Pending

Incidence of Intraoperative Vasoplegic Syndrome in Lung Transplantation
Successful Bridge to Lung Transplant in a Patient With Cystic Fibrosis Colonized With Pan-Resistant Burkholderia Multivorans
Comment on: “Cystic fibrosis related bone disease in children: Can it be predicted?”
Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis
Treatment of intestinal and liver features in cystic fibrosis mice by the osmotic laxative polyethylene glycol
When is Burkholderia cepacia complex truly eradicated in adults with cystic fibrosis? A 20-year follow up study
Pharmacy compounded pilocarpine: An adequate solution to overcome shortage of pilogel® discs for sweat testing in patients with cystic fibrosis
Cystic fibrosis: A changing landscape
The positive impact of journaling on adolescents with cystic fibrosis
Transcriptional profiling and genetic analysis of a cystic fibrosis airway-relevant model shows asymmetric responses to growth in a polymicrobial community
Effects of Azithromycin on Airway Oxidative Stress Markers in Patients With Bronchiectasis
A Cross-Sectional Study of Pediatric Feeding Disorder in Children with Cystic Fibrosis
Selenoneine-inspired selenohydantoins with glutathione peroxidase-like activity
Phase 2a, 28-day Investigational Use Study of ARINA-1 in Non-CF Bronchiectasis With Excess Mucus and Cough
A Study to Assess the Efficacy, Safety, and Tolerability of Brensocatib in Participants With Non-Cystic Fibrosis Bronchiectasis
Breathing Training and Exercise Capacity in Non-CFB
Molecular epidemiology and genomic dynamics of Pseudomonas aeruginosa isolates causing relapse infections
Examining the activity of cefepime-taniborbactam against Burkholderia cepacia complex and Burkholderia gladioli isolated from cystic fibrosis patients in the United States
Airway Microbiome of Cystic Fibrosis Patients
Advances in the Use of N -Acetylcysteine in Chronic Respiratory Diseases
Clinical and Genetic Characteristics of a Patient with Cystic Fibrosis with a Complex Allele [E217G;G509D] and Functional Evaluation of the CFTR Channel
The Anion Channel TMEM16a/Ano1 Modulates CFTR Activity, but Does Not Function as an Apical Anion Channel in Colonic Epithelium from Cystic Fibrosis Patients and Healthy Individuals
Dynamic Hyperinflation While Exercising-A Potential Predictor of Pulmonary Deterioration in Cystic Fibrosis
Advances in the Cystic Fibrosis Drug Development Pipeline
Development of Quinazolinone Derivatives as Modulators of Virulence Factors of Pseudomonas aeruginosa Cystic Fibrosis Strains
Sputum from People with Cystic Fibrosis Reduces the Killing of Methicillin-Resistant Staphylococcus aureus by Neutrophils and Diminishes Phagosomal Production of Reactive Oxygen Species
Preclinical Investigation of a Lipoglycopeptide Dry Powder Inhalation Therapy for the Treatment of Pulmonary MRSA Infection
Recent advances in proteomic-based diagnostics of cystic fibrosis
Optimal Therapeutic Drug Monitoring Strategy for IV Aminoglycosides and IV Vancomycin in People with Cystic Fibrosis: A Systematic Review
Quantitative Evaluation of CFTR Gene Expression: A Comparison between Relative Quantification by Real-Time PCR and Absolute Quantification by Droplet Digital PCR
Reduced Sphingosine in Cystic Fibrosis Increases Susceptibility to Mycobacterium abscessus Infections
Impaired Nitric Oxide Synthetase Activity in Primary Ciliary Dyskinesia-Data-Driven Hypothesis
Fibroblast Growth Factor 23 Signaling Does Not Increase Inflammation from Pseudomonas aeruginosa Infection in the Cystic Fibrosis Bronchial Epithelium
Advancements in Chitosan-Based Nanoparticles for Pulmonary Drug Delivery
Cystic fibrosis autoantibody signatures associate with Staphylococcus aureus lung infection or cystic fibrosis-related diabetes
Neuropsychiatric symptoms in a patient under cystic fibrosis transmembrane conductance regulator modulators treatment: a case report
Changes in fecal elastase-1 following initiation of CFTR modulator therapy in pediatric patients with cystic fibrosis
Transplantation of iPSC-derived airway epithelia with a collagen scaffold into the nasal cavity
Sustained Improvement of Cystic Fibrosis Lung Disease Following Initiation of Elexacaftor/Tezacaftor/Ivacaftor Therapy: Lessons from Real-World Studies
Phoenix from the Ashes: Celebrating the 2023 North American Cystic Fibrosis Conference
Prebiotics for people with cystic fibrosis
Neonatal Screening for Cystic Fibrosis in Hungary-First-Year Experiences
Computed tomography reveals hypertrophic remodelling of the diaphragm in cystic fibrosis but not in COPD
The Impact of CFTR Modulating Therapy on Chronic Lung Infection in Patients with Cystic Fibrosis
Cystic Fibrosis Airways: Does Disease Stem from Faulty Stem Cells?
Prevalence of adverse events in cystic fibrosis patients treated with elexacaftor/tezacaftor/ivacaftor: Experience of the regional referral center in Tuscany, Italy
Electronic home monitoring of children with cystic fibrosis to detect and treat acute pulmonary exacerbations and its effect on 1-year FEV1
Evolution of lasR mutants in polymorphic Pseudomonas aeruginosa populations facilitates chronic infection of the lung
Phase 1 Study to Assess Safety and Efficacy of ANG003
Structure-based discovery of CFTR potentiators and inhibitors
Small molecule correctors divert CFTR-F508del from ERAD by stabilizing sequential folding states
A Novel Mutation in Cystic Fibrosis Presenting as Pseudo Bartter Syndrome: A Case Report
Epithelial alarmins: a new target to treat chronic respiratory diseases
Periodontal Effects of the Reversible Dipeptidyl Peptidase 1 Inhibitor Brensocatib in Bronchiectasis
Decreased plasma levels of sphingolipids and total cholesterol in adult cystic fibrosis patients
In vitro and in silico evaluation of the serrapeptase effect on biofilm and amyloids of Pseudomonas aeruginosa
Correction to: L1077P CFTR pathogenic variant function rescue by Elexacaftor-Tezacaftor-Ivacaftor in cystic fbrosis patient-derived air-liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients
Gastroesophageal reflux burden in youth with CF treated with elexacaftor-tezacaftor-ivacaftor
Rapid-Acting Insulin Used to Treat a Case of Early Cystic Fibrosis-Related Diabetes Complicated by Post Prandial Hypoglycemia
Comparison of amikacin lung delivery between AKITA® and eFlow rapid® nebulizers in healthy controls and patients with CF: A randomized cross-over trial
Education and implementation of home spirometry in an adolescent cystic fibrosis population
Automatic analysis of bronchus-artery dimensions to diagnose and monitor airways disease in cystic fibrosis
Effects of elexacaftor/tezacaftor/ivacaftor on liver fibrosis markers in adults with cystic fibrosis
Saline Enema Administration in Meconium Obstruction of Prematurity and Impact on the Resolution, Feeds, Microbiome, and Gut-brain Axis.
Transgenic ferret models define pulmonary ionocyte diversity and function
Pulmonary delivery systems for antimicrobial peptides
The Cystic Fibrosis Learning Network: A mixed methods evaluation of program goals, attributes, and impact
Very early onset perinatal constipation: Can it be cow’s milk protein allergy?
CFTR Folding: From Structure and Proteostasis to Cystic Fibrosis Personalized Medicine
Pharmacokinetic drug-drug interactions between Paxlovid® (nirmatrelvir/ritonavir) and CFTR modulators for cystic fibrosis
Exploring The “Misfolding Problem” by Systematic Discovery and Analysis of Functional-But-Degraded Proteins
Use of CFTR modulators in special populations, part 2: Severe lung disease
Stenotrophomonas maltophilia in people with Cystic Fibrosis: a systematic review of prevalence, risk factors and management
The non-invasive evaluation of liver involvement in patients with cystic fibrosis: A prospective study
Parallel evolution of linezolid-resistant Staphylococcus aureus in patients with cystic fibrosis
Cystic fibrosis and CFTR-related disorder with electrolyte imbalance at diagnosis: clinical features and outcome in an Italian cohort
Hope in Every Breath: Navigating the Therapeutic Landscape of Cystic Fibrosis
Effects of insulin therapy optimization with sensor augmented pumps on glycemic control and body composition in people with cystic fibrosis-related diabetes
Characterization of impaired beta and alpha cell function in response to an oral glucose challenge in cystic fibrosis: a cross-sectional study
Prevalence of Low Testosterone in Men with Cystic Fibrosis and CBAVD: a cross-sectional study using a large, multi-institutional database
Optimization of performance of Dutch newborn screening for cystic fibrosis
CTNNAL1 deficiency suppresses CFTR expression in HDM-induced asthma mouse model through ROCK1-CAL signaling pathway
SNSP113 (PAAG) improves mucociliary transport and lung pathology in the Scnn1b-Tg murine model of CF lung disease
Improvement in cystic fibrosis newborn screening program outcomes with genetic counseling via telemedicine
Real-life experience with a generic formulation of lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for the Phe508del CFTR mutation
Continuous Glucose Monitoring Systems and the Efficacy of Acarbose Treatment in Cystic Fibrosis-related Dysglycemia
NaCl-induced modulation of species distribution in a mixed P. aeruginosa / S. aureus /B. cepacia biofilm
Changes of androgen and corticosterone metabolites excretion and conversion in cystic fibrosis
A pediatric cystic fibrosis arthropathy case who responded to Elexacaftor/Tezacaftor/Ivacaftor therapy
Life-changing cystic fibrosis treatment wins US$3-million Breakthrough Prize
Reproducibility of in-home CFRD screening using continuous glucose monitoring and mixed meal tolerance test
Red ginseng aqueous extract improves mucociliary transport dysfunction and histopathology in CF rat airways
Global disparities in cystic fibrosis outcomes prior to CFTR modulators: A CF registries cohort study in South Africa and Canada
Improvement in Lung Clearance Index and Chest CT Scores with Elexacaftor/Tezacaftor/Ivacaftor Treatment in People with Cystic Fibrosis Aged 12 Years and Older – The RECOVER Study
Ceftolozane/Tazobactam Continuous Infusion for Infective Exacerbations of Cystic Fibrosis and Bronchiectasis
Genetic therapies in cystic fibrosis
Non-cystic fibrosis bronchiectasis: a retrospective review of clinical, radiological, microbiological and lung function profile at a tertiary care center of low-middle income country
Expression of gain-of-function CFTR in cystic fibrosis airway cells restores epithelial function better than wild-type or codon-optimized CFTR
A retrospective study on newborn screening for metabolic disorders
Engineering in vitro models of cystic fibrosis lung disease using neutrophil extracellular trap inspired biomaterials

Cystic fibrosis: Does it matter to avoid crushing Elexacaftor/Tezacaftor/Ivacaftor (ETI) tablets?
Spanish adaptation and validation of the child- and parent-report cystic fibrosis questionnaire-revised (CFQ-R)
Are we there yet? The ongoing journey of cystic fibrosis care
The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches
CFTR modulator therapy: transforming the landscape of clinical care in cystic fibrosis
Trial emulation with observational data in cystic fibrosis
Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy
Advancing the pipeline of cystic fibrosis clinical trials: a new roadmap with a global trial network perspective
End-of-life care in cystic fibrosis in the era of new therapies
Cystic fibrosis-related mortality in the United States from 1999 to 2020: an observational analysis of time trends and disparities
Theratyping Cystic Fibrosis patients to guide Elexacaftor-Tezacaftor-Ivacaftor out of label prescription
Inflammatory Activity of Epithelial Stem Cell Variants from Cystic Fibrosis Lung Is Not Resolved by CFTR Modulators
Evolving nutritional needs, obesity, and overweight status in cystic fibrosis
Lung Transplant READY CF 2: CARING CF Ancillary RCT
Long-term clinical outcomes of elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis and advanced pulmonary disease
Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics
Quality over quantity: the next ACT in airway clearance in cystic fibrosis
Innovation in care closer to home for people with cystic fibrosis: The importance of evaluating and collaborating
Applications and Research Advances in the Delivery of CRISPR/Cas9 Systems for the Treatment of Inherited Diseases
Pathogenic Relationships in Cystic Fibrosis and Renal Diseases: CFTR, SLC26A9 and Anoctamins
Modulation of Plasmatic Matrix Metalloprotease 9: A Promising New Tool for Understanding the Variable Clinical Responses of Patients with Cystic Fibrosis to Cystic Fibrosis Transmembrane Conductance Regulator Modulators
Cystic Fibrosis and Cancer: Unraveling the Complex Role of CFTR Gene in Cancer Susceptibility
Effect of Lactobacillaceae Probiotics on Colonic Microbiota and Metabolite Production in Cystic Fibrosis: A Comparative In Vitro Study
A Pilot Randomized Trial of Pediatric Cystic Fibrosis Pulmonary Exacerbations Treatment Strategies
Lung Transplant READY CF 2: A Multi-site RCT
Protective role of CFTR during fungal infection of cystic fibrosis bronchial epithelial cells with Aspergillus fumigatus
Spatial lipidomics reveals biased phospholipid remodeling in acute Pseudomonas lung infection
Cystic fibrosis liver disease in the post-modulator era
Clinical characteristics and genetic mutations of 10 Chinese children with cystic fibrosis and cystic frbrosis transmembrane conductance regulator-related disorders
Cystic fibrosis research: The only constant is change
Elexacaftor-Tezacaftor-Ivacaftor Therapy for Chronic Sinus Disease in Cystic Fibrosis
A liposomal platform for the delivery of ion channel proteins for treatment of channelopathies – Application in therapy of cystic fibrosis
L1077P CFTR pathogenic variant function rescue by Elexacaftor-Tezacaftor-Ivacaftor in cystic fibrosis patient-derived air-liquid interface (ALI) cultures and organoids: in vitro guided personalized therapy of non-F508del patients
Predictive modeling of antibiotic eradication therapy success for new-onset Pseudomonas aeruginosa pulmonary infections in children with cystic fibrosis
A Chinese pediatric cystic fibrosis patient with the c.1624G>T, p. Gly542x/c.223C>T, p. Arg75x genotype
Mechanisms of ventilatory limitation to maximum exercise in children and adolescents with chronic airway diseases
Differentiation and Characterization of Cystic Fibrosis Transmembrane Conductance Regulator Knockout Human Pluripotent Stem Cells into Salivary Gland Epithelial Progenitors
Relationship of muscle thickness, strength, and diaphragm function in adults with cystic fibrosis
Delivering a New Future for People With Cystic Fibrosis
“I think most people feel like healthcare professionals tell them to take their treatments and judge them for not taking them”: reflexive thematic analysis of the views of adults with cystic fibrosis on how treatment adherence is discussed in healthcare
Prospective evaluation of nontuberculous mycobacteria disease in cystic fibrosis: The design of the PREDICT study
Prevalence and impact of human papillomavirus (HPV) in adults with cystic fibrosis: A self-reported cross-sectional Canadian survey
Food insecurity screening and local food access: Contributions to nutritional outcomes among children and adults with cystic fibrosis in the United States
Analysis of Pseudomonas aeruginosa transcription in an ex vivo cystic fibrosis sputum model identifies metal restriction as a gene expression stimulus
Diagnostic challenges in CFTR-related metabolic syndrome: Where the guidelines fall short
CFTR potentiator ivacaftor protects against noise-induced hair cell loss by increasing Nrf2 and reducing oxidative stress
Quality of life in children with skin disease: A Spanish sample
A systematic review of the clinical impact of small colony variants in patients with cystic fibrosis
Falling through the Cracks-The Impact of Care Gaps on Lung Function Loss in Cystic Fibrosis
Neuropsychiatric adverse effects from CFTR modulators deserve a serious research effort
Experiences of cystic fibrosis newborn screening and genetic counseling
Telerehabilitation and telemonitoring interventions programs used to improving quality of life in people with cystic fibrosis: A systematic review
Real world effectiveness of airway clearance techniques in children with cystic fibrosis
Food for thought: The revolution stirring within nutritional care priorities in cystic fibrosis
Update on Cystic Fibrosis in Pediatric Patients
Drug-induced eRF1 degradation promotes readthrough and reveals a new branch of ribosome quality control
Imaging Findings in Cirrhotic Liver: Pearls and Pitfalls for Diagnosis of Focal Benign and Malignant Lesions
Transduction of ferret surface and basal cells of airways, lung, liver and pancreas via intratracheal or intravenous delivery of AA1 or 6
Study of ARO-RAGE in Healthy Subjects and Patients With Inflammatory Lung Disease
Internet-delivered Cystic Fibrosis Mental Health Prevention, Wellness, Resource Program: How Does it Work?
Exploring the methylation status of CFTR and PKIA genes as potential biomarkers for lung adenocarcinoma
Use of adenine base editing and homology-independent targeted integration strategies to correct the cystic fibrosis causing variant, W1282X
Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis
What do people with cystic fibrosis eat? Diet quality, macronutrient and micronutrient intakes (compared to recommended guidelines) in adults with cystic fibrosis-A systematic review
Extracellular traps are evident in Romanowsky-stained smears of bronchoalveolar lavage from children with non-cystic fibrosis bronchiectasis
Higher levels of Pseudomonas aeruginosa LasB elastase expression are associated with early-stage infection in cystic fibrosis patients
Impact of COVID-19 social distancing recommendations on pulmonary function, nutritional status, and morbidity in patients with cystic fibrosis
Longitudinal Profiling of the Intestinal Microbiome in Children with Cystic Fibrosis Treated with Elexacaftor-Tezacaftor-Ivacaftor
Combination Treatment to Improve Mucociliary Transport of Pseudomonas aeruginosa Biofilms
Impact of pharmacy services on people with cystic fibrosis (PwCF) immunization rates
The effect of inspiratory muscle training in PCD and CF patients: A pilot study
Esophageal squamous cell carcinoma at the site of tracheo-esophageal fistula repair in a patient with cystic fibrosis
Epithelial TNF controls cell differentiation and CFTR activity to maintain intestinal mucin homeostasis
Delivery Characterization of SPL84 Inhaled Antisense Oligonucleotide Drug for 3849 + 10 kb C- > T Cystic Fibrosis Patients
Breath of fresh insight: unraveling the evolution of our understanding of cystic fibrosis pulmonary exacerbations
Terpenoids as Human Neutrophil Elastase (HNE) Inhibitors: a comprehensive review of Natural Anti-inflammatory Isoprenoids
A Double-Blind, Active-Controlled, Multiple-Ascending Dose Study of Aerosolized RSP-1502 in Subjects With CF and Chronic PA Lung Infection
Editorial: New insights into caring for pediatric patients with cystic fibrosis
Carrier Screening Programs for Cystic Fibrosis, Fragile X Syndrome, Hemoglobinopathies and Thalassemia, and Spinal Muscular Atrophy: A Health Technology Assessment
South African Thoracic Society position statement on the management of non-cystic fibrosis bronchiectasis in adults: 2023
Extracellular vesicles from BALF of pediatric cystic fibrosis and asthma patients increase epithelial sodium channel activity in small airway epithelial cells
The development and characterization of in vivo-like three-dimensional models of bronchial epithelial cell lines
Pancreatic duct organoid swelling is chloride-dependent
Oral Glucose Tolerance Testing Using Candy: A Sweet Solution to Improve Screening in Children with Cystic Fibrosis?
CFTR Modulates Hypothalamic Neuron Excitability to Maintain Female Cycle
Infection, Allergy, and Inflammation: The Role of Aspergillus fumigatus in Cystic Fibrosis
Advancing Treatment Strategies: A Comprehensive Review of Drug Delivery Innovations for Chronic Inflammatory Respiratory Diseases
Stability Considerations for Bacteriophages in Liquid Formulations Designed for Nebulization
Next-Generation Sequencing for Screening Analysis of Cystic Fibrosis: Spectrum and Novel Variants in a South-Central Italian Cohort
Elexacaftor Mediates the Rescue of F508del CFTR Functional Expression Interacting with MSD2
Parents’ Knowledge of the Impact of Cystic Fibrosis on the Quality of Life of Children and Adolescents Suffering from This Disease as an Element of Patient Safety
Addressing Symptom Burden and Palliative Care Needs in Cystic Fibrosis: A Narrative Review of the Literature
Diagnostic and Communication Challenges in Cystic Fibrosis Newborn Screening
Clinical and Experimental Determination of Protection Afforded by BCG Vaccination against Infection with Non-Tuberculous Mycobacteria: A Role in Cystic Fibrosis?
Compounding Achromobacter Phages for Therapeutic Applications
Biologically Relevant Murine Models of Chronic Pseudomonas aeruginosa Respiratory Infection
The Development and Evaluation of iCF-PWR for Healthy Siblings of Individuals With Cystic Fibrosis
Druggable redox pathways against Mycobacterium abscessus in cystic fibrosis patient-derived airway organoids
International consensus recommendations for the use of prolonged-infusion beta-lactam antibiotics: Endorsed by the American College of Clinical Pharmacy, British Society for Antimicrobial Chemotherapy, Cystic Fibrosis Foundation, European Society of Clinical Microbiology and Infectious Diseases, Infectious Diseases Society of America, Society of Critical Care Medicine, and Society of Infectious Diseases Pharmacists: An executive summary
International consensus recommendations for the use of prolonged-infusion beta-lactam antibiotics: Endorsed by the American College of Clinical Pharmacy, British Society for Antimicrobial Chemotherapy, Cystic Fibrosis Foundation, European Society of Clinical Microbiology and Infectious Diseases, Infectious Diseases Society of America, Society of Critical Care Medicine, and Society of Infectious Diseases Pharmacists

Chronic daily respiratory care needs in people with cystic fibrosis treated with highly effective cystic fibrosis transmembrane conductance regulator modulators
Health inequities in the modulator era
Tezacaftor/Ivacaftor therapy has negligible effects on the cystic fibrosis gut microbiome
Comparison between Gibson-Cooke and Macroduct Methods in the Cystic Fibrosis Neonatal Screening Program and in Subjects Who Are Cystic Fibrosis Screen-Positive with an Inconclusive Diagnosis
The small non-coding RNA B11 regulates multiple facets of Mycobacterium abscessus virulence
Children with cystic fibrosis are still receiving inconclusive diagnosis despite undergoing newborn screening
Skin-interfaced Colorimetric Bifluidic Sweat Sensor Device for the Diagnosis of Cystic Fibrosis (CF)
Fibrosing Colonopathy Presenting in a Patient with Cystinosis
Effectiveness of an Oral Gastrografin Regimen for Complete Distal Intestinal Obstruction Syndrome
The association between gut microbiome and growth in infants with cystic fibrosis
The prevalence of vascular and metabolic complications after lung transplant in people with cystic fibrosis in a Dutch cohort
A systematic review on the use of bacteriophage in treating Staphylococcus aureus and Pseudomonas aeruginosa infections in cystic fibrosis
A qualitative exploration of parenthood experiences and needs among people with cystic fibrosis
Ceftazidime-assisted synthesis of ultrasmall chitosan nanoparticles for biofilm penetration and eradication of Pseudomonas aeruginosa
Cystic fibrosis year in review 2022
A new era in cystic fibrosis care: always changing and adapting
Antifungal susceptibility patterns for Aspergillus, Scedosporium and Exophiala isolates recovered from cystic fibrosis patients against amphotericin B, and three triazoles and their impact after long term therapies
Cystic Fibrosis–Related Diabetes
SARS-CoV-2 infection in children with cystic fibrosis: A cross-sectional multicenter study in Spain. New waves, new knowledge
Effects of an early rehabilitation program for adult cystic fibrosis patients during hospitalization: a randomized clinical trial
Synthesis and Evaluation of Bithiazole Derivatives As Potential α-Sarcoglycan Correctors
CFTR-rich ionocytes mediate chloride absorption across airway epithelia
TGF-β1 inhibition of ACE2 mediated by miRNA uncovers novel mechanism of SARS-CoV-2 pathogenesis
Elexacaftor/Tezacaftor/Ivacaftor Improves Bronchial Artery Dilatation Detected by Magnetic Resonance Imaging in Patients with Cystic Fibrosis
The Effects of Elexacaftor, Tezacaftor, and Ivacaftor (ETI) on Blood Glucose in Patients With Cystic Fibrosis: A Systematic Review
Preventable Hazards from in Vitro Fertilization – A Case Series of CF Patients from Bulgaria
A VirB4 ATPase of the mobile accessory genome orchestrates core genome-encoded features of physiology, metabolism, and virulence of Pseudomonas aeruginosa TBCF10839
An In Vitro Medium for Modeling Gut Dysbiosis Associated with Cystic Fibrosis
Antibiotic potentiation and inhibition of cross-resistance in pathogens associated with cystic fibrosis
A mesophilic phosphotriesterase-like lactonase SHOWS high stability and proficiency as quorum quenching enzyme
Real world outcomes of CFTR modulator therapy in Australian adults and children
Why cystic fibrosis newborn screening programs have failed to meet original expectations… thus far
The clinical impact of Lumacaftor-Ivacaftor on structural lung disease and lung function in children aged 6-11 with cystic fibrosis in a real-world setting
MRI and Pulmonary Function Tests’ Results as Ventilation Inhomogeneity Markers in Children and Adolescents with Cystic Fibrosis
Fighting Pseudomonas aeruginosa Infections: Antibacterial and Antibiofilm Activity of D-Q53 CecB, a Synthetic Analog of a Silkworm Natural Cecropin B Variant
CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity
Personalized CFTR Modulator Therapy for G85E and N1303K Homozygous Patients with Cystic Fibrosis
Epithelial-Mesenchymal Transition Mechanisms in Chronic Airway Diseases: A Common Process to Target?
Characterization of Mycobacterium abscessus colony-biofilms based on bi-dimensional images
Molecular identification and biofilm formation of aerobic and anaerobic coinfection bacterial isolated from cystic fibrosis patients in southwest Iran from 2014 to 2022
Heterogenous Biofilm Mass-Transport Model Replicates Periphery Sequestration of Antibiotics in P. aeruginosa PAO1 Microcolonies
Effects of elexacaftor-tezacaftor-ivacaftor on daily treatment burden and airflow obstruction in adults with cystic fibrosis
The impact of elexacaftor/tezacaftor/ivacaftor on fat-soluble vitamin levels in people with cystic fibrosis
Cystic fibrosis survival outcomes following second lung transplant: The north American experience
Mixed educational camps for children and adolescents with type 1 diabetes and cystic fibrosis: A new therapeutic alliance
Recurrent atelectasis in an infant : about one case of false-negative newborn screening for cystic fibrosis
Guidance and standard operating procedures for functional exercise testing in cystic fibrosis
Cystic fibrosis: Epidemiology, clinical manifestations, diagnosis and treatment
Exhaled Volatile Organic Compound Profiles Differ between Children with Primary Ciliary Dyskinesia and Cystic Fibrosis
Low-frequency oscillometry indices to assess ventilation inhomogeneity in CF patients
Development and validation of a novel personalized electronic patient-reported outcome measure to assess quality of life (Q-LIFE): a prospective observational study in people with Cystic Fibrosis
Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry
A unique presentation of hyponatremia and seizures in a 2-month-old child with cystic fibrosis: a case report
Impact of loss to follow-up on survival estimation for cystic fibrosis
Affordable CFTR Modulator Drugs for Cystic Fibrosis: All CF Lives Worldwide Matter!
Is Elexacaftor/Tezacaftor/Ivacaftor Effective in Treating Sinonasal Disease in Patients with Cystic Fibrosis?
Effect of CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor on pulmonary ventilation derived by 3D phase-resolved functional lung MRI in cystic fibrosis patients
The Development of a Metabolomic Test to Diagnose and Quantify Pancreatic Exocrine Insufficiency (The DETECTION Study)
Patient and Caregiver Perceptions of Airway Clearance Methods Used for Cystic Fibrosis
Microbial community organization designates distinct pulmonary exacerbation types and predicts treatment outcome in cystic fibrosis
Fecal Dysbiosis and Inflammation in Intestinal-Specific Cftr Knockout Mice on Regimens Preventing Intestinal Obstruction
Experimentally Evolved Staphylococcus aureus Survives in the Presence of Pseudomonas aeruginosa by Acquiring Mutations in the Amino Acid Transporter, GltT
Protospacer modification improves base editing of a canonical splice site variant and recovery of CFTR function in human airway epithelial cells
Mathematical models of cystic fibrosis as a systemic disease
Hidden CFSPID in CF patient registries? The Italian CF Registry experience
Identification of α-Tocopherol succinate as an RFFL-substrate interaction inhibitor inducing peripheral CFTR stabilization and apoptosis
Reducing unnecessary testing on sputum specimens from patients with cystic fibrosis: pathology stewardship in microbiology
Cell-free DNA Detects P. aeruginosa Lung Infection in Modulator-treated People with Cystic Fibrosis
Impact of intrinsic and extrinsic risk factors on early-onset lung disease in cystic fibrosis
Normative data for aquagenic wrinkling of palms in children 1-15 years of age: A cross-sectional study
Gene Therapy for Cystic Fibrosis: Recent Advances and Future Prospects
Cystic fibrosis and chronic pulmonary infection with Pseudomonas aeruginosa: Is Gross Domestic Product (GDP) involved?
Effects of elexacaftor / tezacaftor / ivacaftor triple combination therapy on glycaemic control and body composition in patients with cystic fibrosis-related diabetes
Long-term therapy with elexacaftor/tezacaftor/ivacaftor (ETI) in cystic fibrosis: improved clinical outcomes but infection and inflammation persist
Systemic Disease and the Liver Part 2: Pregnancy-Related Liver Injury, Sepsis/Critical Illness, Hypoxia, Psoriasis, Scleroderma/Sjogren’s Syndrome, Sarcoidosis, Common Variable Immune Deficiency, Cystic Fibrosis, Inflammatory Bowel Disease, and Hematologic Disorders
Hyperammonemia Syndrome After Lung Transplantation: A Double-Hit Fatal Syndrome. A Case Report
Advanced pulmonary drug delivery formulations for the treatment of cystic fibrosis
Neutrophil lymphocyte ratio, mean platelet volume, and immunoreactive trypsinogen as early inflammatory biomarkers for cystic fibrosis in infancy: A retrospective cohort study
Lung function decline is mitigated following liver transplantation in people with cystic fibrosis: A retrospective cohort study
The Weariness of Hoping: Synchronizing Affect While Awaiting Organ Transplantation for Cystic Fibrosis in Germany
Identification of two distinct phylogenomic lineages and model strains for the understudied cystic fibrosis lung pathogen Burkholderia multivorans
Bioequivalence and Food Effect Bioavailability Study of Lumacaftor Film-Coated Tablets
Transforming Growth Factor ß1 and Gap Junction Protein Alpha 4 Gene Heterogeneity in Relation to the Severity of Clinical Disease in Cystic Fibrosis
Phosphodiesterase inhibitors and lung diseases
Occupations and balance during the transition to motherhood with a lifetime chronic illness: A scoping review examining cystic fibrosis, asthma, and Type-1 diabetes
Identifying the need for a UK colorectal cancer screening programme for patients with cystic fibrosis (CF): 10-year retrospective review of colonoscopy and colorectal cancer outcomes at a single CF centre
Feasibility of home initiation of an airway clearance device (SIMEOX) by telecare in people with non-cystic fibrosis bronchiectasis: a pilot study
The impact of CFTR modulator triple therapy on type 2 inflammatory response in patients with cystic fibrosis
Acinar cystic transformation in the pancreatic tail
Weight increase in people with cystic fibrosis on CFTR modulator therapy is mainly due to increase in fat mass
In Vitro Susceptibility Testing of Imipenem- Relebactam and Tedizolid against 102 Mycobacterium abscessus isolates
Aiming to Improve Equity in Pulmonary Health: Cystic Fibrosis
Nasal polyposis from cystic fibrosis in children – the experience of a single center
Unplanned pregnancies following the introduction of elexacaftor/tezacaftor/ivacaftor therapy in women with cystic fibrosis
Cystic Fibrosis
Pseudomonas aeruginosa is oxygen-deprived during infection in cystic fibrosis lungs, reducing the effectiveness of antibiotics
Bacteriophage Therapy for Pan-Drug-Resistant Pseudomonas aeruginosa in Two Persons With Cystic Fibrosis
Bacteriophage-Antibiotic Combination Therapy against Pseudomonas aeruginosa
In Vitro Activity of Finafloxacin against Panels of Respiratory Pathogens
How Does the Corrected Exhalyzer Software Change the Predictive Value of LCI in Pulmonary Exacerbations in Children with Cystic Fibrosis?

Pulmonary Function Tests in the Evaluation of Early Lung Disease in Cystic Fibrosis
A Proteomic Survey of the Cystic Fibrosis Transmembrane Conductance Regulator Surfaceome
Involvement of Acquired Tobramycin Resistance in the Shift to the Viable but Non-Culturable State in Pseudomonas aeruginosa
Bacterial Biofilm Formation on Biomaterials and Approaches to Its Treatment and Prevention
Reproductive Counseling and Care in Cystic Fibrosis: A Multidisciplinary Approach for a New Therapeutic Era
Antibacterial and Antibiofilm Effects of Lactobacilli Strains against Clinical Isolates of Pseudomonas aeruginosa under Conditions Relevant to Cystic Fibrosis
Lactobacillus Probiotic Strains Differ in Their Ability to Adhere to Human Lung Epithelial Cells and to Prevent Adhesion of Clinical Isolates of Pseudomonas aeruginosa from Cystic Fibrosis Lung
L138ins Variant of the CFTR Gene in Russian Infertile Men
Telemedicine and Its Application in Cystic Fibrosis
Evolving Nutritional Needs in Cystic Fibrosis
Lung Transplantation in a New Era in the Field of Cystic Fibrosis
Improving Protection to Prevent Bacterial Infections: Preliminary Applications of Reverse Vaccinology against the Main Cystic Fibrosis Pathogens
Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges
Deferiprone-gallium-protoporphyrin (IX): A promising treatment modality against Mycobacterium abscessus
Evolution of Lung Function in Pregnant Women With Cystic Fibrosis
Traits, trends and hits of orphan drug designations in cystic fibrosis
In vivo impact of tubulin polymerization promoting protein (Tppp) knockout to the airway inflammatory response
Preservation of β-cell function in pancreatic insufficient cystic fibrosis with highly effective CFTR modulator therapy
Cystic fibrosis screen-positive neonates with one pathogenic variant still warrant sweat testing
Rapid desensitization to elexacaftor/tezacaftor/ivacaftor in a cystic fibrosis patient using fatty snack for improved absorption
Unsupervised home spirometry is not equivalent to supervised clinic spirometry in children and young people with cystic fibrosis: Results from the CLIMB-CF study
Alcohol-Induced Mucociliary Dysfunction: Role of Defective CFTR Channel Function
Comparative genomics of clinical Stenotrophomonas maltophilia isolates reveals regions of diversity which correlate with colonization and persistence in vivo
Prospective study of quantitative liver MRI in cystic fibrosis: feasibility and comparison to PUSH cohort ultrasound
Ovarian reserve in women with cystic fibrosis: is this a cause of sub-fertility?
The Alfred Step Test Exercise Protocol (A-STEP), for Adults With Cystic Fibrosis.
Temporal Changes on Pulmonary Artery Size on Computed Tomography in Adults With Cystic Fibrosis
The natural history of cystic fibrosis liver disease a prospective cohort study
Perceptions and Use of E-cigarettes among Young Adults with Cystic Fibrosis: An Observational Study
Pediatric Pulmonology 2022 year in review: Rare and diffuse lung disease
Comparative effects of CFTR modulators on phagocytic, metabolic and inflammatory profiles of CF and nonCF macrophages
Compound heterozygous variants in CFTR with potentially reducing ATP-binding ability identified in Chinese infertile brothers with isolated congenital bilateral absence of vas deferens
Allergic bronchopulmonary aspergillosis presenting as complete lung collapse with respiratory failure
The lung microbiome in cystic fibrosis: A gap of knowledge still to be filled
Cystic fibrosis to CFSPID: Burden of care vs need and rational approach to weaning therapies
Incidence of nontuberculous mycobacteria infections among persons with cystic fibrosis in the United States (2010-2019)
Cystic fibrosis related bone disease in children: Can it be predicted?
Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient Registry
Clinical, paraclinical, and genetic profile of patients with cystic fibrosis from Colombian Caribbean
Development of a Time-Dependent Oral Colon Delivery System of Anaerobic Odoribacter splanchnicus for Bacteriotherapy
Baihe Yuzi Prescription improves asthenospermia by up-regulating the expression of CFTR in the sperm
Telehealth use in Australian cystic fibrosis centers: Clinician experiences
Clinical and genetic characteristics of children with cystic fibrosis in Henan China: A single-center retrospective analysis
Sesquiterpene lactones improve secretory diarrhea symptoms by inhibiting intestinal Ca2+ -activated Cl- channel activities directly and indirectly
Prevalence, risk factors and outcomes of cardiac disease in cystic fibrosis: A multinational retrospective cohort study
Safety and effectiveness of treatment with elexacaftor, tezacaftor and ivacaftor in adults with cystic fibrosis
Factors associated with lung function response with oral antibiotic treatment of pulmonary exacerbations in cystic fibrosis
Genome-scale metabolic modeling of Aspergillus fumigatus strains reveals growth dependencies on the lung microbiome
Phenotypic and genotypic characteristics of Pseudomonas aeruginosa isolated from cystic fibrosis patients with chronic infections
Therapeutic drug monitoring-guided dosing for pediatric cystic fibrosis patients: recent advances and future outlooks
Assessment of the Glycan-Binding Profile of Pseudomonas aeruginosa PAO1
Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity
Simplifying CFTR modulation therapy in cystic fibrosis
Interventions for improving adherence to airway clearance treatment and exercise in people with cystic fibrosis
Web-Based Survey Piloting Process as a Model for Developing and Testing Past Contraceptive Use and Pregnancy History: Cystic Fibrosis Case Study
Scorpionfish BPI is highly active against multiple drug-resistant Pseudomonas aeruginosa isolates from people with cystic fibrosis
Continuing Medical Education Questions: December 2021
Ethnic differences in staphylococcus aureus acquisition in cystic fibrosis
Airway macrophages display decreased expression of receptors mediating and regulating scavenging in early cystic fibrosis lung disease
Multimodal analysis of granulocytes, monocytes, and platelets in patients with cystic fibrosis before and after Elexacaftor-Tezacaftor-Ivacaftor treatment
ALPINE2: Efficacy and safety of 14-day vs 28-day inhaled aztreonam for Pa eradication in children with cystic fibrosis
Improvement of lipid and lipoprotein profiles in children and adolescents with cystic fibrosis on CFTR modulator therapy
Sweat conductivity diagnostic accuracy for cystic fibrosis: a systematic review and meta-analysis
Emulated trial investigating effects of multiple treatments: estimating combined effects of mucoactive nebulisers in cystic fibrosis using registry data
Microbiology sampling in non-cystic fibrosis bronchiectasis cases from northern Alberta
Olive Leaf Extract (OLE) as a Novel Antioxidant That Ameliorates the Inflammatory Response in Cystic Fibrosis
Epidemiological Profile of Hospitalized Patients with Cystic Fibrosis in Brazil Due to Severe Acute Respiratory Infection during the COVID-19 Pandemic and a Systematic Review of Worldwide COVID-19 in Those with Cystic Fibrosis
It Takes Two to Tango! Protein-Protein Interactions behind cAMP-Mediated CFTR Regulation
Tissue-Specific Regulation of CFTR Gene Expression
Site-Specific RNA Editing of Stop Mutations in the CFTR mRNA of Human Bronchial Cultured Cells
Establishing a Cystic Fibrosis Learning Network: Interventions to promote collaboration and data-driven improvement at scale
Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes
Role of innate immunity and systemic inflammation in cystic fibrosis disease progression
Innovative Strategy toward Mutant CFTR Rescue in Cystic Fibrosis: Design and Synthesis of Thiadiazole Inhibitors of the E3 Ligase RNF5
Ceftolozane/tazobactam heteroresistance in cystic fibrosis-related Pseudomonas aeruginosa infections
Poor Functional Status at the Time of Waitlist for Pediatric Lung Transplant is Associated with Worse Pre-Transplant Outcomes
Computational Exploration of Potential CFTR Binding Sites for Type I Corrector Drugs
Evaluation of the Use of Appetite Stimulants in Pediatric Patients with Cystic Fibrosis
A novel in vitro model to study prolonged Pseudomonas aeruginosa infection in the cystic fibrosis bronchial epithelium
Mrs4 loss of function in fungi during adaptation to the cystic fibrosis lung
Variability of Clinical Presentation in Patients Heterozygous for the F508del Cystic Fibrosis Variant: A Series of Three Cases and a Review of the Literature
Successful treatment of distal intestinal obstruction syndrome with N-acetylcysteine and polyethylene glycol via colonoscopy
Etiology and clinical characteristics of a non-cystic fibrosis bronchiectasis cohort in a middle eastern population
Poly(sebacic acid) microparticles loaded with azithromycin as potential pulmonary drug delivery system: Physicochemical properties, antibacterial behavior, and cytocompatibility studies
Ceftolozane-Tazobactam against Pseudomonas aeruginosa Cystic Fibrosis Clinical Isolates in the Hollow-Fiber Infection Model: Challenges Imposed by Hypermutability and Heteroresistance
Preclinical testing of antimicrobials for cystic fibrosis lung infections: current needs and future priorities
Intestine-lung Axis of Cystic Fibrosis Patients Treated With the Combination Elexacaftor/Tezacaftor/Ivacaftor
Engineering in vitro models of cystic fibrosis lung disease using neutrophil extracellular trap inspired biomaterials
Nebulization of PEGylated recombinant human deoxyribonuclease I using vibrating membrane nebulizers: A technical feasibility study
Meconium ileus and pancreatic sufficiency with D1152H mutation: A case report and review of the literature
Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children
High-resolution cryo-EM structure of the Pseudomonas bacteriophage E217
Airway infections as a risk factor for Pseudomonas aeruginosa acquisition and chronic colonisation in children with cystic fibrosis
Body mass index and nutritional intake following Elexacaftor/Tezacaftor/Ivacaftor modulator therapy in adults with cystic fibrosis
More is better? Hypertonic saline dose and response for cystic fibrosis: efficacy, tolerability and implications for clinical practice
A novel high-resolution melting analysis strategy for detecting cystic fibrosis-causing variants
Aquagenic Wrinkling of the Palms in a cohort of Greek children diagnosed with Cystic Fibrosis: Associated Clinical Parameters
Longitudinal Effects of Elexacaftor/Tezacaftor/Ivacaftor on Sputum Viscoelastic Properties, Airway Infection and Inflammation in Patients with Cystic Fibrosis
How workplaces produce or reduce disability along the career paths of young people with cystic fibrosis
Diabetes of the Exocrine Pancreas: Implications for Pharmacological Management

Evaluating Bronchodilator Response in Patients With Bronchiectasis
Development and validation of a short form psychometric tool assessing the caregiving Challenge of Living with Cystic Fibrosis (CLCF-SF) in a child
Liquid chromatographic determination of lumacaftor in the presence of ivacaftor and identification of five novel degradation products using high-performance liquid chromatography ion trap time-of-flight mass spectrometry
Proliferative activity of antigen-specific CD154+ T cells against bacterial and fungal respiratory pathogens in cystic fibrosis decreases after initiation of highly effective CFTR modulator therapy
Late Diagnosis in the Era of Universal Newborn Screening Negatively Effects Short- and Long-Term Growth and Health Outcomes in Infants with Cystic Fibrosis
Oral non-viral gene delivery platforms for therapeutic applications
Impact of lumacaftor/ivacaftor and tezacaftor/ivacaftor on treatment response in pulmonary exacerbations of F508del/F508del cystic fibrosis
Development of the intestinal microbiome in cystic fibrosis in early life
Three-dimensional Ultrashort Echo Time MRI: A More Sensitive Tool for Monitoring Patients with Cystic Fibrosis
Generating High-Resolution Synthetic CT from Lung MRI with Ultrashort Echo Times: Initial Evaluation in Cystic Fibrosis
Lung Function in Patients with Cystic Fibrosis before and during CFTR -Modulator Therapy Using 3D Ultrashort Echo Time MRI
Class-Driven Synergy and Antagonism between a Pseudomonas Phage and Antibiotics
Results of Lung Transplantation for Cystic Fibrosis With Selected Donors Over 65 Years Old
Impact of Model-Informed Precision Dosing on Achievement of Vancomycin Exposure Targets in Pediatric Patients with Cystic Fibrosis
Hyperbilirubinemia and Gilbert’s syndrome in Cystic Fibrosis patients treated with elexacaftor/tezacaftor/ivacaftor
Replacing Plain Radiograph with ultra-low dose CT thorax in cystic fibrosis (CF) in the era of CFTR modulation and its impact on cumulative effective dose
Experience With Elexacaftor/Tezacaftor/Ivacaftor in Patients With Cystic Fibrosis and Advanced Disease
Author Correction: Predicting glycemic control status and high blood glucose levels through voice characteristic analysis in patients with cystic fibrosis-related diabetes (CFRD)
Outer membrane translocation of pyocins via the copper regulated TonB-dependent transporter CrtA
Stenotrophomonas maltophilia natural history and evolution in the airways of adults with cystic fibrosis
Impact of mucus and biofilm on antimicrobial photodynamic therapy: Evaluation using Ruthenium(II) complexes
Bayesian estimation of Pseudomonas aeruginosa viscoelastic properties based on creep responses of wild type, rugose, and mucoid variant biofilms
Unraveling Genomic Diversity in Pseudomonas aeruginosa Cystic Fibrosis Lung Infection and Its Impact on Antimicrobial Resistance
Spectrum of PRSS1 , SPINK1 , CTRC , CFTR , and CPA1 Gene Variants in Chronic Pancreatitis Patients in Russia
Role of hyperglycemia in cystic fibrosis pulmonary exacerbations
Undernutrition is still highly frequent in hospitalized children with cystic fibrosis
Longitudinal mental health trends in cystic fibrosis
Changes in bone turnover after high-dose vitamin D supplementation during acute pulmonary exacerbation in cystic fibrosis
Cystic Fibrosis-Associated Gastrointestinal Disease in Neonates
Retracted: Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China
Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
Pre-Lung transplant reflux testing demonstrates high prevalence of gastroesophageal reflux in cystic fibrosis and reduces chronic rejection risk
Early Detection and Staging of Lung Fibrosis Enabled by Collagen-Targeted MRI Protein Contrast Agent
Implementation and Evaluation of Ultra Low-Dose CT in Early Cystic Fibrosis Lung Disease
Diagnostic value of nasal nitric oxide for children with primary ciliary dyskinesia
SEXUAL AND REPRODUCTIVE HEALTH AMONG MEN WITH CYSTIC FIBROSIS
Eradication of Burkholderia cepacia complex in cystic fibrosis patients with inhalation of amiloride and tobramycin combined with oral cotrimoxazole
The revolution of personalized pharmacotherapies for cystic fibrosis: what does the future hold?
Staphylococcus aureus Small-Colony Variants from Airways of Adult Cystic Fibrosis Patients as Precursors of Adaptive Antibiotic-Resistant Mutations
Activity of Delafloxacin and Comparator Fluoroquinolones against Multidrug-Resistant Pseudomonas aeruginosa in an In Vitro Cystic Fibrosis Sputum Model
Genome Editing for Cystic Fibrosis
YES1 Kinase Mediates the Membrane Removal of Rescued F508del-CFTR in Airway Cells by Promoting MAPK Pathway Activation via SHC1
Dysfunctions of Neutrophils in the Peripheral Blood of Children with Cystic Fibrosis
Continuous Glucose Monitoring as an Additional Tool in Early Cystic Fibrosis-Related Diabetes Monitoring and in Evaluation of Short-Term Sitagliptin Response
A Cell-Based Optimised Approach for Rapid and Efficient Gene Editing of Human Pluripotent Stem Cells
Theratyping of the Rare CFTR Genotype A559T in Rectal Organoids and Nasal Cells Reveals a Relevant Response to Elexacaftor (VX-445) and Tezacaftor (VX-661) Combination
Toward a Systematic Assessment of Sex Differences in Cystic Fibrosis
Future Comorbidities in an Aging Cystic Fibrosis Population
Identification by Reverse Vaccinology of Three Virulence Factors in Burkholderia cenocepacia That May Represent Ideal Vaccine Antigens
CT imaging shows specific pancreatic abnormalities in persons with cystic fibrosis related diabetes
Body composition and body mass index measures from 8 to 18 years old in children with cystic fibrosis
Cystic Fibrosis screen positive, inconclusive diagnosis (CFSPID) to Cystic Fibrosis: Detecting disease with serial assessments
Pseudo-Bartter syndrome in an infant without obvious underlying conditions: A case report
Laparoscopic modified fundus-down cholecystectomy technique: an alternative method for performing a safe laparoscopic cholecystectomy: how to article
Genomic and Functional Characterization of Longitudinal Pseudomonas aeruginosa Isolates from Young Patients with Cystic Fibrosis
Dietary intake, weight status, pulmonary function, and metabolic profile in children with cystic fibrosis with or without pancreatic sufficiency
Substrate-dependent metabolomic signatures of myeloperoxidase activity in airway epithelial cells: Implications for early cystic fibrosis lung disease
Weight a minute: Exploring the effect on weight and body composition after the initiation of elexacaftor/tezacaftor/ivacaftor in adults with CF
Hypoglycemia in children and young adults with cystic fibrosis during oral glucose tolerance testing vs. continuous glucose monitoring
A transistor model for the cystic fibrosis transmembrane conductance regulator
Immunohistochemical detection of MUC5AC and MUC5B mucins in ferrets
The relation between inspiratory muscle strength and bacterial colonization and other clinical factors in patients with non-cystic fibrosis bronchiectasis
Thinking outside the box: A review of gastrointestinal symptoms and complications in cystic fibrosis
Saline at lower tonicity in CF (SALTI-CF) trial comparing 0.9% v 3% v 6% nebulised saline
Bacteriophage Therapy of Multidrug-Resistant Achromobacter in an 11-year-old Boy With Cystic Fibrosis Assessed by Metagenome Analysis
Changes in glucose tolerance in people with cystic fibrosis after initiation of first-generation CFTR modulator treatment
A wearable colorimetric sweat pH sensor-based smart textile for health state diagnosis
Rhinorrhea and increased chloride secretion through the CFTR chloride channel-a systematic review
Antimicrobial susceptibility of ceftolozane-tazobactam against multidrug-resistant Pseudomonas aeruginosa isolates from Melbourne, Australia
Genome Editing in Ferret Airway Epithelia Mediated by CRISPR/nucleases Delivered with Amphiphilic Shuttle Peptides
Histone acetyltransferases derived RW20 protects and promotes rapid clearance of Pseudomonas aeruginosa in zebrafish larvae
Elexacaftor-tezacaftor-ivacaftor in patients with cystic fibrosis ineligible for clinical trials: a 24-week observational study
The role of individual exopolysaccharides in antibiotic tolerance of Pseudomonas aeruginosa aggregates
Clinical and functional efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis carrying the N1303K mutation
Heterogeneity of weight gain after initiation of Elexacaftor/Tezacaftor/Ivacaftor in people with cystic fibrosis
Antibiotic Regimen Changes During Cystic Fibrosis Pediatric Pulmonary Exacerbation Treatment
Cystic fibrosis modulator therapy can reverse cystic bronchiectasis
Fluoroquinolone Use Among Hospitalized Children: Diagnosis-Based Stratification to Identify Stewardship Targets
Therapeutic interventions alter ecological interactions among cystic fibrosis airway microbiota
Effects of lumacaftor-ivacaftor therapy on cystic fibrosis transmembrane conductance regulator function in F508del homozygous patients with cystic fibrosis aged 2-11 years
Adjunctive nano-curcumin therapy improves inflammatory and clinical indices in children with cystic fibrosis: A randomized clinical trial
Q1291H-CFTR molecular dynamics simulations and ex vivo theratyping in nasal epithelial models and clinical response to elexacaftor/tezacaftor/ivacaftor in a Q1291H/F508del patient
Glycomimetic antagonists of BC2L-C lectin: insights from molecular dynamics simulations
Reducing treatment burden in the era of CFTR modulators – Authors’ reply
Structural changes in lung morphology detected by MRI after modulating therapy with elexacaftor/tezacaftor/ivacaftor in adolescent and adult patients with cystic fibrosis
Reanalysis of N2 -lung clearance index and the comparison to SF6 -lung clearance index and magnetic resonance imaging
Influenza A induced cystic fibrosis transmembrane conductance regulator dysfunction increases susceptibility to Streptococcus pneumoniae
Nebulised hypertonic saline for cystic fibrosis
Cystic Fibrosis: “Ionocyte Modulators”?
Ceftolozane/tazobactam plus tobramycin against free-floating and biofilm bacteria of hypermutable Pseudomonas aeruginosa epidemic strains: resistance mechanisms and synergistic activity: Running title: Ceftolozane/tazobactam plus tobramycin against Pseudomonas biofilm
Protocol for generating airway organoids from 2D air liquid interface-differentiated nasal epithelia for use in a functional CFTR assay
A DFT study of the gallium ion-binding capacity of mature Pseudomonas aeruginosa biofilm extracellular polysaccharide
Cross-talk between CFTR and sphingolipids in cystic fibrosis
Seroprevalence and clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis
Massive and Lengthy Clonal Nosocomial Expansion of Mycobacterium abscessus subsp. massiliense among Patients Who Are Ventilator Dependent without Cystic Fibrosis
The RISE study protocol: resilience impacted by positive stressful events for people with cystic fibrosis
A comprehensive review of cystic fibrosis in Africa and Asia
Lung transplantation for cystic fibrosis complicated by cirrhosis: A case report
Detection of bile acids in bronchoalveolar lavage fluid defines the inflammatory and microbial landscape of the lower airways in infants with cystic fibrosis
In Vitro Susceptibility of Achromobacter Species Isolated from Cystic Fibrosis Patients: a 6-Year Survey

Updated recommendations for CFTR carrier screening: A position statement of the American College of Medical Genetics and Genomics (ACMG)
Combating polymicrobial biofilm: recent approaches
Treatment of Fetal Cystic Fibrosis With Cystic Fibrosis Transmembrane Conductance Regulator Modulation Therapy
Physical activity level and sedentary behavior in patients with bronchiectasis: A systematic review of outcome measures and determinants
Repurposing High-Throughput Screening Identifies Unconventional Drugs with Antibacterial and Antibiofilm Activities against Pseudomonas aeruginosa under Experimental Conditions Relevant to Cystic Fibrosis
Allergic Bronchopulmonary Aspergillosis: A Case Report
Commensal colonization reduces Pseudomonas aeruginosa burden and subsequent airway damage
Olfaction, BMI, and quality of life with cystic fibrosis combination therapy
Changes of Erythrocyte Fatty Acids after Supplementation with Highly Concentrated Docosahexaenoic Acid (DHA) in Pediatric Cystic Fibrosis: A Randomized Double-Blind Controlled Trial
Investigating the Inhibition of FTSJ1, a Tryptophan tRNA-Specific 2′-O-Methyltransferase by NV TRIDs, as a Mechanism of Readthrough in Nonsense Mutated CFTR
Antimicrobial resistance of Pseudomonas aeruginosa in a cystic fibrosis population after introduction of a novel cephalosporin/β-lactamase inhibitor combination
In host evolution of Exophiala dermatitidis in cystic fibrosis lung micro-environment
Alcohol and opiate misuse in adults with cystic fibrosis
Prevalence of tracheobronchomalacia is higher than previously reported in children with cystic fibrosis
Digital technology for delivering and monitoring exercise programs for people with cystic fibrosis
A case of localized paranasal sinusitis associated with Burkholderia cenocepacia ST 1880 in a cystic fibrosis patient
Transcriptional Profiling and Genetic Analysis of a Cystic Fibrosis Airway-Relevant Model Shows Asymmetric Responses to Growth in a Polymicrobial Community
Divergent host innate immune response to the smooth-to-rough M. abscessus adaptation to chronic infection
Characterisation of key genotypic and phenotypic traits of clinical cystic fibrosis Staphylococcus aureus isolates
Regionally distinct progenitor cells in the lower airway give rise to neuroendocrine and multiciliated cells in the developing human lung
Identification and characterization of aptameric inhibitors of human neutrophil elastase
A refresh of the top 10 research priorities in cystic fibrosis
Potential implicit bias in attribution of adverse events in randomized controlled trials in cystic fibrosis
Microbiota and fungal-bacterial interactions in the Cystic Fibrosis lung
Chronic airway epithelial hypoxia exacerbates injury in muco-obstructive lung disease through mucus hyperconcentration
Exoproducts of the Most Common Achromobacter Species in Cystic Fibrosis Evoke Similar Inflammatory Responses In Vitro
Editorial for “Non-Contrast-Enhanced Functional Lung MRI to Evaluate Treatment Response of Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis: A Pilot Study”
Baseline reticular basement membrane morphology is related to subsequent spirometry deterioration in pediatric chronic airway inflammation: A follow-up study
Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?
Cystic fibrosis fitness during inpatient treatment (CF-FIT): A pre-post pilot study
Cystic Fibrosis: A Review
Genetic attributes of Iranian cystic fibrosis patients: the diagnostic efficiency of CFTR mutations in over a decade
Additional evidence on the phenotype produced by combination of CFTR 1677delTA alleles and their relevance in causing CFTR-related disease
Real-world outcomes and direct care cost before and after elexacaftor/tezacaftor/ivacaftor initiation in commercially insured members with cystic fibrosis
A provider survey of cystic fibrosis related diabetes screening and management practices at North American CF centers
Improved engineering of Pseudomonas aeruginosa to study the adaptation of pyoverdine production under intra- or inter- specific bacterial competition
Investigating Pseudomonas aeruginosa population structure and frequency of cross-infection in UK cystic fibrosis clinics – a reference laboratory perspective
Editorial: Optimism grows after 10 years of modulator therapies in Cystic Fibrosis
A glycosylated hemoglobin A1c above 6% (42 mmol/mol) is associated with a high risk of developing Cystic Fibrosis-Related Diabetes and a lower probability of weight gain in both adults and children with Cystic Fibrosis
Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis
Control of mitochondrial functions by Pseudomonas aeruginosa in cystic fibrosis
Psychosocial needs and interventions for young children with cystic fibrosis and their families
Cellular heterogeneity in the 16HBE14o- airway epithelial line impacts biological readouts
Positive and negative impacts of elexacaftor/tezacaftor/ivacaftor: Healthcare providers’ observations across US centers
Non-Contrast-Enhanced Functional Lung MRI to Evaluate Treatment Response of Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis: A Pilot Study
Pulmonary bacteriophage and cystic fibrosis airway mucus: friends or foes?
Biomarkers of nitrosative stress in exhaled breath condensate and serum among patients with cystic fibrosis
Measuring energy requirements during pulmonary exacerbations of cystic fibrosis using indirect calorimetry
Outcome of Patients with cystic fibrosis colonized by Scedosporium and Lomentospora species: a longitudinal cohort study
Humoral and cell-mediated immune responses to BNT162b2 vaccine against SARS-CoV-2 in people with cystic fibrosis
Clinician perspectives on barriers and solutions to symptom management in cystic fibrosis
The efficacy of biosynthesized silver nanoparticles against Pseudomonas aeruginosa isolates from cystic fibrosis patients
Factors associated with frequent high-cost individuals with cystic fibrosis and their healthcare utilization and cost patterns
How personality influences health outcomes and quality of life in adult patients with cystic fibrosis
Computed cardiopulmonography and the idealised lung clearance index, iLCI2.5 , in early stage cystic fibrosis
Intralesional microbleeding in resected thymic cysts indeterminate on imaging
Human Mesenchymal Stem Cell (hMSC) Donor Potency Selection for the “First in Cystic Fibrosis” Phase I Clinical Trial (CEASE-CF)
Drug-drug interactions involving CFTR modulators: a review of the evidence and clinical implications
May Podcast: Cystic fibrosis
Time-to-first-isolation of methicillin-resistant Staphylococcus aureus (MRSA) in cystic fibrosis (CF): An underutilised metric in infection control?
The Impact of Cystic Fibrosis- and Noncystic Fibrosis-Bronchiectasis on Pulmonary Artery Wall Thickness and Right Heart Functions Assessed by Speckle-Tracking Echocardiography
Identification of Virulence Factors Involved in a Murine Model of Severe Achromobacter xylosoxidans Infection
Burkholderia cepacia infection in children without cystic fibrosis: a clinical analysis of 50 cases
An rRNA fragment in extracellular vesicles secreted by human airway epithelial cells increases the fluoroquinolone sensitivity of P. aeruginosa
Evaluation of Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) in Cystic Fibrosis (CF) Participants 12 to Less Than 24 Months of Age
General trends in the effects of VX-661 and VX-445 on the plasma membrane expression of clinical CFTR variants
Nocturnal High-Flow Nasal Cannula Therapy and Sinonasal Symptoms During Cystic Fibrosis Exacerbations
Biofilm-forming strains of P. aeruginosa and S. aureus isolated from cystic fibrosis patients differently affect inflammatory phenotype of macrophages
Impact of extended sinus surgery on allograft infection, allograft function and overall survival in cystic fibrosis lung transplant recipients
Crosslink between SARS-CoV-2 replication and cystic fibrosis hallmarks
Effectiveness of the use of an oscillating positive expiratory pressure device in bronchiectasis with frequent exacerbations: a single-arm pilot study
Current Practice, Barriers to, and Facilitators of Exercise Testing and Training by Physiotherapists in Cystic Fibrosis Specialized Centres in Canada
Exploring the barriers and enablers experienced by people with Cystic Fibrosis and their healthcare professionals in accessing, utilising and delivering maternity and Cystic Fibrosis care during the pre-conception to post-partum period: A mixed methods systematic review protocol
Chronic Pancreatitis Associated With a Variant of CFTR Gene Treated With Total Pancreatectomy and Autologous Islet Cell Transplantation
The role of shear viscosity as a biomarker for improving chronic kidney disease detection using shear wave elastography: A computational study using a validated finite element model
Automatic bronchus and artery analysis on chest computed tomography to evaluate the effect of inhaled hypertonic saline in children aged 3-6 years with cystic fibrosis in a randomized clinical trial
Repurposing small molecules for Nephronophthisis and related renal ciliopathies
Effect of Hypertonic Saline on Lung Function as Add-On Treatment in People with Cystic Fibrosis Receiving Dornase-alfa: A Cystic Fibrosis Foundation Patient Registry Analysis
Adolescent Patient with Cystic Fibrosis Presents with Suspected Ludwig Angina
Sex differences in outcomes of people with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor
Quantifying variation in home spirometry in people with cystic fibrosis during baseline health, and associations with clinical outcomes
Predicting glycemic control status and high blood glucose levels through voice characteristic analysis in patients with cystic fibrosis-related diabetes (CFRD)
Renal Ciliopathies: Promising drug targets and prospects for clinical trials
Mechanisms of organ fibrosis: Emerging concepts and implications for novel treatment strategies
A genomic data archive from the Network for Pancreatic Organ donors with Diabetes
A Pilot Study of Lung Clearance Index as a Useful Outcome Marker in the Follow-Up of Pediatric Patients with Non-Cystic Fibrosis Bronchiectasis?
How Three Self-Secreted Biofilm Exopolysaccharides of Pseudomonas aeruginosa , Psl, Pel, and Alginate, Can Each Be Exploited for Antibiotic Adjuvant Effects in Cystic Fibrosis Lung Infection
Efficacy and Safety of Dry Powder Antibiotics: A Narrative Review
Endotypes of Nasal Polyps in Children: A Multidisciplinary Approach
Dry Powder Inhalation for Lung Delivery in Cystic Fibrosis
Eugenol Essential Oil and Nanoemulsion as Antihydatic Agents with Antifibrotic and Immunomodulatory Effects in Cystic Echinococcosis
CAGE sequencing reveals CFTR-dependent dysregulation of type I IFN signaling in activated cystic fibrosis macrophages
Parent and adolescent perceptions of cystic fibrosis management responsibility: A mixed-methods study
Airway clearance after highly effective CFTR modulators: Normalizing life and reducing treatment burden
Scedosporium/Lomentospora Species Induce the Production of Siderophores by Pseudomonas aeruginosa in a Cystic Fibrosis Mimic Environment
Registry-based study in people with cystic fibrosis and an R117H variant treated with ivacaftor
Nutritional considerations for a new era: A CF foundation position paper
Telehealth in Cystic Fibrosis. A systematic review incorporating a novel scoring system and expert weighting to identify a ‘top 10 manuscripts’ to inform future best practices implementation
Pyochelin biotransformation by Staphylococcusaureus shapes bacterial competition with Pseudomonas aeruginosa in polymicrobial infections
ERS International Congress 2022: highlights from the Paediatrics Assembly

Dietary intake assessment in children with cystic fibrosis using 3-day food diaries: a single-centre study
Real-life impact of highly effective CFTR modulator therapy in children with cystic fibrosis
Patient Attitudes Regarding Health Care Clinician Communication in Adolescents with Cystic Fibrosis
Radiological and long-term clinical response to elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease
The upper airway microbiome in Hispanic children with cystic fibrosis
Impact of Elexacaftor-Tezacaftor-Ivacaftor on lung disease in cystic fibrosis
Evolution of Mycobacterium abscessus in the human lung: Cumulative mutations and genomic rearrangement of porin genes in patient isolates
METABOLISM AND PHARMACOKINETICS OF VITAMIN D in PATIENTS with Cystic Fibrosis
Association of Pseudomonas aeruginosa infection stage with lung function trajectory in children with cystic fibrosis
Clinical and genetic features of cystic fibrosis in Japan
Virtual monitoring in CF – the importance of continuous monitoring in a multi-organ chronic condition
The Impact of CFTR Modulator Triple Therapy on Type 2 Inflammatory Response in Patients with Cystic Fibrosis
Evaluation of the quality of life and associated factors of a group of children and adolescents with cystic fibrosis in the northern region of Portugal: a cross-sectional pilot study
Comparative in vitro activities of eravacycline in combination with colistin, meropenem, or ceftazidime against various Achromobacter spp. strains isolated from patients with cystic fibrosis
Diabetes and prediabetes in children with cystic fibrosis
Prevalence of antibiotic resistance of Staphylococcus aureus in cystic fibrosis infection: A systematic review and meta-analysis
Short chain fatty acids reduce the respiratory burst of human neutrophils in response to cystic fibrosis isolates of Staphylococcus aureus
Chemical Modification of a Bacterial Siderophore by a Competitor in Dual-Species Biofilms
Characterization of sleep in emerging adults with cystic fibrosis on elexacaftor/tezacaftor/ivacaftor
A real-life study of elexacaftor-tezacaftor-ivacaftor therapy in people with cystic fibrosis in Brazil
CFTR and dystrophin encoding plasmids carrying both luciferase reporter gene, nuclear import specific sequences and triple helix sites
Effect of elexacaftor/tezacaftor/ivacaftor on airway and systemic inflammation in cystic fibrosis
The prevalence of children in the UK Cystic Fibrosis Registry on long term anti-Pseudomonas aeruginosa (PA) inhaled antibiotics who become culture negative for PA and a survey of practice for discontinuing treatment
Center-level self-study identifies opportunities to advance equity in cystic fibrosis clinical trial participation
Persistent delay in maturation of the developing gut microbiota in infants with cystic fibrosis
Parallel Evolution of Linezolid Resistant Staphylococcus aureus in Patients with Cystic Fibrosis
Critical role of down-regulated in adenoma bicarbonate transporter in linaclotide stimulated intestinal bicarbonate secretion
Corrigendum: Elexacaftor-Tezacaftor-Ivacaftor treatment reduces abdominal symptoms in cystic fibrosis-early results obtained with the CF-specific CFAbd-Score
Acute Pancreatitis in a Previously Exocrine Pancreatic Insufficient Cystic Fibrosis Patient Who Had Improved Pancreatic Function After Being Treated With Lumacaftor/Ivacaftor
Label-free intratissue activity imaging of alveolar organoids with dynamic optical coherence tomography
Small Bowel Hemolymphangioma Treated by Polypectomy in a Pediatric Patient with Cystic Fibrosis
Impact of omega-3 supplementation on children and adolescents patients with cystic fibrosis: A systematic review and meta-analysis of randomized-controlled trials
An Electrocardiogram Study to Evaluate the Effect of Vanzacaftor on the QT/QTc Interval in Healthy Participants
A Study to Test How Well BI 1291583 is Tolerated by People With Cystic Fibrosis Bronchiectasis (Clairafly™)
N-of-1 Analytics Makerspace: A Prototype for Cystic Fibrosis Self-Care and Co-Care
Proinflammatory indicators and the relevance of echocardiography in children with cystic fibrosis
Profile of the intestinal microbiota of patients with cystic fibrosis: A systematic review
Cystic fibrosis in a transformative era: Adapting to changing mental health needs
The cell envelope of Mycobacterium abscessus and its role in pathogenesis
16S rRNA-Based Microbiota Profiling Assists Conventional Culture Analysis of Airway Samples from Pediatric Cystic Fibrosis Patients
Dipeptidyl peptidase-1 inhibition with brensocatib reduces the activity of all major neutrophil serine proteases in patients with bronchiectasis: results from the WILLOW trial
In Vitro Effect of Combined Hypertonic Saline and Salbutamol on Ciliary Beating Frequency and Mucociliary Transport in Human Nasal Epithelial Cells of Healthy Volunteers and Patients with Cystic Fibrosis
Brazilian guidelines for the pharmacological treatment of the pulmonary symptoms of cystic fibrosis. Official document of the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association)
Menstrual Dysfunction in Adolescents with Chronic Illness: A Systematic Review
Therapeutic potential of phytochemicals for cystic fibrosis
Inflammation as a Regulator of the Airway Surface Liquid pH in Cystic Fibrosis
Correlation of Electrophysiological and Fluorescence-Based Measurements of Modulator Efficacy in Nasal Epithelial Cultures Derived from People with Cystic Fibrosis
The Expression of Affective Temperaments in Cystic Fibrosis Patients: Psychopathological Associations and Possible Neurobiological Mechanisms
Assessing accuracy of testing and diagnosis in cystic fibrosis
Neutrophil Elastase decreases SARS-CoV-2 Spike protein binding to human bronchial epithelia by clipping ACE-2 ectodomian from the epithelial surface
An Overview of Recently Published Patents on Pulmonary Drug Delivery Devices
Possibilities of using recombinant human deoxyribonuclease I in otorhinolaryngology
Individualized approach to elexacaftor/tezacaftor/ivacaftor dosing in cystic fibrosis, in response to self-reported anxiety and neurocognitive adverse events: A case series
Biliary Atresia and Rare Concurrent Cystic Fibrosis Variant: Case Report and Management Considerations
Transient Receptor Potential Vanilloid (TRPV4) channel inhibition: A novel promising approach for the treatment of lung diseases
Allergic bronchopulmonary aspergillosis: A multidisciplinary review
Eradication of Nontuberculous Mycobacteria in People with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor: A Multicenter Cohort Study
Impact of COVID-19 pandemic on health care system, work, and mental well-being of people with cystic fibrosis
Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes
The Epithelial Sodium Channel-An Underestimated Drug Target
Efficacy of Pirfenidone and Nintedanib in Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis: A Systematic Review
The CFTR Gene Germline Heterozygous Pathogenic Variants in Russian Patients with Malignant Neoplasms and Healthy Carriers: 11,800 WGS Results
Lung Ultrasound in the Evaluation of Lung Disease Severity in Children with Clinically Stable Cystic Fibrosis: A Prospective Cross-Sectional Study
The centripetal endoscopic sinus surgery in patients with cystic fibrosis: A preliminary study
Features of CFTR mRNA and implications for therapeutics development
No Stone Left Unturned: Pediatric Pancreatic Stones Presenting With Obstructive Jaundice
CFTR:F508d/A613T Mutation Is Associated With Recurrent Episodes of Pancreatitis
Use of cystic fibrosis inhaled medication before and after elexacaftor/tezacaftor/ivacaftor initiation
Sputum handling for rheology
Cystic Fibrosis: From Tragedy to Triumph
Muscle oxygen utilization and ventilatory parameters during exercise in people with cystic fibrosis: Role of HbA1c
Changes in liver biochemistry and tacrolimus levels following the introduction of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and liver transplant
Repeat or single-dose lentiviral vector administration to mouse lungs? It’s all about the timing
Novel tricyclic pyrrolo-quinolines as pharmacological correctors of the mutant CFTR chloride channel
High prevalence of lipopolysaccharide mutants and R2-Pyocin susceptible variants in Pseudomonas aeruginosa populations sourced from cystic fibrosis lung infections
Single-arm, open-labelled, safety and tolerability of intrabronchial and nebulised bacteriophage treatment in children with cystic fibrosis and Pseudomonas aeruginosa
A Systematic Review of Cystic Fibrosis in Children: Can Non-Medical Therapy Options Lead to a Better Mental Health Outcome?
Raised Intracranial Pressure in Three Children with Cystic Fibrosis Receiving Elexacaftor-Tezacaftor-Ivacaftor Modulator Therapy
Integrating Primary Care into the Management of Cystic Fibrosis
Telemedicine and remote monitoring in cystic fibrosis
Virtual Reality Based Tele-Exercises on Exercise Capacity in Cystic Fibrosis
“It is like a pet in a way”: The self-management experiences of people with cystic fibrosis diabetes
Effective pharmacy provider handoff communication for transition of patient care in CF
Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged ≥6 Years with Cystic Fibrosis and At Least One F508del Allele: A Phase 3, Open-Label Clinical Trial
Genomic features, antimicrobial susceptibility, and epidemiological insights into Burkholderia cenocepacia clonal complex 31 isolates from bloodstream infections in India
Effects of elexacaftor/tezacaftor/ivacaftor therapy on mental health of patients with cystic fibrosis
Elexacaftor – Tezacaftor – Ivacaftor treatment improves systemic infection parameters and Pseudomonas aeruginosa colonization rate in patients with cystic fibrosis a monocentric observational study
Can the data of microbiome be used to predict the presence of Burkholderia spp in pulmonary microbiota of cystic fibrosis patients?
Manifestation and staging of arthropathy in cystic fibrosis. Defining different stages of cystic fibrosis arthropathy using ultrasound imaging and clinical scoring
Corrigendum to “Detecting clusters of high nontuberculous mycobacteria infection risk for persons with cystic fibrosis – An analysis of U.S. counties” [Tuberculosis 138 (2023) 102296]
Feasibility of dynamic chest radiography to calculate lung volumes in adult people with cystic fibrosis: a pilot study
FDA-approved drug screening in patient-derived organoids demonstrates potential of drug repurposing for rare cystic fibrosis genotypes
Evaluation of Long-Term Safety and Efficacy of Vanzacaftor/Tezacaftor/Deutivacaftor in Cystic Fibrosis Participants 1 Year of Age and Older
Evaluation of serum VIP and aCGRP during pulmonary exacerbation in cystic fibrosis: A longitudinal pilot study of patients undergoing antibiotic therapy
Determination of ivacaftor by liquid chromatography techniques in pharmaceutical formulation with interlaboratory comparison and characterization of five novel degradation products by high-performance liquid chromatography ion trap time-of-flight mass spectrometry
A cystic fibrosis patient
Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis
Validation of the 25 level modified shuttle test in children with cystic fibrosis
Pilot of a therapist-guided digital mental health intervention (eHealth CF-CBT) for adults with cystic fibrosis
Benefit-risk assessment of brensocatib for treatment of non-cystic fibrosis bronchiectasis

The Bronchiectasis Exacerbation Diary: a novel patient-reported outcome for non-cystic fibrosis bronchiectasis
Discovery of dysregulated circular RNAs in whole blood transcriptomes from cystic fibrosis patients – implication of a role for cellular senescence in cystic fibrosis
Impact of elexacaftor/tezacaftor/ivacaftor on fat-soluble vitamin levels in children with cystic fibrosis
Growth trajectories in young children with cystic fibrosis: Where are we going?
Lung function and secondhand smoke exposure among children with cystic fibrosis: A Bayesian meta-analysis
Sonic Hedgehog Signaling is Essential for Pulmonary Ionocyte Specification in Human and Ferret Airway Epithelia
The multiple ubiquitination mechanisms in CFTR peripheral quality control
Novel therapeutic approach for the treatment of cystic fibrosis based on freeze-dried tridrug microparticles to treat cystic fibrosis
Use of Omadacycline as a Component of Mycobacterium Abscessus Eradication in an Adolescent With Cystic Fibrosis
Furin as a therapeutic target in cystic fibrosis airways disease
Erratum to “Vitamin D for the Immune System in Cystic Fibrosis (DISC): a double-blind, multicenter, randomized, placebo-controlled clinical trial”
Upper airway microbiota development in infants with cystic fibrosis diagnosed by newborn screen
Partners in research: The success with therapies research consortium and the CF community unite to improve self-management
Is it time to end race and ethnicity adjustment for pediatric pulmonary function tests?
Meconium thorax observed in a Chinese neonate with cystic fibrosis
Disparities and therapeutic advances in cystic fibrosis
Lung clearance index in children with cystic fibrosis previously diagnosed with CRMS/CFSPID: A monocentric prospective experience
Extracellular vesicles of Pseudomonas aeruginosa downregulate pyruvate fermentation enzymes and inhibit the initial growth of Staphylococcus aureus
Assessment of the Glycan-Binding Profile of Pseudomonas aeruginosa PAO1
COVID-19 in a Cystic Fibrosis Patient
ROCK STUDY in CF: sustained anti-inflammatory effects of lumacaftor-ivacaftor in sputum and peripheral blood samples of adult patients with cystic fibrosis-an observational study
Transition Care in Cystic Fibrosis
Usability preferences of people living with cystic fibrosis about a lung transplant education website
Cystic Fibrosis-Asthma Overlap Syndrome. Combination of Cystic Fibrosis Transmembrane Conductance Regulator Modulators and Type 2 Targeted Biologic Treatment for Asthma
Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes
Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes
Inhibition and eradication of Pseudomonas aeruginosa biofilms by secondary metabolites of Nocardiopsis lucentensis EMB25
Deciphering hERG Mutation in Long QT Syndrome Type 2 Using Antisense Oligonucleotide-Mediated Techniques: Lessons from Cystic Fibrosis
Inhaled mRNA therapy for treatment of cystic fibrosis: Interim results of a randomized, double-blind, placebo-controlled phase 1/2 clinical study
Microevolution of Pseudomonas aeruginosa in the airways of people with cystic fibrosis
Survey of exercise testing and training in cystic fibrosis clinics in the UK: a decade of progress
Variability of the sweat test in children with Cystic Fibrosis previously CRMS/CFSPID: A retrospective monocenter experience
Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data
Antibiotic Therapy for Pulmonary Exacerbations in Cystic Fibrosis-A Single-Centre Prospective Observational Study
A Novel Zinc (II) Porphyrin Is Synergistic with PEV2 Bacteriophage against Pseudomonas aeruginosa Infections
Changes in Essential Fatty Acids and Ileal Genes Associated with Metabolizing Enzymes and Fatty Acid Transporters in Rodent Models of Cystic Fibrosis
In Vivo Inflammation Caused by Achromobacter spp. Cystic Fibrosis Clinical Isolates Exhibiting Different Pathogenic Characteristics
Aspergillus fumigatus Supernatants Disrupt Bronchial Epithelial Monolayers: Potential Role for Enhanced Invasion in Cystic Fibrosis
Bone Disease in Long-Term Lung Transplant Survivors
Anti-Pseudomonas aeruginosa Vaccines and Therapies: An Assessment of Clinical Trials
Serum Appetite-Regulating Hormone Levels in Cystic Fibrosis Patients: Influence of the Disease Severity and the Type of Bacterial Infection-A Pilot Study
The effect of CFTR modulators on structural lung disease in cystic fibrosis
Effect of Nebulized BromAc on Rheology of Artificial Sputum: Relevance to Muco-Obstructive Respiratory Diseases
The Cystic Fibrosis Transmembrane Conductance Regulator Gene (CFTR) Is under Post-Transcriptional Control of microRNAs: Analysis of the Effects of agomiRNAs Mimicking miR-145-5p, miR-101-3p, and miR-335-5p
Airway inflammation and lung function in cystic fibrosis
Long-term effects of lumacaftor/ivacaftor on paranasal sinus abnormalities in children with cystic fibrosis detected with magnetic resonance imaging
Cost-effectiveness analysis of genetic tools to predict treatment response in patients with cystic fibrosis
Willingness of people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor (ETI) to participate in randomized modulator and inhaled antimicrobial clinical trials
Targeted locus amplification reveals heterogeneity between and within CFTR genotypes and association with CFTR function in patient-derived intestinal organoids
Discovery of novel inhibitors for Pseudomonas aeruginosa lipase enzyme from in silico and in vitro studies
Self-reported quantity and quality of sleep in children and adolescents with a chronic condition compared to healthy controls
Procedural anxiety among children and adolescents with cystic fibrosis and their parents
Critical disease burdens of Australian adults with cystic fibrosis: Results from an online survey
Identification of Burkholderia cenocepacia non-coding RNAs expressed during Caenorhabditis elegans infection
Competitive survival of clonal serial Pseudomonas aeruginosa isolates from cystic fibrosis airways in human neutrophils
Exploration of the relationship between cumulative exposure to tobramycin and ototoxicity in patients with cystic fibrosis
Human epididymis protein 4 (HE4) plasma concentration inversely correlates with the improvement of cystic fibrosis lung disease in p.Phe508del-CFTR homozygous cases treated with the CFTR modulator lumacaftor/ivacaftor combination
Mobile health platform for self-management of pediatric cystic fibrosis: Impact on patient-centered care outcomes
Transcriptomic analysis of lung development in wildtype and CFTR-/- sheep suggests an early inflammatory signature in the CF distal lung
Depression, anxiety, and resilience during COVID-19 in Dutch patients with cystic fibrosis or primary ciliary dyskinesia and their caregivers
Disruptions in the Cystic Fibrosis Community’s Experiences and Concerns During the COVID-19 Pandemic: Topic Modeling and Time Series Analysis of Reddit Comments
Sex-biased expression of selected chromosome x-linked microRNAs with potent regulatory effect on the inflammatory response in children with cystic fibrosis: A preliminary pilot investigation
Synthesis and screening of cyclic diketone indanedione derivatives as future scaffolds for neutrophil elastase inhibition
Eradication of Mycobacterium abscessus infection in cystic fibrosis with initiation of Elexacaftor/Tezacaftor/Ivacaftor
Cystic Fibrosis in the Kidney: Monitoring the Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in Urine After a Short Pause of Therapy
Identification of Single Nucleotide Variants in SLC26A9 Gene in Patients with Cystic Fibrosis (p.Phe508del Homozygous) and its Association to Orkambi® (Lumacaftor and Ivacaftor) Response in vitro
Periodontitis Risk Factors in Adults with Cystic Fibrosis: A pilot study
Impact of chronic medication de-escalation in patients with cystic fibrosis taking elexacaftor, tezacaftor, ivacaftor: A retrospective review
Use of CFTR modulators in special populations, part 3: Solid organ transplant
How I treat … vitamin D intoxication in a child with cystic fibrosis
Pathogenesis of Achromobacter xylosoxidans respiratory infections: colonization and persistence of airway epithelia and differential gene expression in synthetic cystic fibrosis sputum medium
Mrs4 loss of function in fungi during adaptation to the cystic fibrosis lung
Allergic bronchopulmonary aspergillosis in India
A Participatory Approach Involving Patients with Cystic Fibrosis and Healthcare Professionals for the Co-Design of an Adherence-Enhancing Intervention Toolkit
Nutritional impact of CFTR modulators in children with cystic fibrosis
Survival-Adjusted FEV1 and BMI Percentiles for Patients with Cystic Fibrosis before the Era of Triple CFTR Modulator Therapy in Germany
Mycobacterium malmoense Infection in a Patient With Adult Cystic Fibrosis: A Case Report
The association between Attention-Deficit-Hyperactivity-Disorder (ADHD) symptoms and disease severity in people with Cystic Fibrosis (pwCF)
Simultaneous multiple breath washout and oxygen-enhanced magnetic resonance imaging in healthy adults
Digital technology for early identification of exacerbations in people with cystic fibrosis
Oral health and cystic fibrosis in pediatrics: A survey of health professionals
Comment on “Efficacy of hypertonic saline versus isotonic saline among children with cystic fibrosis: A systematic review and meta-analysis”
Single nucleotide variants in Pseudomonas aeruginosa populations from sputum correlate with baseline lung function and predict disease progression in individuals with cystic fibrosis
Airway and Lung Organoids from Human-Induced Pluripotent Stem Cells Can Be Used to Assess CFTR Conductance
Clinical and Functional Characteristics of the E92K CFTR Gene Variant in the Russian and Turkish Population of People with Cystic Fibrosis
Molecular and Functional Characteristics of Airway Epithelium under Chronic Hypoxia
Clinical Consequences and Functional Impact of the Rare S737F CFTR Variant and Its Responsiveness to CFTR Modulators
How Staphylococcus aureus and Pseudomonas aeruginosa Hijack the Host Immune Response in the Context of Cystic Fibrosis
Passaging Primary Human Bronchial Epithelia Reduces CFTR-Mediated Fluid Transport and Alters mRNA Expression
Real-world disparities and ethical considerations with access to CFTR modulator drugs: Mind the gap!
Differential effects of ELX/TEZ/IVA on organ-specific CFTR function in two patients with the rare CFTR splice mutations c.273+1G>A and c.165-2A>G
Drug desensitization to lumacaftor/ivacaftor: A fast lane to drug tolerance
Calibration and validation of modeled 5-year survival predictions among people with cystic fibrosis treated with the cystic fibrosis transmembrane conductance regulator modulator ivacaftor using United States registry data
Airway clearance techniques compared to no airway clearance techniques for cystic fibrosis
Outpatient management of pulmonary exacerbations in children with cystic fibrosis
Use of A-STEP Test in Cystic Fibrosis Patients
Improvements in glucose regulation in children and young people with cystic fibrosis related diabetes following initiation of Elexacaftor/Tezacaftor/Ivacaftor
Notch signaling inactivation by small molecule gamma-secretase inhibitors restores the multiciliated cell population in the airway epithelium
Implementation of standardized cystic fibrosis care algorithm to improve the center data-quality improvement project international collaboration
Delayed cystic fibrosis diagnosis due to presumed celiac disease-A case report from Syria

Exercise testing and counselling within the cystic fibrosis centres in France: A national survey
A foetus with cystic fibrosis – To treat or not to treat?
Risk factors for more rapid progression of severe liver fibrosis in children with cystic fibrosis-related liver disease: A multi-center study validated by liver biopsy
Heterogeneous liver on research ultrasound identifies children with cystic fibrosis at high risk of advanced liver disease
Therapeutic drug monitoring in cystic fibrosis and associations with pulmonary exacerbations and lung function
Screening strategies for glucose tolerance abnormalities and diabetes in people with cystic fibrosis
Racial Disparities in Mental Health Disorders in Youth with Chronic Medical Conditions
Association between unplanned pregnancies and maternal exacerbations in cystic fibrosis
A compensatory RNase E variation increases Iron Piracy and Virulence in multidrug-resistant Pseudomonas aeruginosa during Macrophage infection
Gaps in Cystic Fibrosis Care Are Associated with Reduced Lung Function in the U.S. Cystic Fibrosis Foundation Patient Registry
Post-approval studies with the CFTR modulators Elexacaftor-Tezacaftor-Ivacaftor
Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis
An ALARMINg Type 2 Response in Cystic Fibrosis – The Key to Understanding ABPA?
Study to Assess Amphotericin B Cystetic for Inhalation (ABCI) Doses in Healthy Volunteers & People With Cystic Fibrosis
PEP and OPEP Devices in Cystic Fibrosis
Tweaking the catalytic efficiency of the CFTR ion channel
Ivacaftor therapy post myocardial infarction augments systemic inflammation and evokes contrasting effects with respect to tissue inflammation in brain and lung
COMPLICATIONS AND PRACTICE VARIATION IN THE USE OF PERIPHERALLY INSERTED CENTRAL VENOUS CATHETERS IN PEOPLE WITH CYSTIC FIBROSIS
Easy-to-Build and Reusable Microfluidic Device for the Dynamic Culture of Human Bronchial Cystic Fibrosis Epithelia
A multidisciplinary model for prenatal care for patients with cystic fibrosis in the era of highly effective modulator therapy
Blue flash sheds light on the roles of individual phosphoserines in CFTR channel activation
Diffusion magnetic resonance imaging for kidney cyst volume quantification and non-cystic tissue characterisation in ADPKD
Ventilatory Threshold and Risk of Pulmonary Exacerbations in Cystic Fibrosis
The synthetic aminoglycoside ELX-02 induces readthrough of G550X-CFTR producing super-functional protein that can be further enhanced by CFTR modulators
Online Detection of HCN in Humid Exhaled Air by Gas Flow-Assisted Negative Photoionization Mass Spectrometry
National integration of mental health screening and treatment into specialized care for cystic fibrosis: What predicts success?
The outcome of COVID-19 in children with chronic lung disease: Systematic review and meta-analyses
Health-related quality of life following lung transplantation for cystic fibrosis: A systematic review
Infection Dynamics of Aspergillus fumigatus in Adults with Cystic Fibrosis (CF)
Peer-supporting for Teenagers With Cystic Fibrosis in the Transition of Care From Paediatric to Adult Services
Morphologically intact airways in lung fibrosis have an abnormal proteome
Six-minute walk test and factors affecting exercise capacity in children with cystic fibrosis
Magnetic resonance imaging detects onset and association with lung disease severity of bronchial artery dilatation in cystic fibrosis
Personalized medicine: Function of CFTR variant p.Arg334Trp is rescued by currently available CFTR modulators
Chronic inhaled antibiotic therapy in people with cystic fibrosis with Pseudomonas aeruginosa infection in Germany
Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond
Correlation between CT imaging and symptom scores in cystic fibrosis associated chronic sinusitis
Provider Perspectives on Fertility and Fertility Preservation Discussions Among Women With Cystic Fibrosis
Cystic Fibrosis Screen Positive, Inconclusive Diagnosis Genotypes in the Cystic Fibrosis Registry
Prenatal cystic fibrosis transmembrane conductance regulator modulator therapy: A promising way to change the impact of cystic fibrosis
“Using chest computed tomography and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis.” A. Campredon, E. Battistella, C. Martin, et al. Eur Respir J 2022; 59: 2101344
Computed tomography body composition and clinical outcomes following lung transplantation in cystic fibrosis
Distribution and Expression of Pulmonary Ionocyte-Related Factors CFTR, ATP6V0D2, and ATP6V1C2 in the Lungs of Yaks at Different Ages
Is There a Difference in Immune Response to SARS-CoV-2 Vaccination between Liver and Lung Transplant Patients with Cystic Fibrosis?
Lack of association between leptin concentrations and cystic fibrosis: A meta-analysis and regression
Prevalence of pulmonary artery dilation in non-cystic fibrosis bronchiectasis: A CT analysis from a cohort of the US Bronchiectasis and Nontuberculous Mycobacteria Research Registry
Pre-emptive transjugular intrahepatic portosystemic shunt in pediatric cystic fibrosis-related liver disease and portal hypertension: prospective long-term results
Chinese experts consensus statement: diagnosis and treatment of cystic fibrosis (2023)
Expert guidelines on exercise and physical activity for people with cystic fibrosis
Psychological interventions for improving adherence to inhaled therapies in people with cystic fibrosis
What is the Perceived Role of the Dietitian Amongst People with Cystic Fibrosis? Results of an International survey
Semaglutide in CFRD
Monoclonal antibody therapy in cystic fibrosis and asthma
Current and Emerging Inhaled Antibiotics for Chronic Pulmonary Pseudomonas aeruginosa and Staphylococcus aureus Infections in Cystic Fibrosis
Lytic Bacteriophage Is a Promising Adjunct to Common Antibiotics across Cystic Fibrosis Clinical Strains and Culture Models of Pseudomonas aeruginosa Infection
Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation
Efficacy and Safety of Elexacaftor-Tezacaftor-Ivacaftor in the Treatment of Cystic Fibrosis: A Systematic Review
CFTR Modulators Rescue the Activity of CFTR in Colonoids Expressing the Complex Allele p.[R74W;V201M;D1270N]/dele22_24
Sleep-Disordered Breathing and Chronic Respiratory Infections: A Narrative Review in Adult and Pediatric Population
The Association between CFTR Gene Mutation Heterozygosity and Asthma Development: A Systematic Review
Advances in Cystic Fibrosis Research in Qatar: A Commentary
Distribution of OGTT-Related Variables in Patients with Cystic Fibrosis from Puberty to Adulthood: An Italian Multicenter Study
Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis
Experiences and perceptions of receiving and prescribing rehabilitation in adults with cystic fibrosis undergoing lung transplantation
Immunoreactive Trypsinogen in Infants Born to Women with Cystic Fibrosis Taking Elexacaftor-Tezacaftor-Ivacaftor
Neutrophil Defect and Lung Pathogen Selection in Cystic Fibrosis
Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density but lung infections generally persist
Association of low-density neutrophils with lung function and disease progression in adult cystic fibrosis
Distinct Screening Approaches Uncover PA14_36820 and RecA as Negative Regulators of Biofilm Phenotypes in Pseudomonas aeruginosa PA14
Effects of Lumacaftor-Ivacaftor on Airway Microbiota-Mycobiota and Inflammation in Patients with Cystic Fibrosis Appear To Be Linked to Pseudomonas aeruginosa Chronic Colonization
High-Throughput Screen Reveals the Structure-Activity Relationship of the Antimicrobial Lasso Peptide Ubonodin
AN 84-YEAR-OLD PATIENT WITH CAROLI SYNDROME: WHAT IS THE PROGNOSIS OF THIS CONDITION?
Early and sustained improvements of lung clearance index from two to sixteen weeks of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis-a real world study
CFTR High Expresser Cells in cystic fibrosis and intestinal diseases
Outcomes of children with cystic fibrosis screen positive, inconclusive diagnosis/CFTR related metabolic syndrome
Mycobacterium abscessus DosRS two-component system controls a species-specific regulon required for adaptation to hypoxia
Normalizing salt content by mixing native human airway mucus samples normalizes sample rheology
Evaluating the efficacy of inhaled amikacin as an adjunct to intravenous combination therapy (ceftazidime and amikacin) in pediatric cystic fibrosis pulmonary exacerbation
PK-PD Evaluation of Inhaled Microparticles loaded with Ciprofloxacin-Copper complex in a Rat Model of Chronic Pseudomonas aeruginosa Lung Infection
Autoimmunity in people with cystic fibrosis
Cervical mucus quality in females with and without cystic fibrosis
A paradigm shift in cystic fibrosis nutritional care: Clinicians’ views on the management of patients with overweight and obesity
The Burkholderia contaminans prevalent phenotypes as possible markers of poor clinical outcomes in chronic lung infection of children with cystic fibrosis
Systematic review and meta-analysis: Associations of vitamin D with pulmonary function in children and young people with cystic fibrosis
Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study
Perioperative Outcomes During Double-Lung Transplantation and Retransplantation in Cystic Fibrosis Patients: A Monocentric Cohort Study
Metal complexes with valuable biomolecules produced by Pseudomonas aeruginosa : a review of the coordination properties of pyocyanin, pyochelin and pyoverdines
Who Modifies the Modifiers: A High Resolution View of the Genetic Modifiers of Cystic Fibrosis
Regulated cell death pathways in kidney disease
Assessment of adaptive immune responses of dairy cows with Burkholderia contaminans -induced mastitis
Cystic fibrosis transmembrane conductance regulator modulators attenuate platelet activation and aggregation in blood of healthy donors and COVID-19 patients
Cystic Fibrosis Reprograms Airway Epithelial IL-33 Release and Licenses IL-33 Dependent Inflammation
Ionocyte-Specific Regulation of CFTR
BEAT CF pulmonary exacerbations core protocol for evaluating the management of pulmonary exacerbations in people with cystic fibrosis
Enhanced Optimal Parameter-Based Nebulizer Design for Flow Analysis of Fluticasone Propionate
CFTR function, pathology and pharmacology at single-molecule resolution
Regarding the article entitled “Effect of elexacaftor/tezacaftor/ivacaftor on annual rate of lung function decline in people with cystic fibrosis”
So You Want to Build Your Disease’s First Online Patient Registry: An Educational Guide for Patient Organizations Based on US and European Experience
Effects of Lumacaftor/Ivacaftor on Cystic Fibrosis Disease Progression in Children 2 through 5 Years of Age Homozygous for F508del-CFTR: A Phase 2 Placebo-controlled Clinical Trial
Increasing incidence rate of breast cancer in cystic fibrosis – relationship between pathogenesis, oncogenesis and prediction of the treatment effect in the context of worse clinical outcome and prognosis of cystic fibrosis due to estrogens

Liver Assessment in Patients with Ataxia-Telangiectasia: Transient Elastography Detects Early Stages of Steatosis and Fibrosis
Acute cholecystitis in cystic fibrosis patients after initiation of treatment with elexacaftor/tezacaftor/ivacaftor
Drug Hypersensitivity Reactions in Patients with Cystic Fibrosis: Potential Value of the Lymphocyte Toxicity Assay to Assess Risk
Ivacaftor: Five-year outcomes in the West of Scotland cystic fibrosis population
Association of Oxygen Therapy with the Natural Disease Progression of Cystic Fibrosis: A Multi-State Model of the European Cystic Fibrosis Society Patient Registry
Development of a Cystic Fibrosis Primary Palliative Care Intervention: Qualitative Analysis of Patient and Family Caregiver Preferences
Cystic Lymphatic Malformation with Lymphedema Treated by Lymphaticovenular Anastomosis Combined with Ethanol Sclerotherapy
Intussusception of the appendix in a young adult: an important differential diagnosis of abdominal pain in cystic fibrosis patients?
Effects of postage on recovery of pathogens from cystic fibrosis sputum samples
Safety and efficacy of a new supplementation protocol in patients with cystic fibrosis and vitamin D deficiency
Iron bioavailability regulates Pseudomonasaeruginosa interspecies interactions through type VI secretion expression
Proteome of airway surface liquid and mucus in newborn wildtype and cystic fibrosis piglets
Down-expression of Foxj1 on airway epithelium with impaired cilia architecture in non-cystic fibrosis bronchiectasis implies disease severity
The impact of elexacaftor/tezacaftor/ivacaftor on body composition in a small cohort of youth with cystic fibrosis
In Utero Mapping and Development Role of CFTR in Lung and Gastrointestinal Tract of Cystic Fibrosis Patients
Medical traumatic stress in cystic fibrosis: A qualitative analysis
The STING/TBK1/IRF3/IFN type I pathway is defective in cystic fibrosis
Concern over tobacco and marijuana perceptions and use among adolescents and young adults with cystic fibrosis
Cystic fibrosis prevalence in the United States and participation in the Cystic Fibrosis Foundation Patient Registry in 2020
Association between nebuliser therapies adherence and visit-to-visit variability of FEV1 in patients with cystic fibrosis
Phase 3 Open-Label Clinical Trial of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged 2 Through 5 Years with Cystic Fibrosis and at Least One F508del Allele
Genetic Modifiers of Cystic Fibrosis Lung Disease Severity: Whole Genome Analysis of 7,840 Patients
Can percussive intrapulmonary ventilation improve the efficacy of physiotherapy in children with cystic fibrosis?
Genome-engineering technologies for modeling and treatment of cystic fibrosis
Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis
Isolated abnormal FEF75% detects unsuspected bronchiolar obstruction in CF children
Burkholderia cepacia infection associated with sickle cell disease: An uncommon entity
Sweat conductivity for diagnosing cystic fibrosis after positive newborn screening: prospective, diagnostic test accuracy study
Acute pancreatitis in pancreatic-insufficient cystic fibrosis patients treated with CFTR modulators
Upper Airway Disease in Adults with Cystic Fibrosis in the Era of CFTR Modulators
Feasibility of free-breathing 19 F MRI image acquisition to characterize ventilation defects in CF and healthy volunteers at wash-in
Efficacy of Omadacycline-Containing Regimen in a Mouse Model of Pulmonary Mycobacteroides abscessus Disease
FEED-Cystic Fibrosis (FEED-CF)
Biomaterial therapeutic strategies for treatment of bacterial lung infections
Adherence, reliability, and variability of home spirometry telemonitoring in cystic fibrosis
“Just Move It . . . Move It”: A Multidisciplinary Motivational Approach to Improve Physical Activity in Children With Cystic Fibrosis
Early Glucose abnormalities in cystic fibrosis – how best to measure them, and what is to be done about them?
Oral care considerations for people with cystic fibrosis: a cross-sectional qualitative study
CFTR Inhibitors Display In Vitro Antiviral Activity against SARS-CoV-2
Effectiveness of Autogenic Drainage in Improving Pulmonary Function in Patients with Cystic Fibrosis
Mycobacterium abscessus Infections in Cystic Fibrosis Individuals: A Review on Therapeutic Options
Revisiting Host-Pathogen Interactions in Cystic Fibrosis Lungs in the Era of CFTR Modulators
Concomitant Use of Elexacaftor/Tezacaftor/Ivacaftor and Etanercept in a Cystic Fibrosis Patient with Juvenile Idiopathic Arthritis
Potentiometric Chloride Ion Biosensor for Cystic Fibrosis Diagnosis and Management: Modeling and Design
Functional restoration of a CFTR splicing mutation through RNA delivery of CRISPR adenine base editor
Advanced Cystic Fibrosis Lung Disease and Lung Transplantation in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulators
Post-transplant outcomes among cystic fibrosis patients undergoing lung transplantation colonized by Burkholderia: A single center cohort study
Influence of environmental factors and polymorphic loci rs6580502 of the SPINK1 gene, rs10273639 of the PRSS1 gene, rs213950 of the CFTR gene on the risk of developing acute alcoholic-alimentary pancreatitis
The Cystic Fibrosis Upper and Lower Airway Metagenome
Comprehensive semen examination in patients with pancreatic-sufficient and pancreatic-insufficient cystic fibrosis
The impact of telehealth based care on paediatric cystic fibrosis outcomes
Quality of anthropometric data measured in children and adolescents with cystic fibrosis: a scoping review
Clinical and functional course of patients with cystic fibrosis treated with lumacaftor/ivacaftor at a children’s hospital. A case series
Lung Function Decline in Cystic Fibrosis: Impact of Data Availability and Modeling Strategies on Clinical Interpretations
Position paper: Models of post-transplant care for individuals with cystic fibrosis
Regulatory T cell enhancement in adults with cystic fibrosis receiving Elexacaftor/Tezacaftor/Ivacaftor therapy
Metabolism-based approaches for autosomal dominant polycystic kidney disease
Inherited causes of exocrine pancreatic insufficiency in pediatric patients: clinical presentation and laboratory testing
Genomic analysis of Staphylococcus aureus sequential isolates from lungs of patients with cystic fibrosis
Canadian Cystic Fibrosis-related Diabetes Clinical Practice Survey: Analysis of Current Practices and Gaps in Clinical Care
Incidence of transaminitis in adults with cystic fibrosis taking elexacaftor/tezacaftor/ivacaftor
Influence on sputum microbiology with CFTR modulator (tezacaftor-ivacaftor) in adult patients with cystic fibrosis: Multicenter study
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis
Safety of elexacaftor/tezacaftor/ivacaftor dose reduction: mechanistic exploration through physiologically-based pharmacokinetic modeling and a clinical case series
Advances in inhaled antibiotics for management of respiratory tract infections
Phage therapy in a lung transplant recipient with cystic fibrosis infected with multidrug-resistant Burkholderia multivorans
SLC26A9 in airways and intestine: secretion or absorption?
Sharing decisions on reproductive goals: A mixed-methods study of the views of women who have cystic fibrosis
The two faces of pyocyanin – why and how to steer its production?
Effect of budesonide on pulmonary activity of multidrug resistance-associated protein 1 assessed with PET imaging in rats
High Deductible Insurance Plans Impart Economic Burden for People with Cystic Fibrosis
Whole Exome Sequencing Identifies a Rare CFTR Mutation in Brothers With Anomalies of the Vas Deferens: a case study
Early life growth trajectories in cystic fibrosis are associated with lung function at age six
Investigating serum extracellular vesicles in Cystic Fibrosis
Mechanisms of iron homeostasis in Pseudomonas aeruginosa and emerging therapeutics directed to disrupt this vital process
De Novo Generation of Pulmonary Ionocytes from Normal and Cystic Fibrosis Human Induced Pluripotent Stem Cells
Cystic fibrosis liver disease in children – A review of our current understanding
Exploring the nature of perceived treatment burden: a study to compare treatment burden measures in adults with cystic fibrosis [version 1; peer review: 2 approved]