibacterial and carbonic anhydrase inhibitors from Aerva persica (Burm.f.) Merr.: experimental and first-principles approaches.
A simple preservation method for the storage of Aspergillus fumigatus and Scedosporium apiospermum filamentous fungi isolated from the sputum of patients with cystic fibrosis (CF).
A Phase 3b, Randomized, Double blind, Controlled Study Evaluating the Efficacy and Safety of VX-445/Tezacaftor/Ivacaftor in Cystic Fibrosis Subjects, Homozygous for F508del
A Study Evaluating the Long-term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis (CF) Subjects 6 Years and Older and F/MF Genotypes
MR Imaging of Lung in the Follow-up Assessment of Cystic Fibrosis
TRPV4 and purinergic receptor signalling pathways are separately linked in airway epithelia to CFTR and TMEM16A chloride channels.
Targeting Nonsense: Optimization of 1,2,4-Oxadiazole TRIDs to Rescue CFTR Expression and Functionality in Cystic Fibrosis Cell Model Systems.
Novel ANO1 Inhibitor from Mallotus apelta Extract Exerts Anticancer Activity through Downregulation of ANO1.
Characterization of the Burkholderia cenocepacia J2315 Surface-Exposed Immunoproteome.
Factors influencing clinical trial participation for adult and pediatric patients with cystic fibrosis.
Effects of Innovative Aerobic Exercise Training in Cystic Fibrosis
How can we relieve gastrointestinal symptoms in people with cystic fibrosis? An international qualitative survey.
Noninvasive Ventilation in Cystic Fibrosis: Clinical Indications and Outcomes in a Large UK Adult Cystic Fibrosis Center.
Differential effects of the cystic fibrosis lung inflammatory environment on mesenchymal stromal cells.
Clinical and microbiological monitoring of Cystic Fibrosis patients, three years of follow-up via Tele-Medicine: an empirical research.

Mucinous cystic adenoma with inflammatory cell infiltration around the splenic artery mimicking pancreatic cancer: a case report.
Bronchiectasis Associated with Electronic Cigarette Use: A Case Series.
Effects of a long-term exercise program on motor performance in children and adolescents with CF.
Azole-Resistant Aspergillus fumigatus Among Danish Cystic Fibrosis Patients: Increasing Prevalence and Dominance of TR34/L98H.
Mechanism-Based Personalized Medicine for Cystic Fibrosis by Suppressing Pseudo Exon Inclusion.
Rheumatoid arthritis in a patient with cystic fibrosis: challenging treatment options.
Molar mass effect in food and health.
Genistein-Calcitriol Mitigates Hyperosmotic Stress-Induced TonEBP, CFTR Dysfunction, VDR Degradation and Inflammation in Dry Eye Disease.
Patients with Cystic Fibrosis and Advanced Lung Disease Benefit from Lumacaftor/Ivacaftor Treatment.
Evolutionary genomics of niche-specific adaptation to the cystic fibrosis lung in Pseudomonas aeruginosa.
Current development of CFTR potentiators in the last decade.
CFTR mutation compromises spermatogenesis by enhancing miR-15b maturation and suppressing its regulatory target CDC25A†.
[Appendiceal diverticulosis, a potential mimic of low grade appendiceal mucinous neoplasm: analysis of 20 cases].
A tale of two pancreases: exocrine pathology and endocrine dysfunction.
Paediatric nasal irrigation: The “fencing” method.
Vitamin E supplementation in people with cystic fibrosis.
The impact of segmentation on whole-lung functional MRI quantification: Repeatability and reproducibility from multiple human observers and an artificial neural network.
Aerosolized lancovutide in adolescents (≥12 years) and adults with cystic fibrosis – a randomized trial.
Airway Inflammation and Host Responses in the Era of CFTR Modulators.
Treatment of pulmonary exacerbations in cystic fibrosis.
Light at the End of Tunnel-Managing Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis.
Risk of malignancy in Caroli disease and syndrome: A systematic review.
Cystic Fibrosis and COVID-19.
Patterns of Health Insurance Coverage and Lung Disease Progression in Adolescents and Young Adults with Cystic Fibrosis.
Allergic Bronchopulmonary Aspergillosis in Children with Cystic Fibrosis: An Update on the Newest Diagnostic Tools and Therapeutic Approaches.
Recommended shielding against COVID-19 impacts physical activity levels in adults with cystic fibrosis.
A Phylogeny-Informed Proteomics Approach for Species Identification within the Burkholderia cepacia complex.
Interferon-gamma-activated macrophages infected with Burkholderia cenocepacia process and present bacterial antigens to T-cells by class I and II major histocompatibility complex molecules.
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
Transition From Pediatric to Adult Care for Young Adults With Chronic Respiratory Disease.
Using Near-Peer Teaching to Address Concepts of Cystic Fibrosis in Undergraduate Medical Learners.
Th17 associated cytokines in sputum samples from patients with cystic fibrosis.
[Cystic fibrosis : A systemic problem].
Impact of Pharmacy Technicians as part of an Integrated Health-System Pharmacy Team on Improvement of Medication Access in the Care of Cystic Fibrosis Patients.
Sleep Screening for Cystic Fibrosis Patients: A Survey of Cystic Fibrosis Programs.
Do Pulmonary and Extrapulmonary Features Differ Among Cystic Fibrosis, Primary Ciliary Dyskinesia and Healthy Children?
G551D Mutation Impairs Protein Kinase A (PKA)-dependent Activation of CFTR Channel that can be Restored by Novel Gain-Of-Function (GOF) Mutations.
Evaluation of ELX/TEZ/IVA in Cystic Fibrosis (CF) Subjects 2 Through 5 Years
Hepatitis B in Cystic Fibrosis and Latent Tuberculosis Respectively
Worsening pulmonary outcomes during sex reassignment therapy in a transgender female with cystic fibrosis (CF) and asthma/allergic bronchopulmonary aspergillosis: a case report.
Accelerated Approval or Risk Reduction? How Response Biomarkers Advance Therapeutics through Clinical Trials in Cystic Fibrosis.
Analysis of hospital management of chronic respiratory diseases in light of the “Maps of Health Needs” project in Poland.
Erratum: Cardiopulmonary Exercise Testing Provides Additional Prognostic Information in Cystic Fibrosis.
The effects of taurine supplementation on obesity, blood pressure and lipid profile: A meta-analysis of randomized controlled trials.
Optimization of nitric oxide donors for investigating biofilm dispersal response in Pseudomonas aeruginosa clinical isolates.
Transparency and diversity in cystic fibrosis research.
Transparency and diversity in cystic fibrosis research.
Transparency and diversity in cystic fibrosis research – Authors’ reply.
Relationship Between Estrogen Treatment and Skeletal Health in Women With Cystic Fibrosis.
Comparison of Meal-Time Dosing of Insulin in Cystic Fibrosis Related Diabetes
Short-Chain Fatty Acids Promote Mycobacterium avium subsp. hominissuis Growth in Nutrient-Limited Environments and Influence Susceptibility to Antibiotics.
From Mouse to Man and Back: Closing the Correlation Gap between Imaging and Histopathology for Lung Diseases.
Cystic fibrosis newborn screening: the importance of bloodspot sample quality.
Cyst growth in ADPKD is prevented by pharmacological and genetic inhibition of TMEM16A in vivo.
Crushing pancreatic enzymes with enteral feeds in an extremely premature infant with cystic fibrosis-a novel and effective technique.
The role of biochemical testing in cystic fibrosis.
Three-dimensional magnetic resonance imaging ultrashort echo-time cones for assessing lung density in pediatric patients.
Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis.
Ivacaftor Modifies Cystic Fibrosis Neutrophil Phenotype in Subjects with R117H Residual Function CFTR Mutations.
Correction to: Physiologically Based Pharmacokinetic Modeling of CFTR Modulation in People with Cystic Fibrosis Transitioning from Mono or Dual Regimens to Triple-Combination Elexacaftor/Tezacaftor/Ivacaftor.
O-Methylation of the glycopeptidolipid acyl chain defines surface hydrophobicity of Mycobacterium abscessus and macrophage invasion.
Activity of airway antimicrobial peptides against cystic fibrosis pathogens.
Sleep disorders are distinctively associated with exercise intolerance and sedentary behavior in children with cystic fibrosis.
[Clinical features of cystic fibrosis associated allergic bronchopulmonary aspergillosis in children].
Risk factor identification in cystic fibrosis by flexible hierarchical joint models.
BPI-ANCA is expressed in the airways of cystic fibrosis patients and correlates to platelet numbers and Pseudomonas aeruginosa colonization.
Phenotypic and molecular characteristics of CF patients carrying the I1234V mutation.
Impact of SARS-CoV-2 infection in patients with cystic fibrosis in Spain: Incidence and results of the national CF-COVID19-Spain survey.
Not Quite the Bully in the Schoolyard: Staphylococcus aureus Can Survive and Co-exist with Pseudomonas aeruginosa in the Cystic Fibrosis Lung.
Benign postcricoid hypertrophy: Case report and review of the literature.
Ivacaftor improves lung disease in patients with advanced CF carrying CFTR mutations that confer residual function.
Chronic rhinosinusitis in COPD: A prevalent but unrecognized comorbidity impacting health related quality of life.
Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations.
Fatal respiratory infection due to ST308 VIM-1-producing Pseudomonas aeruginosa in a lung transplant recipient: case report and review of the literature.
Cardiorespiratory fitness on a treadmill in an adult cystic fibrosis population.
Flagellin shifts 3D bronchospheres towards mucus hyperproduction.
Novel Anti-Inflammatory Approaches for Cystic Fibrosis Lung Disease: Identification of Molecular Targets and Design of Innovative Therapies.
The Resolution Approach to Cystic Fibrosis Inflammation.
Lactose-Induced Chronic Diarrhea Results From Abnormal Luminal Microbial Fermentation and Disorder of Ion Transport in the Colon.
CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Human Lung Microvascular Endothelial Cells Controls Oxidative Stress, Reactive Oxygen-Mediated Cell Signaling and Inflammatory Responses.
The value of nasal nitric oxide measurement in the diagnosis of primary ciliary dyskinesia.
“Reactance inversion” at low frequencies in a child undergoing treatment of a cystic fibrosis exacerbation.
Role of Cystic Fibrosis Bronchial Epithelium in Neutrophil Chemotaxis.
Deletion of cftr Leads to an Excessive Neutrophilic Response and Defective Tissue Repair in a Zebrafish Model of Sterile Inflammation.
Recent advances in phenotypic drug discovery.
An Open Label Phase 2 Trial of Metformin for Improving CFTR Function in CFRD
A Clinical Study to Investigate Safety, Tolerability and Distribution of CHF 6333 After One or After Repeated Inhalation in Patients With Cystic Fibrosis (CF) and in Patients With Non Cystic Fibrosis (NCFB) Bronchiectasis
NETwork of Linoleic Acid Supplementation in Cystic Fibrosis
CFTR Depletion Confers Hypersusceptibility to Mycobacterium fortuitum in a Zebrafish Model.
Approaches for Genetic Discoveries in the Skin Commensal and Pathogenic Malassezia Yeasts.
Contribution of Short Chain Fatty Acids to the Growth of Pseudomonas aeruginosa in Rhinosinusitis.
Senescence and autophagy in usual interstitial pneumonia of different etiology.
Manuka honey versus saline sinus irrigation in the treatment of cystic fibrosis-associated chronic rhinosinusitis: A randomized pilot trial.
Allosteric folding correction of F508del and rare CFTR mutants by elexacaftor-tezacaftor-ivacaftor (Trikafta) combination.
The Ubiquitin System and A20: Implications in Health and Disease.
Evaluation of Short Antibiotic Combination Courses Followed by Aerosols in Cystic Fibrosis
Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: A retrospective registry cohort study.
How good is end of life care for patients dying with cystic fibrosis?
The psychosocial burden of inflammatory bowel disease in adolescents and young adults.