Pending

Study to Determine the Efficacy&Safety of ARV-1801/ACG-701 for the Treatment of Cystic Fibrosis Pulmonary Exacerbations
Clinician perspectives on assessing for disordered eating and body image disturbance in adolescents and young adults with cystic fibrosis
Divergent dynamics of inflammatory mediators and multiplex PCRs during airway infection in cystic fibrosis patients and healthy controls: Serial upper airway sampling by nasal lavage
S945L-CFTR molecular dynamics, functional characterization and tezacaftor/ivacaftor efficacy in vivo and in vitro in matched pediatric patient-derived cell models
SIMPLIFYing cystic fibrosis treatment in a post-modulator era
Restoring myocardial infarction-induced long-term memory impairment by targeting the cystic fibrosis transmembrane regulator
Approaches to the management of haemoptysis in young people with cystic fibrosis
Effects of long-term tobramycin inhalation solution (TIS) once daily on exacerbation rate in patients with non-cystic fibrosis bronchiectasis
Prevalence and Characteristics of Cystic Fibrosis in Omani Children: A Multi-center Cross-sectional Study
Exploring parental cystic fibrosis disclosure to well children
Elexacaftor-Tezacaftor-Ivacaftor corrects monocyte microbicidal deficiency in cystic fibrosis
Normalization of circulating neutrophil counts after 12 months of elexacaftor-tezacaftor-ivacaftor in patients with advanced cystic fibrosis
Moonlighting chaperone activity of the enzyme PqsE contributes to RhlR-controlled virulence of Pseudomonas aeruginosa
Diagnosis of cystic fibrosis in adults: Australian Cystic Fibrosis Data Registry data, 2000-2019
The Effects of Antibiotic Combination Treatments on Pseudomonas aeruginosa Tolerance Evolution and Coexistence with Stenotrophomonas maltophilia
Impact of the COVID-19 pandemic and associated lockdown measures on the management, health, and behavior of the cystic fibrosis population in France during 2020 (MUCONFIN)
Manuka honey in combination with azithromycin shows potential for improved activity against Mycobacterium abscessus
Role of Inhalational Aztreonam Lysine in Lower Airway Infections in Cystic Fibrosis: An Updated Literature Review
Clinical characteristics and outcomes of incident cases of COVID-19 in unvaccinated adult cystic fibrosis patients in southern Brazil: a prospective cohort study conducted during the first year of the COVID-19 pandemic
Acneiform Eruption Following Elexacaftor-Tezacaftor-Ivacaftor Treatment in Patients With Cystic Fibrosis
Noninvasive prenatal screening for cystic fibrosis using circulating trophoblasts: Detection of the 50 most common disease-causing variants
Diverticulitis and Diverticulosis of the Appendix: A Case Series
Need to study simplification of gastrointestinal medication regimen in cystic fibrosis in the era of highly effective modulators
German Newborn Screening for Cystic Fibrosis – Parental perspectives and suggestions for improvements
Impact of Elexacaftor-Tezacaftor-Ivacaftor on Bacterial Colonization and Inflammatory Responses in Cystic Fibrosis
Pulmonary drug delivery technology enables anakinra repurposing in cystic fibrosis
The association of fecal calprotectin and respiratory exacerbation in cystic fibrosis patients
Real-life efficacy and safety of elexacaftor/tezacaftor/ivacaftor on severe cystic fibrosis lung disease patients
Congenital absence of the vas deferens with hypospadias or without hypospadias: Phenotypic findings and genetic considerations
Inhaled Antibiotics in Non-cystic Fibrosis Bronchiectasis (NCFB): A Systematic Review of Efficacy and Limitations in Adult Patients
The Bep gene cluster in Burkholderia cenocepacia H111 codes for a water-insoluble exopolysaccharide essential for biofilm formation
Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis
Aminoglycoside induced ototoxicity risk in the cystic fibrosis population: The utility of large-scale screening
Cefiderocol resistance genomics in sequential chronic Pseudomonas aeruginosa isolates from cystic fibrosis patients
How macronutrients and pancreatic enzyme supplements dose variability affect fat, protein and starch absorption in children with cystic fibrosis
Anti-Aspergillus fumigatus IgG in patients with bronchiectasis and its relationship with clinical outcome
Impact of N-Acetylcysteine and Antibiotics Against Single and Dual Species Biofilms of Pseudomonas aeruginosa and Achromobacter xylosoxidans
Clinical and Genetic Characterisation of Cystic Fibrosis Patients in Latvia: A Twenty-Five-Year Experience
Modifier Factors of Cystic Fibrosis Phenotypes: A Focus on Modifier Genes
Conjunctival Fluid Secretion Impairment via CaCC-CFTR Dysfunction Is the Key Mechanism in Environmental Dry Eye
New TMA (4,6,4′-Trimethyl angelicin) Analogues as Anti-Inflammatory Agents in the Treatment of Cystic Fibrosis Lung Disease
The importance of aeroallergen sensitivity in children with cystic fibrosis
A 3D stack-of-spirals approach for rapid hyperpolarized 129 Xe ventilation mapping in pediatric cystic fibrosis lung disease
Permissive and nonpermissive channel closings in CFTR revealed by a factor graph inference algorithm
Pediatric Pulmonology 2021 Year in Review: Rare and Diffuse Lung Disease
Rectal organoid-guided CFTR modulator therapy restores lung function in a CF patient with the rare 1677delTA/R334W genotype
Day 5 versus day 3 embryo biopsy for preimplantation genetic testing for monogenic/single gene defects
Longitudinal Study of Therapeutic Adherence in a Cystic Fibrosis Unit: Identifying Potential Factors Associated with Medication Possession Ratio
Airspace Enlargement with Fibrosis in a Young Heavy Smoker Mimicking Diffuse Cystic Lung Disease
Culture independent markers of nontuberculous mycobacterial (NTM) lung infection and disease in the cystic fibrosis airway
Clinical outcomes associated with Achromobacter species infection in people with cystic fibrosis
Delayed Spontaneous Pneumothorax in a Previously Healthy Nonventilated COVID-19 Patient
Antibody deficiencies with normal IgG in adults with Non-cystic fibrosis bronchiectasis or recurrent pneumonia: Cross-sectional study
Cefiderocol to manage chronic, multi-drug-resistant Burkholderia cepacia complex infection in a patient with cystic fibrosis: a case report
Real-world data on the efficacy and safety of tezacaftor-ivacaftor in adults living with cystic fibrosis homozygous for F508del and heterozygous for F508del and a residual function mutation
REFINEMENT OF NEWBORN SCREENING FOR CYSTIC FIBROSIS WITH NEXT GENERATION SEQUENCING
The Fatty Acid Imbalance of Cystic Fibrosis Exists at Birth Independent of Feeding, in Pig and Ferret Models
Long-Term Use Of Inhaled Hypertonic Saline In Children And Adolescents With Cystic Fibrosis: Experience From A Tertiary Care Center In A Low And Middle Income Country
Missed Cystic Fibrosis Newborn Screening Cases due to Immunoreactive Trypsinogen Levels below Program Cutoffs: A National Survey of Risk Factors
Clinical efficacy of elexacaftor-tezacaftor-ivacaftor in an adolescent with homozygous G85E cystic fibrosis
Nebulized levofloxacin for chronic Burkholderia cenocepacia pulmonary infection in cystic fibrosis: A case report
Oxygen-induced hypercapnia: physiological mechanisms and clinical implications
Identification of Novel F508del-CFTR traffic correctors among triazole derivatives
Using Hygge to Promote Wellness and Coping With a Chronic Illness
Cystic Fibrosis Screen Positive, Inconclusive Diagnosis Genotypes in People with Cystic Fibrosis from the U.S. Patient Registry
Perceptions of airway gene therapy for cystic fibrosis
Cystic fibrosis treatment: hope for all?
Physical activity and exercise training in cystic fibrosis
Steps Ahead: Optimising physical activity in adults with cystic fibrosis: A pilot randomised trial using wearable technology, goal setting and text message feedback
Altered fecal microbiome and metabolome in adult patients with non-cystic fibrosis bronchiectasis
Patient registry data highlights international differences in survival in cystic fibrosis
Radiological Changes After Treatment With Triple Therapy in Cystic Fibrosis. Are Bronchiectasis Irreversible?
Vasoactive intestinal peptide and cystic fibrosis transmembrane conductance regulator contribute to the transepithelial calcium transport across intestinal epithelium-like Caco-2 monolayer
Survival of people with cystic fibrosis in Australia
Bronchial artery diameter in massive hemoptysis in cystic fibrosis
Prevalence and Risk Factors for Low Bone Mineral Density in Adults With Cystic Fibrosis
Personalized medicine approaches in cystic fibrosis related pancreatitis
Corneal Refractive Surgery Considerations in Patients with Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders
Surface Dependent Inhibition of Mycobacterium abscessus by Diverse Pseudomonas aeruginosa Strains
Boosting Lung Accumulation Of Gallium With Inhalable Nano-Embedded Microparticles For The Treatment Of Bacterial Pneumonia
An unusual case of cystic fibrosis with pancytopenia due to copper deficiency and blindness caused by vitamin A deficiency: A case-report
Successful pregnancy in a cystic fibrosis patient with a severe impairment of lung function receiving Elexacaftor-Tezacaftor-Ivacaftor
Atypical Presentation of Cystic Fibrosis in An Infant
Experience to date with CFTR modulators during pregnancy and breastfeeding in the British Columbia Cystic Fibrosis clinic
High-quality read-based phasing of cystic fibrosis cohort informs genetic understanding of disease modification
Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
Drug treatment of cystic fibrosis
The long-term effects of insulin use in incident cystic fibrosis-related diabetes: a target trial emulated using longitudinal national registry data
Assessment of factors and interventions towards therapeutic adherence among persons with non-cystic fibrosis bronchiectasis
Long-term effectiveness of dual CFTR modulator treatment of cystic fibrosis
Synthesis and Biological Characterization of Fluorescent Cyclipostins and Cyclophostin Analogues: New Insights for the Diagnosis of Mycobacterial-Related Diseases
Systems-Wide Dissection of Organic Acid Assimilation in Pseudomonas aeruginosa Reveals a Novel Path To Underground Metabolism
CFTR modulates aquaporin-mediated glycerol permeability in mouse Sertoli cells
Cystic Fibrosis-Related Diabetes: Clinical approach and knowledge gaps
Distinct Long- and Short-Term Adaptive Mechanisms in Pseudomonas aeruginosa
Inhaled anti-pseudomonal antibiotics for long-term therapy in cystic fibrosis
COVID-19 Antibody Responses in Cystic Fibrosis
Effect of Trikafta on bone density, body composition and exercise capacity in CF: a pilot study
Effects of elexacaftor-tezacaftor-ivacaftor discontinuation in cystic fibrosis
Quantitation of cystic fibrosis triple combination therapy, elexacaftor/tezacaftor/ivacaftor, in human plasma and cellular lysate

European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance
Chest magnetic resonance imaging in cystic fibrosis: technique and clinical benefits
Fungal microbiota dynamics and its geographical, age and gender variability in patients with cystic fibrosis
Contemporary cystic fibrosis incidence rates in Canada and the United States
Screening by high-throughput sequencing for pathogenic variants in cystic fibrosis: Benefit of introducing personalized therapies
MR Imaging of Germ Cell and Sex Cord Stromal Tumors
Impact of COVID-19 on Lung Disease in People with Cystic Fibrosis: A 6-Month Follow-Up Study on Respiratory Outcomes
The Efficacy of an N-Acetylcysteine-Antibiotic Combination Therapy on Achromobacter xylosoxidans in a Cystic Fibrosis Sputum/Lung Cell Model
Effect of Dornase Alfa on the Lung Clearance Index in Children with Cystic Fibrosis: A Lesson from a Case Series
Physical Activity and Quality of Life among Patients with Cystic Fibrosis
Effectiveness of Physical Exercise Interventions on Pulmonary Function and Physical Fitness in Children and Adults with Cystic Fibrosis: A Systematic Review with Meta-Analysis
“What Are the Applications for Remote Rehabilitation Management in Cystic Fibrosis?”: A Scoping Review Protocol
Genetic Deletion of Mmp9 Does Not Reduce Airway Inflammation and Structural Lung Damage in Mice with Cystic Fibrosis-like Lung Disease
Anti-Virulence Potential of a Chionodracine-Derived Peptide against Multidrug-Resistant Pseudomonas aeruginosa Clinical Isolates from Cystic Fibrosis Patients
Clinical Advances in Cystic Fibrosis
Role of Bronchial Artery Embolization as Early Treatment Option in Stable Cystic Fibrosis Patients with Sub-Massive Hemoptysis: Personal Experience and Literature Review
Vitamin Status in Children with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutation
Essential Fatty Acid Deficiency in Cystic Fibrosis Disease Progression: Role of Genotype and Sex
Pulmonary Safety Profile of Esc Peptides and Esc-Peptide-Loaded Poly(lactide-co-glycolide) Nanoparticles: A Promising Therapeutic Approach for Local Treatment of Lung Infectious Diseases
Probiotic knowledge of adults with cystic fibrosis is limited but is associated with probiotic use: A cross-sectional survey study
The Value of the Patient Voice: A Review of Salt in My Soul by Mallory Smith
Cystic Fibrosis
The periplasmic chaperone Skp prevents misfolding of the secretory lipase A from Pseudomonas aeruginosa
Cystic fibrosis with liver involvement in adults has a benign course. Results from a tertiary referral centre cohort
Immunoreactive trypsinogen in healthy newborns and infants with cystic fibrosis
Embedded Specialist Palliative Care in Cystic Fibrosis: Results of a Randomized Feasibility Clinical Trial
Higher Mortality Rates Associated with Clostridioides difficile Infection in Hospitalized Children with Cystic Fibrosis
Cystic fibrosis-related mortality trends in Mexico between 1999 – 2020
Immunoglobulin A Vasculitis in a Child: Secondary to COVID-19 or Cystic Fibrosis?
Clearing Up the Smoke: Physical and Mental Health Considerations Regarding Cannabis Use in Adolescents with Cystic Fibrosis
Rounding out the pediatric CF care team: Including the profession of occupational therapy
Case Report of a Pediatric Chinese Cystic Fibrosis Patient with the c.1521_1523delCTT/c.3874-4522A>G Genotype
Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands
Carbohydrate metabolism impairment in children and adolescents with cystic fibrosis
Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials
Toward a Broader Understanding of Cystic Fibrosis Epidemiology and Its Impact on Clinical Manifestations
Genetics of Cystic Fibrosis: Clinical Implications
Update on Innate and Adaptive Immunity in Cystic Fibrosis
Novel Applications of Biomarkers and Personalized Medicine in Cystic Fibrosis
Emerging Approaches to Monitor and Modify Care in the Era of Cystic Fibrosis Transmembrane Conductance Regulators
The Impact of Highly Effective Modulator Therapy on Cystic Fibrosis Microbiology and Inflammation
Novel Approaches to Multidrug-Resistant Infections in Cystic Fibrosis
Update on Clinical Outcomes of Highly Effective Modulator Therapy
Nontuberculous Mycobacterial Infections in Cystic Fibrosis
Non-Modulator Therapies: Developing a Therapy for Every Cystic Fibrosis Patient
Updates in Nutrition Management of Cystic Fibrosis in the Highly Effective Modulator Era
Update in Advancing the Gastrointestinal Frontier in Cystic Fibrosis
Transitions of Care in Cystic Fibrosis
Endocrine Complications of Cystic Fibrosis
Management of Mental Health in Cystic Fibrosis
Family Planning and Reproductive Health in Cystic Fibrosis
Update on Lung Transplantation for Cystic Fibrosis
Cystic Fibrosis in the Era of Highly Effective CFTR Modulators
Sinusitis, otitis media and diffuse bronchiectasis in both lungs
The effect of N-acetylcysteine in patients with non-cystic fibrosis bronchiectasis (NINCFB): study protocol for a multicentre, double-blind, randomised, placebo-controlled trial
Natural Products Lysobactin and Sorangicin A Show In Vitro Activity against Mycobacterium abscessus Complex
Parallel Evolution of Pseudomonas aeruginosa during a Prolonged ICU-Infection Outbreak
Persistent pleuritic chest pain in a patient with cystic fibrosis
Management of pregnancy in cystic fibrosis
Transition from paediatric to adult care in cystic fibrosis
Toxoplasma gondii infection triggers ongoing inflammation mediated by increased intracellular Cl- concentration in airway epithelium
Risk factors and outcomes of non-tuberculous mycobacteria infection in lung transplant recipients: A systematic review and meta-analysis
Marginal association of fasting blood glucose with the risk of cystic fibrosis-related diabetes
Salicylate attenuates gentamicin-induced ototoxicity and facilitates the recovery in the basilar papilla of the lizard Podarcis siculus
Interplay between biofilm microenvironment and pathogenicity of Pseudomonas aeruginosa in cystic fibrosis lung chronic infection
Significance and potential role of eosinophils in non-cystic fibrosis bronchiectasis
Transcription factor FoxM1 promotes cyst growth in PKD1 mutant ADPKD
The Effects of Virtual Reality Physiotherapy Interventions on Cardiopulmonary Function and Breathing Control in Cystic Fibrosis: A Systematic Review
Rate of Lung Function Decline in People with Cystic Fibrosis Having a Residual Function Gene Mutation
The relationship between weight and pulmonary outcomes in overweight and obese people with cystic fibrosis: A retrospective observational study
An Unusual Case of Type 2 Fronto-Ethmoidal Mucopyocele
The effect of Pseudomonas aeruginosa eradication regimens on chronic colonization and clinical outcomes in pediatric patients with cystic fibrosis
Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis
Development of novel therapeutics for all individuals with CF (the future goes on)
Can Identifying Pulmonary Exacerbation Phenotypes Guide New Treatment Approaches for Cystic Fibrosis?
Challenged Urine Bicarbonate Excretion as a Measure of Cystic Fibrosis Transmembrane Conductance Regulator Function in Cystic Fibrosis
The Clinical Yield of Bronchoscopy in The Management of Cystic Fibrosis – A Retrospective Multicenter Study
Outcome of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis and solid organ transplantation
A Case of Allergic Bronchopulmonary Aspergillosis With Bronchial Asthma
Quick and wide-range taxonomical repertoire establishment of the cystic fibrosis lung microbiota by tandem mass spectrometry on sputum samples
Serum levels of hormones regulating appetite in patients with cystic fibrosis – a single-center, cross-sectional study
Efficacy of selective histone deacetylase 6 inhibition in mouse models of Pseudomonas aeruginosa infection: A new glimpse for reducing inflammation and infection in cystic fibrosis
Chest radiography and computed tomography imaging in cystic fibrosis: current challenges and new perspectives
Clinical findings of methicillin-resistant Staphylococcus aureus in cystic fibrosis
Gene therapy for cystic fibrosis: Challenges and prospects
Modulation of Biofilm Mechanics by DNA Structure and Cell Type
Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders
The In Vivo and In Vitro Assessment of Pyocins in Treating Pseudomonas aeruginosa Infections
Antimicrobial Treatment on a Catheter-Related Bloodstream Infection (CRBSI) Case Due to Transition of a Multi-Drug-Resistant Ralstonia mannitolilytica from Commensal to Pathogen during Hospitalization
Virtual Drug Repositioning as a Tool to Identify Natural Small Molecules That Synergize with Lumacaftor in F508del-CFTR Binding and Rescuing
Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with CFTR Mutations Causing Pancreatic Insufficiency
Complementary Dual Approach for In Silico Target Identification of Potential Pharmaceutical Compounds in Cystic Fibrosis
Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease
Kidney Cyst Lining Epithelial Cells Are Resistant to Low-Dose Cisplatin-Induced DNA Damage in a Preclinical Model of Autosomal Dominant Polycystic Kidney Disease
Serratiopeptidase Affects the Physiology of Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients
Drug Repurposing for Cystic Fibrosis: Identification of Drugs That Induce CFTR-Independent Fluid Secretion in Nasal Organoids
Barriers for Sports and Exercise Participation and Corresponding Barrier Management in Cystic Fibrosis
The Effectiveness of Ultraviolet-C (UV-C) Irradiation on the Viability of Airborne Pseudomonas aeruginosa
Dynamic Perfluorinated Gas MRI Shows Improved Lung Ventilation in People with Cystic Fibrosis after Elexacaftor/Tezacaftor/Ivacaftor: An Observational Study
Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium

Quantifying Intracellular Viral Pathogen: Specimen Preparation, Visualization and Quantification of Multiple Immunofluorescent Signals in Fixed Human Airway Epithelium Cultured at Air-Liquid Interface
Identification of Exhaled Metabolites in Children with Cystic Fibrosis
Recovery of ΔF508-CFTR Function by Citrate
Positive impact of insulin treatment on clinical trend in cystic fibrosis patients: a retrospective study
Short Chain Fatty Acids: Fundamental mediators of the gut-lung axis and their involvement in pulmonary diseases
Cftr deletion in mouse epithelial and immune cells differentially influence the intestinal microbiota
3D Single-Breath Chemical Shift Imaging Hyperpolarized Xe-129 MRI of Healthy, CF, IPF, and COPD Subjects
Personalized Selection of a CFTR Modulator for a Patient with a Complex Allele [L467F;F508del]
Small Colonies, Bigger Problems? New Evidence That Staphylococcus aureus Small Colony Variants Can Worsen Lung Inflammation in Cystic Fibrosis Rats
Physiologically Based Pharmacokinetic Modeling To Guide Management of Drug Interactions between Elexacaftor-Tezacaftor-Ivacaftor and Antibiotics for the Treatment of Nontuberculous Mycobacteria
Newly Discovered Cutting-Edge Triple Combination Cystic Fibrosis Therapy: A Systematic Review
Use of systemic steroids during pulmonary exacerbations in children and adolescent with Cystic fibrosis
Impact of cholesterol and Lumacaftor on the folding of CFTR helical hairpins
Cystic Fibrosis: Highly Effective Targeted Therapeutics and the Impact on Sex and Racial Disparities
Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI
Parental Preferences about Policy Options Regarding Disclosure of Incidental Genetic Findings in Newborn Screening: Using Videos and the Internet to Educate and Obtain Input
Update on cystic fibrosis : From neonatal screening to causal treatment
The relationship between lung disease severity and the sputum proteome in cystic fibrosis
Patient engagement in the development of CF-CBT: A cystic fibrosis-specific cognitive-behavioral intervention for adults
A pathogenic variant of TULP3 causes renal and hepatic fibrocystic disease
Ion channels as a therapeutic target for renal fibrosis
The Impact of Elexacaftor/Ivacaftor/Tezacaftor on Cystic Fibrosis Patients Who Acquire COVID-19 Infection
Myofibroblast depletion reduces kidney cyst growth and fibrosis in autosomal dominant polycystic kidney disease
Changes in exercise endurance and inspiratory capacity after lumacaftor/ivacaftor therapy in 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. Authors’ reply
Frequencies of pathogenic CFTR variants in Greek cystic fibrosis patients with allergic bronchopulmonary aspergillosis and Aspergillus fumigatus chronic colonization: A retrospective cohort study
Narrowing the Differential Diagnosis of Cystic Lesions in Smokers with Expiratory CT Acquisition Using the Cyst-Airway Communication Hypothesis
Serum kisspeptin and proopiomelanocortin in cystic fibrosis: a single study
New concepts in antimicrobial resistance in cystic fibrosis respiratory infections
Corrigendum for Update on the management of vitamins and minerals in cystic fibrosis. Nutr Clin Pract. 2022;37(5):1074-1087
Effects of Telerehabilitation-based Exercises in Cystic Fibrosis
Coronary artery disease in patients with cystic fibrosis – A case series and review of the literature
Amikacin Liposomal Inhalation Suspension in the Treatment of Mycobacterium abscessus Lung Infection: A French Observational Experience
Family case studies: absence of Pseudomonas aeruginosa transmission in bronchiectasis
Nonsense codons suppression. An acute toxicity study of three optimized TRIDs in murine model, safety and tolerability evaluation
Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor
Unified Airway-Cystic Fibrosis
Real life evaluation of the multi-organ effects of Lumacaftor/Ivacaftor on F508del homozygous cystic fibrosis patients
Identification and characterization of bacteria isolated from patients with cystic fibrosis in Jordan
Inhaled Corticosteroids in Adults with Non-cystic Fibrosis Bronchiectasis: From Bench to Bedside. A Narrative Review
Molecular structures reveal synergistic rescue of Δ508 CFTR by Trikafta modulators
Bicarbonate defective CFTR variants increase risk for chronic pancreatitis: A meta-analysis
Novel Substituted Cyclopropyl Compounds as CFTR Modulators for Treating Cystic Fibrosis
A single heterozygous nonsense mutation in the TTC21B gene causes adult-onset nephronophthisis 12: A case report and review of literature
Effect of Ultraviolet-c (UVc) light and ozone on the survival of Mycobacterium abscessus complex organisms associated with cystic fibrosis
Helping map the taxonomical position of the Nontuberculous Mycobacteria (NTM) in cystic fibrosis
Allergic bronchopulmonary aspergillosis in association with rheumatic heart disease: report of three cases
Assessment of Cystic Fibrosis Distribution Based on Air Pollution by Geographical Information System (GIS)
Effect of Physical Activity Program on the Quality of Life of Children with Cystic Fibrosis at School Age: A Randomized Clinical Trail
Production of CFTR Mutant Gene Model by Homologous Recombination System
Statistical Evaluation of Metaproteomics and 16S rRNA Amplicon Sequencing Techniques for Study of Gut Microbiota Establishment in Infants with Cystic Fibrosis
Airway clearance physiotherapy and health-related quality of life in cystic fibrosis
Discovery of N -β-l-Fucosyl Amides as High-Affinity Ligands for the Pseudomonas aeruginosa Lectin LecB
Antibiotic Resistance in Pseudomonas
Cell Envelope Stress Response in Pseudomonas aeruginosa
Pseudomonas aeruginosa in the Cystic Fibrosis Lung
Mixed Populations and Co-Infection: Pseudomonas aeruginosa and Staphylococcus aureus
Association between exercise and risk of cardiovascular diseases in patients with non-cystic fibrosis bronchiectasis
The risk of colorectal cancer in individuals with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: An English population-based study
Effects of prolonged proton pump inhibitor treatment on nutritional status and respiratory infection risk in cystic fibrosis: A matched cohort study
Variability of clinically measured lung clearance index in children with cystic fibrosis
Aryl Hydrocarbon Receptor Agonism Antagonizes the Hypoxia-driven Inflammation in Cystic Fibrosis
Identification of cystic fibrosis transmembrane conductance regulator as a prognostic marker for juvenile myelomonocytic leukemia via the whole-genome bisulfite sequencing of monozygotic twins and data mining
Synthesis of C6-modified mannose 1-phosphates and evaluation of derived sugar nucleotides against GDP-mannose dehydrogenase
Specific circulating neutrophils subsets are present in clinically stable adults with cystic fibrosis and are further modulated by pulmonary exacerbations
Determination of Cystic Fibrosis Mutation Frequency in Preterm and Term Neonates with Respiratory Tract Problems
Now What? Collective Sensemaking and Sensegiving in the Cystic Fibrosis Community in Sweden During the Initial Phase of the COVID-19 Pandemic
Pulmonary disorders in pregnancy: Bronchiectasis, cystic fibrosis, sarcoidosis and interstitial diseases
Guidelines for Cystic Fibrosis Carrier Screening in the Prenatal/Preconception Period
Inhalable ceftazidime-roflumilast powder targeting infection and inflammation: Influence of incorporating roflumilast into ceftazidime-leucine co-amorphous formulation
CFTR Modulators and Reduction of Airway Inflammation in Cystic Fibrosis: How Much is Enough?
An inflammatory Signature of Glucose Impairment in Cystic Fibrosis
Plasticity towards Rigidity: A Macrophage Conundrum in Pulmonary Fibrosis
Modulator Combination Improves In Vitro the Microrheological Properties of the Airway Surface Liquid of Cystic Fibrosis Airway Epithelia
Detection of Disease-Causing CFTR Variants In State Newborn Screening Programs
Genotype-phenotype correlations of cystic fibrosis in siblings compound heterozygotes for rare variant combinations: Review of literature and case report
Improved Trimethylangelicin Analogs for Cystic Fibrosis: Design, Synthesis and Preliminary Screening
Synthesis and Biomedical Applications of Mucin Mimic Materials
Clinically undetected polyclonal heteroresistance among Pseudomonas aeruginosa isolated from cystic fibrosis respiratory specimens
Evidence of validity of internal structure of the Functional Assessment of Chronic Illness Therapy-Spiritual Well-Being Scale (FACIT-Sp-12) in Brazilian adolescents with chronic health conditions
Deletion of sphingosine kinase 2 attenuates cigarette smoke-mediated chronic obstructive pulmonary disease-like symptoms by reducing lung inflammation
MRI evaluation of right heart functions in children with mild cystic fibrosis
Idiopathic pulmonary fibrosis: Diagnosis, biomarkers and newer treatment protocols
ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria
The efficacy of azithromycin to prevent exacerbation of non-cystic fibrosis bronchiectasis: a meta-analysis of randomized controlled studies
129 Xe and Free-Breathing 1 H Ventilation MRI in Patients With Cystic Fibrosis: A Dual-Center Study
Investigation of the Mechanism and Chemistry Underlying Staphylococcus aureus’ Ability to Inhibit Pseudomonas aeruginosa Growth In Vitro
Metabolic Mechanism and Physiological Role of Glycerol 3-Phosphate in Pseudomonas aeruginosa PAO1
Airway surface hyperviscosity and defective mucociliary transport by IL-17/TNF-α are corrected by beta-adrenergic stimulus
CGM patterns in adults with cystic fibrosis-related diabetes before and after elexacaftor-tezacaftor-ivacaftor therapy
Restoring airway epithelial homeostasis in Cystic Fibrosis
Structure basis of CFTR folding, function and pharmacology
Discovery of Homogentisic Acid as a Precursor in Trimethoprim Metabolism and Natural Product Biosynthesis
Meconium Ileus, Distal Intestinal Obstruction Syndrome, and Other Gastrointestinal Pathology in the Cystic Fibrosis Patient
Quantity and quality of airway clearance in children and young people with cystic fibrosis
Sexual and reproductive health experiences and care of adult women with cystic fibrosis
High Rates of Extensively Drug-Resistant Pseudomonas aeruginosa in Children with Cystic Fibrosis
ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction
Activity of newest generation β-lactam/β-lactamase inhibitor combination therapies against multidrug resistant Pseudomonas aeruginosa
Pleiotropic modifiers of age-related diabetes and neonatal intestinal obstruction in cystic fibrosis

Thoracic configuration in patients ageing with cystic fibrosis: A retrospective single-blinded cohort study
Exploring the impact of elexacaftor-tezacaftor-ivacaftor treatment on opinions regarding airway clearance techniques and nebulisers: TEMPO a qualitative study in children with cystic fibrosis, their families and healthcare professionals
Limited evidence of patient-to-patient transmission of Staphylococcus aureus strains between children with cystic fibrosis, Queensland, Australia
Changing racial and ethnic differences for lung transplantation in cystic fibrosis
Pharmacological inhibitors of the cystic fibrosis transmembrane conductance regulator exert off-target effects on epithelial cation channels
Investigating the genetic profile of familial atypical cystic fibrosis patients (DeltaF508-CFTR) with neonatal biliary atresia
Retrospective Review of Vancomycin Monitoring via Trough Only vs. Two- Point Estimated Area Under the Curve in Pediatric and Adult Patients with Cystic Fibrosis
Scars of COVID-19: A bibliometric analysis of post-COVID-19 fibrosis
Longitudinal Assessment of Educational Risk for K-12 Students with Cystic Fibrosis
Cough suppression and HRQoL in adult people with cystic fibrosis: an unexplored correlation
Trends in and Outcomes of Deliveries Complicated by Cystic Fibrosis
Assessing the Occurrence of Reproductive Health Counseling in Adolescents with Cystic Fibrosis
Comparison of continuous glucose monitoring to reference standard oral glucose tolerance test for the detection of dysglycemia in cystic Fibrosis: A systematic review
Parent’s Report on Oral Health-Related Quality of Life of Children with Cystic Fibrosis
Phage therapy for pulmonary infections: lessons from clinical experiences and key considerations
Increased expression of glucagon-like peptide-1 and cystic fibrosis transmembrane conductance regulator in the ileum and colon in mouse treated with metformin
In Silico Identification of Potential Insect Peptides against Biofilm-Producing Staphylococcus aureus
In vivo correction of cystic fibrosis mediated by PNA nanoparticles
Disulfiram Is Effective against Drug-Resistant Mycobacterium abscessus in a Zebrafish Embryo Infection Model
False negatives in the newborn screening for Cystic Fibrosis in Western Andalusia: Results from a 10- year experience
Young’s syndrome, a rare syndrome that can cause infertility and mimics cystic fibrosis and immotile-cilia syndrome: a case report
Single-Cell RNA-Sequencing Reveals New Basic and Translational Insights in the Cystic Fibrosis Lung
Pseudomonas aeruginosa utilises the host-derived polyamine spermidine to facilitate antimicrobial tolerance
The Role of the Physical Therapist in Cystic Fibrosis Care
Coexistence of Cystic Fibrosis with other genetic disorders: A case series
Beta-adrenergic sweat test in children with inconclusive cystic fibrosis diagnosis: do we need new reference ranges?
Reproductive genetic carrier screening for cystic fibrosis, fragile X syndrome and spinal muscular atrophy: patterns of community and healthcare provider participation in a Victorian screening program
Evolution of Quorum Sensing in Pseudomonas aeruginosa Can Occur via Loss of Function and Regulon Modulation
Impact of Patient-Specific Aminoglycoside Monitoring for Treatment of Pediatric Cystic Fibrosis Pulmonary Exacerbations
Monitoring disease progression in childhood bronchiectasis
Effects of a therapeutic exercise program in children with non-cystic fibrosis bronchiectasis: A pilot randomized controlled trial
Serum leptin and neuropeptide Y in patients with cystic fibrosis-A single center study
Evaluation of phages and liposomes as combination therapy to counteract Pseudomonas aeruginosa infection in wild-type and CFTR -null models
ULTRASONOGRAPHIC PRESENTATION OF NODULAR CYSTIC FAT NECROSIS AFTER A LOW-VELOCITY TRAUMA: A CASE REPORT
Genetic factors help explain the variable responses of young children with cystic fibrosis to vitamin D supplements
The SLC26A9 inhibitor S9-A13 provides no evidence for a role of SLC26A9 in airway chloride secretion but suggests a contribution to regulation of ASL pH and gastric proton secretion
Role of physical activity and airway clearance therapy in cystic fibrosis: moving forward in a rapidly changing landscape
Web-based physical activity promotion in young people with CF: a randomised controlled trial
Enabling Asymmetric Synthesis of ABBV-3748, a Corrector Compound for the Treatment of Cystic Fibrosis
Evaluation of the Interstitial Histological Lesions in Pulmonary Langerhans Cell Histiocytosis
Jaw and neck muscle activity during chewing and craniocervical dysfunction index in children, adolescents, and adults with and without cystic fibrosis: a comparative analysis
Body mass index and additional risk factors for cancer in adults with cystic fibrosis
Imipenem/Cilastatin/Relebactam Pharmacokinetics, Safety, and Outcomes in Adults and Adolescents With Cystic Fibrosis
Closed-loop for People Living With Cystic Fibrosis Related Diabetes
Distal Intestinal Obstruction Syndrome Relieved by Ileoscopy in a Patient With Cystic Fibrosis: A Case Report and Literature Review
Intercellular communication and social behaviors in mycobacteria
Mechanism of Xiaoqinglong Decoction and Qingqi Huatan Pills in improving pathological airway mucus based on nuclear factor-κB/microRNA-494 signaling regulation of mucin 5AC and cystic fibrosis transmembrane conductance regulator
Targeting a Multidrug-Resistant Pathogen: First Generation Antagonists of Burkholderia cenocepacia ‘s BC2L-C Lectin
Multicenter Surveillance of Cystic Fibrosis in Korean Children
Take on transplant: human-centered design of a patient education tool to facilitate informed discussions about lung transplant among people with cystic fibrosis
Personalized tobramycin dosing in children with cystic fibrosis: a comparative clinical evaluation of log-linear and Bayesian methods
Targeted AntiBiotics for Chronic pulmonary diseases (TARGET ABC): can targeted antibiotic therapy improve the prognosis of Pseudomonas aeruginosa-infected patients with chronic pulmonary obstructive disease, non-cystic fibrosis bronchiectasis, and asthma? A multicenter, randomized, controlled, open-label trial
Genomic epidemiology of Mycobacterium abscessus in a Canadian cystic fibrosis centre
Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites
Antimicrobial susceptibility and genotyping of microorganisms isolated from sputum culture of children with cystic fibrosis in an Iranian referral children’s hospital
Impact of Antibiotics on the Lung Microbiome and Lung Function in Children With Cystic Fibrosis 1 Year After Hospitalization for an Initial Pulmonary Exacerbation
Acute Infection with a Tobramycin-Induced Small Colony Variant of Staphylococcus aureus Causes Increased Inflammation in the Cystic Fibrosis Rat Lung
Generation of two TMEM16A knockout iPSC clones each from a healthy human iPSC line, from a Cystic Fibrosis patient specific line with p.Phe508del mutation and from the gene corrected iPSC line
Clinical outcomes of adults and children with cystic fibrosis during the COVID-19 pandemic
Impact of Elexacaftor/Tezacaftor/Ivacaftor Therapy on the Cystic Fibrosis Airway Microbial Metagenome
Intra- and Interspecies Spread of a Novel Conjugative Multidrug Resistance IncC Plasmid Coharboring bla OXA-181 and armA in a Cystic Fibrosis Patient
Telemonitoring for Cystic fibrosis patients of Bambino Gesù Children’s Hospital during COVID-19
Evaluation of nutritional status through bioimpedance analysis in a group of Cystic Fibrosis patients
Coxsackievirus B infections are common in Cystic Fibrosis and experimental evidence supports protection by vaccination
Innovative cystic fibrosis drug development: A perspective
Tranexamic Acid Use for Massive Hemoptysis in a Child: A Case Report
Allergic Bronchopulmonary Aspergillosis (ABPA) Diagnosis Missed in the Context of Asthma Exacerbation Due to Medication Nonadherence
Lymph node regression grading of locally advanced rectal cancer treated with neoadjuvant chemoradiotherapy
A pilot study investigating the effects of a manuka honey sinus rinse compared to a standard sinus rinse on sino-nasal outcome test scores in cystic fibrosis patients
Editorial overview – 2022 respiratory issue: Cystic fibrosis pathophysiology, models, and novel therapies
Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis
Cytokine patterns in cystic fibrosis patients with different microbial infections in oropharyngeal samples
Author’s reply: “Serum bile acids in cystic fibrosis patients-Glycodeoxycholic acid as a potential marker of liver disease”
Advent of elexacaftor/tezacaftor/ivacaftor for cystic fibrosis treatment: What consequences on Aspergillus-related diseases? Preliminary insights
One-minute sit-to-stand test is practical to assess and follow the muscle weakness in cystic fibrosis
Appetite stimulants for people with cystic fibrosis
Multidrug-Resistant Bacteria in Children and Adolescents with Cystic Fibrosis
Non-Cystic Fibrosis Bronchiectasis in Pediatric Age: A Case Series in a Metropolitan Area of Northern Italy
Mucins and CFTR: Their Close Relationship
Evaluation of the Complex p.[Leu467Phe;Phe508del] CFTR Allele in the Intestinal Organoids Model: Implications for Therapy
In Vitro Rescue of the Bile Acid Transport Function of ABCB11 Variants by CFTR Potentiators
The CFTR Amplifier Nesolicaftor Rescues TGF-β1 Inhibition of Modulator-Corrected F508del CFTR Function
Modulator Therapy in Cystic Fibrosis Patients with cis Variants in F508del Complex Allele: A Short-Term Observational Case Series
Trikafta-Extending Its Success to Less Common Mutations
Malassezia Folliculitis following Triple Therapy for Cystic Fibrosis
α-Tocopherol Pharmacokinetics in Adults with Cystic Fibrosis: Benefits of Supplemental Vitamin C Administration
Effects of Ceragenins and Antimicrobial Peptides on the A549 Cell Line and an In Vitro Co-Culture Model of A549 Cells and Pseudomonas aeruginosa
Self-Emulsifying Formulations to Increase the Oral Bioavailability of 4,6,4′-Trimethylangelicin as a Possible Treatment for Cystic Fibrosis
Clinical associations with carriage of pulmonary Stenotrophomonas maltophilia
Feasibility of unsedated lung MRI in young children with cystic fibrosis
The natural history and genetic diversity of Haemophilus influenzae infecting the airways of adults with cystic fibrosis
Standardizing Treatments for Pulmonary Exacerbations – Aminoglycoside Study
Integrating specialist palliative care to improve care and reduce suffering: cystic fibrosis (InSPIRe:CF) – study protocol for a multicentre randomised clinical trial
Evaluation of Growth Hormone Deficiency in Children with Cystic Fibrosis
Real-world feasibility of short-term, unsupervised home spirometry in CF
Significance of Molecular Identification of Genomic Variants of Pseudomonas aeruginosa in Children with Cystic Fibrosis in Mexico
Lysosomal cathepsin D mediates endogenous mucin glycodomain catabolism in mammals
COPD and the modulation of CFTR by acute exposure to cigarette smoke
Withaferin A targets the membrane of Pseudomonas aeruginosa and mitigates the inflammation in zebrafish larvae; an in vitro and in vivo approach
Mucus aberrant properties in CF: Insights from cells and animal models

Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis
Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor
Short-term effects of a novel bronchial drainage device: A pilot cohort study in subjects with cystic fibrosis
Bone metabolism and incretin hormones following glucose ingestion in young adults with pancreatic insufficient cystic fibrosis
Biphasic regulation of CFTR expression by ENaC in epithelial cells: The involvement of Ca2+ -modulated cAMP production
Beyond borders: cystic fibrosis survival between Australia, Canada, France and New Zealand
Efficacy and Mode of Action of a Direct Inhibitor of Mycobacterium abscessus InhA
DNA Methyltransferase Regulates Nitric Oxide Homeostasis and Virulence in a Chronically Adapted Pseudomonas aeruginosa Strain
Is Measuring Physical Literacy in School-Aged Children With Cystic Fibrosis or Congenital Heart Disease Needed?
Italian Cystic Fibrosis Registry (ICFR). Report 2019-2020
Association between phenotypic and genotypic characteristics and disease severity in individuals with cystic fibrosis
Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era
Pseudomonas aeruginosa Strains from Both Clinical and Environmental Origins Readily Adopt a Stable Small-Colony-Variant Phenotype Resulting from Single Mutations in c-di-GMP Pathways
Progress and challenges in fungal lung disease in cystic fibrosis
Infection control in cystic fibrosis: evolving perspectives and challenges
Is Cystic Fibrosis Contributing Significantly to Infant Mortality Rate in India?
Preclinical evaluation of the ENaC inhibitor AZD5634 and implications on human translation
Fibrosis quística: patogenia bacteriana y moduladores del CFTR (regulador de conductancia transmembranal de la fibrosis quística)
Dental Caries and Associated Salivary Biomarkers in Patients with Cystic Fibrosis
Low Diversity and Instability of the Sinus Microbiota over Time in Adults with Cystic Fibrosis
Treatment of Pseudomonas aeruginosa infectious biofilms: Challenges and strategies
Diet quality in cystic fibrosis – associations with patient reported outcome measures and enablers and barriers to eating a healthy diet: A protocol paper for a mixed methods study
Trajectories of early growth and subsequent lung function in cystic fibrosis: An observational study using UK and Canadian registry data
CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway
Drug-induced acne with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis
The feasibility of collecting the physiotherapy outcomes airway clearance, physical activity and fitness for the Australian Cystic Fibrosis Data Registry
Mental health screening in Cystic Fibrosis as an intervention: Patient and caregiver feedback on improving these processes
A Study to Evaluate the Relative Bioavailability of a Fixed-dose Combination Tablet of VX-121/Tezacaftor/Deutivacaftor
Differential Performance and Lung Deposition of Levofloxacin with Different Nebulisers Used in Cystic Fibrosis
KDM2A and KDM3B as Potential Targets for the Rescue of F508del-CFTR
Ultrastructural Characterization of Human Bronchial Epithelial Cells during SARS-CoV-2 Infection: Morphological Comparison of Wild-Type and CFTR-Modified Cells
The NHE3 Inhibitor Tenapanor Prevents Intestinal Obstructions in CFTR-Deleted Mice
Exercise Capacity and Physical Activity in Non-Cystic Fibrosis Bronchiectasis after a Pulmonary Rehabilitation Home-Based Programme: A Randomised Controlled Trial
Competitive fitness of Pseudomonas aeruginosa isolates in human and murine precision-cut lung slices
Cystic fibrosis – A look at Belgium in 2022
Mediation of the total effect of cystic fibrosis-related diabetes on mortality: A UK Cystic Fibrosis Registry cohort study
Progress in Respiratory Gene Therapy
Longitudinal Evolution of the Pseudomonas-Derived Cephalosporinase (PDC) Structure and Activity in a Cystic Fibrosis Patient Treated with β-Lactams
A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase
Xylitol vs Saline Nasal Irrigations in CF-CRS
Tromethamine improves mucociliary clearance in cystic fibrosis pigs
New Recommendations of a Height-Based Dosing Regimen of Tobramycin for Cystic Fibrosis in Adults: A Population Pharmacokinetic Analysis
In vitro synergy between manuka honey and amikacin against Mycobacterium abscessus complex shows potential for nebulisation therapy
Airway Infection in Cystic Fibrosis: Microbiology and Management
Adaptation and Evolution of Pathogens in the Cystic Fibrosis Lung
Antimicrobial Stewardship in Cystic Fibrosis
The Elusive Role of Airway Infection in Cystic Fibrosis Exacerbation
Drugs, Drugs, Drugs: Current Treatment Paradigms in Cystic Fibrosis Airway Infections
The Sense and Nonsense of Antimicrobial Susceptibility Testing in Cystic Fibrosis
Exploring the Cystic Fibrosis Lung Microbiome: Making the Most of a Sticky Situation
Opportunistic Pathogens in Cystic Fibrosis: Epidemiology and Pathogenesis of Lung Infection
Community Perspective on Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis
Health-related quality of life in a prospective study of ultrasound to detect cystic fibrosis-related liver disease in children
Using the genome to correct the ion transport defect in cystic fibrosis
A Study to Assess the Pharmacokinetics and Safety of Co-administered Oral Galicaftor, Navocaftor, and ABBV-576 in Healthy Adults for the Treatment of Cystic Fibrosis
Generation of two induced pluripotent stem cell lines (RCMGi005-A/B) from human skin fibroblasts of a cystic fibrosis patient with homozygous F508del mutation in CFTR gene
The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus
Glycemic indices at night measured by CGM are predictive for a lower pulmonary function in adults but not in children with cystic fibrosis
Clinimetric analysis of outcome measures for airway clearance in people with cystic fibrosis: a systematic review
KCa3.1 potentiation stimulates Cl- secretion in F508del and G551D CFTR corrected primary human bronchial epithelial cells
The circadian system in cystic fibrosis mice is regulated by histone deacetylase 6
Development of algorithm for diagnosis of Cystic Fibrosis in absence of sweat chloride testing in resource limited setting
Pulmonary exacerbations, airway pathogens, and long-term course of lung clearance index in children and young adults with Cystic Fibrosis
The role of mechanosensitive ion channels in the gastrointestinal tract
Niche, not phylogeny, governs the response to oxygen availability among diverse Pseudomonas aeruginosa strains
Experiments with LDA and Top2Vec for embedded topic discovery on social media data-A case study of cystic fibrosis
Clinical outcomes of digital health in adults with cystic fibrosis
Research advances on the regulatory mechanism of sweat secretion ion channels of eccrine sweat glands
Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
Faecal calprotectin and rectal histological inflammatory markers in cystic fibrosis: a single-centre study
Real-world Efficacy and Safety of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulator Therapy in Adult Patients With Cystic Fibrosis (CF)
The association between Sodium Urinary Discharge (FENa) and growth parameters in pediatrics with cystic fibrosis
In response to “who are the 10%? – Non eligibility of cystic fibrosis (CF) patients for highly effective modulator therapies”
Intracellular replication of Inquilinus limosus in bronchial epithelial cells
Analysis of cystic fibrosis patient survival confirms STAT3 as a CF modifying gene with changing impact over time
Combining Gallium Protoporphyrin and Gallium Nitrate Enhances In Vitro and In Vivo Efficacy against Pseudomonas aeruginosa : Role of Inhibition of Bacterial Antioxidant Enzymes and Resultant Increase in Cytotoxic Reactive Oxygen Species
Prevalence, trends and outcomes of long-term inhaled antibiotic treatment in people with cystic fibrosis without chronic Pseudomonas aeruginosa infection – A European cystic fibrosis patient registry data analysis
Keratin 8 is a scaffolding and regulatory protein of ERAD complexes
Detecting Ion Levels, Changes to Monitor Health
Systemic Corticosteroids in the Management of Pediatric Cystic Fibrosis Pulmonary Exacerbations
Exercise performance in children and adolescents with cystic fibrosis with and without abnormal glucose tolerance: a single center cross-sectional study
High expression of CFTR in cumulus cells from mature oocytes is associated with high-quality of oocyte and subsequent embryonic development
Case report of a Hispanic female with cystic fibrosis and short stature
Evaluation of an association between RANKL and OPG with bone disease in people with cystic fibrosis
SOCIAL DETERMINANTS OF HEALTH SCREENING AND INTERVENTION: A CYSTIC FIBROSIS QUALITY IMPROVEMENT PROCESS
Polyguluronate simulations shed light onto the therapeutic action of OligoG CF-5/20
Clinical phenotypes and genotypic spectrum of cystic fibrosis with pancreatic insufficiency in children
Phase 1 Study to Assess the Safety and Pharmacokinetics of Elexacaftor/Tezacaftor/Ivacaftor in Subjects Without Cystic Fibrosis With Moderate Hepatic Impairment
Long-term azithromycin in children with bronchiectasis unrelated to cystic fibrosis: treatment effects over time
Self-reported dental attendance, oral hygiene habits, and dietary habits of adults with cystic fibrosis
Insulinogenic index and early phase insulin secretion predict increased risk of worsening glucose tolerance and of cystic fibrosis-related diabetes
Availability of CFTR modulators in countries of Eastern Europe: The reality in 2022
Comment on: “Serum bile acids in cystic fibrosis patients – glycodeoxycholic acid as a potential marker of liver disease”
Physical activity assessment and vascular function in adults with cystic fibrosis and their non-CF peers
Immunogenicity of BNT162b2 mRNA-Based Vaccine against SARS-CoV-2 in People with Cystic Fibrosis According to Disease Characteristics and Maintenance Therapies
Effects of Levofloxacin, Aztreonam, and Colistin on Enzyme Synthesis by P. aeruginosa Isolated from Cystic Fibrosis Patients
Antibiotherapy in Children with Cystic Fibrosis-An Extensive Review
Differential CFTR-Interactome Proximity Labeling Procedures Identify Enrichment in Multiple SLC Transporters
Conformational Variability in Ground-State CFTR Lipoprotein Particle Cryo-EM Ensembles
Achromobacter spp. Adaptation in Cystic Fibrosis Infection and Candidate Biomarkers of Antimicrobial Resistance

Combined Treatment of Bronchial Epithelial Calu-3 Cells with Peptide Nucleic Acids Targeting miR-145-5p and miR-101-3p: Synergistic Enhancement of the Expression of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene
Can Leukotriene Receptor Antagonist Therapy Improve the Control of Patients with Severe Asthma on Biological Therapy and Coexisting Bronchiectasis? A Pilot Study
Advances in Preclinical In Vitro Models for the Translation of Precision Medicine for Cystic Fibrosis
Cystic Fibrosis and Oxidative Stress: The Role of CFTR
Usefulness of Muscle Ultrasonography in the Nutritional Assessment of Adult Patients with Cystic Fibrosis
Ruthenium(II) Polypyridyl Complexes for Antimicrobial Photodynamic Therapy: Prospects for Application in Cystic Fibrosis Lung Airways
Therapeutic Drug Monitoring of Ivacaftor, Lumacaftor, Tezacaftor, and Elexacaftor in Cystic Fibrosis: Where Are We Now?
Implementation of Model-Based Dose Adjustment of Tobramycin in Adult Patients with Cystic Fibrosis
BEGIN Novel ImagiNG Biomarkers
Response to inhaled ceftazidime in patients with non-cystic fibrosis bronchiectasis and chronic bronchial infection unrelated to Pseudomonas aeruginosa
A missense mutation in the proprotein convertase gene furinb causes hepatic cystogenesis during liver development in zebrafish
Cystic fibrosis transmembrane conductance regulator modulators and the exocrine pancreas: A scoping review
Whole-genome analysis of Haemophilus influenzae strains isolated from persons with cystic fibrosis
Lower airway microbiota and decreasing lung function in young Brazilian cystic fibrosis patients with pulmonary Staphylococcus and Pseudomonas infection
Allergic Bronchopulmonary Aspergillosis in Children Presenting as Lung Masses
Functional Evaluation and Genetic Landscape of Children and Young Adults Referred for Assessment of Bronchiectasis
Risk factors for severe COVID-19 in people with cystic fibrosis: A systematic review
Outcome of lung transplantation in cystic fibrosis patients with severe asymmetric chest cavities
Commentary: Creative solutions for lung transplantation with chest wall asymmetry and cystic fibrosis
Neuromodulation for Children With Cystic Fibrosis Experiencing Chronic Abdominal Pain
T helper 2 Mechanisms involved in Human Rhinovirus Infections and Asthma
Historical note: Horace Dobell’s report of cystic fibrosis in 1872
The effect of a training webinar on decreasing inter-observer variability in making a radiologic diagnosis of bronchiectasis
Supplemental Oxygen Alters the Airway Microbiome in Cystic Fibrosis
Impact of pharmacy services on time to elexacaftor-tezacaftor-ivacaftor initiation
The Value of Sputum Polymerase Chain Reaction for Detection of Nontuberculous Mycobacteria in Cystic Fibrosis Patients with Negative Nontuberculous Mycobacteria Sputum Culture
A successful treatment of a lobar atelectasis in a patient with Cystic Fibrosis
The Utility of Glucose Area Under the Curve from The Oral Glucose Tolerance Test as a Screening Tool for Cystic Fibrosis -Related Diabetes
The respiratory microbiota alpha-diversity in chronic lung diseases: first systematic review and meta-analysis
Update on the management of vitamins and minerals in cystic fibrosis
Impact of Pancreatitis-Associated Protein on Newborn Screening Outcomes and Detection of CFTR-Related Metabolic Syndrome (CRMS)/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): A Monocentric Prospective Pilot Experience
PHLPP1 regulates CFTR activity and lumen expansion through AMPK
Profiling the response to Lumacaftor-Ivacaftor in children with Cystic Fibrosis New insight from a French-Italian real-life cohort
Pulmonary medication adherence among children and adults with Cystic Fibrosis: Is there an association with disease severity?
Impact of antibiotic eradication therapy of Pseudomonas aeruginosa on long term lung function in cystic fibrosis
Carbon nanoparticles adversely affect CFTR expression and toxicologically relevant pathways
An optimized protocol for assessment of sputum macrorheology in health and muco-obstructive lung disease
Oligonucleotide-based therapies for cystic fibrosis
Three Complex alleles associated with N1303K mutation and their molecular consequences
Nitric oxide inhibits alginate biosynthesis in Pseudomonas aeruginosa and increases its sensitivity to tobramycin by downregulating algU gene expression
Human pluripotent stem cell-derived macrophages host Mycobacterium abscessus infection
Long-term follow-up and liver outcomes in children with cystic fibrosis and nodular liver on ultrasound in a multi-center study
The disease-specific clinical trial network for primary ciliary dyskinesia: PCD-CTN
Pharmacokinetic/Pharmacodynamic Evaluation of the Dipeptidyl Peptidase 1 Inhibitor Brensocatib for Non-cystic Fibrosis Bronchiectasis
A Study Assessing KB407 for the Treatment of Cystic Fibrosis
Prevalence, clinical features, and outcomes of COVID-19 in persons with cystic fibrosis: a systematic review protocol
Cystic Fibrosis Modulator Therapies
β-sitosterol ameliorates inflammation and Pseudomonas aeruginosa lung infection in a mouse model
Prevalence of unmet pain and symptom management needs in adults with cystic fibrosis
Antibiotics Drive Expansion of Rare Pathogens in a Chronic Infection Microbiome Model
Bacterial Culture Underestimates Lung Pathogen Detection and Infection Status in Cystic Fibrosis
CFTR Modulators: From Mechanism to Targeted Therapeutics
Time to change course and tackle CF related obesity
Frailty predicts outcomes in cystic fibrosis patients listed for lung transplantation
Partner perspectives on women’s sexual and reproductive healthcare in cystic fibrosis
Microrheology of Mucin-Albumin Assembly Using Diffusing Wave Spectroscopy
Effects of Mucin and DNA Concentrations in Airway Mucus on Pseudomonas aeruginosa Biofilm Recalcitrance
Identification of differentially expressed genes and their major pathways among the patient with COVID-19, cystic fibrosis, and chronic kidney disease
Reduced Expression of TMEM16A Impairs Nitric Oxide-Dependent Cl- Transport in Retinal Amacrine Cells
Cystic fibrosis transmembrane regulator correction attenuates heart failure-induced lung inflammation
Multidrug-resistant Pseudomonas aeruginosa is predisposed to lasR mutation through up-regulated activity of efflux pumps in non-cystic fibrosis bronchiectasis patients
Laboratory biomarkers in the diagnosis and follow-up of treatment of allergic bronchopulmonary aspergillosis in cystic fibrosis
Structural characterization of the novel stress response facilitator (SrfA) from Pseudomonas aeruginosa
Predicting risk-adjusted incidence rates of methicillin-resistant Staphylococcus Aureus and Pseudomonas Aeruginosa in cystic fibrosis programs in the United States
Elexacaftor-Tezacaftor-Ivacaftor Overdose in an Adolescent Female with Cystic Fibrosis
Association between early chronic methicillin-susceptible Staphylococcus aureus colonization and lung function in children with cystic fibrosis
G378X-I148T CFTR variant: A new complex allele in a cystic fibrosis newborn with pancreatic insufficiency
Super resolution microscopy analysis reveals increased Orai1 activity in asthma and cystic fibrosis lungs
Sweat Proteomics in Cystic Fibrosis: Discovering Companion Biomarkers for Precision Medicine and Therapeutic Development
Pseudomonas Aeruginosa Lung Infection Subverts Lymphocytic Responses through IL-23 and IL-22 Post-Transcriptional Regulation
Probiotics Administration in Cystic Fibrosis: What Is the Evidence?
Clinical Features and Treatment Outcomes of Pulmonary Mycobacterium avium-intracellulare Complex With and Without Coinfections
Personalized medicine with drugs targeting the underlying protein defect in cystic fibrosis: is monitoring of treatment response necessary?
The Microbiology of Non-aeruginosa Pseudomonas Isolated From Adults With Cystic Fibrosis: Criteria to Help Determine the Clinical Significance of Non-aeruginosa Pseudomonas in CF Lung Pathology
Silver-manganese nanocomposite modified screen-printed carbon electrode in the fabrication of an electrochemical, disposable biosensor strip for cystic fibrosis
A Giant Sporadic Intra-abdominal Desmoid Tumor in a Male Patient: A Case Report
Cystic fibrosis and jejunoileal atresia: A clinical case
Cystic fibrosis transmembrane conductance regulator (CFTR): a missing link between smoking and chronic airway diseases?
Evidence for Early CFTR Modulator Treatment for Children with Cystic Fibrosis Keeps Growing
Antimicrobial peptides for tackling cystic fibrosis related bacterial infections: A review
Antibiotics and outcomes of CF pulmonary exacerbations in children infected with MRSA and Pseudomonas aeruginosa
Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
Physical activity and exercise training in cystic fibrosis
Molecular Epidemiology of Mycobacterium abscessus Isolates Recovered from German Cystic Fibrosis Patients
Congenital etiologies of exocrine pancreatic insufficiency
Disease severity of people with cystic fibrosis carrying residual function mutations: Data from the ECFS Patient Registry
Chronic rhinosinusitis in people with Cystic Fibrosis: Expanding evidence and future directions
CFTR gene variants, air pollution, and childhood asthma in a California Medicaid population
Cystic fibrosis exacerbations treated with medical therapy with and without concurrent sinus surgery
Achromobacter spp. prevalence and adaptation in cystic fibrosis lung infection
Eating Disorders in Adolescents and Young Adults with Cystic Fibrosis
FEASIBILITY AND ACCEPTABILITY OF A CF-SPECIFIC COGNITIVE-BEHAVIORAL PREVENTIVE INTERVENTION FOR ADULTS INTEGRATED INTO TEAM-BASED CARE
Spectrum of Cystic Fibrosis Conductance Regulator Gene Mutations Reported in Pakistani Descent Cystic Fibrosis Patients
Current knowledge in the use of bacteriophages to combat infections caused by Pseudomonas aeruginosa in cystic fibrosis
Considering the impact of patient ethnicity on cystic fibrosis related bone disease
Novel Macrocyclic 1,3,4-Oxadiazoles as CFTR Modulators for Treating Cystic Fibrosis
A systematic review of lung clearance index in non-cystic fibrosis, non-primary ciliary dyskinesia bronchiectasis
A step forward for an intermediate cystic fibrosis population
Improving the management of chronic Pseudomonas aeruginosa infection in lower respiratory tract
Stress and mental health in adolescents and young adults with cystic fibrosis one year after the COVID pandemic: Findings from an Italian sample

Plasma and cellular ivacaftor concentrations in patients with CF
Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index
Modelling Successful Self-Management in Adults With Cystic Fibrosis: Vicarious Self-Efficacy From Videos of ‘People Like Me’
Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia
Heterogeneous spectrum of CFTR gene mutations in Chinese patients with CAVD and the dilemma of genetic blocking strategy
Surgical considerations in cystic fibrosis: what every general surgeon needs to know
A novel free-breathing abdominal RAVE T2 /T1 hybrid MRI sequence in patients with cystic fibrosis: Preliminary results
Global prevalence of non-tuberculous mycobacteria in adults with non-cystic fibrosis bronchiectasis 2006-2021: a systematic review and meta-analysis
Impact of highly effective CFTR modulator therapy on digital clubbing in patients with cystic fibrosis
Developing an ecological approach to physical activity promotion in adults with Cystic fibrosis
Inhaled antibiotics for pulmonary exacerbations in cystic fibrosis
Real-world Associations of US Cystic Fibrosis Newborn Screening Programs With Nutritional and Pulmonary Outcomes
Pilot Trial of ExACT (Exercise as Airway Clearance Therapy) for People With Cystic Fibrosis
Educational achievements of children aged 10-11 years with cystic fibrosis. A data linkage study in Wales
Effects of the Sex Steroid Hormone Estradiol on Biofilm Growth of Cystic Fibrosis Pseudomonas aeruginosa Isolates
A retrospective correlative profiling of lung functions, microbiological, radiological, periodontal, hematological parameters in noncystic fibrosis bronchiectasis patients of North India
Corrigendum to “Using a learning health system to understand the mismatch between medicines supply and actual medicines use among adults with cystic fibrosis” [J Cyst Fibros (2022), 21/2, 323-331]
The omicron wave modifies the COVID-19 paradigm in children with cystic fibrosis
Continuous glucose monitoring versus self-monitoring of blood glucose in the management of cystic fibrosis related diabetes: A systematic review and meta-analysis
A multimodal iPSC platform for cystic fibrosis drug testing
Non-cell-autonomous activation of Hedgehog signaling contributes to disease progression in a mouse model of renal cystic ciliopathy
Therapeutic applications of curcumin nanomedicine formulations in cystic fibrosis
Editorial: Mechanisms of Novel Drugs and Gene Modifiers in the Treatment of Cystic Fibrosis
Insulin-Like Growth Factor Binding Protein (IGFBP-6) as a Novel Regulator of Inflammatory Response in Cystic Fibrosis Airway Cells
Oxidative stress and impaired insulin secretion in cystic fibrosis pig pancreas
Trends in Growth and Maturation in Children with Cystic Fibrosis Throughout Nine Decades
Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study
Gut Bifidobacteria enrichment following oral Lactobacillus-supplementation is associated with clinical improvements in children with cystic fibrosis
Small molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough
Depression, anxiety, substance misuse and self-harm in children and young people with rare chronic liver disease
Is there a role of viral infection in cystic fibrosis exacerbation in children?
A female with excessive wrinkling of the hands
Monoallelic pathogenic ALG5 variants cause atypical polycystic kidney disease and interstitial fibrosis
Biologic drugs in treating allergic bronchopulmonary aspergillosis in patients with cystic fibrosis: a systematic review
Cystic fibrosis associated with Wernicke’s encephalopathy in an older adult
Clinical and gene mutation features of cystic fibrosis: an analysis of 8 cases
Variation in lung function and clinical aspects in adults with cystic fibrosis
Characterization of the cluster MabR prophages of Mycobacterium abscessus and Mycobacterium chelonae
The Use of Thiocyanate Formulations to Create Manganese Porphyrin Antioxidants That Supplement Innate Immunity
IL-17 Cytokines and Chronic Lung Diseases
Going the Extra Mile: Why Clinical Research in Cystic Fibrosis Must Include Children
Nontuberculous Mycobacterial Lung Disease in the Patients with Cystic Fibrosis-A Challenging Diagnostic Problem
Health-Related Quality of Life in Adults with Cystic Fibrosis: Familial, Occupational, Social, and Mental Health Predictors
A Cross-Sectional Study of the Marital Attitudes of Pregnant Women at Risk for Cystic Fibrosis and Psychological Impact of Prenatal Screening
A Splice Switch in SIGIRR Causes a Defect of IL-37-Dependent Anti-Inflammatory Activity in Cystic Fibrosis Airway Epithelial Cells
Emerging Concepts in Defective Macrophage Phagocytosis in Cystic Fibrosis
Macrophage Lysosomal Alkalinization Drives Invasive Aspergillosis in a Mouse Cystic Fibrosis Model of Airway Transplantation
Identification of Pathogenic Variant Burden and Selection of Optimal Diagnostic Method Is a Way to Improve Carrier Screening for Autosomal Recessive Diseases
Serum Fibrinogen as a Biomarker for Disease Severity and Exacerbation in Patients with Non-Cystic Fibrosis Bronchiectasis
Auxological and Endocrinological Features in Children and Adolescents with Cystic Fibrosis
Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis
Loss of Serum Glucocorticoid-Inducible Kinase 1 SGK1 Worsens Malabsorption and Diarrhea in Microvillus Inclusion Disease (MVID)
The Use of Artificial Sputum Media to Enhance Investigation and Subsequent Treatment of Cystic Fibrosis Bacterial Infections
Activity of Drug Combinations against Mycobacterium abscessus Grown in Aerobic and Hypoxic Conditions
Mycobacterium abscessus : It’s Complex
Newborn Screening for Cystic Fibrosis: A Qualitative Study of Successes and Challenges from Universal Screening in the United States
Pseudomonas aeruginosa : Recent Advances in Vaccine Development
Absence of EPAC1 Signaling to Stabilize CFTR in Intestinal Organoids
Novel Treatment Approach for Aspergilloses by Targeting Germination
Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
Nebulizer Cleaning Practices and Adherence to Nebulized Therapies in People With Cystic Fibrosis When Traveling
Abdominal ultrasound findings in adult patients with cystic fibrosis
Pediatric Medical Traumatic Stress in Inflammatory Bowel Disease, Pancreatitis and Cystic Fibrosis
Aquagenic keratoderma
Telehealth and CFTR modulators: Accelerating innovative models of cystic fibrosis care
Reproducibility of pulmonary magnetic resonance angiography in adults with muco-obstructive pulmonary disease
Clinician attitudes and practices on pregnancy planning and care in cystic fibrosis
Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study
An invisible threat? Aspergillus positive cultures and co-infecting bacteria in airway samples
Disparities in first evaluation of infants with cystic fibrosis since implementation of newborn screening
Unveiling Co-Infection in Cystic Fibrosis Airways: Transcriptomic Analysis of Pseudomonas aeruginosa and Staphylococcus aureus Dual-Species Biofilms
The Role of Pulmonary Function Test in Perioperative Management of Patients with Cystic Fibrosis
Resistance Training Recommendations for Children and Adolescents With Cystic Fibrosis-Related Diabetes
The Role of PaFicT in Pseudomonas aeruginosa Persister Cell Formation
Non-invasive diagnostics of pathogenic bacteria using a breath sampler in children with cystic fibrosis
Treatment preference amongst people with cystic fibrosis: the importance of reducing treatment burden
Different management approaches and outcome for infants with an inconclusive diagnosis following newborn screening for cystic fibrosis (CRMS/CFSPID) and Pseudomonas aeruginosa isolation
Staphylococcus aureus and Pseudomonas aeruginosa Isolates from the Same Cystic Fibrosis Respiratory Sample Coexist in Coculture
Perspectives on Genetic Medicine for Cystic Fibrosis
PlayPhysio: Making Physio Fun
Survival difference between high-risk and low-risk CFTR genotypes after lung transplant
Surface Motility Favors Codependent Interaction between Pseudomonas aeruginosa and Burkholderia cenocepacia
Specificity of Immunoglobulin Response to Nontuberculous Mycobacteria Infection in People with Cystic Fibrosis
Exploring the role of antibiotics and steroids in managing respiratory diseases
Evaluating Differences in the Disease Experiences of Minority Adults With Cystic Fibrosis
Genetic spectrum of Chinese children with cystic fibrosis: comprehensive data analysis from the main referral centre in China
Resistance evolution can disrupt antibiotic exposure protection through competitive exclusion of the protective species
Secondary Metabolites Produced during Aspergillus fumigatus and Pseudomonas aeruginosa Biofilm Formation
The In Vitro Replication Cycle of Achromobacter xylosoxidans and Identification of Virulence Genes Associated with Cytotoxicity in Macrophages
A New Era for Cystic Fibrosis and CFTR Modulator Trials in Infants
Extreme electrolyte depletion associated with septic shock as a form of presentation of cystic fibrosis
Content validation for the Chilean population of the quality of life assessment instrument in children, adolescents and adults with Cystic Fibrosis: CFQ-R CYSTIC FIBROSIS QUESTIONNAIRE-REVISED version in Spanish, Chile
CFTR genotype analysis of Asians in international registries highlights disparities in the diagnosis and treatment of Asian patients with cystic fibrosis
Reducing human DNA bias in cystic fibrosis airway specimens for microbiome analysis
Azithromycin and cystic fibrosis
The potential of volatile organic compound analysis for pathogen detection and disease monitoring in patients with cystic fibrosis
The changing epidemiology of cystic fibrosis: the implications for adult care
Elexacaftor-Tezacaftor-Ivacaftor improves exercise capacity in adolescents with cystic fibrosis
Drugs in Clinical Development to Treat Autosomal Dominant Polycystic Kidney Disease
Potential opportunities and challenges for infection prevention and control for cystic fibrosis in the modern era

WNK Inhibition Increases Surface Liquid pH and Host Defense in Cystic Fibrosis Airway Epithelia
If At First You Don’t Succeed, Trikafta Again
Effectiveness and Safety of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Children With Cystic Fibrosis: A Meta-Analysis
Rechallenge of Elexacaftor/Tezacaftor/Ivacaftor After Skin Rash in Two Pediatric Patients
ACCESS 2: AI for pediatriC diabetiC Eye examS Study 2
Generation of induced pluripotent stem cell line (RCMGi008-A) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/CFTRdele2.3 mutations in CFTR gene
Development and outcomes of a patient driven cystic fibrosis quality of care improvement project
Chronic rhinosinusitis is associated with non-cystic fibrosis bronchiectasis – a new clinical implication?
A case of Elexacaftor-Tezacaftor-Ivacaftor induced rash resolving without interruption of treatment
Longitudinal assessment of renal function after lung transplantation for cystic fibrosis: transition from post-operative acute kidney injury to acute kidney disease and chronic kidney failure
Pharmacokinetics of Intermittent Dosed Intravenous Vancomycin in Adult Persons with Cystic Fibrosis
Membrane lipid renovation in Pseudomonas aeruginosa – implications for phage therapy?
The Exocrine Pancreas in Cystic Fibrosis in the Era of CFTR Modulation: A Mini Review
Psychological status of mothers of children with cystic fibrosis and primary ciliary dyskinesia
Epithelial damage in the cystic fibrosis lung: the role of host and microbial factors
Antibiotic allergy in children with cystic fibrosis: a retrospective case-control study
Limitations of the dichotomized 6-minute walk distance when computing lung allocation score for cystic fibrosis: a 16-year retrospective cohort study
ScRNA-seq expression of IFI27 and APOC2 identifies four alveolar macrophage superclusters in healthy BALF
Erratum: “Volatile organic compound breath signatures of children with cystic fibrosis by real-time SESI-HRMS”. Ronja Weber, Naemi Haas, Astghik Baghdasaryan, Tobias Bruderer, Demet Inci, Srdjan Micic, Nathan Perkins, Renate Spinas, Renato Zenobi and Alexander Moeller for the Paediatric Exhalomics Group at the University Children’s Hospital Zurich. ERJ Open Res 2020; 6: 00171-2019
Pregnancy experience in the setting of cystic fibrosis: A systematic review and thematic synthesis
Effects of Hypoxia on Respiratory Diseases: Perspective View of Epithelial Ion Transport
A Phase 1b/2 Trial of the Safety and Microbiological Activity of Bacteriophage Therapy in Cystic Fibrosis Subjects Colonized With Pseudomonas Aeruginosa
Improving nutrition in cystic fibrosis: A systematic literature review
Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3B, Randomized, Placebo-Controlled Study
The Cystic Fibrosis Action Plan: a low health literacy, pictographic self-management tool with clinical automation
Time Free From Hospitalization in Children and Adolescents With Cystic Fibrosis: Findings From FEV1 , Lung Clearance Index and Peak Work Rate
Pharmacological Responses of the G542X-CFTR to CFTR Modulators
Management of Cepacia Syndrome in an Immunocompetent Non-Cystic Fibrosis Adult Patient
The pharmacology of the TMEM16A channel: therapeutic opportunities
The role of viral infections in pulmonary exacerbations of patients with non-cystic fibrosis bronchiectasis: A systematic review
Feasibility of the A-STEP for the assessment of exercise capacity in people with cystic fibrosis
Development of excipients free inhalable co-spray-dried tobramycin and diclofenac formulations for cystic fibrosis using two and three fluid nozzles
Daily versus three-times-weekly azithromycin in Chinese patients with non-cystic fibrosis bronchiectasis: protocol for a prospective, open-label and randomised controlled trial
Multi-omic comparisons between CFBE41o- cells stably expressing wild-type CFTR and F508del-mutant CFTR
Cystic fibrosis related diabetes is not associated with maximal aerobic exercise capacity in cystic fibrosis: a cross-sectional analysis of an international multicenter trial
Effects of a Long-Term Monitored Exercise Program on Aerobic Fitness in a Small Group of Children with Cystic Fibrosis
Experiences and Perspectives of Individuals with Cystic Fibrosis and Their Families Related to Food Insecurity
Main Metabolites of Pseudomonas aeruginosa : A Study of Electrochemical Properties
Effects of a Long-Term Wearable Activity Tracker-Based Exercise Intervention on Cardiac Morphology and Function of Patients with Cystic Fibrosis
Mucosal Immunity in Cystic Fibrosis
Effects of GLP-1 and GIP on Islet Function in Glucose Intolerant, Pancreatic Insufficient Cystic Fibrosis
Gold nanocluster adjuvant enables the eradication of persister cells by antibiotics and abolishes the emergence of resistance
2D Shear Wave Elastography, a promising screening tool for Cystic Fibrosis liver disease, shows a correlation between vitamin D and liver stiffness
Correlation among clinical, functional and morphological indexes of the respiratory system in non-cystic fibrosis bronchiectasis patients
The changing landscape of the cystic fibrosis lung environment: From the perspective of Pseudomonas aeruginosa
Bilateral Pseudomonas aeruginosa keratitis as presenting feature in an infant of cystic fibrosis
Adverse Childhood Experiences (ACEs) in Adults with Cystic Fibrosis
Persistent Aspergillus fumigatus infection in cystic fibrosis: impact on lung function and role of treatment of asymptomatic colonization-a registry-based case-control study
Ivacaftor, not ivacaftor/lumacaftor, associated with lower pulmonary inflammation in preschool cystic fibrosis
Microsporidial myositis, keratitis and hypercalcaemia in a cystic fibrosis lung transplant recipient
Generation of Airway Epithelial Cell Air-Liquid Interface Cultures from Human Pluripotent Stem Cells
Real Life With Tezacaftor and Ivacaftor in Adult Patients With Cystic Fibrosis: Spanish Multicenter Study
Characterization of the Achromobacter xylosoxidans Type VI Secretion System and Its Implication in Cystic Fibrosis
Heart rate variability, exercise capacity and levels of daily physical activity in children and adolescents with mild-to-moderate cystic fibrosis
CF Patients’ Airway Epithelium and Sex Contribute to Biosynthesis Defects of Pro-Resolving Lipids
Allicin Facilitates Airway Surface Liquid Hydration by Activation of CFTR
Peer i-Coaching for Activated Self-Management Optimization in Adolescents and Young Adults With Chronic Conditions
A Study Evaluating the Long-term Safety and Efficacy of VX-121 Combination Therapy
Future therapies for cystic fibrosis
Pediatric Pulmonary Year in Review 2021: Sleep Medicine
An Update on Ductal Plate Malformations and Fibropolycystic Diseases of the Liver
Reclassifying inconclusive diagnosis for Cystic Fibrosis with new generation sweat test
The U-shaped association of post-lung transplant mortality with pretransplant eGFR underscores possible limitations of creatinine-based estimation equations for risk stratification
MicroRNA global profiling in cystic fibrosis cell lines reveals dysregulated pathways related with inflammation, cancer, growth, glucose and lipid metabolism, and fertility: an exploratory study
The Role of Hypoxia in Improving the Therapeutic Potential of Mesenchymal Stromal Cells. A Comparative Study From Healthy Lung and Congenital Pulmonary Airway Malformations in Infants
Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: Early report
Prevalence and characteristics of cystic fibrosis liver disease: a study highlighting the lack of histological diagnosis
Targeting fibrosis, mechanisms and cilinical trials
Exposure to bile and gastric juice can impact the aerodigestive microbiome in people with cystic fibrosis
Behavioral Health Screening in Military Cystic Fibrosis Centers: A Survey
A Phase 3, Open-Label Study of Lumacaftor/Ivacaftor in Children 1 to Less Than 2 Years of Age With Cystic Fibrosis Homozygous for F508del-CFTR
Pharmacokinetics and Safety Assessment of VX-121/Tezacaftor/Deutivacaftor in Participants With Moderate Hepatic Impairment
Modulation, microbiota and inflammation in the adult CF gut: A prospective study
A Middle East systematic review and meta-analysis of prevalence and antibiotic susceptibility pattern in MRSA Staphylococcus aureus isolated from patients with cystic fibrosis
Stem Cells, Cell Therapies, and Bioengineering in Lung Biology and Disease 2021
In Vitro Comparison of Aztreonam/Amoxicillin-Clavulanate Versus Aztreonam/Ceftazidime-Avibactam on Ceftazidime-Avibactam Resistant Stenotrophomonas maltophilia
Expression of active B-Raf proto-oncogene in kidney collecting ducts induces cyst formation in normal mice and accelerates cyst growth in mice with polycystic kidney disease
ELX/TEZ/IVA use in cystic fibrosis liver disease: Is the perspective of improved lung function worth the risk?
Pancreatic, hepatobiliary, and gastrointestinal manifestations of children with cystic fibrosis: A 10-year experience from a tertiary care center in southern India
Collaboration Between Two CF Centers; one in USA and one in Turkey before and during CoV2 Pandemic
Antimicrobial Peptides against Multidrug-Resistant Pseudomonas aeruginosa Biofilm from Cystic Fibrosis Patients
Crosstalk between lysine methyltransferase Smyd2 and TGF-β-Smad3 signaling promotes renal fibrosis in autosomal dominant polycystic kidney disease
An Update on CFTR Drug Discovery: Opportunities and Challenges
Hemoptysis and the Risk for Lung Transplant or Death without Transplant in Individuals with Cystic Fibrosis in the United States
T-cell-mediated hypersensitivity to lumacaftor and ivacaftor in cystic fibrosis
Correlation of Gut Microbiota, Vitamin D Status, and Pulmonary Function Tests in Children With Cystic Fibrosis
Editorial: Genome Editing to Treat Cystic Fibrosis and Other Pulmonary Diseases
Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis
Systemic oxidative stress in children with cystic fibrosis with bacterial infection including Pseudomonas Aeruginosa
Comparative analysis of respiratory symptom scores to detect acute respiratory events in children with cystic fibrosis
Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study
Evaluation of Aminopyrrolidine Amide to improve chloride transport in CFTR-defective cells
High prevalence of Merkel cell polyomavirus is associated with dysregulation in transcript levels of TLR9 and type I IFNs in a large cohort of CF patients from the Italian (Lazio) reference center for cystic fibrosis
Eruptive melanocytic naevi following initiation of elexacaftor/ivacaftor/tezacaftor for cystic fibrosis
Pseudomonas aeruginosa: pathogenesis, virulence factors, antibiotic resistance, interaction with host, technology advances and emerging therapeutics
Functional role of the ER stress transducer IRE1α in CF airway epithelial inflammation
Being affiliated to a cystic fibrosis centre is important for parents’ everyday life
Short-acting inhaled bronchodilators for cystic fibrosis
Eating Disorders and Disordered Eating Behaviors in Cystic Fibrosis: A Neglected Issue
One Size Does Not Fit All: The Past, Present and Future of Cystic Fibrosis Causal Therapies

CFTR Rescue by Lumacaftor (VX-809) Induces an Extensive Reorganization of Mitochondria in the Cystic Fibrosis Bronchial Epithelium
CFTR and Gastrointestinal Cancers: An Update
Sleep, Sedentary Time and Physical Activity Levels in Children with Cystic Fibrosis
The effect of cystic fibrosis transmembrane conductance regulator modulators on impaired glucose tolerance and cystic fibrosis related diabetes
Altered intravenous drug disposition in people living with cystic fibrosis: a meta-analysis integrating top-down and bottom-up data
PF-06409577 inhibits renal cyst progression by concurrently inhibiting the mTOR pathway and CFTR channel activity
TMED3 Complex Mediates ER Stress-Associated Secretion of CFTR, Pendrin, and SARS-CoV-2 Spike
Mannose-Binding Lectin (MBL) and Gap Junction Protein Alpha 4 (GJA4) Gene Heterogeneity in Relation to Severity of Clinical Disease in Cystic Fibrosis
COVID-19 Lockdown Impacts Among Patients with Cystic Fibrosis: An Italian Regional Reference Centre Experience
Macrocycle-stabilization of its interaction with 14-3-3 increases plasma membrane localization and activity of CFTR
Analyzing the use and impact of elexacaftor/tezacaftor/ivacaftor on total cost of care and other health care resource utilization in a commercially insured population
The thematic role of extracellular loop of VraG in activation of the membrane sensor GraS in a cystic fibrosis MRSA strain differs in nuance from the CA-MRSA strain JE2
Activity of N -Acetylcysteine Alone and in Combination with Colistin against Pseudomonas aeruginosa Biofilms and Transcriptomic Response to N -Acetylcysteine Exposure
Detection of Metallo Beta-Lactamase (MBL) Producing Pseudomonas aeruginosa in a Tertiary Care Hospital, Ghanpur, Medchal, India
Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway disease
Strain-specific predation of Bdellovibrio bacteriovorus on Pseudomonas aeruginosa with a higher range for cystic fibrosis than for bacteremia isolates
Exercise versus airway clearance techniques for people with cystic fibrosis
Emerging medicines to improve the basic defect in cystic fibrosis
Taxonomic position, antibiotic resistance and virulence factors of clinical Achromobacter isolates
Beneficial short-term effect of autogenic drainage on peripheral resistance in childhood cystic fibrosis disease
Synergistic Activity of Repurposed Peptide Drug Glatiramer Acetate with Tobramycin against Cystic Fibrosis Pseudomonas aeruginosa
A novel role for CFTR interaction with LH and FGF in azoospermia and epididymal maldevelopment caused by cryptorchidism
Revealing the human mucinome
Du modèle médical vers un modèle social de l’autonomie des personnes atteintes de mucoviscidose. Une étude sociologique du point de vue des acteurs professionnels et associatifs
Temporal Modulation of Drug Desensitization Procedures
Editorial: Co-Infection and Consequences in Cystic Fibrosis
Satisfaction and Concerns with Telemedicine Endocrine Care of Patients with Cystic Fibrosis
Development of an Interdisciplinary Telehealth Care Model in a Pediatric Cystic Fibrosis Center
Elexacaftor-Tezacaftor-Ivacaftor Treatment Reduces Abdominal Symptoms in Cystic Fibrosis-Early results Obtained With the CF-Specific CFAbd-Score
The Impact of Air Pollution on the Course of Cystic Fibrosis: A Review
COMMUNICATING RESEARCH FINDINGS TO PATIENTS:: Comparison of readability of Patient Lay Summaries written by eight clinical disciplines of the Cystic Fibrosis (CF) Multidisciplinary Team (MDT)
Elexacaftor-Tezacaftor-Ivacaftor improve Gastro-Oesophageal reflux and Sinonasal symptoms in advanced cystic fibrosis
Clearing the air: Uniquely engaging furin as an approach to cystic fibrosis therapy
Acute Pulmonary Exacerbation Phenotypes in Patients with Cystic Fibrosis
CF-Seq, an accessible web application for rapid re-analysis of cystic fibrosis pathogen RNA sequencing studies
The Beneficial Role of Probiotic Lactobacillus in Respiratory Diseases
Ivacaftor Alters Macrophage and Lymphocyte Infiltration in the Lungs Following Lipopolysaccharide Exposure
Metabolomic Biomarkers to Predict and Diagnose Cystic Fibrosis Pulmonary Exacerbations: A Systematic Review
Defining and identifying early-onset lung disease in cystic fibrosis with cumulative clinical characteristics
CFTR RNA- and DNA-based therapies
Prospectively evaluating maternal and fetal outcomes in the era of CFTR modulators: the MAYFLOWERS observational clinical trial study design
Anesthesia and Mucociliary Clearance: Implications for Cystic Fibrosis
Evaluation of VX-121/Tezacaftor/Deutivacaftor in Cystic Fibrosis (CF) Participants 1 Through 11 Years of Age
Molecular mechanisms of Cystic Fibrosis – how mutations lead to misfunction and guide therapy
Food-web and metabolic interactions of the lung inhabitants Streptococcus pneumoniae and Pseudomonas aeruginosa
Impact of illness on mothers of children with cystic fibrosis in Tunisia: A qualitative interview study: Impact of cystic fibrosis on Tunisian mothers
Socioeconomic determinants of respiratory health in patients with cystic fibrosis: implications for treatment strategies
Basic Pancreatic Lesions: Radiologic-pathologic Correlation
Prolonged treatment with inhaled ampicillin in children with non-cystic fibrosis bronchiectasis
Pilot Study to Evaluate a Voice Journaling Application in Adolescents With Cystic Fibrosis
Geographical distribution of cystic fibrosis carriers as population genetic determinant of COVID-19 spread and fatality in 37 countries
The Role of Bronchoscopy in the Management of Children With Cystic Fibrosis
Cystic fibrosis: Quality of life and radiological monitoring
Long-term Follow-up in Adult Patients with Cystic Fibrosis and Deep Intronic Splicing Variants
Can we Train the Immune System of Patients With Cystic Fibrosis?
Effects of Controlled Voluntary Increase in the Ventilatory Demand on Respiratory System Resistance in Healthy and Non-Cystic Fibrosis Bronchiectasis Subjects: A Cross-Sectional Study
Mycobacterium abscessus : insights from a bioinformatic perspective
Analysis of the genotypic profile and its relationship with the clinical manifestations in people with cystic fibrosis: study from a rare disease registry
Coefficient of Fat Absorption to Measure the Efficacy of Pancreatic Enzyme Replacement Therapy in People With Cystic Fibrosis: Gold Standard or Coal Standard?
Calprotectin: the link between acute lung injury and gastrointestinal injury in Covid-19: Ban or boon
Mucus-targeting therapies of defective mucus clearance for cystic fibrosis: A short review
Severity of the S1251N allele in cystic fibrosis is affected by the presence of the F508C variant in cis
Poor Tolerability of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Lung Transplant Recipients
Discovery and SAR of 4-Aminopyrrolidine-2-carboxylic Acid Correctors of CFTR for the Treatment of Cystic Fibrosis
Biologically synthesized silver nanoparticles as potent antibacterial effective against multi drug-resistant Pseudomonas aeruginosa
CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France
Caroli’s disease incidentally discovered in a 16-years-old female: a case report
Risk Factors of Incident Lung Cancer in Patients with Non-Cystic Fibrosis Bronchiectasis: A Korean Population-Based Study
Nanomechanics combined with HDX reveals allosteric drug binding sites of CFTR NBD1
rAAV-MEDIATED EDITING OF THE G551D CFTR MUTATION IN FERRET AIRWAY BASAL CELLS
Five-year performance analysis of a cystic fibrosis newborn screening program in northeastern Brazil
Lubiprostone is non-selective activator of cAMP-gated ion channels and Clc-2 has a minor role in its prosecretory effect in intestinal epithelial cells
New Insights into Clinical and Mechanistic Heterogeneity of the Acute Respiratory Distress Syndrome: Summary of the Aspen Lung Conference 2021
Acute kidney injury in cystic fibrosis patients treated with intravenous colistimethate sodium or tobramycin
The effect of probiotic administration on metabolomics and glucose metabolism in CF patients
The Short Term Influence of Chest Physiotherapy on Lung Function Parameters in Children With Cystic Fibrosis and Primary Ciliary Dyskinesia
“Acquiring the Skills Needed to Communicate What Is in Our Teams’ Heart: Love, Compassion and Partnership”: Qualitative Analysis of Intact Multidisciplinary Teams’ Experience of Relationship-Centered Communication Training
Use of mucoactive agents in cystic fibrosis: A consensus survey of Italian specialists
The inhibition of CFTR in the descended testis of SD rats with unilateral cryptorchidism induced by di-(2-ethylhexyl) phthalate (DEHP)
Routine spirometry in cystic fibrosis patients: impact on pulmonary exacerbation diagnosis and FEV1 decline
Diabetic foot infection caused by bacteria of the Burkholderia cepacia complex: report of an unusual case and a scoping literature review
Early Disseminated Mycobacterium Abscessus Complex Infection in an Infant with Coexisting Cystic Fibrosis and Progressive Familial Intrahepatic Cholestasis: Case report and literature review
Structural Insights into the Inhibition Site in the Phosphorylcholine Phosphatase Enzyme of Pseudomonas aeruginosa
Antibiotic hypersensitivity in cystic fibrosis – low frequency of anaphylaxis over 16,000 courses
Rifaximin for Treatment of Bloating in Children and Adults With Cystic Fibrosis
Treatment With LAU-7b Complements CFTR Modulator Therapy by Improving Lung Physiology and Normalizing Lipid Imbalance Associated With CF Lung Disease
Disease Prevention and Treatment Using β-Carotene: the Ultimate Provitamin A
Video Game-Based Exercise in Children and Adolescents with Non-Cystic Fibrosis Bronchiectasis: A Randomized Comparative Study of Aerobic and Breathing Exercises
Pharmacogenomics: a tool to improve medication safety and efficacy in patients with cystic fibrosis
The first lung retransplantation with “ECMO bridge” in the Czech Republic – case report
Change in exercise capacity measured by Cardio-pulmonary Exercise Testing (CPET) in Danish people with cystic fibrosis after initiation of treatment with Lumacaftor/Ivacaftor and Tezacaftor/Ivacaftor
No patient left behind! Therapeutic options for cystic fibrosis patients living with lung transplantation
Changes in airway bacterial communities occur soon after initiation of antibiotic treatment of pulmonary exacerbations in cystic fibrosis
Up-to-date incidence and initial characteristics of cystic fibrosis in Tunisia
CFTR heterozygosity in severe asthma with recurrent airway infections: a retrospective review
Depression and anxiety prevalence in people with cystic fibrosis and their caregivers: a systematic review and meta-analysis
The effect of azithromycin on structural lung disease in infants with cystic fibrosis (COMBAT CF): a phase 3, randomised, double-blind, placebo-controlled clinical trial
Ivacaftor in Omani children with cystic fibrosis caused by p.Ser549Arg CFTR mutation
COMBATing airway inflammation in infants with cystic fibrosis
The Progress of the Prevention and Treatment of Vitamin D to Tuberculosis

First report on the prevalence of bacteria in cystic fibrosis patients (CF) in a tertiary care center in Saudi Arabia
Liver disease in cystic fibrosis patients in a tertiary care center in Saudi Arabia
Isolation, cultivation, and application of primary respiratory epithelial cells obtained by nasal brushing, polyp samples, or lung explants
Efficient suppression of endogenous CFTR nonsense mutations using anticodon-engineered transfer RNAs
Loss-of-function CFTR p.G970D missense mutation might cause congenital bilateral absence of the vas deferens and be associated with impaired spermatogenesis
The negative impact of chronic rhinosinusitis on the health-related quality of life among adult patients with cystic fibrosis
Genomics of Diversification of Pseudomonas aeruginosa in Cystic Fibrosis Lung-like Conditions
The Genetic relatedness of Burkholderia contaminans clinical isolates from cystic fibrosis and non-cystic fibrosis patients in Argentina
Regulation of Biofilm Exopolysaccharide Biosynthesis and Degradation in Pseudomonas aeruginosa
The sweat chloride test has lived up to the changes in CF care
The MUC5B Promoter Polymorphism is Not Associated With Non-ILD Chronic Respiratory Diseases or Post-transplant Outcome
Translational Research in Cystic Fibrosis: From Bench to Beside
Metabolomics of airways disease in cystic fibrosis
The effect of antibiotic changes during treatment of cystic fibrosis pulmonary exacerbations
Splicing mutations in the CFTR gene as therapeutic targets
Development and internal validation of a prognostic model of 2-year death or lung transplant for cystic fibrosis
Improvement in sinonasal quality of life indicators for pediatric patients with cystic fibrosis treated with elexacaftor-tezacaftor- ivacaftor
Effectiveness of omalizumab as treatment of allergic pulmonary aspergillosis in patients with cystic fibrosis and asthma
The Relation of CFTR-Genotype and Associated Comorbidities to Development of Pulmonary Atelectasis in Cystic Fibrosis Patients
A Public Dialogue to Inform the Use of Wider Genomic Testing When Used as Part of Newborn Screening to Identify Cystic Fibrosis
The prevalence of common CFTR gene mutations and polymorphisms in infertile Iranian men with very severe oligozoospermia
Accounting for population structure in genetic studies of cystic fibrosis
High-risk twin pregnancy: case report of an adolescent patient with cystic fibrosis and systemic lupus erythematosus
The Human ATP-Binding Cassette (ABC) Transporter Superfamily
Increased sputum lactate during oral glucose tolerance test in cystic fibrosis
Inhibition of the sodium-dependent HCO3 – transporter SLC4A4, produces a cystic fibrosis-like airway disease phenotype
Overcoming Health Disparities in Access to CFTR Modulator Therapies: One Child’s Journey with Cystic Fibrosis
Competition between Pseudomonas aeruginosa and Staphylococcus aureus is dependent on intercellular signaling and regulated by the NtrBC two-component system
Improved in-vivo airway gene transfer via magnetic-guidance, with protocol development informed by synchrotron imaging
PILOT STUDY OF INFLAMMATORY BIOMARKERS IN MATCHED INDUCED SPUTUM AND BRONCHOALVEOLAR LAVAGE OF 2-YEAR-OLDS WITH CYSTIC FIBROSIS
Towards Innovative Antibacterial-Correctors for Cystic Fibrosis Targeting the Lung Microbiome with a Multifunctional Effect
Unconventional T Cell Immunity in the Lungs of Young Children with Cystic Fibrosis
Characterization of the Extracellular Volatile Metabolome of Pseudomonas Aeruginosa Applying an in vitro Biofilm Model under Cystic Fibrosis-Like Conditions
T405, a New Penem, Exhibits In Vivo Efficacy against M. abscessus and Synergy with β-Lactams Imipenem and Cefditoren
Changes in Microbiome Dominance Are Associated With Declining Lung Function and Fluctuating Inflammation in People With Cystic Fibrosis
Prevalence and Determinants of Wheezing and Bronchodilatation in Children With Cystic Fibrosis: A Retrospective Cohort Study
Establishment of a CFTR-based detection method for the second messenger cAMP in the cytoplasm
Who are the 10%? – Non eligibility of cystic fibrosis (CF) patients for highly effective modulator therapies
Gene-specific nonsense-mediated mRNA decay targeting for cystic fibrosis therapy
Links between Disease Severity, Bacterial Infections and Oxidative Stress in Cystic Fibrosis
Gene Polymorphism of Biotransformation Enzymes and Ciprofloxacin Pharmacokinetics in Pediatric Patients with Cystic Fibrosis
Colonic Fluid and Electrolyte Transport 2022: An Update
Complex Presentation of Hao-Fountain Syndrome Solved by Exome Sequencing Highlighting Co-Occurring Genomic Variants
Evaluating the Effect of Kaftrio on Perspectives of Health and Wellbeing in Individuals with Cystic Fibrosis
Oral Hygiene and Periodontal Treatment Needs in Adult Patients with Cystic Fibrosis (CF)
Increased Safety Behavior and COVID-19-Related Fear in Adults with Cystic Fibrosis during the Pandemic
Oral Care Recommendation for Cystic Fibrosis Patients-Recommendation for Dentists
Association between Smoking Status and Incident Non-Cystic Fibrosis Bronchiectasis in Young Adults: A Nationwide Population-Based Study
Human Amniotic Mesenchymal Stem Cells and Fibroblasts Accelerate Wound Repair of Cystic Fibrosis Epithelium
Essential Oils Biofilm Modulation Activity and Machine Learning Analysis on Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients
Evaluation of a Novel Chromogenic Medium for the Detection of Pseudomonas aeruginosa in Respiratory Samples from Patients with Cystic Fibrosis
The Role of Zinc in the Pathogenesis of Lung Disease
Covalent Inhibition of the Human 20S Proteasome with Homobelactosin C Inquired by QM/MM Studies
Elexacaftor-Tezacaftor-Ivacaftor as a Final Frontier in the Treatment of Cystic Fibrosis: Definition of the Clinical and Microbiological Implications in a Case-Control Study
Liposome-Encapsulated Tobramycin and IDR-1018 Peptide Mediated Biofilm Disruption and Enhanced Antimicrobial Activity against Pseudomonas aeruginosa
Current clinical opinion on CFTR dysfunction and patient risk of pancreatitis: diagnostic and therapeutic considerations
Viruses to the rescue-Use of bacteriophage to treat resistant pulmonary infections
Children with cystic fibrosis have elevated levels of fecal chitinase-3-like-1
A Commensal Streptococcus Dysregulates the Pseudomonas aeruginosa Nitrosative Stress Response
Antitoxin CrlA of CrlTA Toxin-Antitoxin System in a Clinical Isolate Pseudomonas aeruginosa Inhibits Lytic Phage Infection
New drugs in cystic fibrosis: what has changed in the last decade?
Genomic characterization of lytic bacteriophages targeting genetically diverse Pseudomonas aeruginosa clinical isolates
High Adherence, Microbiological Control and Reduced Exacerbations in Patients With Non-Cystic Fibrosis Bronchiectasis Treated With Nebulised Colistin. A Prospective Observational Study
Polymicrobial infections can select against Pseudomonas aeruginosa mutators because of quorum-sensing trade-offs
Symptom Based Performance of Airway Clearance After Starting Highly Effective Modulators for Cystic Fibrosis (SPACE-CF)
ERS International Congress 2021: highlights from the Paediatric Assembly
VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non-G551D-CFTR gating mutations
Characteristics of Stenotrophomonas maltophilia isolates from cystic fibrosis patients in Russia
The Use of Machine Learning Techniques to Predict Diabetes in Patients with Cystic Fibrosis
Author Reply to: Empowering Without Misinforming Adolescents and Young Adults with Cystic Fibrosis. Comment on “Perceptions of Social Media Use to Augment Health Care Among Adolescents and Young Adults With Cystic Fibrosis: Survey Study”
Empowering Without Misinforming Adolescents and Young Adults with Cystic Fibrosis. Comment on “Perceptions of Social Media Use to Augment Health Care Among Adolescents and Young Adults With Cystic Fibrosis: Survey Study”
Evaluation of nutritional status and related factors in children with cystic fibrosis
Current state of CFTR modulators for treatment of Cystic Fibrosis
Coexistence of pan-hypogammaglobulinaemia and primary ciliary dyskinesia
Reversible bronchiectasis caused by influenza virus mimicking Williams-Campbell syndrome
Glycosylation increases the anti-QS as well as anti-biofilm and anti-adhesion ability of the cyclo (L-Trp-L-Ser) against Pseudomonas aeruginosa
Analysis of CFTR gene expression as an immunological and prognostic biomarker in pan-cancers
Pulmonary hemorrhage in a neonate born to a woman with cystic fibrosis treated with targeted cystic fibrosis transmembrane conductance regulator modulator elexacaftor-tezacaftor-ivacaftor during pregnancy
Fever in a Patient with a Central Venous Catheter Colonized by Pandoraea pnomenusa
Isolation of Exophiala dermatitidis is not associated with worse clinical outcomes during acute pulmonary exacerbations in cystic fibrosis
Mental health screening of patients with cystic fibrosis through telehealth during COVID-19: Evaluation of feasibility and process adoption
Identification of IQGAP1 as a SLC26A4 (Pendrin)-Binding Protein in the Kidney
Effect of Triple Combination CFTR Modulator Therapy on Sleep in Adult Patients with Cystic Fibrosis
First in Poland Simultaneous Liver-Lung Transplantation With Liver-First Approach for Recipient Due to Cystic Fibrosis: A Case Report
Parenthood challenged by cystic fibrosis. Experience of affected parents
Complex and unexpected outcomes of antibiotic therapy against a polymicrobial infection
Preliminary results of abdominal simultaneous multi-slice accelerated diffusion-weighted imaging with motion-correction in patients with cystic fibrosis and impaired compliance
Objective and patient-based measures of chronic rhinosinusitis in people with cystic fibrosis treated with highly effective modulator therapy
Does the effect of comprehensive respiratory physiotherapy home-program differ in children with cystic fibrosis and non-cystic fibrosis bronchiectasis?
Effect of airway clearance therapies on mucociliary clearance in adults with cystic fibrosis: A randomized controlled trial
Dysregulation of the Scribble/YAP/β-catenin axis sustains the fibroinflammatory response in a PKHD1-/- mouse model of congenital hepatic fibrosis
Fibrotic Cystic Lung Disease Post Hematopoietic Stem Cell Transplant: Who is the Culprit?
Editorial: Cystic Fibrosis in Children
Cystic fibrosis learning network telehealth innovation lab during the COVID-19 pandemic: a success QI story for interdisciplinary care and agenda setting
Inhibition mechanism of the chloride channel TMEM16A by the pore blocker 1PBC
JMM Profile: Achromobacter xylosoxidans : the cloak-and-dagger opportunist
Diagnosis and predicted outcomes of patients with cystic fibrosis related liver disease considered for lung transplantation
Genomics of diversification of Pseudomonas aeruginosa in cystic fibrosis lung-like conditions
Evaluation of Frequency and Characteristics of Drug Allergy in Pediatric Patients with Cystic Fibrosis
Infection and Inflammation MUC Up the Cystic Fibrosis Airway

Novel CFTR Modulators for Treating Cystic Fibrosis
Parenting Children with Cystic Fibrosis: Developmental Acquisition of Expertise
Association between stool consistency and clinical variables among infants with cystic fibrosis: Findings from the BONUS study
Research letter: The impact of elexacaftor/tezacaftor/ivacaftor on adherence to nebulized maintenance therapies in people with cystic fibrosis
Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis
Dornase Alfa
IgA Serological Response for the Diagnosis of Mycobacterium abscessus Infections in Patients with Cystic Fibrosis
Comparison of Airway Clearance Therapies for Cystic Fibrosis: A Cochrane Review Summary With Commentary
Recruitment of monocytes primed to express heme oxygenase-1 ameliorates pathological lung inflammation in cystic fibrosis
Validation of nasal tracheal aspiration in children with lung disease
Asymptomatic Pyuria as a Prognostic Biomarker in Autosomal Dominant Polycystic Kidney Disease
Vitamin D Receptor Activation Exerts Anti-Secretory Actions In Colonic Epithelial Cells
How Should the Effects of CFTR Modulator Therapy on Cystic Fibrosis Lung Disease be Monitored?
Animal models of cystic fibrosis in the era of highly effective modulator therapies
Rapid chloride and bicarbonate determination by capillary electrophoresis for confirmatory testing of cystic fibrosis infants with volume-limited sweat specimens
ELEXACAFTOR/TEZACAFTOR/IVACAFTOR IN CHILDREN AGED 6-11 YEARS WITH CYSTIC FIBROSIS, AT LEAST ONE F508DEL ALLELE, AND ADVANCED LUNG DISEASE: A 24-WEEK OBSERVATIONAL STUDY
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