Pending

Elevation of Sweat Chloride Levels in a Patient with CFTR-Related Metabolic Syndrome Receiving Dexmethylphenidate and Guanfacine
Changes in share wave elastography after Lumacaftor/Ivacaftor treatment in children with cystic fibrosis
The first report on CFTR mutations of meconium ileus in cystic fibrosis population in Saudi Arabia: A single center review
The first report on the association of celiac disease and cystic fibrosis in a tertiary care center in Saudi Arabia
Muc5b Contributes to Mucus Abnormality in Rat Models of Cystic Fibrosis
Ultrasound-guided placement of Long Peripheral Cannula in children with Cystic Fibrosis
Opposite regulation of F508del-CFTR biogenesis by four poly-lysine ubiquitin chains In vitro
An Update on CFTR Modulators as New Therapies for Cystic Fibrosis
Host and pathogen response to bacteriophage engineered against Mycobacterium abscessus lung infection
Prevalence of Post-Glucose Challenge Hypoglycemia in Adult Patients With Cystic Fibrosis and Relevance to the Risk of Cystic Fibrosis-Related Diabetes
Development and Evaluation of a Pharmacist-Driven Vitamin D Protocol for a Cystic Fibrosis Clinic
CFTR Modulator Therapy in an Individual With Cystic Fibrosis Caused by a N1303K CFTR Variant and Infected With Mycobacterium abscessus
CHST11-mediated microenvironment events contribute to pulmonary fibrosis and cancer progression
Constipation in Children and Adolescents: Evaluation and Treatment
Model-based Bayesian inference of the ventilation distribution in patients with Cystic Fibrosis from multiple breath washout, with comparison to ventilation MRI
In vitro activity of ceragenins against Burkholderia cepacia complex
Identification of Key Factors for Anoxic Survival of B. cenocepacia H111
Giving Drugs a Second Chance: Antibacterial and Antibiofilm Effects of Ciclopirox and Ribavirin against Cystic Fibrosis Pseudomonas aeruginosa Strains
Novel CFTR Activator Cact-3 Ameliorates Ocular Surface Dysfunctions in Scopolamine-Induced Dry Eye Mice
Surface Hydration Protects Cystic Fibrosis Airways from Infection by Restoring Junctional Networks
Positively Charged Gold Quantum Dots: An Nanozymatic “Off-On” Sensor for Thiocyanate Detection
A Compositional Analysis of Physical Activity, Sedentary Time, and Sleep and Associated Health Outcomes in Children and Adults with Cystic Fibrosis
Renal Function in Patients with Cystic Fibrosis: A Single-Center Study
Magnesium Status and Calcium/Magnesium Ratios in a Series of Cystic Fibrosis Patients
CFTR dysfunction and targeted therapies: A vision from non-cystic fibrosis bronchiectasis and COPD
Loss of Endothelial CFTR Drives Barrier Failure and Edema Formation in Lung Infection and Can Be Targeted by CFTR Potentiation
VX770 Modulated Bicarbonate Conductance in CFTR-expressing FRT Cell
Potent and selective inhibition of anion exchange activity of SLC26A9 by A9-301
Kinetic Characterization of a Crystallized Putative Hydroxybutyrate Dehydrogenase from the Opportunistic Pathogen Burkholderia cenocepacia
Pharmacological effect of pinostrobin on CFTR-mediated Cl- secretion and renal cyst development in in vitro model of polycystic kidney disease
Loss of Club Cell Creb Mitigates IL-1B-mediated Muco-obstructive Phenotypes in Male Murine Airways
Alternative Chloride Channels are Increased in the CFTR-/- Rat Airway
Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by the Nuclear Bile Acid Receptor, Farnesoid X Receptor
Colistin Action Against Planktonic Pseudomonas aeruginosa
Evaluation of Small Molecule Inhibitors of Pseudomonas Virulence factor LasB as Non-Traditional Immunotherapeutics
In vitro model of pulmonary candidiasis for testing novel therapeutics
Select Amino Acids Increase Anion Secretion in Human Bronchial Epithelial Cells (HBEC) with F508del and Nonsense Mutations: Alternative Approach to Restoring Airway Anion Secretion in Cystic Fibrosis
Effect of Ciprofloxacin on Planktonic Pseudomonas aeruginosa
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Plays an Important Role in Fetal Human Colon Cell Migration and Proliferation
Corneal opacification, an atypical presentation of cystic fibrosis: a case report and review of the literature
In vitro Activity of Antivirulence Drugs Targeting the las or pqs Quorum Sensing Against Cystic Fibrosis Pseudomonas aeruginosa Isolates
Adaptive Immune Response to Mycobacterium abscessus Complex (MABSC) in Cystic Fibrosis and the Implications of Cross-Reactivity
Limitations of standard cost-effectiveness methods for health technology assessment of treatments for rare, chronic diseases: a case study of treatment for cystic fibrosis
Taxonomic position, antibiotic resistance and virulence factor production by Stenotrophomonas isolates from patients with cystic fibrosis and other chronic respiratory infections
Pseudomonas aeruginosa Alters Peptidoglycan Composition under Nutrient Conditions Resembling Cystic Fibrosis Lung Infections
Atomic-scale interactions between quorum sensing autoinducer molecules and the mucoid P. aeruginosa exopolysaccharide matrix
Telemedicine of patients with cystic fibrosis during the COVID-19 pandemic
Optimization of a Liposomal DNase I Formulation with an Extended Circulating Half-Life
Prevalence and subtyping of biofilms present in bronchoalveolar lavage from children with protracted bacterial bronchitis or non-cystic fibrosis bronchiectasis: a cross-sectional study
Urinary bicarbonate and metabolic alkalosis during exacerbations in cystic fibrosis
Potential Cross-Transmission of Mycobacterium abscessus among Non-Cystic Fibrosis Patients at a Tertiary Hospital in Japan
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles
Tissue-localized immune responses in people with cystic fibrosis and respiratory nontuberculous mycobacteria infection
Reducing exacerbations in children and adults with primary ciliary dyskinesia using erdosteine and/or azithromycin therapy (REPEAT trial): study protocol for a multicentre, double-blind, double-dummy, 2×2 partial factorial, randomised controlled trial
Nephronophthisis: a pediatric case report
Non-Cystic Fibrosis Bronchiectasis Increases the Risk of Lung Cancer Independent of Smoking Status
Functional Evaluation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in the Endocervix
Rapid Viscoelastic Characterization of Airway Mucus using a Benchtop Rheometer
Primary Human Nasal Epithelial Cells: Biobanking in the Context of Precision Medicine
Discovering Common Pathophysiological Processes between COVID-19 and Cystic Fibrosis by Differential Gene Expression Pattern Analysis
Short-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor Combination on Glucose Tolerance in Young People With Cystic Fibrosis-An Observational Pilot Study
Cancer in Cystic Fibrosis: Do Not Neglect Gynecologic Cancers
Prostaglandin E2 : A Potential Link Between Cystic Fibrosis and Cancer
[Translated article] Aquagenic Keratoderma: Treatment Update
Caution advised in the use of CFTR modulator treatment for individuals harboring specific CFTR variants
Anesthetic impacts on pulmonary function: Implications for cystic fibrosis
Pathological remodeling of distal lung matrix in end-stage cystic fibrosis patients
The effect of TGF-β1 polymorphisms on pulmonary disease progression in patients with cystic fibrosis
The impact of FDA and EMA regulatory decision-making process on the access to CFTR modulators for the treatment of cystic fibrosis
Amino Acids Improve Aerosolization and Chemical Stability of Potential Inhalable Amorphous Spray-dried Ceftazidime for Pseudomonas aeruginosa Lung Infection
Systematic review on fecal calprotectin in cystic fibrosis
Clinical outcomes of a large cohort of individuals with the F508del/5T;TG12 CFTR genotype
Association between early respiratory viral infections and structural lung disease in infants with cystic fibrosis
Alterations in innate immune responses of patients with chronic rhinosinusitis related to cystic fibrosis
A dual center and dual vendor comparison study of automated perfusion-weighted phase-resolved functional lung magnetic resonance imaging with dynamic contrast-enhanced magnetic resonance imaging in patients with cystic fibrosis
Susceptibility of Burkholderia cepacia Complex to Ceftazidime/Avibactam and Standard Drugs of Treatment for Cystic Fibrosis Patients
Increased intracellular Cl- concentration mediates neutrophil extracellular traps formation in atherosclerotic cardiovascular diseases
Blood mRNA biomarkers distinguish variable systemic and sputum inflammation at treatment initiation of inhaled antibiotics in cystic fibrosis: A prospective non-randomized trial
Impacts of Nontuberculous Mycobacteria Isolates in Non-cystic Fibrosis Bronchiectasis: A 16-Year Cohort Study in Taiwan
Sputum neutrophil elastase and its relation to pediatric bronchiectasis severity: A cross-sectional study
Chronic cough in cystic fibrosis: the effect of modulator therapy on objective 24-h cough monitoring
Distribution of Achromobacter Species in 12 French Cystic Fibrosis Centers in 2020 by a Retrospective MALDI-TOF MS Spectrum Analysis
Cystic Fibrosis and Diagnostic Imaging
Angiographic and histopathological study on bronchial-to-pulmonary vascular anastomoses on explants from patients with cystic fibrosis after bronchial artery embolisation
Extreme electrolyte depletion associated with septic shock as a form of presentation of cystic fibrosis
Exercise in Child Health
Early Pseudomonas aeruginosa predicts poorer pulmonary function in preschool children with cystic fibrosis
Cystic fibrosis and alpha-1 antitrypsin deficiency: case report and review of literature
Invited editorial: Q and A on hereditary lung cancer
CFTR protein quantification as a cystic fibrosis diagnostic biomarker in dried blood spots using multiple reaction monitoring tandem mass spectrometry
The psychological implications and health risks of cystic fibrosis pre- and post- CFTR modulator therapy
COVID-19 infection and nocardiosis causing the death of an adolescent with cystic fibrosis
Exploring YAP1-centered networks linking dysfunctional CFTR to epithelial-mesenchymal transition
Results of Lung Transplantations Among Cystic Fibrosis Patients: A Single-Center Study
Cystic fibrosis year in review 2021
Impaired regulation of PMCA activity by defective CFTR expression promotes epithelial cell damage in alcoholic pancreatitis and hepatitis
Role of Cellular Metabolism in the Formation of Neutrophil Extracellular Traps in Airway Diseases
Synergy Between Pseudomonas aeruginosa Filtrates And Voriconazole Against Aspergillus fumigatus Biofilm Is Less for Mucoid Isolates From Persons With Cystic Fibrosis
Cardiac sarcoidosis in an adult person with cystic fibrosis: a case report
Diffuse pulmonary calcification in allergic bronchopulmonary aspergillosis

COVID-19 infection in an infant with cystic fibrosis: A case report and possible therapeutic effect of hypertonic saline
Cystic Fibrosis-Related Liver Disease is an Independent Risk Factor For Mortality And Increased Health Care Resource Utilization In Hospitalized Pediatric Patients With Cystic Fibrosis
Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis: Authors’ Reply
Staphylococcus aureus detection from CF respiratory samples is improved using alternative media
Difficult gall bladder in adolescents with cystic fibrosis and symptomatic cholelithiasis: What is the best treatment choice?
The Role of HLA-DRB1 Alleles in Pulmonary Cystic Fibrosis
Breath biomarkers associated withnontuberculosis mycobacteria disease status in persons with cystic fibrosis: a pilot study
Antibacterial contact-dependent proteins secreted by Gram-negative cystic fibrosis respiratory pathogens
CFTR mRNAs with nonsense codons are degraded by the SMG6-mediated endonucleolytic decay pathway
Role of inhaled antibiotics in children and adolescents with cystic fibrosis: Experience from the tertiary care center
Cystic Fibrosis Airway Mucus Hyperconcentration Produces a Vicious Cycle of Mucin, Pathogen, and Inflammatory Interactions that Promote Disease Persistence
Continuous glucose monitoring and dysglycaemia in young children with cystic fibrosis: A case series
Effectiveness of positive expiratory pressure on patients over 16 years of age with cystic fibrosis: systematic review and meta-analysis
Esophagogastric variceal bleeding as debut of Caroli’s syndrome
From a medical model to a social model of autonomy for people with cystic fibrosis. A sociological study of the views of professional and associative stakeholders
Pharmacist-administered audiology screening for pediatric cystic fibrosis patients exposed to high-dose aminoglycosides: a pilot study
Sustained inhibition of ENaC in CF: Potential RNA-based therapies for mutation-agnostic treatment
Novel adamantyl clubbed iminothiazolidinones as promising elastase inhibitors: design, synthesis, molecular docking, ADMET and DFT studies
A Pharmacokinetic Analysis of Tobramycin in Patients Less than Five Years of Age with Cystic Fibrosis: Assessment of Target Attainment with Extended-Interval Dosing through Simulation
Sputum Metabolites Associated with Nontuberculous Mycobacterial Infection in Cystic Fibrosis
Use of inhaled antibiotics among Danish patients with cystic fibrosis
Can continuous glucose monitoring predict cystic fibrosis-related diabetes and worse clinical outcome?
Diagnosis of cystic fibrosis-related diabetes: too early or too late?
Cumulative Incidence and Risk Factors for Severe COVID-19 in French People with Cystic Fibrosis
Paths to Motherhood for Women with Cystic Fibrosis
Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients
Molecular Dynamics and Theratyping in Airway and Gut Organoids Reveal R352Q-CFTR Conductance Defect
Molecular Insight into Gene Response of Diorcinol- and Rubrolide-Treated Biofilms of the Emerging Pathogen Stenotrophomonas maltophilia
Genetic and Transcriptomic Characteristics of RhlR-Dependent Quorum Sensing in Cystic Fibrosis Isolates of Pseudomonas aeruginosa
Reporting two novel mutations in two Iranian families with cystic fibrosis, molecular and bioinformatic analysis
Romosozumab used to treat a patient with cystic fibrosis-related osteoporosis
Diabetic Ketoacidosis as a Unique Presentation of Cystic Fibrosis
Contribution of Afferent Renal Nerves to Cystogenesis and Arterial Pressure Regulation in a Preclinical Model of Autosomal Recessive Polycystic Kidney Disease
Complete Genome Sequence of Stenotrophomonas maltophilia Podophage Pepon
Downregulation of epithelial sodium channel (ENaC) activity in cystic fibrosis cells by epigenetic targeting
Antipseudomonal treatment decisions during CF exacerbation management
Seamless Gene Correction in the Human Cystic Fibrosis Transmembrane Conductance Regulator Locus by Vector Replacement and Vector Insertion Events
Diagnostics of the Epstein-Barr Virus Before and After Lung Transplantation in a Patient With Developing Posttransplant Lymphoproliferative Disease: A Case Report
Effect of Bronchoscopic Interventions on Long-Term Lung Function Among Lung Transplant Recipients due to Cystic Fibrosis: A Single-Center Study
Immunogenicity of the COVID-19 BNT162b2 vaccine in adolescents and young adults with cystic fibrosis
Ways of coping and survival in Cystic Fibrosis: a 20-year longitudinal study
Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics
Overview of CF lung pathophysiology
The Octopus Sign-A New HRCT Sign in Pulmonary Langerhans Cell Histiocytosis
Investigation of Direct and Retro Chromone-2-Carboxamides Based Analogs of Pseudomonas aeruginosa Quorum Sensing Signal as New Anti-Biofilm Agents
Cilia Stimulatory and Antibacterial Activities of T2R Bitter Taste Receptor Agonist Diphenhydramine: Insights into Repurposing Bitter Drugs for Nasal Infections
Oral Glucose Tolerance Test in Patients with Cystic Fibrosis Compared to the Overweight and Obese: A Different Approach in Understanding the Results
Theratyping of the Rare CFTR Variants E193K and R334W in Rectal Organoid-Derived Epithelial Monolayers
Secretory Immunoglobulin A Immunity in Chronic Obstructive Respiratory Diseases
CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells
Highway to Cell: Selection of the Best Cell-Penetrating Peptide to Internalize the CFTR-Stabilizing iCAL36 Peptide
Pseudomonas aeruginosa PAO1 Is Attracted to Bovine Bile in a Novel, Cystic Fibrosis-Derived Bronchial Epithelial Cell Model
Clinical Impact of Aspergillus fumigatus in Children with Cystic Fibrosis
The Human Mycobiome in Chronic Respiratory Diseases: Current Situation and Future Perspectives
Pharmacological Effects of Panduratin A on Renal Cyst Development in In Vitro and In Vivo Models of Polycystic Kidney Disease
Influence of Bronchoscopic Interventions on Graft Function of Double Lung Transplant Recipients due to Cystic Fibrosis
Combined machine learning and pharmacophore based virtual screening approaches to screen for antibiofilm inhibitors targeting LasR of Pseudomonas aeruginosa
Alterations of mucosa-attached microbiome and epithelial cell numbers in the cystic fibrosis small intestine with implications for intestinal disease
An observational study of Pseudomonas aeruginosa in adult long-term ventilation
Novel CFTR modulator combinations maximise rescue of G85E and N1303K in rectal organoids
Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A case series
DNA Sequencing Analysis of Cystic Fibrosis Transmembrane Regulator Gene Identifies Cystic Fibrosis-Associated Variants in the Severe Asthma Research Program
At-home Compounding Preparation of Slow Desensitization of Elexacaftor/Tezacaftor/Ivacaftor for Delayed Hypersensitivity Rash
Co-Operative Biofilm Interactions between Aspergillus fumigatus and Pseudomonas aeruginosa through Secreted Galactosaminogalactan Exopolysaccharide
The Presence of Exophiala dermatitidis in the Respiratory Tract of Cystic Fibrosis Patients Accelerates Lung Function Decline: A Retrospective Review of Lung Function
Molecular Epidemiological Characteristics of Mycobacterium abscessus Complex Derived from Non-Cystic Fibrosis Patients in Japan and Taiwan
Interbacterial Antagonism Mediated by a Released Polysaccharide
Losartan ameliorates TGF-β1-induced CFTR dysfunction and improves correction by cystic fibrosis modulator therapies
Histomorphological Changes in a Rat Model of Polycystic Ovary Syndrome and the Contribution of Stevia Leaf Extract in Modulating the Ovarian Fibrosis, VEGF, and TGF-β Immunoexpressions: Comparison with Metformin
The role of Hyaluronan synthesis and degradation in the critical respiratory illness COVID-19
Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated with one Month of Lumacaftor/ivacaftor
Current prices versus minimum costs of production for CFTR modulators
Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D
Non-visualisation of fetal gallbladder in a Chinese cohort
Investigation of interspecies crosstalk between probiotic Bacillus subtilis BR4 and Pseudomonas aeruginosa using metabolomics analysis
Pediatric Paranasal Sinuses- Development, Growth, Pathology & Functional Endoscopic Sinus Surgery
Living with cystic fibrosis during the COVID-19 pandemic: a social connectedness perspective
A new era has dawned for persons with cystic fibrosis; however many knowledge gaps exist in our efforts to improve care
Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosis
The Effect of Dysglycaemia on Changes in Pulmonary and Aerobic Function in Cystic Fibrosis
Bone accrual and structural changes over one year in youth with cystic fibrosis
Clinical practice versus guidelines for the screening of cystic fibrosis-related diabetes: A French survey from the 47 centers
Using Structure-guided Fragment-Based Drug Discovery to Target Pseudomonas aeruginosa Infections in Cystic Fibrosis
A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein
Application of gap time analysis with flexible hazards to pulmonary exacerbations in the EPIC observational study
‘We’re kind of like genetic nomads’: Parents’ experiences of biographical disruption and uncertainty following in/conclusive results from newborn cystic fibrosis screening
Occurrence of Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations in Patients with Allergic Bronchopulmonary Aspergillosis Complicating Asthma
Spatial covariance analysis reveals the residue-by-residue thermodynamic contribution of variation to the CFTR fold
Constipation and cystic fibrosis. Slow movement
Evaluation of clinically relevant changes in the lung clearance index in children with cystic fibrosis and healthy controls
Evaluating FEV1 Decline in Diagnosis and Management of Pulmonary Exacerbations in Children with Cystic Fibrosis
Mental health care needs in cystic fibrosis: A scoping review
A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus
Study to Evaluate Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) Long-term Safety and Efficacy in Subjects Without F508del
Coming Full Circle: Reflections and Inspirations from a Cystic Fibrosis Patient Scientist Panel
Role of Efflux in Antibiotic Resistance of Achromobacter xylosoxidans and Achromobacter insuavis Isolates From Patients With Cystic Fibrosis
Repeatability of lung clearance index in infants with cystic fibrosis and recurrent wheeze
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice
Re: Impact of spiroware re-analysis method on multiple-breath washout outcomes in children with cystic fibrosis; M.A. Oestreich, F. Wyler, P. Latzin et al
CFTR modulator use and risk of nontuberculous mycobacteria positivity in cystic fibrosis, 2011-2018

Targeting the EGFR-ERK axis using the compatible solute ectoine to stabilize CFTR mutant F508del
Characterization of skeletal muscle wasting pathways in diaphragm and limb muscles of cystic fibrosis mice
Correlates of Pancreatic Enzyme Replacement Therapy Intake in Adults with Cystic Fibrosis: Results of a Cross-Sectional Study
Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis
CFTR Modulator Therapies: Potential Impact on Airway Infections in Cystic Fibrosis
The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis
Comparison of Bioelectrical Impedance Analysis with DXA in Adolescents with Cystic Fibrosis before and after a Resistance Training Intervention
Cystic Fibrosis-Related Diabetes in Poland
Bronchiectasis – Exercise as Therapy (BREATH): rationale and study protocol for a multi-center randomized controlled trial
Biochemical, Biophysical, and Immunological Characterization of Respiratory Secretions in Severe SARS-CoV-2 (COVID-19) Infections
Feasibility and efficacy of combined pancreatic islet-lung transplantation in cystic fibrosis related diabetes -PIM study: a multicenter phase 1-2 trial
Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis: Correspondence
Mucus Clearance Strategies in Mechanically Ventilated Patients
Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis
Transcatheter coil embolization of a complex pulmonary artery pseudoaneurysm with thyrocervical trunk-pulmonary arterial fistulization in a patient with cystic fibrosis and massive hemoptysis
High cystic fibrosis transmembrane conductance regulator expression in childhood B-cell acute lymphoblastic leukemia acts as a potential therapeutic target
“The Stakes Are Higher”- Patient and Caregiver Perspectives on Cystic Fibrosis Research and Personalized Medicine
Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry
Genetic evidence supports the development of SLC26A9 targeting therapies for the treatment of lung disease
Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse Model
Safety of chronic hypertonic bicarbonate inhalation in a cigarette smoke-induced airway irritation guinea pig model
Oxylipin profile in saliva from patients with cystic fibrosis reveals a balance between pro-resolving and pro-inflammatory molecules
Ciliary and immune dysfunctions and their genetic background in patients with non-cystic fibrosis bronchiectasis in Central Iran
Distinct proteostasis states drive pharmacologic chaperone susceptibility for Cystic Fibrosis Transmembrane Conductance Regulator misfolding mutants
Medical interventions for chronic rhinosinusitis in cystic fibrosis
Lung parenchymal calcifications in a child with cystic fibrosis
Curvilinearity provides additional information to lung clearance index only in a minority of children with early cystic fibrosis lung disease
A pilot study of disease related education and psychotherapeutic support for unresolved grief in parents of children with CF
Brazilian Guidelines for Nutrition in Cystic Fibrosis
Developmental Functioning Outcomes in Infants with Cystic Fibrosis: a 24- to 36-Month Follow-Up Study
Cefiderocol Pharmacokinetics in Adult Patients With Cystic Fibrosis
Surprise Foreign Body Aspiration with Pistachios in a Patient with Cystic Fibrosis with Persistent Atelectasis on Chest Radiography
Glby, Encoded by MAB_3167c , Is Required for In Vivo Growth of Mycobacteroides abscessus and Exhibits Mild β-Lactamase Activity
Persistent Rhinovirus Infection in a Child With Leukemia
Proteostasis Regulators in Cystic Fibrosis: Current Development and Future Perspectives
Microbial Community Composition in Explanted Cystic Fibrosis and Control Donor Lungs
Targeting the Pseudomonas aeruginosa Virulence Factor Phospholipase C With Engineered Liposomes
Reinfection or relapse? A case study of whole genome sequencing guided genomic characterization of Mycobacterium abscessus chronic infection in a cystic fibrosis patient
Association between elevated peripheral blood eosinophil count and respiratory outcomes in adults with cystic fibrosis
Objective Nebuliser Adherence Data as “Proof” of Adherence in the Management of Cystic Fibrosis: A Qualitative Interview Study
SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair Modulation
Moving up: Healthcare transition experiences of adolescents and young adults with cystic fibrosis
Club cell secretory protein and lung function in children with cystic fibrosis
Pathophysiology of Lung Disease and Wound Repair in Cystic Fibrosis
Expression of ACE2 – A Key SARS-CoV-2 Entry Factor – Is Not Increased in the Nasal Mucosa of People with Cystic Fibrosis
TMEM16A (ANO1) as a therapeutic target in cystic fibrosis
High frequency of complex CFTR alleles associated with c.1521_1523delCTT (F508del) in Russian cystic fibrosis patients
Lung clearance index to characterize clinical phenotypes of children and adolescents with cystic fibrosis
The 49th parallel: Does geographic position affect longevity of patients with cystic fibrosis?
Mucus concentration-dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis
Efficacy and Safety of Triple Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Patients With Cystic Fibrosis: A Meta-Analysis of Randomized Controlled Trials
Network Analysis to Identify Multi-Omic Correlations in the Lower Airways of Children With Cystic Fibrosis
Efficacy of N-acetylcysteine on idiopathic or postinfective non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis protocol
The dilemma of initiating ELX/TEZ/IVA in a CF patient recovering from acute-on-chronic liver failure
Prepuberal insulin secretory indices are long term predictors of short adult stature in cystic fibrosis
Eosinophilic Esophagitis in Cystic Fibrosis: A Case Series with Long-Term Follow-up
The Ubiquitin Ligase RNF34 Participates in the Peripheral Quality Control of CFTR (RNF34 Role in CFTR PeriQC)
A Shaving Proteomic Approach to Unveil Surface Proteins Modulation of Multi-Drug Resistant Pseudomonas aeruginosa Strains Isolated From Cystic Fibrosis Patients
A PI3Kγ mimetic peptide triggers CFTR gating, bronchodilation, and reduced inflammation in obstructive airway diseases
Using A Work System Framework to Investigate Pharmacists’ Roles in Cystic Fibrosis Management
Azithromycin inhibits mucin secretion, mucous metaplasia, airway inflammation and airways hyperresponsiveness in mice exposed to house dust mite extract
MRI lung lobe segmentation in pediatric cystic fibrosis patients using a recurrent neural network trained with publicly accessible CT datasets
Things come in threes: A new complex allele and a novel deletion within the CFTR gene complicate an accurate diagnosis of cystic fibrosis
Azole-Induced Myositis after Combined Lung-Liver Transplantation
Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis
Histological Manifestations of Diabetic Kidney Disease and its Relationship with Insulin Resistance
Cross-talk between enhancers, structural elements and activating transcription factors maintains the 3D architecture and expression of the CFTR gene
Significance of plasma fibrinogen and malondialdehyde in the post-inflammatory period in patients with cystic fibrosis
Complete Genome Sequence of Burkholderia cenocepacia Phage Paku
COVID-19 in a pregnant cystic fibrosis carrier with myasthenia gravis: A case report
Factors in childhood associated with lung function decline to adolescence in cystic fibrosis
Global access to affordable CFTR modulator drugs: Time for action!
Krüppel-Like Factor 5 regulates CFTR expression through repression by maintaining chromatin architecture coupled with direct enhancer activation
Characterization of glucose metabolism in youth with vs. without cystic fibrosis liver disease: A pilot study
The Changing Landscape of Nutrition in Cystic Fibrosis: The Emergence of Overweight and Obesity
Journey on VX-809-Based Hybrid Derivatives towards Drug-like F508del-CFTR Correctors: From Molecular Modeling to Chemical Synthesis and Biological Assays
Defining and Treating Cystic Fibrosis Liver Disease: Some Things Old and Some Things New
Pseudomonas aeruginosa polysaccharide Psl supports airway microbial community development
Exposure to the Pseudomonas aeruginosa secretome alters the proteome and secondary metabolite production of Aspergillus fumigatus
An 18-Year Dataset on the Clinical Incidence and MICs to Antibiotics of Achromobacter spp. (Labeled Biochemically or by MAL-DI-TOF MS as A. xylosoxidans ), Largely in Patient Groups Other than Those with CF
Elastase-Activated Antimicrobial Peptide for a Safer Pulmonary Treatment of Cystic Fibrosis Infections
Lung-Directed Bacteriotherapy in Cystic Fibrosis: Could It Be an Option?
Structural Comparative Modeling of Multi-Domain F508del CFTR
The Relation between Vitamin D Level and Lung Clearance Index in Cystic Fibrosis-A Pilot Study
The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs
Feasibility and Efficacy of Telehealth-Based Resistance Exercise Training in Adolescents with Cystic Fibrosis and Glucose Intolerance
Biofilm on Toothbrushes of Children with Cystic Fibrosis: A Potential Source of Lung Re-Infection after Antibiotic Treatment?
Metrics of Antifungal Effects of Ciprofloxacin on Aspergillus fumigatus Planktonic Growth and Biofilm Metabolism; Effects of Iron and Siderophores
Targeted Gene Insertion for Functional CFTR Restoration in Airway Epithelium
One year monitoring of SARS-CoV-2 prevalence in a German cohort of patients with cystic fibrosis
Inherited pancreatic exocrine insufficiency and pancreatitis: When children transition to adult care
Digestive outcomes in Cystic fibrosis
Improving Therapeutic Adherence With a Co-constructed Program Involving Both Patients and Health Care Professionals
Description of the Short-term Effects of KAFTRIO® by Continuous Monitoring With the PHEAL-CR-K Application in Real Life in Patients With Cystic Fibrosis Eligible for KAFTRIO® Treatment
Caregiver burden in cystic fibrosis: a systematic literature review
Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs
A Potent Inhibitor of the Cystic Fibrosis Transmembrane Conductance Regulator Blocks Disease and Morbidity Due to Toxigenic Vibrio cholerae
Does ibuprofen affect the expression of alginate genes in pathogenic Pseudomonas aeruginosa strains?
State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the “iMAging managEment of cySTic fibROsis” (MAESTRO) consortium
Alginate oligosaccharides enhance diffusion and activity of colistin in a mucin-rich environment

Respiratory physiotherapy in patients with Cystic Fibrosis and upper limb deep vein thrombosis
Measurement of exhaled nitric oxide fraction in lung diseases
The Use of Tobramycin for Pseudomonas aeruginosa : A Review
Cystic fibrosis transmembrane conductance regulator prevents ischemia/reperfusion induced intestinal apoptosis via inhibiting PI3K/AKT/NF-kappaB pathway
Cystic fibrosis patients on cystic fibrosis transmembrane conductance regulator modulators have a reduced incidence of cirrhosis
Pseudomonas aeruginosa modulates neutrophil granule exocytosis in an in vitro model of airway infection
Resolution of lobe collapse in a child with cystic fibrosis with M. abscessus using serial intrabronchial rhDNase
CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor
CFTR-mediated monocyte/macrophage dysfunction revealed by cystic fibrosis proband-parent comparisons
Systematic estimation of cystic fibrosis prevalence in Chinese and genetic spectrum comparison to Caucasians
Strong and consistent associations of precedent chronic rhinosinusitis with risk of non-cystic fibrosis bronchiectasis
The Impact of COVID-19 in Cystic Fibrosis
Cystic Fibrosis in 2021: “The Times They Are A-Changin”
Association Between Evolution of Mucus Plugging, Parenchymal Alterations and Air Trapping on Computed Tomography and Risk of Exacerbations in Adults With Cystic Fibrosis
Variation in CFTR-dependent ‘β-sweating’ among healthy adults
Virulence Mechanisms of Mycobacterium abscessus : Current Knowledge and Implications for Vaccine Design
Novel cystic fibrosis transmembrane conductance regulator variant in a cystic fibrosis patient
Is exercise and electrostimulation effective in improving muscle strength and cardiorespiratory fitness in children with cystic fibrosis and mild-to-moderate pulmonary impairment?: Randomized controlled trial
Decreased efficacy of antimicrobial agents in a polymicrobial environment
Mutational Spectrum of the CFTR Gene in the Kazakhstan Population
HALT-ing Nontuberculous Mycobacteria in CF Centers. Is There Something in The Water?
The NSAID glafenine rescues class 2 CFTR mutants via cyclooxygenase 2 inhibition of the arachidonic acid pathway
Pinostrobin inhibits renal CFTR-mediated Cl- secretion and retards cyst growth in cell-derived cyst and polycystic kidney disease rats
Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis
Health care costs in a randomized trial of antimicrobial duration among cystic fibrosis patients with pulmonary exacerbations
A prospective randomised controlled mixed-methods pilot study of home monitoring in adults with cystic fibrosis
Use of Dynamic Chest Radiography to Assess Treatment of Pulmonary Exacerbations in Cystic Fibrosis
Impact of lockdown during the COVID-19 pandemic on health status in patients with cystic fibrosis: a mono-centre observational study
Clinical Outcomes of Antipseudomonal versus Other Antibiotics Among Children with Cystic Fibrosis without Pseudomonas aeruginosa
Targeting the E1 ubiquitin-activating enzyme (UBA1) improves elexacaftor/tezacaftor/ivacaftor efficacy towards F508del and rare misfolded CFTR mutants
Informatic analysis of the pulmonary microecology in non-cystic fibrosis bronchiectasis at three different stages
Predictive value of impulse oscillometry and multiple breath washout parameters in pediatric patients with cystic fibrosis pulmonary exacerbation
PBPK-led guidance for cystic fibrosis patients taking elexacaftor-tezacaftor-ivacaftor with nirmatrelvir-ritonavir for the treatment of COVID-19
Charting a New Path: A Single-cell Atlas of Porcine CF Airways at Birth
Novel volumetric capnography indices measure ventilation inhomogeneity in cystic fibrosis
Elexacaftor/Tezacaftor/Ivacaftor as a Bridge to Lung Retransplant in a Recipient With Cystic Fibrosis
Three-hole oesophagectomy following bilateral lung transplant for cystic fibrosis
The effect of inhaled hypertonic saline on lung structure in children aged 3-6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled trial
Effect of chest physiotherapy on cystic fibrosis sputum nanostructure: an experimental and theoretical approach
Inhaled Dry Powder Mannitol Treatment in Pediatric Patients with Cystic Fibrosis: Evaluation of Clinical Data in a Real-World Setting
Efficacy of Intensive Cholecalciferol Monitoring and Supplementation on Serum vitD Levels in Pediatric Patients With CF
N-Acetyl-Cysteine Increases Activity of Peanut-Shaped Gold Nanoparticles Against Biofilms Formed by Clinical Strains of Pseudomonas aeruginosa Isolated from Sputum of Cystic Fibrosis Patients
Letter in Reply: Can We Justify Cystic Fibrosis Mutational Analysis among Omani Neonates?
Can We Justify Cystic Fibrosis Mutational Analysis among Omani Neonates?
Commentary: Filling in the cracks: How to improve survival for patients with cystic fibrosis
Cough in adolescent with cystic fibrosis, from nightmare to COVID-19 stigma: A qualitative thematic analysis
What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?
Iron Deficiency in Cystic Fibrosis: A Cross-Sectional Single-Centre Study in a Referral Adult Centre
Intestinal Absorption of Lipids Using a Pancreatic Enzyme-Free Nutritional Supplement in Patients with Cystic Fibrosis: A Randomized, Double-Blind, Crossover Pilot Trial
Left behind: The potential impact of CFTR modulators on racial and ethnic disparities in cystic fibrosis
Longitudinal lung clearance index and association with structural lung damage in children with cystic fibrosis
Comparison of the Lung Clearance Index in Preschool Children with Primary Ciliary Dyskinesia and Cystic Fibrosis
Comparison of continuous glucose monitoring in cystic fibrosis patients with or without pancreatic exocrine insufficiency
CFTR suppresses neointimal formation through attenuating proliferation and migration of aortic smooth muscle cells
Effects of Exercise on Nutritional Status in People with Cystic Fibrosis: A Systematic Review
Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management
Anticipating New Treatments for Cystic Fibrosis: A Global Survey of Researchers
Methods of Sputum and Mucus Assessment for Muco-Obstructive Lung Diseases in 2022: Time to “Unplug” from Our Daily Routine!
Proximity Profiling of the CFTR Interaction Landscape in Response to Orkambi
CFTR, Cell Junctions and the Cytoskeleton
Effectiveness of Video Games as Physical Treatment in Patients with Cystic Fibrosis: Systematic Review
Evaluation of Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) in Cystic Fibrosis Subjects Without an F508del Mutation
Reducing prescribing errors: making electronic prescribing work for cystic fibrosis inpatients
The Prognosis of ART Is Not Altered in Cystic Fibrosis Women: A Case-Report Study
Polycationic Glycopolymer Demonstrates Activity Against Persisters and Biofilms of Non-tuberculosis Mycobacteria Cystic Fibrosis Clinical Isolates in vitro
Pseudomonas aeruginosa Affects Airway Epithelial Response and Barrier Function During Rhinovirus Infection
Establishing Antimicrobial Susceptibility Testing Methods and Clinical Breakpoints for Inhaled Antibiotic Therapy
Interleukin-1 beta is a potential mediator of airway nitric oxide deficiency in cystic fibrosis
Impact of azithromycin on serum inflammatory markers in children with cystic fibrosis and new Pseudomonas
Pilot Study of a CF-specific CBT Intervention for Adolescents
Development and preliminary validation of the challenges of living with cystic fibrosis (CLCF) questionnaire: a 46-item measure of treatment burden for parent/carers of children with CF
Benefits of reviewing pancreatic function in children with cystic fibrosis
Safety of mRNA-based vaccines against SARS-CoV-2 in people with cystic fibrosis aged 12 years and over
The clinical impact of the Covid-19 pandemic first wave on patients with cystic fibrosis in New York
Receiving results of uncertain clinical relevance from population genetic screening: systematic review & meta-synthesis of qualitative research
Association of Body Mass Index With Clinical Outcomes in Patients With Cystic Fibrosis: A Systematic Review and Meta-analysis
Body Mass Index and Clinical Outcomes in Persons Living With Cystic Fibrosis-Is Bigger Always Better?
CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages
Tobramycin safety and efficacy review article
Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro
DMBT1 is upregulated in cystic fibrosis, affects ciliary motility, and is reduced by acetylcysteine
“Be afraid – be very afraid”: Passive air drying of nebulizer parts in cystic fibrosis (CF) – Occult microbiological risks of contamination with Pseudomonas aeruginosa from calyptrate flies (Musca domestica &Calliphora vomitoria)
Ethmoidal mucocele causing proptosis in a pediatric patient with cystic fibrosis: A case report
The role of pharmacy services beyond cystic fibrosis: a case for support in childhood interstitial lung disease
The changing landscape of pediatric lung transplantation
Platelet CFTR inhibition enhances arterial thrombosis via increasing intracellular Cl- concentration and activation of SGK1 signaling pathway
Achromobacter xylosoxidans Purulent Bronchitis in a Previously Healthy Child: An Unexpected Consequence of COVID-19 Infection
Cystic Fibrosis Lung Transplant Recipients 10 years of age or Younger: Predisposing Factors for End-stage Disease
Prevalence of antibiotic resistance of Pseudomonas aeruginosa in cystic fibrosis infection: A systematic review and meta-analysis
3.22 Nutrition in Cystic Fibrosis
Elexacaftor, tezacaftor and ivacaftor: a case of severe rash and approach to desensitisation
A Single-Cell Atlas of Large and Small Airways at Birth in a Porcine Model of Cystic Fibrosis
The Burkholderia cenocepacia iron starvation σ factor, OrbS, possesses an on-board iron sensor
Addition time plays a major role in the inhibitory effect of chitosan on the production of Pseudomonas aeruginosa virulence factors
Patienthèque of Finisterian (South of Brittany) Children With Cystic Fibrosis in the Time of Precision Medicine
Impact of Spiroware re-analysis method on multiple-breath washout outcomes in children with cystic fibrosis
Risk Assessment for Patients with Chronic Respiratory Conditions in the Context of the SARS-CoV-2 Pandemic Statement of the German Respiratory Society with the Support of the German Association of Chest Physicians
The impact of COVID-19 upon the delivery of exercise services within cystic fibrosis clinics in the United Kingdom
Effectiveness of Different Eradication Treatment Protocols for New-Onset Pseudomonas aeruginosa in Children with Cystic Fibrosis
AZD5634, an inhaled ENaC inhibitor, in healthy subjects and patients with cystic fibrosis

Epigenetic regulation of inflammation by microRNAs in post-infectious bronchiolitis obliterans
Functional interplay between CFTR and pendrin: physiological and pathophysiological relevance
Impaired intestinal stem cell activity in ETEC infection: enterotoxins, cyclic nucleotides, and Wnt signaling
Testicular cancer in men with cystic fibrosis
Congenital Bilateral Absence of the Vas Deferens
Burkholderia cepacia Complex Lumbar Spondylodiscitis: A Rare Nosocomial Infection
Cystic fibrosis telemedicine in the era of COVID-19
Staphylococcus aureus in Non-Cystic Fibrosis Bronchiectasis: Prevalence and Genomic Basis of High Inoculum Beta-Lactam Resistance
Achromobacter spp. in a Cohort of Non-Selected Pre- and Post-Lung Transplant Recipients
N-acetylcysteine (NAC) and Its Role in Clinical Practice Management of Cystic Fibrosis (CF): A Review
Utilization of the Healthy Eating Index in Cystic Fibrosis
Nutrition in Cystic Fibrosis-Some Notes on the Fat Recommendations
Neutrophil Dysfunction in the Pathogenesis of Cystic Fibrosis
Cystic fibrosis transmembrane conductance regulator function in patients with chronic pancreatitis
Protein with negative surface charge distribution, Bnr1, shows characteristics of a DNA-mimic protein and may be involved in the adaptation of Burkholderia cenocepacia
In Vivo Activity of Metal Complexes Containing 1,10-Phenanthroline and 3,6,9-Trioxaundecanedioate Ligands against Pseudomonas aeruginosa Infection in Galleria mellonella Larvae
Inhibitor of Hyaluronic Acid Synthesis 4-Methylumbelliferone Suppresses the Secretory Processes That Ensure the Invasion of Neutrophils into Tissues and Induce Inflammation
Respiratory and Peripheral Muscle Weakness and Body Composition Abnormalities in Non-Cystic Fibrosis Bronchiectasis Patients: Gender Differences
Safety and Efficacy of Devices Delivering Inhaled Antibiotics among Adults with Non-Cystic Fibrosis Bronchiectasis: A Systematic Review and a Network Meta-Analysis
Lung Ultrasound in Children with Cystic Fibrosis in Comparison with Chest Computed Tomography: A Feasibility Study
Pulmonary Exacerbations in Pediatric Patients: Retrospective Study in a Portuguese Cystic Fibrosis Center
SLC26A9 as a Potential Modifier and Therapeutic Target in Cystic Fibrosis Lung Disease
Use of Intravenous Pulse Steroids to Treat Allergic Bronchopulmonary Aspergillosis in a Non-Compliant Asthmatic Adolescent
Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study
Factors Predisposing the Response to Lumacaftor/Ivacaftor in People with Cystic Fibrosis
Novel Bacteriophages Show Activity against Selected Australian Clinical Strains of Pseudomonas aeruginosa
The Arginine Catabolism-Derived Amino Acid l-ornithine Is a Chemoattractant for Pseudomonas aeruginosa
Induction of Broad β-lactam Resistance in Achromobacter ruhlandii by Exposure to Ticarcillin Is Primarily Linked to Substitutions in Murein Peptide Ligase Mpl
In vitro assessment of triple combination therapy for the most common disease-associated mutation in cystic fibrosis
National Guidelines for the Performance of the Sweat Test in Diagnosis of Cystic Fibrosis on behalf of the Croatian Society of Medical Biochemistry and Laboratory Medicine and the Cystic Fibrosis Centre – Paediatrics and adults, University Hospital Centre Zagreb
Prevalence and Impact of Rheumatologic Pain in Cystic Fibrosis Adult Patients
Detection of DZIP1L mutations by whole-exome sequencing in consanguineous families with polycystic kidney disease
A highly selective, cell-permeable furin inhibitor BOS-318 rescues key features of cystic fibrosis airway disease
Natural killer cells kill extracellular Pseudomonas aeruginosa using contact-dependent release of granzymes B and H
Serum anti-PAD4 autoantibodies are present in cystic fibrosis children and increase with age and lung disease severity
Improvements in anthropometric measures and gastrointestinal tolerance in patients with cystic fibrosis by using a digestive enzyme cartridge with overnight enteral nutrition
Bicarbonate transport of airway surface epithelia in luminally perfused mice bronchioles
Neutrophil extracellular traps in chronic lung disease: implications for pathogenesis and therapy
Age at menarche in girls with cystic fibrosis and asthma
Collaborative research protocol to define patient-reported experience measures of the cystic fibrosis care pathway in France: the ExPaParM study
Body functions and structure, activity, and participation limitations of adult cystic fibrosis patients under the international classification of functioning, disability, and health framework
A Pandemic Lesson for Global Lung Diseases: Exacerbations are Preventable
Cystic Fibrosis-Related Diabetes Mellitus and Pregnancy: A Retrospective Study
Differential correlation network analysis of rectal transcriptomes reveals cystic fibrosis-related disturbance
Gait and functional balance in non-CF bronchiectasis
A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant
Expression of cystic fibrosis lung disease modifier genes in human airway models
An Open-label, Phase 1/2 Trial of Gene Therapy 4D-710 in Adults With Cystic Fibrosis
Study of Satisfaction of Patients and Care Providers for an Alternate Follow-up With In-hospital Consults and Tele-consult for Patients With Cystic Fibrosis
Two systematic cochrane reviews of the prevention and treatment of distal intestinal obstruction syndrome in cystic fibrosis
How the sweat gland reveals levels of CFTR activity
Repurposing the Veterinary Antibiotic Apramycin for Antibacterial and Antibiofilm Activity Against Pseudomonas aeruginosa From Cystic Fibrosis Patients
Complete Genome Sequencing and Comparative Analysis of the Clinically-Derived Apiotrichum mycotoxinivorans Strain GMU1709
Cystic fibrosis in the 21st century: what every radiologist should know
Cystic fibrosis: candidate selection and impact of the cystic fibrosis transmembrane conductance regulator therapy
Development of a novel rapamycin loaded nano- into micro-formulation for treatment of lung inflammation
Parenthood impacts short-term health outcomes in people with cystic fibrosis
Chest x-rays are less sensitive than multiple breath washout examinations when it comes to detecting early cystic fibrosis lung disease
Alkalosis-induced hypoventilation in cystic fibrosis: The importance of efficient renal adaptation
Commensal Bacteria in the Cystic Fibrosis Airway Microbiome Reduce P. aeruginosa Induced Inflammation
Outcomes and survival following lung transplantation in non-cystic fibrosis bronchiectasis
Controlled inhalation improves central and peripheral deposition in cystic fibrosis patients with moderate lung disease
The Effect Of Telerehabilitation On Quality Of Life, Anxiety And Depression In Children With Cystic Fibrosis And Caregivers: A Single-Blind Randomized Trial
Neutrophil-derived extracellular vesicles promote feed-forward inflammasome signaling in cystic fibrosis airways
High affinity iron uptake by pyoverdine in Pseudomonas aeruginosa involves multiple regulators besides Fur, PvdS, and FpvI
Everything in excess is opposed to nature, even vitamin D: a case report
Mutation profiling of the c.1521_1523delCTT (p.Phe508del, F508del) CFTR allele using haplotype-resolved long-read next generation sequencing
Men’s sexual and reproductive health in cystic fibrosis in the era of highly effective modulator therapies-A qualitative study
CFTR modulator therapy alters plasma sphingolipid profiles in people with cystic fibrosis
Clinical outcomes in cystic fibrosis at 6 years of age with tricare insurance coverage
CONCERNS FOR PEOPLE WITH CYSTIC FIBROSIS (PWCF) WHEN TRAVELLING PRE COVID-19
A Survey: Understanding the Health and Perspectives of People with CF Not Benefiting from CFTR Modulators
Managing Pulmonary Infection in Adults With Cystic Fibrosis: Adult Cystic Fibrosis Series
Feasibility of Home-based Exercise Program for Adults With Cystic Fibrosis
Inflammation, Fibrosis and Cancer: Mechanisms, Therapeutic Options and Challenges
Intestinal Inflammation and Alterations in the Gut Microbiota in Cystic Fibrosis: A Review of the Current Evidence, Pathophysiology and Future Directions
Assays of CFTR Function In Vitro, Ex Vivo and In Vivo
Airway Delivery of Hydrogel-Encapsulated Niclosamide for the Treatment of Inflammatory Airway Disease
Unprecedented Epimerization of an Azithromycin Analogue: Synthesis, Structure and Biological Activity of 2′-Dehydroxy-5″-Epi-Azithromycin
How can the transition of adolescents from a children’s to an adult CF center be improved? Analysis of adolescents’ and parents’ needs during the post-transfer period
Guanylin and uroguanylin: a promising nexus in intestinal electrolyte and fluid homeostasis
CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system
Precision Medicine Based on CFTR Genotype for People with Cystic Fibrosis
REAL-world clinical effectiveness of ivacaftor therapy in the first 24 months in two infants with cystic fibrosis and different gating mutations-A case report
Biocompatible antimicrobial colistin loaded calcium phosphate nanoparticles for the counteraction of biofilm formation in cystic fibrosis related infections
Investigation of respiratory rate in patients with cystic fibrosis using a minimal-impact biomotion system
Cystic Fibrosis and Sleep Circadian Rhythms
A Qualitative Exploration into Experiences and Attitudes Regarding Psychosocial Challenges, Self-compassion, and Mindfulness in a Population of Adults with Cystic Fibrosis
Chasing a Breath of Fresh Air in Cystic Fibrosis (CF): Therapeutic Potential of Selective HDAC6 Inhibitors to Tackle Multiple Pathways in CF Pathophysiology
Changes in Care during the COVID-19 Pandemic for People with Cystic Fibrosis
The rapid reduction of infection-related visits and antibiotic use among people with cystic fibrosis after starting Elexacaftor-Tezacaftor-Ivacaftor
Editorial: Functional Characterization and Pharmaceutical Targets in Common and Rare CFTR Dysfunctions
Dynamic Upper and Lower Airway Microbiotas in Paediatric Bronchiectasis Exacerbations: A Pilot Study
The P2X7 Receptor in Cystic Fibrosis Monocytes: Linking CFTR Deficiency to Inflammation
Towards a new definition of non-cystic fibrosis bronchiectasis
Continuing Medical Education Questions: December 2021
Integrative pan cancer analysis reveals the importance of CFTR in lung adenocarcinoma prognosis
Implementing the use of objective medication adherence data in routine clinical practice via the digital CFHealthHub platform: situation analysis and strategy development using the theoretical domains framework
Is Positive Expiratory Pressure Physiotherapy an Effective Method for Airway Clearance in People With Cystic Fibrosis? A Cochrane Review Summary With Commentary
Hypervitaminosis A with fulminant secondary intracranial hypertension following personalized medicine-based Elexacaftor/Tezacaftor/Ivacaftor initiation in a preadolescent with cystic fibrosis

Pancreatic Insufficiency in a Child With p.Gly542* and c.2657+5G>A Heterozygote CFTR : A Case Report
Relationship Between the Development of Impaired Glucose Tolerance, the Phenotype of CFLD, and the Risk of Liver Fibrosis
Worldwide rates of diagnosis and effective treatment for cystic fibrosis
A Hyperglycemic Microenvironment Inhibits Tendon-to-Bone Healing through the let-7b-5p/CFTR Pathway
Seasonal variation of lung function in cystic fibrosis: longitudinal modeling to compare a Midwest US cohort to international populations
Influence of Comorbidities and Airway Clearance on Mortality and Outcomes of Patients With Severe Bronchiectasis Exacerbations in Taiwan
Engaging Stakeholders in the Development of a Reproductive Goals Decision AID for Women with Cystic Fibrosis
Leveraging TOPMed imputation server and constructing a cohort-specific imputation reference panel to enhance genotype imputation among cystic fibrosis patients
Study to Assess the Safety and PK of Oral and IV Xenleta in Adults With Cystic Fibrosis
Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis
Synthesis and bioactivity of readily hydrolysable novel cationic lipids for potential lung delivery application of mRNAs
Human papillomavirus (HPV) and cervical dysplasia in adult female cystic fibrosis (CF) lung transplant recipients
Preliminary method for profiling volatile organic compounds in breath that correlate with pulmonary function and other clinical traits of subjects diagnosed with cystic fibrosis: a pilot study
Genetic analysis and intracytoplasmic sperm injection outcomes of Chinese patients with congenital bilateral absence of vas deferens
Gastroparesis in Cystic Fibrosis
Static mucus impairs bacterial clearance and allows chronic infection with Pseudomonas aeruginosa in the cystic fibrosis rat
Identification and selection of healthy spermatozoa in heterozygous carriers of the Phe508del-variant of the CFTR-gene in assisted reproduction
Nutrition management in adults with cystic fibrosis
When is cystic fibrosis not cystic fibrosis? The importance of appropriately classifying patients
Genomic diversity and antimicrobial resistance of Prevotella species isolated from chronic lung disease airways
Porphyromonas pasteri and Prevotella nanceiensis in the sputum microbiota are associated with increased decline in lung function in individuals with cystic fibrosis
Hybrid Lipid/Polymer Nanoparticles to Tackle the Cystic Fibrosis Mucus Barrier in siRNA Delivery to the Lungs: Does PEGylation Make the Difference?
Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype
The Predictive Role of Psychological Status and Disease Severity Indexes on Quality of Life Among Patients with Non-CF Bronchiectasis
Genetic and Clinical Demographics of Adult Cystic Fibrosis Patients in a Middle Eastern Population
Maternal and Fetal Problems in Patients with Non-Cystic Fibrosis Bronchiectasis During Pregnancy
The Frequency of Obstructive Sleep Apnea in Patients with Non-cystic Fibrosis Bronchiectasis
Association Between Cystic Fibrosis Severity Markers and CFTR Genotypes in Turkish Children
Food Insecurity and Mental Health During the COVID-19 Pandemic in Cystic Fibrosis Households
New concepts in the pathogenesis of cystic fibrosis-related diabetes
Measuring the effect of elexacaftor/tezacaftor/ivacaftor combination therapy on the respiratory pump in people with CF using dynamic chest radiography
Functionally Additive Fixed Positive and Negative Charges in the CFTR Channel Pore Control Anion Binding and Conductance
The Success of the Cystic Fibrosis Registry of Turkey for Improvement of Patient Care
Pseudomonas aeruginosa adaptation in cystic fibrosis patients increases C5a levels and promotes neutrophil recruitment
Early Growth Response 1 Suppresses Macrophage Phagocytosis by Inhibiting NRF2 Activation Through Upregulation of Autophagy During Pseudomonas aeruginosa Infection
What Is Most Suitable for Children With Cystic Fibrosis-The Relationship Between Spirometry, Oscillometry, and Multiple Breath Nitrogen Washout
Assessing the Utility of an Outpatient Exercise Program for Children With Cystic Fibrosis: A Quality Improvement Project
Role of Salivary Biomarkers in Cystic Fibrosis: A Systematic Review
Impaired Regulatory Volume Decrease and Characterization of Underlying Volume-Activated Currents in Cystic Fibrosis Human Cholangiocyte Cell Line
The First 4 Years – Outcome of Children Identified by Newborn Screening for CF in Germany
Generation of human induced pluripotent stem cells from cystic fibrosis patient carrying nonsense mutation (p.S308X) in CFTR gene
PROBIOTIC USE IN ADULTS WITH CYSTIC FIBROSIS IS COMMON AND INFLUENCED BY GASTROINTESTINAL HEALTH NEEDS: A CROSS SECTIONAL SURVEY STUDY
Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis
Cystic fibrosis foundation otolaryngology care multidisciplinary consensus recommendations
Evaluating the alginate oligosaccharide (OligoG) as a therapy for Burkholderia cepacia complex cystic fibrosis lung infection
Bone mineral density screening by DXA for people with cystic fibrosis: A registry analysis of patient and program factors influencing rates of screening
Radiomics derived morphological features predict pulmonary function response under lumacaftor-ivacaftor in patients with cystic fibrosis
Forskolin-induced Organoid Swelling is Associated with Long-term CF Disease Progression
Fecal Calprotectin and Phenotype Severity in Patients with Cystic Fibrosis: A Systematic Review and Meta-Analysis
The Challenges of Eating Well for People Living with Cystic Fibrosis: an Interview Study Exploring the Use of Mindful Eating Approaches and Behaviours to Support Optimal Nutritional Status
Pseudomonas aeruginosa cytochrome P450 CYP168A1 is a fatty acid hydroxylase that metabolizes arachidonic acid to the vasodilator 19-HETE
Investigating Nontuberculous Mycobacteria Transmission at the Colorado Adult Cystic Fibrosis Program
Clinical Phenotypes of Cystic Fibrosis Carriers
Measuring the Impact of an Empiric Antibiotic Algorithm for Pulmonary Exacerbation in Children and Young Adults with Cystic Fibrosis
Ultrasound-Derived Diaphragm Contractile Reserve as a Marker of Clinical Status in Patients With Cystic Fibrosis
Roscovitine Worsens Mycobacterium abscessus Infection by Reducing DUOX2-Mediated Neutrophil Response
Type 2 inflammation in cystic fibrosis: New insights
Discovery of Novel Inhibitors of Uridine Diphosphate-N-Acetylenolpyruvylglucosamine Reductase (MurB) from Pseudomonas aeruginosa, an Opportunistic Infectious Agent Causing Death in Cystic Fibrosis Patients
Combined Host- and Pathogen-Directed Therapy for the Control of Mycobacterium abscessus Infection
Introducing a MAP for adherence care in the paediatric cystic fibrosis clinic: a multiple methods implementation study
The lung microbiota in children with cystic fibrosis captured by induced sputum sampling
Mycobacterium abscessus drug discovery using machine learning
International Approaches to Management of CFTR-Related Metabolic Syndrome/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis
Poly (acetyl, arginyl) glucosamine disrupts Pseudomonas aeruginosa biofilms and enhances bacterial clearance in a rat lung infection model
Computational investigations on the potential role of hygrophorones as quorum sensing inhibitors against LasR protein of Pseudomonas aeruginosa
Determinants of Daily Physical Activity Level in Patients With Cystic Fibrosis – Pilot Study Protocol
Inhibiting Mycobacterium abscessus Cell Wall Synthesis: Using a Novel Diazabicyclooctane β-Lactamase Inhibitor To Augment β-Lactam Action
Cystic Fibrosis: A Disease in Transformation, Yet More Work to be Done!
Cytotoxic activity of Staphylococcus aureus isolates from a cohort of Mexican children with cystic fibrosis
Mucoid Coating Provides a Growth Advantage to Pseudomonas aeruginosa at Oil-Water Interfaces
Molecular Mechanisms of Staphylococcus and Pseudomonas Interactions in Cystic Fibrosis
Sustained Reduction in Time to Data Entry in the Cystic Fibrosis Foundation Registry
Molecular dynamics and functional characterization of I37R-CFTR lasso mutation provide insights into channel gating activity
A flexible summary statistics-based colocalization method with application to the mucin cystic fibrosis lung disease modifier locus
Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains
Lived experiences of individuals with cystic fibrosis on CFTR-modulators
Cystic fibrosis: a call for papers for ECFS 2022
Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme
Aquagenic wrinkling of the palms in cystic fibrosis patients treated with ivacaftor
The Effect of Dynasore Upon the Negative Interaction Between ENaC and CFTR Channels in Xenopus laevis Oocytes
Five cases of missed cystic fibrosis heterozygous mutations identified after a positive newborn screen on a sibling
Treatment of cystic fibrosis related bone disease
Weight loss and cystic disease progression in autosomal dominant polycystic kidney disease
Yield of chest computed tomography angiogram in cystic fibrosis patients with suspected pulmonary embolism
Lipid-driven CFTR clustering is impaired in CF and restored by corrector drugs
Fat-Soluble Vitamins in Standard vs. Liposomal Form Enriched with Vitamin K2 in Cystic Fibrosis: A Randomized Multi-Center Trial
Correction: Dagenais et al. Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review. J. Clin. Med. 2021, 10, 23
First Case of COVID-19 Treated with Monoclonal Anti-Spike Antibodies in a Patient with Cystic Fibrosis in Romania
Therapeutic CFTR Correction Normalizes Systemic and Lung-Specific S1P Level Alterations Associated with Heart Failure
Activation of Notch3 in Renal Tubular Cells Leads to Progressive Cystic Kidney Disease
The Use of Targeted Monoclonal Antibodies in the Treatment of ABPA-A Case Series
Aerosol-Mediated Non-Viral Lung Gene Therapy: The Potential of Aminoglycoside-Based Cationic Liposomes
The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis
National multi-centre study found a low prevalence of severely impaired lung function in children and adolescents
Case Report: Japanese Siblings of Cystic Fibrosis With a Novel Large Heterozygous Deletion in the CFTR Gene
Review article: epidemiology, pathogenesis and management of liver disease in adults with cystic fibrosis
Cellular and molecular architecture of submucosal glands in wild-type and cystic fibrosis pigs
Formation of Calprotectin-Derived Peptides in the Airways of Children with Cystic Fibrosis
Body Mass Index Recovery Following Lung Transplant for Cystic Fibrosis
The physical therapy consultation: A qualitative study of the experience of parents of infants with cystic fibrosis in Australia

Malignancies in patients with cystic fibrosis: a case series
Simulated intravenous versus inhaled tobramycin with and without intravenous ceftazidime evaluated against hypermutable Pseudomonas aeruginosa via a dynamic biofilm model and mechanism-based modeling
ADHERENCE TO INHALED THERAPIES OVER 4 YEARS IN PEOPLE WITH CYSTIC FIBROSIS
Nasal epithelial cells as a gold-standard predictive model for personalized medicine in cystic fibrosis
Development of Inhibitors of SAICAR Synthetase (PurC) from Mycobacterium abscessus Using a Fragment-Based Approach
Metabolic reprogramming in a slowly developing orthologous model of polycystic kidney disease
Challenging the paradigm: moving from umbrella labels to treatable traits in airway disease
Cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)
Progress in precision medicine in cystic fibrosis: a focus on CFTR modulator therapy
Preferred health outcome states following treatment for pulmonary exacerbations of cystic fibrosis
The role of inspiratory capacity and tidal flow in diagnosing exercise ventilatory limitation in Cystic Fibrosis
The genetics of autosomal recessive polycystic kidney disease (ARPKD)
Geographic distribution and phenotype of European people with cystic fibrosis carrying A1006E mutation
Elevation of alkaline phosphatase and long-term drug therapy for cystic fibrosis
Abdominal Surgical Procedures in Adult Patients with Cystic Fibrosis: What are the Risks?
Bone Marrow Transplantation Rescues Monocyte Recruitment Defect and Improves Cystic Fibrosis in Mice
Role and mechanism(s) of incretin-dependent therapies for treating diabetes mellitus
An LC-MS/MS method for simultaneous analysis of the cystic fibrosis therapeutic drugs colistin, ivacaftor and ciprofloxacin
Swyer-James syndrome: A cause of adult-onset dyspnea in a patient with adult polycystic kidney disease
Contemporary N(2) and SF(6) multiple breath washout in infants and toddlers with cystic fibrosis
Role of AxyABM overexpression in acquired resistance in Achromobacter xylosoxidans
Successful non-surgical treatment of pseudomonas choroidal abscess in cystic fibrosis with previous double lung transplantation
The future of dry powder inhaled therapy: Promising or Discouraging for systemic disorders?
Advanced detection and sensing strategies of Pseudomonas aeruginosa and quorum sensing biomarkers: A review
Bacterial low-abundant taxa are key determinants of a healthy airway metagenome in the early years of human life
Cystic fibrosis related liver disease and endocrine considerations
Multilocus Sequence Typing Reveals Extensive Genetic Diversity of the Emerging Fungal Pathogen Scedosporium aurantiacum
Sequence diversity of the Pseudomonas aeruginosa population in loci that undergo microevolution in cystic fibrosis airways
Stage-Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function
Laparoscopic morphological aspects and tentative explanation of the aetiopathogenesis of isolated endometriosis of the sciatic nerve: a review based on 267 patients
Trikafta Rescues CFTR and Lowers Monocyte P2X7R-Induced Inflammasome Activation in Cystic Fibrosis
The effect of Control IQ hybrid closed loop technology on glycemic control in adolescents and adults with cystic fibrosis related diabetes
Receptor-mediated activation of CFTR via prostaglandin signaling pathways in the airway
The Changing Face of Cystic Fibrosis: An Update for Anesthesiologists
Diagnostic Uncertainty, Microbes, and the Isolation of People with Cystic Fibrosis
Vitamins A, D, E status as related to supplementation and lung disease markers in young children with cystic fibrosis
Exon-skipping antisense oligonucleotides for cystic fibrosis therapy
Open reading frame correction using splice-switching antisense oligonucleotides for the treatment of cystic fibrosis
Environment in the lung of cystic fibrosis patients stimulates the expression of biofilm phenotype in Mycobacterium abscessus
What Is Cystic Fibrosis?
Annual decline in lung function in adults with sickle cell disease is similar to that observed in adults with cystic fibrosis
Report of two events of nosocomial outbreak and pseudo-outbreak due to contamination with Achromobacter spp
Trabecular bone score in people with cystic fibrosis
Rare Trafficking CFTR Mutations Involve Distinct Cellular Retention Machineries and Require Different Rescuing Strategies
Bicarbonate Transport in Cystic Fibrosis and Pancreatitis
Rescue of Mutant CFTR Trafficking Defect by the Investigational Compound MCG1516A
Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation
Correlation between clinical-functional parameters and number of lobes involved in non-cystic fibrosis bronchiectasis
Treatment of Polarized Cystic Fibrosis Airway Cells With HGF Prevents VX-661-Rescued F508del-CFTR Destabilization Caused by Prolonged Co-exposure to VX-770
POINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? Yes
COUNTERPOINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? No
Revisiting a diagnosis of cystic fibrosis – Uncertainties and considerations
Exploring provider attitudes and perspectives related to men’s health in cystic fibrosis
Focusing on powder processing in Dry Powder Inhalation product development, manufacturing and performance
Understanding motivation for Australian adolescents and young adults with cystic fibrosis: Modifiable factors to support self-management
Mechanism of CFTR correction by type I folding correctors
CFTR variants are associated with chronic bronchitis in smokers
Scrutiny of Metal Ion Binding Sites in Different Alginate Lyases through In Silico Analysis
CFTR deficiency aggravates Ang II induced vasoconstriction and hypertension by regulating Ca2+ influx and RhoA/Rock pathway in VSMCs
What is the role of Achromobacter species in patients with cystic fibrosis?
Cardiac involvement in cystic fibrosis evaluated using cardiopulmonary magnetic resonance
Neutrophil respiratory burst activity is not exaggerated in cystic fibrosis
Systems Approaches to Unravel Molecular Function: High-content siRNA Screen Identifies TMEM16A Traffic Regulators as Potential Drug Targets for Cystic Fibrosis
The Pseudomonas aeruginosa homeostasis enzyme AlgL clears the periplasmic space of accumulated alginate during polymer biosynthesis
Evaluation of the safety of cefepime prolonged infusions in pediatric patients with cystic fibrosis
The Effects of Sub-inhibitory Antibiotic Concentrations on Pseudomonas aeruginosa : Reduced Susceptibility Due to Mutations
CF and male health: Sexual and reproductive health, hypogonadism, and fertility
Carbonic anhydrase and soluble adenylate cyclase regulation of cystic fibrosis cellular phenotypes
An update on methods for assessing bone quality and health in Cystic fibrosis
Gestational and pregestational diabetes in pregnant women with cystic fibrosis
Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe
Utilizing in silico and in vitro methods to identify possible binding sites of a novel ligand against Pseudomonas aeruginosa phospholipase toxin ExoU
Cystic Fibrosis Transmembrane Conductance Regulator: Roles in Chronic Obstructive Pulmonary Disease
Prenatal imaging features and perinatal outcomes of foetal volvulus-A literature review
Culture and Imaging of Human Nasal Epithelial Organoids
The nutritional environment is sufficient to select coexisting biofilm and quorum-sensing mutants of Pseudomonas aeruginosa
Depressive symptoms in cystic fibrosis patients and their caretakers are best predicted by their respective sense of belonging
An Overview on the Upper and Lower Airway Microbiome in Cystic Fibrosis Patients
The role of genetic modifiers, inflammation and CFTR in the pathogenesis of Cystic fibrosis related diabetes
Use of estrogen supplementation is associated with higher quality of life scores in women with cystic fibrosis
Non-invasive tools for detection of liver disease in children and adolescents with cystic fibrosis
Case report of compound CFTR variants in Korean siblings with cystic fibrosis: importance of differentiating cystic fibrosis from inflammatory bowel disease
The sodium/glucose cotransporters as potential therapeutic targets for CF lung diseases revealed by human lung organoid swelling assay
What’s in a name: The importance of lung transplant at Cystic Fibrosis Foundation Accredited Care Centers for patients with Cystic Fibrosis
Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation
Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis
Lung nodules due to Candida parapsilosis in a person with cystic fibrosis
Case Report: Embolization of the Left Atrial Coronary Artery in Cystic Fibrosis for Control of Massive Hemoptysis due to Coronary to Bronchial Artery Communication
Esc peptides as novel potentiators of defective cystic fibrosis transmembrane conductance regulator: an unprecedented property of antimicrobial peptides
Trends in Cystic Fibrosis survival over 40 years in South Africa: an observational cohort study
Opportunity for pharmacogenomic testing in patients with cystic fibrosis
RISK FACTORS FOR OBSTRUCTIVE SLEEP APNEA IN CYSTIC FIBROSIS
Variable Susceptibility to Gallium Compounds of Major Cystic Fibrosis Pathogens
Genetic counseling access for parents of newborns who screen positive for cystic fibrosis: consensus guidelines
Pediatric Pulmonology 2020 Year in Review: Rare and Diffuse Lung Disease
Six minute walk test in Italian children with cystic fibrosis aged 6 and 11
Remotely Supervised Exercise for Adults With Cystic Fibrosis
Cystic Fibrosis in Children: A Pediatric Anesthesiologist’s Perspective
Deposition of Inhaled Levofloxacin in Cystic Fibrosis Lungs Assessed by Functional Respiratory Imaging
Probing Allosteric Hsp70 Inhibitors by Molecular Modelling Studies to Expedite the Development of Novel Combined F508del CFTR Modulators

Vitamin D Status in Pediatric and Young Adult Cystic Fibrosis Patients. Are the New Recommendations Effective?
Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood
Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial
Safety and pharmacokinetics of Roscovitine (Seliciclib) in cystic fibrosis patients chronically infected with Pseudomonas aeruginosa, a randomized, placebo-controlled study
Structural lung disease in preschool children with cystic fibrosis: An 18 month natural history study
Comprehensive Analysis of Chemical Structures That Have Been Tested as CFTR Activating Substances in a Publicly Available Database CandActCFTR
Long-term macrolide therapy in asthma
Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies
Portuguese adolescents with cystic fibrosis and their parents: An intervention proposal for nursing clinical practice
Quality improvement and rapid PDSA cycles to maintain routine surveillance of pulmonary pathogens during the COVID-19 pandemic in a pediatric cystic fibrosis clinic
Gamma-glutamyl transpeptidase-to-platelet ratio as a biomarker of liver disease and hepatic fibrosis severity in paediatric Cystic Fibrosis
Clinical complications in children with false-negative results in cystic fibrosis newborn screening
Bactericidal effects and stability of LL-37 and CAMA in the presence of human lung epithelial cells
Specialized pro-resolving mediators in respiratory diseases
A disorder clinically resembling cystic fibrosis caused by biallelic variants in the AGR2 gene
Genome-Wide RNAi Screening Identifies Novel Pathways/Genes Involved in Oxidative Stress and Repurposable Drugs to Preserve Cystic Fibrosis Airway Epithelial Cell Integrity
Standards of Nutritional Care for Patients with Cystic Fibrosis: A Methodological Primer and AGREE II Analysis of Guidelines
New Auranofin Analogs with Antibacterial Properties against Burkholderia Clinical Isolates
Potential Therapeutic Targets for Combination Antibody Therapy against Pseudomonas aeruginosa Infections
A Polyclonal Antibody Raised against the Burkholderia cenocepacia OmpA-like Protein BCAL2645 Impairs the Bacterium Adhesion and Invasion of Human Epithelial Cells In Vitro
Revisiting the Role of Leukocytes in Cystic Fibrosis
High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids
Immunoglobulin A Mucosal Immunity and Altered Respiratory Epithelium in Cystic Fibrosis
Causes of Exocrine Pancreatic Insufficiency Other Than Chronic Pancreatitis
Genetic Modifying Factors of Cystic Fibrosis Phenotype: A Challenge for Modern Medicine
The Need for Precision Therapies as Determined by Genetic Signature for Cystic Fibrosis
Prediction of Real-World Long-Term Outcomes of People with CF Homozygous for the F508del Mutation Treated with CFTR Modulators
In Vitro Activity of 22 Antibiotics against Achromobacter Isolates from People with Cystic Fibrosis. Are There New Therapeutic Options?
Evaluation of Three Culture Media for Isolation of Burkholderia cepacia Complex from Respiratory Samples of Patients with Cystic Fibrosis
Revisiting CFTR Interactions: Old Partners and New Players
Advanced but not mild liver disease is a predictor of decreased survival in children with cystic fibrosis, with far greater impact in females: A 27-year real-life cohort study
Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward
Outcomes following lung re-transplantation in patients with cystic fibrosis
SARS-CoV-2 antibodies among people with cystic fibrosis prior to the vaccination campaign: A seroprevalence study in two specialized centres in Northern Italy
Comparison of the airway microbiota in children with chronic suppurative lung disease
Flagellin From Pseudomonas aeruginosa Modulates SARS-CoV-2 Infectivity in Cystic Fibrosis Airway Epithelial Cells by Increasing TMPRSS2 Expression
Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial
Comparison of biosimilar Tigerase and Pulmozyme in long-term symptomatic therapy of patients with cystic fibrosis and severe pulmonary impairment (subgroup analysis of a Phase III randomized open-label clinical trial (NCT04468100))
MutTMPredictor: Robust and accurate cascade XGBoost classifier for prediction of mutations in transmembrane proteins
Prospects of Inhaled Phage Therapy for Combatting Pulmonary Infections
A Volatile and Dynamic Longitudinal Microbiome Is Associated With Less Reduction in Lung Function in Adolescents With Cystic Fibrosis
Family planning, pregnancy and birth in women with lung conditions: a worldwide survey
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles
Coming Up for Air: The Role of Anaerobes in Cystic Fibrosis
Ceftaroline Versus Vancomycin for the Treatment of Acute Pulmonary Exacerbations of Cystic Fibrosis in Adults
Health-Related Quality of Life Assessment: An Inexpensive Tool for Cystic Fibrosis Care
Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis
Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis
Phosphodiesterase 8A Regulates CFTR Activity in Airway Epithelial Cells
Cystic Fibrosis Mucus Model to Design More Efficient Drug Therapies
Cystic fibrosis: current concepts
The Impact of COVID-19 in Cystic Fibrosis
The Management of Cystic Fibrosis Chronic Rhinosinusitis: An Evidenced-Based Review with Recommendations
Comparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis
Long-term macrolide treatment for non-cystic fibrosis bronchiectasis in children: a meta-analysis
Mutations in CFTR genes are associated with oligoasthenospermia in infertile men undergoing IVF
Impact of the COVID-19 pandemic: How our response is shaping the future of cystic fibrosis care
Financial impacts of the COVID-19 pandemic on cystic fibrosis care: lessons for the future
Financial impacts of the COVID-19 pandemic on cystic fibrosis care: Lessons for the future (Commentary) A.L. Stephenson
Barriers and facilitators to implementing telehealth services during the COVID-19 pandemic: A qualitative analysis of interviews with cystic fibrosis care team members
Design and methods for understanding the state of cystic fibrosis care amid the COVID-19 pandemic
Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis
Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis (Commentary)
Patient and family experience of telehealth care delivery as part of the CF chronic care model early in the COVID-19 pandemic
Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors
Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network
Evaluating barriers to and promoters of telehealth during the COVID-19 pandemic at U.S. cystic fibrosis programs
The effect of the cystic fibrosis care center on outcomes after lung transplantation for cystic fibrosis
Healthcare-associated links in transmission of nontuberculous mycobacteria among people with cystic fibrosis (HALT NTM) study: Rationale and study design
Cystic fibrosis related diabetes (CFRD) prognosis
Cystic fibrosis-related diabetes: The patient perspective
A growing trend: CFTR modulators for cystic fibrosis lung transplant recipients
Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers
Low body mass index as a barrier to lung transplant in cystic fibrosis
Magnetic resonance imaging of the gastrointestinal tract shows reduced small bowel motility and altered chyme in cystic fibrosis compared to controls
Developing Ciprofloxacin Dry Powder for Inhalation: A Story of Challenges and Rational Design in the Treatment of Cystic Fibrosis Lung Infection
Clinical Outcomes of Cystic Fibrosis Patients with Pseudomonas aeruginosa Bloodstream Infections
Management of Multidrug Resistant Infections in Lung Transplant Recipients with Cystic Fibrosis
Eating disorders and body image in cystic fibrosis
The role of modulators in cystic fibrosis related diabetes
Cystic fibrosis-related diabetes: Prevalence, screening, and diagnosis
Impact of Achromobacter spp. Isolation on Clinical Outcomes in Children with Cystic Fibrosis
Evaluation of a novel CFTR potentiator in copd ferrets with acquired cftr dysfunction
The effect of danusertib, an Aurora kinase inhibitor, onto the cytotoxicity, cell cycle and apoptosis in pancreatic ductal adenocarcinoma cells
Perception, experience and relationship with food and eating in adults with cystic fibrosis
Gene mutations in congenital bilateral absence of the vas deferens: An update
Cross-talk of inflammatory mediators and airway epithelium reveals the cystic fibrosis transmembrane conductance regulator as a major target
Acute ST-elevation myocardial infarction in two young women with cystic fibrosis and cystic fibrosis-related diabetes
Autogenic drainage for airway clearance in cystic fibrosis
Major Decrease in Lung Transplantation for Patients with Cystic Fibrosis in France
The Controversy of Drug Hypersensitivity in Patients with Cystic Fibrosis and Review of the Literature
Olfactory dysfunction in people with cystic fibrosis with at least one copy of F508del
Circadian Rhythmicity in Cerebral Microvascular Tone Influences Subarachnoid Hemorrhage-Induced Injury
The journal of cystic fibrosis’ 20th anniversary
Home monitoring for cystic fibrosis: The future is now
The Relationship Between CFTR Gene Mutations and Exercise Capacity
Cystic fibrosis associated liver disease in children
Food protein-induced enterocolitis syndrome in a patient with pseudo-Bartter syndrome associated with cystic fibrosis. A case report
Combining Ivacaftor and Intensive Antibiotics Achieves Limited Clearance of Cystic Fibrosis Infections
Increased susceptibility of cystic fibrosis airway epithelial cells to ferroptosis

Forewarned is forearmed: The cardiovascular time bomb in Cystic Fibrosis
Meteorological Factors Influence the Presence of Fungi in the Air; A 14-Month Surveillance Study at an Adult Cystic Fibrosis Center
Patient and Provider Experience With Cystic Fibrosis Telemedicine Clinic
Cystic Fibrosis: Systems Biology Analysis from Homozygous p.Phe508del Variant Patients’ Samples Reveals Perturbations in Tissue-Specific Pathways
Genetic determinants of virulence and antibiotic resistance are common for Pseudomonas aeruginosa ST235 isolates from cystic fibrosis patients from various geographical regions
In vitro differentiation of ciliated cells in ALI-cultured human airway epithelium – The framework for functional studies on airway differentiation in ciliopathies
Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis
Hepatic inflammation and liver fibrogenesis: a potential target for the treatment of cystic echinococcosis-associated hepatic injury
Real-World Outcomes in Cystic Fibrosis Telemedicine Clinical Care in a Time of a Global Pandemic
Evaluation of Long-term Safety and Efficacy of ELX/TEZ/IVA in Cystic Fibrosis (CF) Participants 2 Years and Older
Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis
CFTR mutational screening by next-generation sequencing reveals novel variants and a high carrier rate in a Middle Eastern population
Clinical validation of digital biomarkers for pediatric patients with asthma and cystic fibrosis – Potential for clinical trials and clinical care
Synergy in Cystic Fibrosis Therapies: Targeting SLC26A9
Defining the Importance of Age-Related Changes in Drug Clearance to Optimizing Aminoglycoside Dosing Regimens for Adult Patients with Cystic Fibrosis
Environmental predictors of pulmonary nontuberculous mycobacteria (NTM) sputum positivity among persons with cystic fibrosis in the state of Florida
Role of the flagellar hook in the structural development and antibiotic tolerance of Pseudomonas aeruginosa biofilms
Nebulisation therapy in patients with cystic fibrosis – consensus of the Polish Cystic Fibrosis Society
Magnetic resonance imaging of cystic fibrosis: Multi-organ imaging in the age of CFTR modulator therapies
A new path for CF clinical trials through the use of historical controls
The safety and sustainability of bottle-pep therapy in pediatric patients with cystic fibrosis
Transcriptome analysis of Pseudomonas aeruginosa biofilm infection in an ex vivo pig model of the cystic fibrosis lung
Goblet Cell Hyperplasia is not Epithelial-Autonomous in the Cftr Knockout Intestine
Translating in vitro CFTR rescue into small molecule correctors for cystic fibrosis using the Library of Integrated Network-based Cellular Signatures drug discovery platform
Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes
Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes. Reply
Comparison of endoscopic sinus surgery timing in lung transplant patients with cystic fibrosis
Frequency of CFTR variants in southern Brazil and indication for modulators therapy in patients with cystic fibrosis
Clinical course and risk factors for severe COVID-19 among Italian patients with cystic fibrosis: a study within the Italian Cystic Fibrosis Society
Not a wild goose chase: long-lasting MRSA negative status following eradication therapy for chronic MRSA infection in patients with cystic fibrosis
Infant BMI or Weight-for-Length and Risk of Undernutrition in Childhood Among Children with Cystic Fibrosis
The Effect of Telerehabilitation on Functional Capacity, Oxidative Stress and Respiratory Parameters in Cystic Fibrosis
Molecular pathology of the R117H cystic fibrosis mutation is explained by loss of a hydrogen bond
Endobronchial Therapy With Gentamicin and Dexamethasone After Airway Clearance by Bronchoscopy in Exacerbation of Non-Cystic Fibrosis Bronchiectasis: A Real-World Observational Study
Editorial: Novel Anti-Inflammatory Approaches for Cystic Fibrosis Lung Disease: Identification of Molecular Targets and Design of Innovative Therapies
Acidic Microenvironment Determines Antibiotic Susceptibility and Biofilm Formation of Pseudomonas aeruginosa
miR-224-5p and miR-545-5p Levels Relate to Exacerbations and Lung Function in a Pilot Study of X-Linked MicroRNA Expression in Cystic Fibrosis Monocytes
Impaired glucose tolerance and indeterminate glycemia in cystic fibrosis
Obesity in cystic fibrosis
Long-Term Outcomes in Real Life of Lumacaftor-Ivacaftor Treatment in Adolescents With Cystic Fibrosis
Incidence of breast cancer in people with cystic fibrosis: A cause for concern?
Development and evaluation of a virtual patient-centered outcomes research training program for the cystic fibrosis community
Sweat metabolomics before and after intravenous antibiotics for pulmonary exacerbation in people with cystic fibrosis
Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis
Synchronous association of hepatocellular carcinoma and cystic echinococcosis with unusual pathology: report on diagnostic and pathological variability
Diagnosis and management of non-cystic fibrosis bronchiectasis
Burden of cystic fibrosis in children <12 years of age prior to the introduction of CFTR modulator therapies
Primary nasal epithelial cells from patients with cystic fibrosis hold promise for guiding precision medicine and expanding treatment
S-nitrosothiols signaling in cystic fibrosis airways
A Comparative Analysis of Pricing and Reimbursement of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Europe
Might Routine Vitamin A Monitoring in Cystic Fibrosis Patients Reduce Virus-Mediated Lung Pathology?
Editorial: Fungal Respiratory Infections in Cystic Fibrosis
Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients
Non-liver mRNA Delivery
The Genetic Analysis of Cystic Fibrosis Patients With Seven Novel Mutations in the CFTR Gene in the Central Anatolian Region of Turkey
Non-Pharmaceutical Techniques for Obstructive Airway Clearance Focusing on the Role of Oscillating Positive Expiratory Pressure (OPEP): A Narrative Review
25-Hydroxy vitamin D and body composition are associated with pulmonary function in non-cystic fibrosis bronchiectasis: A cross-sectional study
Association of site of treatment with clinical outcomes following intravenous antimicrobial treatment of a pulmonary exacerbation
Disconcerting and Counter-Intuitive Findings from a Trial of Exercise in Cystic Fibrosis- Can Exercise Make our Patients Worse?
Inquilinus limosus isolated from a patient with chronic cystic fibrosis. First report in Mexico and evidence that co-infection with Pseudomonas aeruginosa promotes the accelerated and increased formation of extracellular neutrophil traps
Nonspecific binding of common anti-CFTR antibodies in ciliated cells of human airway epithelium
Effects of Training Intensity on Physical Fitness and Body Composition in Cystic Fibrosis
Clinical characteristics and outcome of SARS -CoV-2 infection in patients with cystic fibrosis managed at home
Ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis
Delayed HIV diagnosis in a cystic fibrosis patient: Not just another exacerbation
Gynecologic health care for females with cystic fibrosis
Continuous Glucose Monitoring and HbA1c in Cystic Fibrosis: Clinical Correlations and Implications for CFRD Diagnosis
The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis post-liver transplant: a case series
CFTR limits F-actin formation and promotes morphological alignment with flow in human lung microvascular endothelial cells
Mycobacterium abscessus, a model of resistance to multiple antibiotic classes
A step back in time: The basics of CF care still matter!
Perceptions of barriers to and facilitators of physical activity in adults with cystic fibrosis
Molecular epidemiology and phylogenomic analysis of Mycobacterium abscessus clinical isolates in an Asian population
Emotional Impact of COVID-19 Pandemic on Adults with Cystic Fibrosis
Validity and reliability of the one-minute sit-to-stand test for the measurement of cardio-respiratory responses in children with cystic fibrosis
Cystic fibrosis: a diagnosis in an adolescent
Continuous glucose monitoring systems for monitoring cystic fibrosis-related diabetes
Validation of a Custom Next-Generation Sequencing Assay for Cystic Fibrosis Newborn Screening
Is mHealth a useful therapy for improving physical or emotional health in adolescents with cystic fibrosis? A systematic review
Urticaria multiforme-like eruption due to a novel agent elexacaftor/tezacaftor/ivacaftor in a pediatric patient with cystic fibrosis
Transplantation of human iPS cell-derived airway cells on vitrigel membrane into rat nasal cavity
Collection, Expansion, and Differentiation of Primary Human Nasal Epithelial Cell Models for Quantification of Cilia Beat Frequency
Cystic Fibrosis Newborn Screening: Five-year Experience from a Tertiary Care Center
Assessment of body composition in cystic fibrosis: agreement between skinfold measurement and densitometry
The bitter end: T2R bitter receptor agonists elevate nuclear calcium and induce apoptosis in non-ciliated airway epithelial cells
Evaluation of Pulmonary Structure and Function in Patients with Cystic Fibrosis from Electrical Impedance Tomography Data
Nano- and Macroscale Imaging of Cholesterol Linoleate and Human Beta Defensin 2-Induced Changes in Pseudomonas aeruginosa Biofilms
Sub-Inhibitory Antibiotic Exposure and Virulence in Pseudomonas aeruginosa
Integrity and Stability of PTC Bearing CFTR mRNA and Relevance to Future Modulator Therapies in Cystic Fibrosis
The Effects of Aspergillus fumigatus Colonization on Lung Function in Patients with Cystic Fibrosis
Lung and Gut Microbiota Changes Associated with Pseudomonas aeruginosa Infection in Mouse Models of Cystic Fibrosis
Putative RNA Ligase RtcB Affects the Switch between T6SS and T3SS in Pseudomonas aeruginosa
Methods Used to Evaluate the Immediate Effects of Airway Clearance Techniques in Adults with Cystic Fibrosis: A Systematic Review and Meta-Analysis
miR-125b Promotes Colorectal Cancer Migration and Invasion by Dual-Targeting CFTR and CGN
CFTR Protein: Not Just a Chloride Channel?
TMEM16A/ANO1: Current Strategies and Novel Drug Approaches for Cystic Fibrosis
The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
Apparent Yield Stress of Sputum as a Relevant Biomarker in Cystic Fibrosis
Impact of Airway Inflammation on the Efficacy of CFTR Modulators
Employment Status and Work Ability in Adults with Cystic Fibrosis

The Distribution and Role of the CFTR Protein in the Intracellular Compartments
Identification of cbiO Gene Critical for Biofilm Formation by MRSA CFSa36 Strain Isolated from Pediatric Patient with Cystic Fibrosis
Defective Glyoxalase 1 Contributes to Pathogenic Inflammation in Cystic Fibrosis
Anti-Virulence Properties of Coridothymus capitatus Essential Oil against Pseudomonas aeruginosa Clinical Isolates from Cystic Fibrosis Patients
Geospatial Analysis of Food Deserts and Their Impact on Health Outcomes in Children with Cystic Fibrosis
Vitamin D Status and Risk of Cystic Fibrosis-Related Diabetes: A Retrospective Single Center Cohort Study
Handgrip Strength: Associations with Clinical Variables, Body Composition, and Bone Mineral Density in Adults with Cystic Fibrosis
Genetic Evaluation of Pancreatitis
Genome evolution drives transcriptomic and phenotypic adaptation in Pseudomonas aeruginosa during 20 years of infection
Predictors of mortality in children with cystic fibrosis in India
Lung Transplant READY Pilot Study
A restructuring of microbiome niche space is associated with Elexacaftor-Tezacaftor-Ivacaftor therapy in the cystic fibrosis lung
Differentiation of Cystic Fibrosis-Related Pathogens by Volatile Organic Compound Analysis with Secondary Electrospray Ionization Mass Spectrometry
Consideration of height-based tobramycin dosing regimens for the treatment of adult cystic fibrosis pulmonary exacerbations
Optimising the care and quality of life of people with cystic fibrosis: the influence of cystic fibrosis transmembrane conductance regulator modulators
Discovery of inhibition of Burkholderia cenocepacia, Pseudomonas aeruginosa and Stenotrophomonas maltophilia by the Brown Rot Basidiomycete Fungus, Postia placenta
Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
A Clinician’s guide to vitamin D supplementation for patients with cystic fibrosis
The adverse association between stimulant use for attention deficit hyperactivity disorder (ADHD) and semen parameters
Physical Activity and Sedentary Behavior in Adults With Cystic Fibrosis: Association With Aerobic Capacity, Lung Function, Sleep, Well-Being, and Quality of Life
Parent-Collected Oropharyngeal Swabs in Cystic Fibrosis
Outcomes of Cystic Fibrosis Screening-Positive Infants With Inconclusive Diagnosis at School Age
Transitioning to telehealth during the coronavirus disease 2019 pandemic: Perspectives from partners of women with cystic fibrosis and healthcare providers
Effect of Salbutamol on Lung Ventilation in Children with Cystic Fibrosis: Comprehensive Assessment Using Spirometry, Multiple-Breath Washout, and Functional Lung Magnetic Resonance Imaging
Intravenous or oral antibiotic treatment in adults and children with cystic fibrosis and Pseudomonas aeruginosa infection: the TORPEDO-CF RCT
Hereditary Pancreatitis Related to SPINK-1 Mutation. Is There an Increased Risk of Developing Pancreatic Cancer?
Organic acids and their salts potentiate the activity of selected antibiotics against Pseudomonas aeruginosa biofilms grown in a synthetic cystic fibrosis sputum medium
Body composition in individuals with cystic fibrosis
Fertility preservation in women with cystic fibrosis pre-lung transplantation: A mixed methods study
Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes
Facilitators and barriers to empowerment in children and young people with cystic fibrosis: a meta-synthesis of the qualitative literature
Urinary sodium/creatinine ratio is a predictor for fractional sodium excretion and related to age in patients with cystic fibrosis
Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis
Approach and anesthetic management for kidney transplantation in a patient with bilateral lung transplantation: case report
Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype
Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip: Cystic fibrosis airway chip
Measurements of spontaneous CFTR-mediated ion transport without acute channel activation in airway epithelial cultures after modulator exposure
Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis
Surgical treatment of non-cystic fibrosis bronchiectasis in Central Europe
Right hepatectomy with a cholangiojejunostomy and hepaticojejunostomy for unilobar Caroli’s syndrome
Small-molecule drugs for cystic fibrosis: Where are we now?
A Phase 2a randomized, single-center, double-blind, placebo-controlled study to evaluate the safety and preliminary efficacy of oral iOWH032 against cholera diarrhea in a controlled human infection model
Aspergillus-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?
Abdominal Symptoms Assessed with the CFAbd-Score are Associated with Intestinal Inflammation in Patients with Cystic Fibrosis
An empirical comparison of segmented and stochastic linear mixed effects models to estimate rapid disease progression in longitudinal biomarker studies
Erratum to: Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance
Membrane Transporters of the Major Facilitator Superfamily Are Essential for Long-Term Maintenance of Phenotypic Tolerance to Multiple Antibiotics in E. coli
Management of Cystic Fibrosis during COVID-19: Patient Reported Outcomes based remote follow-up among CF patients in Denmark – A feasibility study
Clinical Effectiveness of Elexacaftor/Tezacftor/Ivacaftor in People with Cystic Fibrosis
Translation, cross-cultural adaptation and psychometric evaluation of the Brazilian version of the Cystic Fibrosis Knowledge Scale (CFKS)
CyFi-MAP: an interactive pathway-based resource for cystic fibrosis
Complete CFTR gene sequencing in 5,058 individuals with cystic fibrosis informs variant-specific treatment
Nontypeable Haemophilus influenzae infection impedes Pseudomonas aeruginosa colonization and persistence in mouse respiratory tract
Conjugation of Silver Nanoparticles with De Novo Engineered Cationic Antimicrobial Peptides: An Exploratory Proposal
Unique Features of Mycobacterium abscessus Biofilms Formed in Synthetic Cystic Fibrosis Medium
Circulating Fibrocyte Level in Children with Cystic Fibrosis
Quality of home spirometry performance amongst adults with cystic fibrosis
Emerging non-pulmonary complications for adults with cystic fibrosis
Genetic Variation in ABCC4 and CFTR and Acute Pancreatitis during Treatment of Pediatric Acute Lymphoblastic Leukemia
Lung Clearance Index in Children with Cystic Fibrosis during Pulmonary Exacerbation
Comprehensive Analysis of Combinatorial Pharmacological Treatments to Correct Nonsense Mutations in the CFTR Gene
Multi-Omics Study of Keystone Species in a Cystic Fibrosis Microbiome
Human Recombinant DNase I (Pulmozyme® ) Inhibits Lung Metastases in Murine Metastatic B16 Melanoma Model That Correlates with Restoration of the DNase Activity and the Decrease SINE/LINE and c-Myc Fragments in Blood Cell-Free DNA
Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge
Cystic Fibrosis Transmembrane Conductance Regulator Folding Mutations Reveal Differences in Corrector Efficacy Linked to Increases in Immature Cystic Fibrosis Transmembrane Conductance Regulator Expression
Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China
Telemedicine in cystic fibrosis
Final results of the southwest German pilot study on cystic fibrosis newborn screening – Evaluation of an IRT/PAP protocol with IRT-dependent safety net: Results of the Southwest German CFNBS pilot study
SPLUNC1 comes of age? Predicting acute exacerbations in cystic fibrosis
Epigenetic reprogramming of airway macrophages promotes polarization and inflammation in muco-obstructive lung disease
The challenges of managing thoracic pain in cystic fibrosis (CF)
Expanding clinical phage microbiology: simulating phage inhalation for respiratory tract infections
Predicting mortality in non-cystic fibrosis bronchiectasis patients using distance-saturation product
Correlating genotype with phenotype using CFTR-mediated whole-cell Cl- currents in human nasal epithelial cells
Unexpected associations between respiratory viruses and bacteria with Pulmonary Function Testing in children suffering from Cystic Fibrosis (MUCOVIB study)
When CFSPID becomes CF
CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the beta-adrenergic sweat rate assay
No drug-drug interaction between tezacaftor-ivacaftor and clofazimine: A case report
Methodological tools to study species of the genus Burkholderia
CFTR mutations causing congenital unilateral absence of the vas deferens (CUAVD) and congenital absence of the uterus (CAU) in a consanguineous family
The effectiveness of exercise interventions to increase physical activity in Cystic Fibrosis: A systematic review
Using negative control outcomes and difference-in-differences to estimate treatment effects in an entirely treated cohort: the effect of ivacaftor in cystic fibrosis
Exploring Associations Between Self-Compassion, Self-Criticism, Mental Health, and Quality of Life in Adults with Cystic Fibrosis: Informing Future Interventions
An Overview of Noninvasive Ventilation in Cystic Fibrosis
Spotlighting “Neutrophil Elastase Triggers the Release of Macrophage Extracellular Traps”: A New Catch in CF?
The determinants of survival among adults with cystic fibrosis-a cohort study
Spontaneous and iatrogenic hypoglycemia in cystic fibrosis
Altered Pseudomonas Strategies to Inhibit Surface Aspergillus Colonies
spa Typing and Molecular Characterization of Antimicrobial Resistance in Staphylococcus aureus Strains from Patients with Cystic Fibrosis
Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy
Fenretinide inhibits vitamin A formation from β-carotene and regulates carotenoid levels in mice
The Epidemiology and Pathogenesis and Treatment of Pseudomonas aeruginosa Infections: An Update
Lung ultrasound for the diagnosis of cystic fibrosis pulmonary exacerbation
A Study Evaluating the Safety of Elexacaftor/Tezacaftor/Ivacaftor in Participants With Cystic Fibrosis (CF)
The Immunomodulatory Effects of Macrolide Antibiotics in Respiratory Disease
Frequency of allele variations in the CFTR gene in a Mexican population
Treating nontuberculous mycobacteria in children with cystic fibrosis: a multicentre retrospective study
Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis
Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis
Overweight and Cystic Fibrosis: An Unexpected Challenge

Therapeutic education and cystic fibrosis
Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter Randomized Controlled Trial (ACTIVATE-CF)
CFTR modulators increase risk of acute pancreatitis in pancreatic insufficient patients with cystic fibrosis
Contrast Enhanced Magnetic Resonance Imaging Does Not Detect a Progression in Lung Morphological Score in Preschool Children with Cystic Fibrosis
Reply to: Contrast Enhanced Magnetic Resonance Imaging Does Not Detect a Progression in Lung Morphological Score in Preschool Children with Cystic Fibrosis
Obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment: a systematic review and meta-analysis of prevalence
Validation of a Spanish version of the Leicester Cough Questionnaire in cystic fibrosis
Toxic Epidermal Necrolysis Induced by Cystic Fibrosis Transmembrane Conductance Regulator modulators
Review of current and future therapeutics in ABPA
Decreased CFTR/PPARγ and increased transglutaminase 2 in nasal polyps
Arm muscle area for the longitudinal assessment of nutritional status in paediatric patients with cystic fibrosis – A single centre experience
Parent-Collected Oropharyngeal Swabs in Cystic Fibrosis
Visualization of Ectopic Serine Protease Activity by Förster Resonance Energy Transfer-Based Reporters
Renal transplantation in a patient with cystic fibrosis: a changing disease landscape
Current evidence on the effect of highly effective CFTR modulation on interleukin-8 in cystic fibrosis
MICROBIOLOGICAL FEATURES OF STAPHYLOCOCCUS AUREUS ISOLATED FROM RESPIRATORY TRACT OF CHILDREN WITH CYSTIC FIBROSIS
Metabolomic profiling of Burkholderia cenocepacia in synthetic cystic fibrosis sputum medium reveals nutrient environment-specific production of virulence factors
Need of gastrointestinal surgery in cystic fibrosis? Do not forget the chest!
Apixaban Causing Hepatic Cystic Bleeding: A Rare but a Life-Threatening Complication
Biofilm Formation in Methicillin-Resistant Staphylococcus aureus Isolated in Cystic Fibrosis Patients Is Strain-Dependent and Differentially Influenced by Antibiotics
Mapping Pulmonary and Systemic Inflammation in Preschool Aged Children With Cystic Fibrosis
COVID-19: Pathophysiology and Implications for Cystic Fibrosis, Diabetes and Cystic Fibrosis-Related Diabetes
Phase II Study of a Nebulised Nitric Oxide Generating Solution in Patients With Cystic Fibrosis
Analysis of multiple gene co-expression networks to discover interactions favoring CFTR biogenesis and ΔF508-CFTR rescue
Gene therapy for cystic fibrosis: new tools for precision medicine
Rugose small colony variant and its hyper-biofilm in Pseudomonas aeruginosa: Adaption, evolution, and biotechnological potential
Pretransplant Quality of Life and Post-Transplant Survival in Adolescents with Cystic Fibrosis
Age-Related Progression of Microvascular Dysfunction in Cystic Fibrosis: New Detection Ways and Clinical Outcomes
Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR
Pseudomonas aeruginosa: an antibiotic resilient pathogen with environmental origin
Non-nuberculous mycobacteria infection treated with intermittently inhaled high-dose nitric oxide
Validation of Therapeutic Efficacy Targeting the Splicing Variants in Cystic Fibrosis and CFTR Pathologies
A Developmental Role of the Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Lung Disease Pathogenesis
One more piece in the pancreatic chloride puzzle
Olfaction before and after initiation of elexacaftor-tezacaftor-ivacaftor in a cystic fibrosis cohort
Testing the effects of combining azithromycin with inhaled tobramycin for P. aeruginosa in cystic fibrosis: a randomised, controlled clinical trial
Respiratory syncytial virus persistent infection causes acquired CFTR dysfunction in human bronchial epithelial cells
Looking Toward the Future: Approaching Care of the Aging CF Patient
Ivacaftor restores delayed mucociliary transport caused by Pseudomonas aeruginosa-induced acquired cystic fibrosis transmembrane conductance regulator dysfunction in rabbit nasal epithelia
A Study of Inhaled KB407 for the Treatment of Cystic Fibrosis
50 Years Ago in TheJournalofPediatrics: Carbenicillin and Cystic Fibrosis
Impact of CFTR large deletions and insertions on the clinical and laboratory severity of cystic fibrosis: a serial case report
Achieving respiratory excellence in pre-school children with cystic fibrosis
Association of bacterial community types, functional microbial processes and lung disease in cystic fibrosis airways
Bacterial Subversion of Autophagy in Cystic Fibrosis
Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease with the Phe508del/minimal function genotype
Genetic mutation analysis of 22 patients with congenital absence of vas deferens: A single-center study†
Effects of Tham Nasal Alkalinization on Airway Microbial Communities: A Pilot Study in Non-CF and CF Adults
Clinical characteristics and outcomes of non-cystic fibrosis patients with Burkholderia cepacia complex bacteremia at a medical center in Taiwan
Understanding the Pancreatic Islet Microenvironment in Cystic Fibrosis and the Extrinsic Pathways Leading to Cystic Fibrosis Related Diabetes
A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients
Cystic fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies
A model for building a national, patient-driven database to track contraceptive use in women with rare diseases
NBD2 Is Required for the Rescue of Mutant F508del CFTR by a Thiazole-Based Molecule: A Class II Corrector for the Multi-Drug Therapy of Cystic Fibrosis
Dysfunction in the Cystic Fibrosis Transmembrane Regulator in Chronic Obstructive Pulmonary Disease as a Potential Target for Personalised Medicine
Pseudomonas aeruginosa mexR and mexEF Antibiotic Efflux Pump Variants Exhibit Increased Virulence
Effect of N-Acetylcysteine in Combination with Antibiotics on the Biofilms of Three Cystic Fibrosis Pathogens of Emerging Importance
Consistent Assignment of Risk and Benign Allele at rs2303153 in the CF Modifier Gene SCNN1B in Three Independent F508del-CFTR Homozygous Patient Populations
Forced Oscillation Technique for Monitoring the Respiratory Status of Children with Cystic Fibrosis: A Systematic Review
Effect of Low Amperage Electric Current on Staphylococcus Aureus-Strategy for Combating Bacterial Biofilms Formation on Dental Implants in Cystic Fibrosis Patients, In Vitro Study
Therapeutic Approaches for Patients with Cystic Fibrosis Not Eligible for Current CFTR Modulators
Adaptation and genomic erosion in fragmented Pseudomonas aeruginosa populations in the sinuses of people with cystic fibrosis
Sleep in children and young adults with cystic fibrosis
Adherence to CF treatment can be improved with the right approach!
Clinical characteristics of people with cystic fibrosis and frequent fungal infection
Comparison of inhaled mannitol/dornase alfa combination and daily dornase alfa alone in children with cystic fibrosis
Antimicrobial effects of Melaleuca alternifolia (tea tree) essential oil against biofilm-forming multi-drug resistant cystic fibrosis-associated Pseudomonas aeruginosa as a single agent and in combination with commonly nebulised antibiotics
A Study to Assess the Safety, Tolerability, and Pharmacokinetics of Brensocatib Tablets in Adults With Cystic Fibrosis
Increasing life expectancy in cystic fibrosis: Advances and challenges
Airway clearance and exercise for people with cystic fibrosis: balancing longevity with life
Population Genomics and Inference of Mycobacterium avium Complex Clusters in Cystic Fibrosis Care Centers, United States
Alkyl-Quinolones derivatives as potential biomarkers for Pseudomonas aeruginosa infection chronicity in Cystic Fibrosis
Bone involvement in young adults with cystic fibrosis – a Portuguese cohort
Escherichia coli cryptic prophages sense nutrients to influence persister cell resuscitation
Isotope dilution LC-MS/MS quantification of the cystic fibrosis transmembrane conductance regulator (CFTR) modulators ivacaftor, lumacaftor, tezacaftor, elexacaftor, and their major metabolites in human serum
Separating the contributions of SLC26A9 and CFTR to anion secretion in primary human bronchial epithelia (HBE)
Functional Restoration of CFTR Nonsense Mutations in Intestinal Organoids
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.