Pending

Pseudomonas aeruginosa aggregates in cystic fibrosis sputum produce exopolysaccharides that likely impede current therapies
Cystic Fibrosis Polymorphic Variants in a Russian Population
Downregulation of epithelial sodium channel (ENaC) activity in human airway epithelia after low temperature incubation
Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs
Virulence attenuating combination therapy: a potential multi-target synergy approach to treat Pseudomonas aeruginosa infections in cystic fibrosis patients
Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis
Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
Attitudes Towards Involving Children in Decision-Making Surrounding Lung Transplantation
Frequent Pet Contact as Risk Factor for Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis
Burkholderia vietnamiensis Causing a Non-lactational Breast Abscess in a Non-cystic Fibrosis Patient in Tamil Nadu, India
Neutrophil dysfunction in cystic fibrosis
Contribution of common CFTR variants (M470V, T854, and Q1463) to cystic fibrosis in Tunisia: haplotype analysis
How Many Maneuvers Should We Do for Maximal Inspiratory and Expiratory Muscle Pressure Testing in Children: A Retrospective Review in Children with Cystic Fibrosis
Disparities in insurance coverage among hospitalized adult congenital heart disease patients before and after the Affordable Care Act
Reproductive Health Counseling and Contraceptive Use in Adolescents with Cystic Fibrosis
Feasibility study on exhaled-breath analysis by untargeted Selected-Ion Flow-Tube Mass Spectrometry in children with cystic fibrosis, asthma, and healthy controls: Comparison of data pretreatment and classification techniques
Cystic fibrosis-related diabetes and lung disease: an update
Aspergillus fumigatus Strain-Specific Conidia Lung Persistence Causes an Allergic Broncho-Pulmonary Aspergillosis-Like Disease Phenotype
Comparative genomics of ST5 and ST30 methicillin-resistant Staphylococcus aureus sequential isolates recovered from paediatric patients with cystic fibrosis
An evaluation of healthcare utilization and clinical charges in children and adults with cystic fibrosis
The Incidence of Glucose Disorders Related to Cystic Fibrosis Could Be Higher with Current Criteria (Re: Carbohydrate Metabolism Changes in Cystic Fibrosis. J Pediatr Endocrinol Metab 2007; 20: 621-663)
Rapid Improvement After Starting Elexacaftor-tezacaftor-ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease
Effects of Lumacaftor-Ivacaftor on Lung Clearance Index, Magnetic Resonance Imaging and Airway Microbiome in Phe508del Homozygous Patients with Cystic Fibrosis
Bewertung der Funktionen des rechten Herzens bei Kindern mit leichter Mukoviszidose
Virtual Screening Approach to Identifying a Novel and Tractable Series of Pseudomonas aeruginosa Elastase Inhibitors
Lung Transplantation for Cystic Fibrosis in Turkey: First Report
Failed eradication therapy of new onset Pseudomonas aeruginosa infections in cystic fibrosis children is associated with bacterial resistance to neutrophil functions
Generation of two induced pluripotent stem cell lines (RCMGi004-A and -B) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/W1282X mutations in CFTR gene
The Upd3 cytokine couples inflammation to maturation defects in Drosophila
Beyond phenotype: The genomic heterogeneity of co-infecting Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients
Outcomes of cystic fibrosis pulmonary exacerbations treated with antibiotics with activity against anaerobic bacteria
Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough
Functional relationship between CFTR and RAC3 expression for maintaining cancer cell stemness in human colorectal cancer
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells
Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort
Williams-Campbell syndrome: an unusual presentation in an adult patient
Change in circulating proteins during treatment of pulmonary exacerbation in patients with cystic fibrosis
Clinical characteristics associated with lung function decline in individuals with adult-diagnosed cystic fibrosis: Contemporary analysis of the Canadian CF Registry
Interval versus constant-load exercise training in adults with Cystic Fibrosis
PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy
Withdrawal of dornase alfa increases ventilation inhomogeneity in children with cystic fibrosis
Outcomes of cystic fibrosis pulmonary exacerbations treated with antibiotics with activity against anaerobic bacteria.
CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.
Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort.
Williams-Campbell syndrome: an unusual presentation in an adult patient.
Change in circulating proteins during treatment of pulmonary exacerbation in patients with cystic fibrosis.
Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough.
Functional relationship between CFTR and RAC3 expression for maintaining cancer cell stemness in human colorectal cancer.
Clinical characteristics associated with lung function decline in individuals with adult-diagnosed cystic fibrosis: Contemporary analysis of the Canadian CF Registry.
Interval versus constant-load exercise training in adults with Cystic Fibrosis.
Beyond phenotype: The genomic heterogeneity of co-infecting Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients.
Prostaglandin E2 stimulates anion and fluid secretion triggered by lipopolysaccharide in rat vaginal epithelium.
Hospice care access inequalities: a systematic review and narrative synthesis.
Immune transcriptomes of highly exposed SARS-CoV-2 asymptomatic seropositive versus seronegative individuals from the Ischgl community.
The Upd3 cytokine couples inflammation to maturation defects in Drosophila.
Inhalable nano into micro dry powders for ivacaftor delivery: The role of mannitol and cysteamine as mucus-active agents.
Genetic markers for treatment-related pancreatitis in a cohort of Hispanic children with acute lymphoblastic leukemia.
Antibiotic use evaluation in hospitalized pediatric patients with respiratory tract infections: A retrospective chart review study.
Virtual Screening Approach to Identifying a Novel and Tractable Series of Pseudomonas aeruginosa Elastase Inhibitors.
Diaphragmatic thickness and excursion by lung ultrasound in pediatric chronic pulmonary diseases.
Lung Transplantation for Cystic Fibrosis in Turkey: First Report.
Failed eradication therapy of new onset Pseudomonas aeruginosa infections in cystic fibrosis children is associated with bacterial resistance to neutrophil functions.
Generation of two induced pluripotent stem cell lines (RCMGi004-A and -B) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/W1282X mutations in CFTR gene.
Editing Myosin VB Gene to Create Porcine Model of Microvillus Inclusion Disease, With Microvillus-Lined Inclusions and Alterations in Sodium Transporters.
Feasibility study on exhaled-breath analysis by untargeted Selected-Ion Flow-Tube Mass Spectrometry in children with cystic fibrosis, asthma, and healthy controls: Comparison of data pretreatment and classification techniques.
GRAF2, WDR44, and MICAL1 mediate Rab8/10/11-dependent export of E-cadherin, MMP14, and CFTR ΔF508.
Burkholderia vietnamiensis Causing a Non-lactational Breast Abscess in a Non-cystic Fibrosis Patient in Tamil Nadu, India.
Neutrophil dysfunction in cystic fibrosis.
Contribution of common CFTR variants (M470V, T854, and Q1463) to cystic fibrosis in Tunisia: haplotype analysis.
How Many Maneuvers Should We Do for Maximal Inspiratory and Expiratory Muscle Pressure Testing in Children: A Retrospective Review in Children with Cystic Fibrosis.
A novel highly bio-available itraconazole formulation (SUBA®-Itraconazole) for anti-fungal prophylaxis in Lung Transplant recipients.
Disparities in insurance coverage among hospitalized adult congenital heart disease patients before and after the Affordable Care Act.
Reproductive Health Counseling and Contraceptive Use in Adolescents with Cystic Fibrosis.
Longitudinal effects of ivacaftor and medicine possession ratio in people with the Gly551Asp mutation: a 5-year study.
Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
Complete Genome Sequence of Burkholderia cenocepacia Phage Mica.
Phenotyping of Rare CFTR Mutations Reveals Distinct Trafficking and Functional Defects.
Current Status of Genetic Diagnosis Laboratories and Frequency of Genetic Variants Associated with Cystic Fibrosis through a Newborn-Screening Program in Turkey.
Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.
Associations Between Religious/Spiritual Coping and Depression Among Adults with Cystic Fibrosis: A 12-Month Longitudinal Study.
Friends or enemies? The complicated relationship between Pseudomonas aeruginosa and Staphylococcus aureus.
Effect of omeprazole on symptoms of gastroesophageal reflux disease in children with cystic fibrosis. A randomized, double-blind, placebo-controlled trial.
Measurement properties of the one-minute sit-to-stand test in children and adolescents with cystic fibrosis: A multicenter randomized cross-over trial.
F1099L-CFTR (c.3297C>G) has Impaired Channel Function and Associates with Mild Disease Phenotypes in Two Pediatric Patients.
First clinical trials of the inhaled epithelial sodium channel inhibitor BI 1265162 in healthy volunteers.
Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis.
Cystic fibrosis transmembrane conductance regulator-associated ligand protects dopaminergic neurons by differentially regulating metabotropic glutamate receptor 5 in the progression of neurotoxin 6-hydroxydopamine-induced Parkinson’s disease model.
Diversity and importance of tracheobronchial glands in mammalian species.
Culture-independent multilocus sequence typing of Pseudomonas aeruginosa for cross-infection screening.
Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with open-label extension.
Human Genetic Variation Influences Enteric Fever Progression.
Contribution of Drugs Interfering with Protein and Cell Wall Synthesis to the Persistence of Pseudomonas aeruginosa Biofilms: An In Vitro Model.
Pulmonary Aspergillosis: Spectrum of Disease.
Erratum to ‘From micro to macro; joining the dots of early CF lung disease’ [Journal of Cystic Fibrosis (2020) 850-851/2102].
Determinants of COVID-19 preventive behaviours among adults with chronic diseases in the USA: an analysis of the nationally representative COVID-19 impact survey.
Percutaneous endoscopic gastrostomy in children: A single center experience in Saudi Arabia.
Differential diagnosis of perinatal Bartter, Bartter and Gitelman syndromes.
Detection of cytosine methylation in Burkholderia cenocepacia by single-molecule real-time sequencing and whole-genome bisulfite sequencing.
Leukocyte Telomere Length Is Not Reduced in Children and Adults with Cystic Fibrosis but Associates with Clinical Characteristics-A Cross-Sectional Study.
Animal models for cystic fibrosis: a systematic search and mapping review of the literature. Part 2: nongenetic models.

The Potential for Phospholipids in the Treatment of Airway Inflammation: An Unexplored Solution.
Targeting G542X CFTR nonsense alleles with ELX-02 restores CFTR function in human-derived intestinal organoids.
Effect of CFTR modulator therapy on cystic fibrosis-related diabetes.
Pediatric pulmonology 2019 year in review: rare and diffuse lung disease.
Asthma in Cystic Fibrosis: Definitions and Implications of This Overlap Syndrome.
History of Newborn Screening for Cystic Fibrosis-The Early Years.
Performance of a Three-Tier (IRT-DNA-IRT) Cystic Fibrosis Screening Algorithm in British Columbia.
It All Depends What You Count-The Importance of Definitions in Evaluation of CF Screening Performance.
Constructing a Bioethical Framework to Evaluate and Optimise Newborn Bloodspot Screening for Cystic Fibrosis.
Newborn Screening for CF across the Globe-Where Is It Worthwhile?
Inconclusive Diagnosis after Newborn Screening for Cystic Fibrosis.
The Role of Extended CFTR Gene Sequencing in Newborn Screening for Cystic Fibrosis.
Processing Newborn Bloodspot Screening Results for CF.
Psychological Impact of NBS for CF.
Pancreatitis-Associated Protein in Neonatal Screening for Cystic Fibrosis: Strengths and Weaknesses.
Production of CFTR-ΔF508 Rabbits.
Prospective Evaluation of Aspergillus fumigatus-Specific IgG in Patients With Cystic Fibrosis.
Comparative Analysis of Clinical Parameters and Sputum Biomarkers in Establishing the Relevance of Filamentous Fungi in Cystic Fibrosis.
COVID-19 and Pediatric Lung Disease: A South African Tertiary Center Experience.
Differential effects on the miRNome of the treatment of human airway epithelial Calu-3 cells with peptide-nucleic acids (PNAs) targeting microRNAs miR-101-3p and miR-145-5p: Next generation sequencing datasets.
Correction of the Gene Defect in Cystic Fibrosis: Is it too late for bone?
Antibiotic Efficacy Testing in an Ex vivo Model of Pseudomonas aeruginosa and Staphylococcus aureus Biofilms in the Cystic Fibrosis Lung.
Pulmonary Infection Secondary to Blastobotrys raffinosifermentans in a Cystic Fibrosis Patient: Review of the Literature.
Telemedicine in Pediatrics: A Systematic Review of Randomized Controlled Trials.
Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor.
First and second wave of SARS-CoV2 in Italian Cystic Fibrosis patients: Data from Italian Cystic Fibrosis Registry.
Home-Care Bronchiectasis
Airway Clearance for Pediatric and Adult Cystic Fibrosis Patients Using a Portable Intra-Pulmonary Percussion Device
Prognosis-Based Early Intervention Strategies to Resolve Exacerbation and Progressive Lung Function Decline in Cystic Fibrosis.
Formulation and In Vitro and In Silico Characterization of “Nano-in-Micro” Dry Powder Inhalers Containing Meloxicam.
Urinary stone disease prevalence and associations in cystic fibrosis.
Pseudomonas aeruginosa Resistance to Bacteriophages and Its Prevention by Strategic Therapeutic Cocktail Formulation.
Complete Genome Sequence of Burkholderia gladioli Phage Maja.
Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis.
Eradication success for non-tuberculous mycobacteria in children with cystic fibrosis.
Balkan Endemic Nephropathy: An Autopsy Case Report.
The effects of the addition of a new airway clearance device to chest physiotherapy in children with cystic fibrosis pulmonary exacerbations.
A Prospective Study of the Effects of Sex Hormones on Lung Function and Inflammation in Women with Cystic Fibrosis.
Multidisciplinary approach to patients with manifestations and pulmonary complications of cystic fibrosis.
Clinical Implications of Polymicrobial Synergism Effects on Antimicrobial Susceptibility.
The Effects of Telerehabilitation on Muscle Function, Physical Activity and Sleep in Cystic Fibrosis During Pandemic
Interspecies Metabolic Complementation in Cystic Fibrosis Pathogens via Purine Exchange.
Considerations for Phage Therapy Against Mycobacterium abscessus.
Gender-affirming hormone therapy in cystic fibrosis – A case of new Pseudomonas infection.
Intestinal TMEM16A control luminal chloride secretion in a NHERF1 dependent manner.
Improvement of Pulmonary Function in Cystic Fibrosis Patients following Endoscopic Sinus Surgery.
Telemedicine Use and Health-related Concerns of Patients with Chronic Conditions During COVID-19: A Survey of Members of Online Health Communities.
The Effects of Nebulizer Hygiene Training on the Practices of Cystic Fibrosis Patients and Caregivers.
Development and in vitro characterization of a novel pMDI diclofenac formulation as an inhalable anti-inflammatory therapy for cystic fibrosis.
A Peptide-Nucleic Acid Targeting miR-335-5p Enhances Expression of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene with the Possible Involvement of the CFTR Scaffolding Protein NHERF1.
Reflections on the Importance of Cost of Illness Analysis in Rare Diseases: A Proposal.
Primary care during the transition to adult care for adolescents involved with pediatric specialty services: a scoping review protocol.
Challenges in the use of highly effective modulator treatment for cystic fibrosis.
Mesenchymal Stem Cell exosome delivered Zinc Finger Protein activation of cystic fibrosis transmembrane conductance regulator.
Histomorphology and Immunohistochemistry of a Congenital Nephromegaly Demonstrate Concurrent Features of Heritable and Acquired Cystic Nephropathies in a Girgentana Goat (Capra falconeri).
Losartan reduces cigarette smoke-induced airway inflammation and mucus hypersecretion.
Avatar acceptability: views from the Australian Cystic Fibrosis community on the use of personalised organoid technology to guide treatment decisions.
Sex disparities in cystic fibrosis: review on the effect of female sex hormones on lung pathophysiology and outcomes.
Longitudinal non-cystic fibrosis trends of pulmonary Mycobacterium abscessus disease from 2010 to 2017: spread of the “globally successful clone” in Asia.
Large pH oscillations promote host defense against human airways infection.
DNAJB12 and Hsp70 Triage Arrested Intermediates of N1303K-CFTR for ER Associated-Autophagy.
Pilocarpine Microneedles for Sweat Induction (PMN-SI)
Microbial Biomarkers of EArly Pseudomonas Aeruginosa Colonization in CHildren With Cystic Fibrosis
Environmental Microbial Contamination during Cystic Fibrosis Group-Based Psychotherapy.
JCF Year in Review.
Sclerosing Polycystic Adenoma.
Cystic Fibrosis.
Patterns of azithromycin use in obstructive airway diseases: a real-world observational study.
From genotype to phenotype: adaptations of Pseudomonas aeruginosa to the cystic fibrosis environment.
Interactions between ABCC4/MRP4 and ABCC7/CFTR in Human Airway Epithelial Cells in Lung Health and Disease.
Within-Host Microevolution of Pseudomonas aeruginosa Urinary Isolates: A Seven-Patient Longitudinal Genomic and Phenotypic Study.
How Clinically Efficient Is Lumacaftor/Ivacaftor for Cystic Fibrosis Patients? An Updated Literature Review.
Intravital Imaging of Pulmonary Immune Response in Inflammation and Infection.
Social Inequities and Cystic Fibrosis Outcomes: We Can Do Better.
Azithromycin and Tobramycin Therapy in Cystic Fibrosis Pulmonary Exacerbations: Less Is More?
Pseudomonas aeruginosa induces p38MAP kinase-dependent IL-6 and CXCL8 release from bronchial epithelial cells via a Syk kinase pathway.
Optimization of anti-pseudomonal antibiotics for cystic fibrosis pulmonary exacerbations: II. Cephalosporins and penicillins latest update.
Modulators of CFTR. Updates on clinical development and future directions.
Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study.
The O2-independent pathway of ubiquinone biosynthesis is essential for denitrification in Pseudomonas aeruginosa.
Lung Microbiome in Cystic Fibrosis.
Serious infectious events and ibuprofen administration in pediatrics: a narrative review in the era of COVID-19 pandemic.
Exploring the Developmental Impact of Cystic Fibrosis on Lung Transplant Candidacy: Considerations for Adulthood.
Pseudomonas aeruginosa Elastase Contributes to the Establishment of Chronic Lung Colonization and Modulates the Immune Response in a Murine Model.
Personalized approaches to bronchiectasis.
Outcomes of Repeat Sweat Testing in Cystic Fibrosis Newborn Screen Positive Infants.
Pseudomonas aeruginosa colonization in cystic fibrosis: Impact on neutrophil functions and cytokine secretion capacity.
Screening of depression and anxiety in adolescents with cystic fibrosis and caregivers in Turkey by PHQ-9 and GAD-7 questionnaires.
Quantitative Evaluation of CFTR Pre-mRNA Splicing Dependent on the (TG)mTn Poly-Variant Tract.
Overcoming Challenges to Make Bacteriophage Therapy Standard Clinical Treatment Practice for Cystic Fibrosis.
Early Diagnosis and Intervention in Cystic Fibrosis: Imagining the Unimaginable.
GLP-1 Agonist Therapy in Cystic Fibrosis-Related Glucose Intolerance
Recognition of Diagnostic Gaps for Laboratory Diagnosis of Fungal Diseases: Expert Opinion from the Fungal Diagnostics Laboratories Consortium (FDLC).
CFTR modulators: transformative therapies for cystic fibrosis.
Cost-effectiveness of implementing routine hearing screening using a tablet audiometer for pediatric cystic fibrosis patients receiving high-dose IV aminoglycosides.
The effectiveness and value of novel treatments for cystic fibrosis.
[Novel therapies in respiratory management].
Feasibility of Outpatient Closed Loop Control With the iLet Bionic Pancreas in Cystic Fibrosis Related Diabetes
Cystic fibrosis-related diabetes onset can be predicted using biomarkers measured at birth.
Molecular mechanism mediating enteric bacterial translocation after severe burn: the role of cystic fibrosis transmembrane conductance regulator.

Metabotypes of Pseudomonas aeruginosa Correlate with Antibiotic Resistance, Virulence and Clinical Outcome in Cystic Fibrosis Chronic Infections.
Synthesis of Tetravalent Thio- and Selenogalactoside-Presenting Galactoclusters and Their Interactions with Bacterial Lectin PA-IL from Pseudomonas aeruginosa.
Prolonged Treatment With Inhaled Ampicillin in Children With Non-cystic Fibrosis Bronchiectasis.
The proteostatic network chaperome is downregulated in F508del homozygote cystic fibrosis.
“Test-retest reliability and minimal detectable change in TGlittre-P test in children and adolescents with cystic fibrosis”.
[Portuguese Newborn Screening Program.]
Overcoming the Limits of Reconditioning: Seventeen Hours of Ex-Vivo Lung Perfusion (EVLP) with Successful Transplantation from Uncontrolled Circulatory Death Donor.
A Mobile Health Platform for Self-Management of Pediatric Cystic Fibrosis: Qualitative Study of Adaptation to Stakeholder Needs and Integration in Clinical Settings.
Peripheral muscle strength is associated with aerobic fitness and use of antibiotics in patients with cystic fibrosis.
Animal Models and Their Role in Understanding the Pathophysiology of Cystic Fibrosis-Associated Gastrointestinal Lesions.
Comparison of Hyperpolarized 3He and 129Xe MR Imaging in Cystic Fibrosis Patients.
A narrative review of electrical impedance tomography in lung diseases with flow limitation and hyperinflation: methodologies and applications.
The fractional excretion of sodium in patients with cystic fibrosis treated with oral sodium chloride.
Preoperative evaluation for lung resection in patients with bronchiectasis: should we rely on standard lung function evaluation?
Analysis of national coverage of neonatal cystic fibrosis screening in Brazil from 2008 to 2017.
Manifesto on United Airways Diseases (UAD): An Interasma (Global Asthma Association – GAA) document.
Therapeutic peptides for the treatment of cystic fibrosis: Challenges and perspectives.
Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators.
In vitro evaluation of drug delivery behavior for inhalable amorphous nanoparticle formulations in a human lung epithelial cell model.
Pharmacokinetic and Pharmacodynamic Optimization of Antibiotic Therapy in Cystic Fibrosis Patients: Current Evidences, Gaps in Knowledge and Future Directions.
Assessing the validity and applicability of the French 3-year prognostic score in the UK cystic fibrosis population – a national cohort study.
Nanoparticles for Delivery of Agents to Fetal Lungs.
Virulence attenuating combination therapy: a potential multi-target synergy approach to treat Pseudomonas aeruginosa infections in cystic fibrosis patients.
New therapies for people with CF in the CFTR modulator world.
From micro to macro; joining the dots of early CF lung disease.
The PROSPECT Is Bright for CFTR Modulators.
Microstructured Lipid Carriers (MLC) Based on N-Acetylcysteine and Chitosan Preventing Pseudomonas aeruginosa Biofilm.
Cellular Redox State Acts as Switch to Determine the Direction of NNT-Catalyzed Reaction in Cystic Fibrosis Cells.
Neutrophil extracellular traps are present in the airways of ENaC-overexpressing mice with cystic fibrosis-like lung disease.
Abnormal glucose tolerance and lung function in children with cystic fibrosis. Comparing oral glucose tolerance test and continuous glucose monitoring.
Incidence and risk factors for respiratory tract bacterial colonization and infection in lung transplant recipients.
Pediatrician intervention impacts parental smoking in cystic fibrosis, diabetes and bronchiolitis.
EGFR activity upregulates lactate dehydrogenase A (LDHA) expression, LDH activity and lactate secretion in cultured IB3-1 cystic fibrosis lung epithelial cells.
Safety of research bronchoscopy with BAL in stable adult patients with cystic fibrosis.
Myriocin modulates the altered lipid metabolism and storage in cystic fibrosis.
Transmission and antibiotic resistance of Achromobacter in cystic fibrosis.
Novel Lytic Phages Protect Cells and Mice against Pseudomonas aeruginosa Infection.
Phenotypic and Genotypic Adaptations in Pseudomonas aeruginosa Biofilms following Long-Term Exposure to an Alginate Oligomer Therapy.
Pediatric Resident Education in Pulmonary (PREP): A Subspecialty Preparatory Boot Camp Curriculum for Pediatric Residents.
The effect of an intensive residential rehabilitation program on body composition in patients with cystic fibrosis.
Early Life Height Attainment in Cystic Fibrosis Is Associated with Pulmonary Function at Age 6 Years.
[Clinical characteristics and gene variants of patients with infantile intrahepatic cholestasis].
A Case of Phage Therapy against Pandrug-Resistant Achromobacter xylosoxidans in a 12-Year-Old Lung-Transplanted Cystic Fibrosis Patient.
Role of Collagen in Airway Mechanics.
Contemporary analysis of ETEST for antibiotic susceptibility and minimum inhibitory concentration agreement against Pseudomonas aeruginosa from patients with cystic fibrosis.
Ultra-low-dose thoracic CT with model-based iterative reconstruction (MBIR) in cystic fibrosis patients undergoing treatment with cystic fibrosis transmembrane conductance regulators (CFTR).
Positive epistasis between disease-causing missense mutations and silent polymorphism with effect on mRNA translation velocity.
Meta-analysis of Efficacy and Safety of Inhaled Ciprofloxacin in Non-cystic Fibrosis Bronchiectasis Patients.
Does Bordetella pertussis vaccine offer any cross-protection against Bordetella bronchiseptica? Implications for pet owners with cystic fibrosis.
Understanding the expanding role of pharmacy services in outpatient cystic fibrosis care.
Cystic Fibrosis Patients of Minority Race and Ethnicity Less Likely Eligible for CFTR Modulators Based on CFTR Genotype.
CF Monocyte Derived Macrophages Have an Attenuated Response to Extracellular Vesicles Secreted by Airway Epithelial Cells.
Gene Therapy for Cystic Fibrosis: Lessons Learned and Paths Forward.
In silico drug repositioning on F508del-CFTR: A proof-of-concept study on the AIFA library.
COVID-19 lockdown beneficial effects on lung function in a cohort of cystic fibrosis patients.
Changes in airway inflammation with pseudomonas eradication in early cystic fibrosis.
Neonatal screening program for five conditions in Honduras.
Sharing Patient-Controlled Real-World Data Through the Application of the Theory of Commons: Action Research Case Study.
The Risks of Complications During Endoscopic Sinus Surgery in Cystic Fibrosis Patients: An Anatomical and Endoscopic Study.
A Randomized, Double-Blind, Placebo-Controlled, Multiple Dose Escalation Study to Evaluate the Safety and Pharmacokinetics of ELX-02 in Healthy Subjects.
Functional Impacts of Aminoglycoside Treatment on Speech Perception and Extended High-Frequency Hearing Loss in a Pediatric Cystic Fibrosis Cohort.
Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Study Design.
Cystic fibrosis transmembrane conductance regulator in follicular fluid and cumulus cells and its relationship with age.
Preemptive non-selective bronchial artery angioembolization to reduce recurrence rate of hemoptysis.
Perioperative Management and Preemptive ECMO Cannulation of a Parturient with Cystic Fibrosis Undergoing Cesarean Delivery.
Applying Petitmengin’s Explicitation Interview Method to Elicit the Lived Experience of Breathing Upon Waking by an Individual With Cystic Fibrosis.
Hearing the Voice of a Shadow Child: Healthy Siblings Experience of Cystic Fibrosis and Other Life-Threatening Conditions.
Virtual Peer Support for People With Cystic Fibrosis and Their Family Members: A Program Evaluation.
Associations between peak oxygen uptake, lung function and bronchiectasis in children with cystic fibrosis in the era of CFTR modulators.
[Guideline for the diagnosis and treatment of patients with cystic fibrosis. Update].
Kill and cure: genomic phylogeny and bioactivity of Burkholderia gladioli bacteria capable of pathogenic and beneficial lifestyles.
diagnosis of endocrine disease: Diagnosing and classifying diabetes in diseases of the exocrine pancreas.
Lung microbiota predict chronic rejection in healthy lung transplant recipients: a prospective cohort study.
Roles of microRNAs in chronic pediatric diseases and their use as potential biomarkers: A review.
Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity.
Is hyperpolarised gas magnetic resonance imaging a valid and reliable tool to detect lung health in cystic fibrosis patients? a cosmin systematic review.
Biomarkers for the diagnosis of allergic bronchopulmonary aspergillosis in cystic fibrosis: a systematic review and meta-analysis.
The Aspergillus fumigatus Secretome Alters the Proteome of Pseudomonas aeruginosa to Stimulate Bacterial Growth: Implications for Co-infection.
Designing the GALAXY study: Partnering with the cystic fibrosis community to optimize assessment of gastrointestinal symptoms.
Signal Synthase-Type versus Catabolic Monooxygenases: Retracing 3-Hydroxylation of 2-Alkylquinolones and Their N-Oxides by Pseudomonas aeruginosa and Other Pulmonary Pathogens.
Diagnosis of cystic fibrosis in adulthood and eligibility for novel CFTR modulator therapy.
Commentary for the article: MicroRNA-1246 regulates proliferation, invasion and differentiation in human vascular smooth muscle cell by targeting cystic fibrosis transmembrane conductance regulator (CFTR).
Pancreatic Cancer-Associated Diabetes is Clinically Distinguishable From Conventional Diabetes.
Towards human translation of lentiviral airway gene delivery for cystic fibrosis: A one-month CFTR and reporter gene study in marmosets.
Complete Genome Sequence of Achromobacter xylosoxidans Myophage Mano.
Unsupervised Phenotypic Clustering for Determining Clinical Status in Children with Cystic Fibrosis.
[Bird fancier’s disease in a child: about a rare and unusual case].
Weak acids as an alternative anti-microbial therapy.
Antibiotic susceptibility of cystic fibrosis lung microbiome members in a multispecies biofilm.
Antibiotics in Adult Cystic Fibrosis Patients: A Review of Population Pharmacokinetic Analyses.
Copper nanoparticles: Biosynthesis, characterization, and protoscolicidal effects alone and combined with albendazole against hydatid cyst protoscoleces.
Cystic Fibrosis: Quality of Life and Radiological Monitoring.
Gasdermin D restricts Burkholderia cenocepacia infection in vitro and in vivo.
Modulation of cAMP metabolism for CFTR potentiation in human airway epithelial cells.
Methods and feasibility study for exome sequencing as a universal second-tier test in newborn screening.
Surgery for Ampullary Cancer in a Patient with Pancreatic Lipomatosis Caused by Cystic Fibrosis.
Influence of relevant cystic fibrosis bacteria on Scedosporium apiospermum and Scedosporium boydii growth and viability.
Exercise as a substitute for traditional airway clearance in cystic fibrosis: a systematic review.
On the relationship between anion binding and chloride conductance in the CFTR anion channel.
A multicentre analysis of Clostridium difficile in persons with Cystic Fibrosis demonstrates that carriage may be transient and highly variable with respect to strain and level: Cystic Fibrosis and Clostridium difficile.

PIPERACILLIN-TAZOBACTAM HYPERSENSITIVITY: A LARGE, MULTICENTRE ANALYSIS.
Assessing the applicability of the new Global Lung Function Initiative reference values for the diffusing capacity of the lung for carbon monoxide in a large population set.
COVID-19 rapid guideline: cystic fibrosis
Korean Red Ginseng aqueous extract improves markers of mucociliary clearance by stimulating chloride secretion.
Same Game, Different Players: Emerging Pathogens of the CF Lung.
Peptide-Conjugated Phosphorodiamidate Morpholino Oligomers Retain Activity against Multidrug-Resistant Pseudomonas aeruginosa In Vitro and In Vivo.
The GENDULF algorithm: mining transcriptomics to uncover modifier genes for monogenic diseases.
Metformin slows liver cyst formation and fibrosis in experimental model of polycystic liver disease.
The biofilm of Burkholderia cenocepacia H111 contains an exopolysaccharide composed of l-rhamnose and l-mannose: Structural characterization and molecular modelling.
Mobilome Analysis of Achromobacter spp. Isolates from Chronic and Occasional Lung Infection in Cystic Fibrosis Patients.
Molecular characterization, expression analysis of 14-3-3 beta/alpha and the effect of RNA interference on ion transporter protein Na+-K+-ATPase, Na+-H+-exchanger and CFTR in turbot (Scophthalmus maximus).
Antipseudomonal and Immunomodulatory Properties of Esc Peptides: Promising Features for Treatment of Chronic Infectious Diseases and Inflammation.
Effects of therapeutic probiotics on modulation of microRNAs.
Relationship between airway dysbiosis, inflammation and lung function in adults with cystic fibrosis.
Patient and Family Perspectives Regarding the Use of Telehealth For Cystic Fibrosis Care.
Clinical implications of fungal isolation from sputum in adult patients with cystic fibrosis.
Comparing Stable Isotope Enrichment by Gas Chromatography with Time-of-Flight, Quadrupole Time-of-Flight, and Quadrupole Mass Spectrometry.
SARS-CoV-2 may hijack GPCR signaling pathways to dysregulate lung ion and fluid transport.
A case of testicular atrophy associated with cystic fibrosis.
Reduction of pulmonary exacerbations in young children with cystic fibrosis during the COVID-19 pandemic.
Epidemiologic Study of Cystic Fibrosis: 25 years of observational research.
Bacteriophage therapy for infections in CF.
Entering the era of highly effective modulator therapies.
Assessment of Distinct Electrophysiological Parameters in Rectal Biopsies for the Choice of the Best Diagnosis/Prognosis Biomarkers for Cystic Fibrosis.
Diaphragmatic Ultrasound in Non-Cystic Fibrosis Bronchiectasis: Relationship to Clinical Parameters.
Where is the Cystic Fibrosis Transmembrane Conductance Regulator?
Colorectal Cancer Screening and Surveillance for Non-Hereditary High-Risk Groups-Is It Time for a Re-Think?
Use of a Connected Companion in Children With Cystic Fibrosis
Current and novel therapeutic strategies for the management of cystic fibrosis.
Biofilms.
Dynamic light scattering microrheology for soft and living materials.
Tyrosine supplementation for phenylketonuria.
HIGH PREVALENCE OF NONTUBERCULOUS MYCOBACTERIA IN CYSTIC FIBROSIS PATIENTS IN TROPICAL FRENCH REUNION ISLAND.
The antimicrobial peptide Gad-1 clears Pseudomonas aeruginosa biofilms under cystic fibrosis conditions.
Exhaled volatile organic compounds detect pulmonary exacerbations early in children with Cystic Fibrosis: results of a one-year observational pilot study.
Introducing the Adult Cystic Fibrosis Series: An Exciting Time of Change, But New Challenges Lie Ahead.
The modified shuttle test as a predictor of risk for hospitalization in youths with cystic fibrosis: A two-year follow-up study: Modified shuttle test as a predictor of hospitalization.
Untapped potential: Therapeutically targeting eicosanoids and endocannabinoids in the lung.
Therapeutic Potential of Antimicrobial Peptides in Polymicrobial Biofilm-Associated Infections.
MicroRNA-1246 regulates proliferation, invasion, and differentiation in human vascular smooth muscle cells by targeting cystic fibrosis transmembrane conductance regulator (CFTR).
Effect of Expiratory Muscle Training on Peak Cough Flow in Children and Adolescents with Cystic Fibrosis: A Randomized Controlled Trial.
Implementation of microbiota analysis in clinical trials for cystic fibrosis lung infection: Experience from the OligoG phase 2b clinical trials.
Looking beyond pulmonary disease in COVID-19: A lesson from patients with cystic fibrosis.
CRISPR/Cas9 Gene Editing Therapies for Cystic Fibrosis.
Clinico-microbiological profile of Burkholderia cepacia keratitis: a case series.
Significant functional differences in differentiated Conditionally Reprogrammed (CRC)- and Feeder-free Dual SMAD inhibited-expanded human nasal epithelial cells.
Ataluren and aminoglycosides stimulate read-through of nonsense codons by orthogonal mechanisms.
A dyadic approach to the delineation of diagnostic entities in clinical genomics.
Positive expiratory pressure (PEP) therapy. What pressures do we achieve in young children with cystic fibrosis? A single-centre study.
Extracellular cyclic dinucleotides induce polarized responses in barrier epithelial cells by adenosine signaling.
Tribulations and (clinical) trials in cystic fibrosis.
Impaired cholesterol metabolism in the mouse model of cystic fibrosis. A preliminary study.
Cystic fibrosis in Tuscany: evolution of newborn screening strategies over time to the present.
SARS-CoV-2 Entry Genes Expression in Relation with Interferon Response in Cystic Fibrosis Patients.
Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs.
Erratum to “Epithelial vectorial ion transport in cystic fibrosis: Dysfunction, measurement, and pharmacotherapy to target the primary deficit”.
Carbapenem-Resistant Pseudomonas aeruginosa in Chronic Lung Infection: Current Resistance Profile and Hypermutability in Patients with Cystic Fibrosis.
A Murine Model for Chronic A. fumigatus Airway Infections.
Feasibility of a Goal-based Agenda Setting Intervention
Thoracic Mobility in Cystic Fibrosis Care
Optimizations of In Vitro Mucus and Cell Culture Models to Better Predict In Vivo Gene Transfer in Pathological Lung Respiratory Airways: Cystic Fibrosis as an Example.
Synergy between Readthrough and Nonsense Mediated Decay Inhibition in a Murine Model of Cystic Fibrosis Nonsense Mutations.
Significance of kidney biopsy in autosomal dominant tubulointerstitial kidney disease-UMOD: is kidney biopsy truly nonspecific?
Comparison of respiratory pathogen colonization and antimicrobial susceptibility in people with cystic fibrosis bronchiectasis versus non-cystic fibrosis bronchiectasis: a protocol for a systematic review.
Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis.
Recurrent massive hemoptysis in a patient with cystic fibrosis: balloon assisted Onyx embolization after bronchial artery coil recanalization.
Repurposing calcium sensing receptor agonist cinacalcet for treatment of CFTR-mediated secretory diarrheas.
Pseudomonas aeruginosa associated with severity of non-cystic fibrosis bronchiectasis measured by the modified bronchiectasis severity score (BSI) and the FACED: The US bronchiectasis and NTM Research Registry (BRR) study.
New Therapeutic Approaches in Cystic Fibrosis.
Development of an intervention to increase adherence to nebuliser treatment in adults with cystic fibrosis: CFHealthHub.
A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay.
The Interplay Between Respiratory Microbiota and Innate Immunity in Flavor E-Cigarette Vaping Induced Lung Dysfunction.
Nephronophthisis due to a novel DCDC2 variant in a patient from African-Caribbean descent: A case report.
Oxygen uptake kinetics during treadmill walking in adolescents with clinically stable cystic fibrosis.
Altered Degranulation and pH of Neutrophil Phagosomes Impacts Antimicrobial Efficiency in Cystic Fibrosis.
A Case of Scedosporium aurantiacum Infection in the United States.
In vivo Evaluation of Mucociliary Clearance in Mice.
Cystic fibrosis or not? Familial occurrence of a rare mutation in the CFTR gene.
Distinctive metabolic profiles between Cystic Fibrosis mutational subclasses and lung function.
Influence of Acid Blockade on the Aerodigestive Tract Microbiome in Children With Cystic Fibrosis.
Obstructive lung diseases and allergic bronchopulmonary aspergillosis.
Erratum to: Epidemiology of Nontuberculous Mycobacteria Infection in Children and Young People With Cystic Fibrosis: Analysis of UK Cystic Fibrosis Registry.
Vitamin E supplementation in people with cystic fibrosis: Summary of a Cochrane review.
VITAMIND AND ITS THERAPEUTIC RELEVANCE IN PULMONARY DISEASES.
Abnormal Lung Clearance Index in Cystic Fibrosis Children with Normal FEV1 and Single-Breath Nitrogen Washout Test.
Predicting Substance Use from Religiosity/Spirituality in Individuals with Cystic Fibrosis.
Bridging the survival gap in cystic fibrosis: An investigation of lung transplant outcomes in Canada and the United States.
OTX2 regulates CFTR expression during endoderm differentiation and occupies 3′ cis-regulatory elements.
Psychological distress in adults with and without cystic fibrosis during the COVID-19 lockdown.
Genetic information from discordant sibling pairs points to ESRP2 as a candidate trans-acting regulator of the CF modifier gene SCNN1B.
Age at Lung Transplant Impacts Post-Transplant Survival in Cystic Fibrosis; Why?
Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation.
Identification of Predictive Epigenetic Biomarkers of Lung Disease Severity in Cystic Fibrosis
Physiometric Response to High-Flow Nasal Cannula Support in Acute Bronchiolitis.
Carbamazepine-induced sperm disorders can be associated with the altered expressions of testicular KCNJ11/miR-let-7a and spermatozoal CFTR/miR-27a.
SARS-CoV-2 related pneumonia in an adult with cystic fibrosis: natural favourable clinical course or effective therapy?
Andrological findings in infertile men with two (biallelic) CFTR mutations: results of a multicentre study in Germany and Austria comprising 71 patients.
Host genetic effects in pneumonia.
The clinical and microbiological utility of inhaled aztreonam lysine for the treatment of acute pulmonary exacerbations of cystic fibrosis: An open-label randomised crossover study (AZTEC-CF).
Long-term Follow-up in Adult Patients with Cystic Fibrosis and Deep Intronic Splicing Variants.

Identification of cell-surface glycans that mediate motility-dependent binding and internalization of Pseudomonas aeruginosa by phagocytes.
VO2max as an exercise tolerance endpoint in people with cystic fibrosis: Lessons from a lumacaftor/ivacaftor trial.
Purinergic receptors in airway hydration.
Continuous and daily oral immunotherapy for peanut allergy: results from a 2-year open-label follow-on study.
Longitudinal Course of Clinical Lung Clearance Index in Children with Cystic Fibrosis.
β-lactam combinations that exhibit synergy against Mycobacteroides abscessus clinical isolates.
Modeling of the Transport and Exchange of a Gas Species in Lungs With an Asymmetric Branching Pattern. Application to Nitric Oxide.
An open-source high-content analysis workflow for CFTR function measurements using the forskolin-induced swelling assay.
Murepavadin antimicrobial activity against and resistance development in cystic fibrosis Pseudomonas aeruginosa isolates.
Authors’ reply re: The outcome of pregnancy in women with cystic fibrosis: a UK population-based descriptive study.
Preferences for Disclosing Adverse Childhood Experiences for Children and Adults with Cystic Fibrosis.
Differentially Expressed Genes Associated with Disease Severity In Siblings with Cystic Fibrosis.
Quantifying the Effects of Antimicrobials on In vitro Biofilm Architecture using COMSTAT Software.
Correlates of distress in young people with cystic fibrosis: the role of self-efficacy and metacognitive beliefs.
Oral cysteamine as an adjunct treatment in cystic fibrosis pulmonary exacerbations: An exploratory randomized clinical trial.
Implementation of a Clinical Tool to Improve Waitlist Mortality in Patients With Cystic Fibrosis
Clinical Biofilm Ring Test® Reveals the Potential Role of β-Lactams in the Induction of Biofilm Formation by P. aeruginosa in Cystic Fibrosis Patients.
Congenital absence of the vas deferens: Cystic fibrosis transmembrane regulatory gene mutations.
Skin Metabolomics.
Appraisal of Clinical Practice Guideline: Physiotherapy for cystic fibrosis in Australia and New Zealand.
Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: A two center case series.
Influence of pre-diabetic and pancreatic exocrine states on pulmonary and nutritional status in adults with Cystic Fibrosis.
A Pilot Study to Assess the Effect of Intermittent iNO on the Treatment of NTM Lung Infection in CF and Non-CF Patients
The Impact of Patch Rx Technologies on Adherence to Vitamin and Trikafta Therapies in Patients With Cystic Fibrosis
CYstic Fibrosis bacterioPHage Study at Yale (CYPHY)
Effects of a Telerehabilitation Approach in Children With Cystic Fibrosis
Video Telehealth Exercise Training in Cystic Fibrosis
Variant classifications, databases and genotype-phenotype correlations.
The Multifaceted Roles of MicroRNAs in Cystic Fibrosis.
Increased Fecal Calprotectin Is Associated with Worse Gastrointestinal Symptoms and Quality of Life Scores in Children with Cystic Fibrosis.
Graphene-Doped Tin Oxide Nanofibers and Nanoribbons as Gas Sensors to Detect Biomarkers of Different Diseases through the Breath.
A Transparent Approach to Calculate Detection Rate and Residual Risk for Carrier Screening.
The impact of SARS-CoV-2 on the cystic fibrosis foundation therapeutics development network.
CFTR-function and ventilation inhomogeneity in individuals with cystic fibrosis.
Vitamin D supplementation in patients with cystic fibrosis: A systematic review and meta-analysis.
Cellulophaga algicola alginate lyase inhibits biofilm formation of a clinical Pseudomonas aeruginosa strain MCC 2081.
Caring for Adolescents with Cystic Fibrosis, in Portugal: The Nurse’s Role.
Results of Tobramycin Inhalation Therapy in Patients with Noncystic Fibrosis Bronchiectasis with Pseudomonas aeruginosa Colonization: Real Life Management.
Care of Cystic Fibrosis Children in COVID-19 Pandemic.
NKCC1: Newly Found as a Human Disease-Causing Ion Transporter.
The clinical use of lung MRI in cystic fibrosis: what, now, how?
The effect of particle size of inhaled tobramycin dry powder on the eradication of Pseudomonas aeruginosa biofilms.
A Population Pharmacokinetic Modeling Approach to Determine the Efficacy of Intravenous Amikacin in Children with Cystic Fibrosis.
Role of Tris-CaEDTA as an adjuvant with nebulised tobramycin in cystic fibrosis patients with Pseudomonas aeruginosa lung infections: A randomised controlled trial.
Ototoxicity in cystic fibrosis patients receiving intravenous tobramycin for acute pulmonary exacerbation: Ototoxicity following tobramycin treatment.
A phase 3, randomized, double-blind, parallel-group study to evaluate tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for F508del-CFTR and a gating mutation.
Impact of biofilm formation and azoles’ susceptibility in Scedosporium/Lomentospora species using an in vitro model that mimics the cystic fibrosis patients’ airway environment.
Human Papilloma Virus Vaccination Among Female Patients Attending French Paediatric Cystic Fibrosis Centres.
Can we Train the Immune System of Patients With Cystic Fibrosis?
Microbiome data enhances predictive models of lung function in people with cystic fibrosis.
[Rare diseases on the agenda for innovation in health: progress and challenges with cystic fibrosis].
Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
Respiratory muscle training for cystic fibrosis.
Glucose tolerance stages in Cystic Fibrosis are idenfied by a unique pattern of defects of Beta-cell function.
Sensitization to A.fumigatus in subjects with non-cystic fibrosis bronchiectasis.
Office-Based Sinus Surgery for Cystic Fibrosis Chronic Rhinosinusitis.
The challenge of medication acquisition in cystic fibrosis; cracking the nut with pharmacy technicians.
Adenocarcinoma arising in the multiple heterotopic submucosal glands of the intestine in a Satoyoshi syndrome patient: A case report.
Current Advances in Burkholderia Vaccines Development.
CRMS/CFSPID Subjects Carrying D1152H CFTR Variant: Can the Second Variant Be a Predictor of Disease Development?
Environmental risks of Pseudomonas aeruginosa-What to advise patients and parents.
Mechanisms and Control Strategies of Antibiotic Resistance in Pathological Biofilms.
[Evaluation and perspective of 20 years of neonatal screening in Galicia. Program results.]
[Results of the neonatal screening on Western Andalusia after a decade of experience.]
[Response of the Catalonia neonatal screening laboratory to the pandemic of SARS-CoV-2.]
[Impact of the inclusion of second-tier tests in the newborn screening program of Catalonia and in other international programs.]
[50 years of the Neonatal Screening Program in Catalonia.]
Healthcare Resource Utilization, Exacerbations, and Readmissions Among Medicare Patients with Chronic Obstructive Pulmonary Disease After Long-Acting Muscarinic Antagonist Therapy Initiation with Soft Mist versus Dry Powder Inhalers.
ELISA Test for the Serological Detection of Scedosporium/Lomentospora in Cystic Fibrosis Patients.
Cystic Fibrosis Point of Personalized Detection (CFPOPD): An Interactive Web Application.
Safety of N-Acetylcysteine at High Doses in Chronic Respiratory Diseases: A Review.
Antimicrobial Susceptibility Testing in Pseudomonas aeruginosa Biofilms: One Step Closer to a Standardized Method.
Pseudomonas aeruginosa Susceptibility Patterns and Associated Clinical Outcomes in People with Cystic Fibrosis following Approval of Aztreonam Lysine for Inhalation.
Burkholderia ubonensis high-level tetracycline resistance is due to efflux pump synergy involving a novel TetA(64) resistance determinant.
In vitro antibacterial activity and in vivo efficacy of sulbactam-durlobactam against pathogenic Burkholderia species.
Human extrahepatic and intrahepatic cholangiocyte organoids show region-specific differentiation potential and model cystic fibrosis-related bile duct disease.
The first report of Methicillin-resistant Staphylococcus aureus (MRSA) in cystic fibrosis (CF) patients in Saudi Arabia.
The human respiratory tract microbial community structures in healthy and cystic fibrosis infants.
Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia.
VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.
Development of a device to measure adherence and pressure characteristics of positive expiratory pressure therapies used by adults with cystic fibrosis.
Autosomal dominant polycystic kidney disease and pioglitazone for its therapy: a comprehensive review with an emphasis on the molecular pathogenesis and pharmacological aspects.
Wheeze and cough measurements at night in children with respiratory symptoms.
Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study.
Amphotericin B induces epithelial voltage responses in people with cystic fibrosis.
[AFU and SALF recommendations for the evaluation of male infertility].
Pseudomonas aeruginosa by Pseudomonas aeruginosa.
Processing of positive newborn screening results: a qualitative exploration of current practice in England.
‘Triple therapy’ (elexacaftor, tezacaftor, ivacaftor) skin rash in patients with cystic fibrosis.
Review of Rapid Advances in Cystic Fibrosis.
The regulatory role of vasoactive intestinal peptide in lacrimal gland ductal fluid secretion: A new piece of the puzzle in tear production.
Bronchial Artery Embolization, an Increasingly Used Method for Hemoptysis; Treatment and Avoidance.
Proximal intestinal obstruction syndrome (PIOS) in a patient with cystic fibrosis: A case report.
Parasexual recombination enables Aspergillus fumigatus to persist in cystic fibrosis.
Preclinical evaluation of the epithelial sodium channel inhibitor BI 1265162 for treatment of cystic fibrosis.
An innovative phase II trial to establish proof of efficacy and optimal dose of a new inhaled epithelial sodium channel inhibitor BI 1265162 in adults and adolescents with cystic fibrosis: BALANCE-CFTM 1.
Cefiderocol for the Treatment of Adult and Pediatric Patients with Cystic Fibrosis and Achromobacter xylosoxidans Infections.
Cyclic Peptidyl Inhibitors against CAL/CFTR Interaction for Treatment of Cystic Fibrosis.
Spellchecking for the Story of Life With CRISPR-Cas9 and Base, Prime Editors.
Optimization of Aztreonam in Combination with Ceftazidime/Avibactam in a Cystic Fibrosis Patient with Chronic Stenotrophomonas maltophilia Pneumonia Using Therapeutic Drug Monitoring: A Case Study.

Bacterial persisters in long-term infection: Emergence and fitness in a complex host environment.
Mepolizumab use in cystic fibrosis-associated allergic bronchopulmonary aspergillosis.
The impact of liver disease on mortality in cystic fibrosis – a systematic review protocol.
Organoids for personalized treatment of Cystic Fibrosis: Professional perspectives on the ethics and governance of organoid biobanking.
Targeting Cystic Fibrosis Inflammation in the Age of CFTR Modulators: Focus on Macrophages.
Rescue of multiple class II CFTR mutations by elexacaftor+ tezacaftor+ivacaftor mediated in part by the dual activities of Elexacaftor as both corrector and potentiator.
Free-breathing MRI for Monitoring Ventilation Changes following Antibiotic Treatment of Pulmonary Exacerbations in Pediatric Cystic Fibrosis.
IL-17A from innate and adaptive lymphocytes contributes to inflammation and damage in cystic fibrosis lung disease.
(R)-Roscovitine and CFTR modulators enhance killing of multi-drug resistant Burkholderia cenocepacia by cystic fibrosis macrophages.
Reciprocal Links Between Physical Health and Coping Among Adolescents With Cystic Fibrosis.
Inhaled tobramycin for chronic infection with pseudomonas aeruginosa in non-cystic fibrosis bronchiectasis: A systematic review and meta-analysis.
Airway Redox Homeostasis and Inflammation Gone Awry: From Molecular Pathogenesis to Emerging Therapeutics in Respiratory Pathology.
Comparative evaluation of the effect of different growth media on in vitro sensitivity to azithromycin in multi-drug resistant Pseudomonas aeruginosa isolated from cystic fibrosis patients.
Phages as immunomodulators and their promising use as anti-inflammatory agents in a cftr loss-of-function zebrafish model.
Lung transplant referrals for individuals with cystic fibrosis: a pediatric perspective on the Cystic Fibrosis Foundation consensus guidelines.
Cystic Fibrosis F508del Carriers and Cancer Risk: Results from the UK Biobank.
Longitudinal Assessment of Patients With Cystic Fibrosis Lung Disease With Multivolume Noncontrast MRI and Spirometry.
Growth, Body Composition, and Lung Function in Prepubertal Children with Cystic Fibrosis Diagnosed by Newborn Screening.
Trends in the Prevalence of Non-tuberculous Mycobacterial Infection in Patients with Non-Cystic Fibrosis Bronchiectasis in South Korea, 2012-2016.
Chronic rhinosinusitis and endoscopic sinus surgery in children admitted for pulmonary exacerbations of cystic fibrosis.
A Cationic Porphyrin, ZnPor, Disassembles Pseudomonas aeruginosa Biofilm Matrix, Kills Cells Directly, and Enhances Antibiotic Activity of Tobramycin.
Understanding Pseudomonas aeruginosa-Host Interactions: The Ongoing Quest for an Efficacious Vaccine.
Triage for Malnutrition Risk among Pediatric and Adolescent Outpatients with Cystic Fibrosis, Using a Disease-Specific Tool.
Essential Oils Biofilm Modulation Activity, Chemical and Machine Learning Analysis. Application on Staphylococcus aureus Isolates from Cystic Fibrosis Patients.
New Insights into the Binding Features of F508del CFTR Potentiators: A Molecular Docking, Pharmacophore Mapping and QSAR Analysis Approach.
Exogenous Nitric Oxide Improves Antibiotic Susceptibility in Resistant Bacteria.
Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251).
Comparative transcriptome analysis unveils the adaptative mechanisms of Scedosporium apiospermum to the microenvironment encountered in the lungs of patients with cystic fibrosis.
Monogenic Diabetes in a Child with Cystic Fibrosis: A Case Report and Review of the Literature.
Effectiveness of Hypertonic Saline Nebulization in Airway Clearance in Children with Non-Cystic Fibrosis Bronchiectasis: A randomized control trial.
Mental Health History and Social Barriers Impacting Caregivers of Infants with Cystic Fibrosis.
carP, encoding a Ca2+-regulated putative phytase, is evolutionarily conserved in Pseudomonas aeruginosa and has potential as a biomarker.
Clinical and molecular characterisation of the R751L-CFTR mutation.
Polyclonality, Shared Strains, and Convergent Evolution in Chronic CF S. aureus Airway Infection.
Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis.
Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19.
Development of a penem antibiotic against Mycobacteroides abscessus.
Evaluation of Rescue Oral Glucocorticoid Therapy during Inpatient Cystic Fibrosis Exacerbations.
Novel Odoribacter splanchnicus Strain and Its Outer Membrane Vesicles Exert Immunoregulatory Effects in vitro.
Novel therapeutic approaches for the management of cystic fibrosis.
Vasculitis in Cystic Fibrosis.
Steps Ahead: optimising physical activity and health in people with cystic fibrosis: Study Protocol for a pilot randomised trial.
Subacute cadmium exposure disrupts the hypothalamic-pituitary-gonadal axis, leading to polycystic ovarian syndrome and premature ovarian failure features in female rats.
A current review of the safety of cystic fibrosis transmembrane conductance regulator modulators.
siRNA delivery to macrophages using aspherical, nanostructured microparticles as delivery system for pulmonary administration.
The effect of self-efficacy, social support and quality of life on readiness for transition to adult care among adolescents with cystic fibrosis in Turkey.
ACT with CF: A telehealth and in-person feasibility study to address anxiety and depressive symptoms among people with cystic fibrosis.
Clinical evaluation of an evidence-based method based on food characteristics to adjust pancreatic enzyme supplements dose in cystic fibrosis.
CFTR plays an important role in the regulation of vascular resistance and high-fructose/salt-diet induced hypertension in mice.
Rescue from Pseudomonas aeruginosa airway infection via stem cell transplantation.
YKL-40 as a clinical biomarker in adult patients with CF: Implications of a CHI3L1 single nucleotide polymorphism in disease severity.
Can we identify cystic fibrosis (CF) from skeletal remains?: A proposed differential diagnosis.
G970R-CFTR Mutation (c.2908G>C) Results Predominantly in a Splicing Defect.
Therapeutic effect of nebulized hypertonic saline for muco-obstructive lung diseases: a systematic review and meta-analysis with trial sequential analysis.
Heme protects Pseudomonas aeruginosa and Staphylococcus aureus from calprotectin-induced iron starvation.
Lung Function Assessment by Impulse Oscillometry in Adults.
Should the dosage of propofol be higher and independent of immunosuppressive therapy in adult cystic fibrosis patients undergoing sedation during flexible video bronchoscopy?
Novel Antioxidant Therapy with the Immediate Precursor to Glutathione, γ-Glutamylcysteine (GGC), Ameliorates LPS-Induced Cellular Stress in In Vitro 3D-Differentiated Airway Model from Primary Cystic Fibrosis Human Bronchial Cells.
Early disease surveillance in young children with cystic fibrosis: A qualitative analysis of parent experiences.
Topographic heterogeneity of lung microbiota in end-stage idiopathic pulmonary fibrosis: the Microbiome in Lung Explants-2 (MiLEs-2) study.
Impaired renal HCO3 – secretion in CFTR deficient mice causes metabolic alkalosis during chronic base-loading.
Modelling experimentally measured of ciprofloxacin antibiotic diffusion in Pseudomonas aeruginosa biofilm formed in artificial sputum medium.
Extensive CFTR Gene Analysis Revealed a Higher Occurrence of Cystic Fibrosis Transmembrane Regulator-Related Disorders (CFTR-RD) among CF Carriers.
Ecological Succession of Polymicrobial Communities in the Cystic Fibrosis Airways.
Multicentre feasibility of multiple-breath washout in preschool children with cystic fibrosis and other lung diseases.
The Lung Microbiome of Three Young Brazilian Patients With Cystic Fibrosis Colonized by Fungi.
Immune defects in patients with pulmonary Mycobacterium abscessus disease without cystic fibrosis.
Non-invasive Ventilation for Children With Chronic Lung Disease.
Sleep-Disordered Breathing in Cystic Fibrosis.
COVID-19 in children with underlying chronic respiratory diseases: survey results from 174 centres.
Early COVID-19 infection after lung transplantation in a patient with cystic fibrosis.
Pseudomonas aeruginosa in the CF Airway: Does it Deserve its Reputation as a Predatory ‘Bully’?
Polysulfated Hyaluronan GM-1111 Inhibits Elastase and Improves Rheology in CF Sputum.
Heterogeneous expression of CFTR in insulin-secreting β-cells of the normal human islet.
Impact of Pseudomonas aeruginosa Infection on Patients with Chronic Inflammatory Airway Diseases.
Local and Systemic Alterations of the L-Arginine/Nitric Oxide Pathway in Sputum, Blood, and Urine of Pediatric Cystic Fibrosis Patients and Effects of Antibiotic Treatment.
Molecular Characterization of the Burkholderia cenocepacia dcw Operon and FtsZ Interactors as New Targets for Novel Antimicrobial Design.
Azithromycin potentiates avian IgY effect against Pseudomonas aeruginosa in a murine pulmonary infection model.
Right Ventricle Dysfunction in Patients With Adult Cystic Fibrosis Enlisted for Lung Transplant.
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).
IgA autoantibodies directed against self DNA are elevated in cystic fibrosis and associated with more severe lung dysfunction.
The Association of Area Deprivation and State Child Health with Respiratory Outcomes of Pediatric Patients with Cystic Fibrosis in the United States.
Stenotrophomonas maltophilia phenotypic and genotypic features through 4-year cystic fibrosis lung colonization.
The Effects of Ivacaftor on Bone Density and Microarchitecture in Children and Adults with Cystic Fibrosis.
PROBIOTICS FOR PEOPLE WITH CYSTIC FIBROSIS (REVIEW).
Repeated isolation of an antibiotic-dependent and temperature-sensitive mutant of Pseudomonas aeruginosa from a cystic fibrosis patient.
Extraction of the molecular level biomedical event trigger based on gene ontology using radial belief neural network techniques.
PEGylation of recombinant human deoxyribonuclease I deceases its transport across lung epithelial cells and uptake by macrophages.
A Scientific Rationale for Using Cystic Fibrosis Transmembrane Conductance Regulator Therapeutics in COVID-19 Patients.
Physiotherapy: At what cost? Parents experience of performing chest physiotherapy for infants with cystic fibrosis.
Development of elexacaftor – tezacaftor – ivacaftor: highly effective CFTR modulation for the majority of people with cystic fibrosis.
The cystic fibrosis gut as a potential source of multidrug resistant pathogens.
Prostaglandin E2 and phagocytosis of inhaled particulate matter by airway macrophages in cystic fibrosis.
Peptidorhamnomannans From Scedosporium and Lomentospora Species Display Microbicidal Activity Against Bacteria Commonly Present in Cystic Fibrosis Patients.
Gene therapy for sickle cell disease.
Trypsin Detection Strategies: A Review.
Effect of host-mimicking medium and biofilm growth on the ability of colistin to kill Pseudomonas aeruginosa.
Mining GWAS and eQTL data for CF lung disease modifiers by gene expression imputation.
Caring through distancing: Spatial boundaries and proximities in the cystic fibrosis clinic.
Lumacaftor/ivacaftor in people with cystic fibrosis with an A455E-CFTR mutation.

Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.
“Adenosine an old player with new possibilities in kidney diseases”: Preclinical evidences and clinical perspectives.
Effects of chlorothalonil, prochloraz and the combination on intestinal barrier function and glucolipid metabolism in the liver of mice.
Long-term azithromycin use is not associated with QT prolongation in children with cystic fibrosis.
Flow Volume Loop and Regional Ventilation Assessment Using Phase-Resolved Functional Lung (PREFUL) MRI: Comparison With 129 Xenon Ventilation MRI and Lung Function Testing.
Chlamydia pneumoniae and Mycoplasma pneumoniae in children with Cystic Fibrosis: impact on bacterial respiratory microbiota diversity.
Delayed age at menarche in chronic respiratory diseases.
Focus on gastroesophageal reflux disease in patients with cystic fibrosis.
The Changing Face of Cystic Fibrosis and Its Implications for Screening.
Why Do We Screen Newborn Infants for Cystic Fibrosis?
Newborn Screening for Cystic Fibrosis: Over the Hump, Still Need to Fine-Tune It.
Differential effects of cotreatment of the antibiotic rifampin with host-directed therapeutics in reducing intracellular Staphylococcus aureus infection.
Mycobacterium Simiae Infection in a Person With Cystic Fibrosis.
“Molecular Mechanisms for the Inflammation-Resolving Actions of Lenabasum”.
Pseudomonas Quinolone Signal-Induced Outer Membrane Vesicles Enhance Biofilm Dispersion in Pseudomonas aeruginosa.
Integrative genomic meta-analysis reveals novel molecular insights into cystic fibrosis and ΔF508-CFTR rescue.
Lung Clearance Index in Children with Sickle Cell Disease.
Aerosolized drug-loaded nanoparticles targeting migration inhibitory factors inhibit Pseudomonas aeruginosa-induced inflammation and biofilm formation.
Technology-Enabled Health Care Collaboration in Pediatric Chronic Illness: Pre-Post Interventional Study for Feasibility, Acceptability, and Clinical Impact of an Electronic Health Record-Linked Platform for Patient-Clinician Partnership.
Variations in Nutrition Practices in Cystic Fibrosis: A Survey of the DIGEST Program.
Subtle relationships between Pseudomonas aeruginosa and fungi in patients with cystic fibrosis.
Riociguat: clinical research and evolving role in therapy.
Emerging Alternatives to Conventional Clinic Visits in the Era of COVID-19: Adoption of Telehealth at VCU Adult Cystic Fibrosis Center.
Letter to the editor of JCF.
Allergic bronchopulmonary aspergillosis in children.
Physical Activity, Sedentary Time and Sleep in Cystic Fibrosis Youth: A Bi-Directional Relationship?
Phospholipase C controls chloride-dependent short-circuit current in human bronchial epithelial cells.
Inhibition of the GDP-d-Mannose Dehydrogenase from Pseudomonas aeruginosa Using Targeted Sugar Nucleotide Probes.
CRISPR-Cas9: A Preclinical and Clinical Perspective for the Treatment of Human Diseases.
Comparison Between Ultra-low-dose Computed Tomography and Lung MRI in Cystic Fibrosis
Proteomic Studies of the Biofilm Matrix including Outer Membrane Vesicles of Burkholderia multivorans C1576, a Strain of Clinical Importance for Cystic Fibrosis.
Pulmonary Manifestations of Genetic Disorders in Children.
[Bronchiectasis not related to cystic fibrosis in children: Guidelines for diagnosis, monitoring and treatment].
Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene.
Animal and Cell Culture Models for Cystic Fibrosis: Which Model is Right for Your Application?
Decoding (patho-)physiology of the lung by advanced in vitro models for developing novel anti-infectives therapies.
Effects of rehabilitation methods on lower-limb muscle function and functional performance in patients with cystic fibrosis: A systematic review.
Urban Life as Risk Factor for Aspergillosis.
Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with cystic fibrosis and meconium peritonitis in a female neonate 4 days of age – case report and review of the literature.
Re: The outcome of pregnancy in women with cystic fibrosis: a UK population-based descriptive study.
Role Of Long-Term Intermittent Use Of Oral Azithromycin On Pulmonary Exacerbations In Cystic Fibrosis Children.
Cystic Fibrosis with Non-G551D Gating Mutations in Italy: Epidemiology and clinical characteristics.
Draft Genome Sequence of Mycobacteroides sp. Strain LB1, Isolated from the Sputum of a Cystic Fibrosis Patient.
Evaluation of Inhaled Tobramycin in Early Eradication of Pseudomonas aeruginosa in Infants With Cystic Fibrosis.
Management of Cepacia Syndrome With a Combination of Intravenous and Inhaled Antimicrobials in a Non-Cystic Fibrosis Pediatric Patient.
Caroli’s Syndrome: An Early Presentation.
The pharmacokinetics of antibiotics in cystic fibrosis.
Recommendations to improve physical activity prescription for the cystic fibrosis population: an Irish perspective.
Small molecule drugs in cystic fibrosis.
Pseudomonas aeruginosa adaptation and evolution in patients with cystic fibrosis.
Prevalence of coccidioidomycosis in cystic fibrosis patients residing in Southern Arizona.
Effectiveness of Short versus Long Course Perioperative Antibiotics in Lung Transplant Recipients with Donor Positive Respiratory Cultures.
Antibacterial Effects of Bicarbonate in Media Modified to Mimic Cystic Fibrosis Sputum.
Defective immunometabolism pathways in cystic fibrosis macrophages.
Engineered mutant α-ENaC subunit mRNA delivered by lipid nanoparticles reduces amiloride currents in cystic fibrosis-based cell and mice models.
Coinfection with Pseudomonas aeruginosa and Aspergillus fumigatus in cystic fibrosis.
Repeat Dosing of AAV2.5T to Ferret Lungs Elicits an Antibody Response That Diminishes Transduction in an Age-Dependent Manner.
Cystic fibrosis year in review 2019: Section 3 multisystem-based care and research.
Current Treatment Options for Cystic Fibrosis-Related Liver Disease.
Estimation of Proinflammatory Factors in the Saliva of Adult Patients with Cystic Fibrosis and Dental Caries.
NMR Profiling of Exhaled Breath Condensate Defines Different Metabolic Phenotypes of Non-Cystic Fibrosis Bronchiectasis.
Crystal Structure of Isoform CBd of the Basic Phospholipase A2 Subunit of Crotoxin: Description of the Structural Framework of CB for Interaction with Protein Targets.
Host-Pathogen Interactions after Lung Transplant: Are Cystic Fibrosis Patients Unique?
Age-Related Changes in the Canine Aorta.
Oxidative stress-induced inflammation in susceptible airways by anthropogenic aerosol.
Evaluation of the health-related quality of life in Turkish cystic fibrosis patients.
Non-Linear Pharmacokinetics of Oral Roscovitine (Seliciclib) in Cystic Fibrosis Patients Chronically Infected with Pseudomonas aeruginosa: A Study on Population Pharmacokinetics with Monte Carlo Simulations.
Rapidly progressive metastatic cholangiocarcinoma in a postpartum patient with cystic fibrosis: a case report.
Ultra-low-dose chest CT in adult patients with cystic fibrosis using a third-generation dual-source CT scanner.
ADIPS 2020 guideline for pre-existing diabetes and pregnancy.
[Evidence-based treatment of cystic fibrosis].
Vitamin D Deficiency Is Associated with Increased Nontuberculous Mycobacteria Risk in Cystic Fibrosis.
Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes.
How to determine the mechanism of action of CFTR modulator compounds: A gateway to theranostics.
Outcomes and endpoints reported in studies of pulmonary exacerbations in people with cystic fibrosis: A systematic review.
Electronic health nudges to improve reproductive health care for women with cystic fibrosis.
Evaluation of Pyocyanin induced systemic pathogenicity of Pseudomonas aeruginosa.
Utilisation, expenditure and cost-effectiveness of cystic fibrosis drugs in Ireland: a retrospective analysis of a national pharmacy claims database.
A SNARE protein Syntaxin 17 captures CFTR to potentiate autophagosomal clearance under stress.
Impact of a Gap Junction Protein Alpha 4 Variant on Clinical Disease Phenotype in F508del Homozygous Patients With Cystic Fibrosis.
Evaluation of quorum-sensing inhibitory effects of extracts of three traditional medicine plants with known antibacterial properties.
Synthesis of the Hydroxamate Siderophore N α-Methylcoprogen B in Scedosporium apiospermum Is Mediated by sidD Ortholog and Is Required for Virulence.
Current Approach in the Diagnosis and Management of Allergic Bronchopulmonary Aspergillosis in Children With Cystic Fibrosis.
The Open Challenge of in vitro Modeling Complex and Multi-Microbial Communities in Three-Dimensional Niches.
Dehydrated patient without clinically evident cause: A case report.
Cystic Fibrosis Diagnosed Using Indigenously Wrapped Sweating Technique: First Large-Scale Study Reporting Socio-Demographic, Clinical, and Laboratory Features among the Children in Bangladesh A Lower Middle Income Country.
Tackling Chronic Inflammation with Withanolide Phytochemicals-A Withaferin a Perspective.
First Wave of COVID-19 in French Patients with Cystic Fibrosis.
Evaluation of pancreatic functions in cases of primary and secondary malnutrition.
Telephone surveillance during 2019 novel coronavirus disease: Is it a helpful diagnostic tool for detecting acute pulmonary exacerbations in children with chronic lung disease?
Addressing nutritional failure in a cystic fibrosis patient using nutritional and psychological intervention.
The global impact of SARS-CoV-2 in 181 people with cystic fibrosis.
Transcriptomic analysis of CFTR-impaired endothelial cells reveals a pro-inflammatory phenotype.
Draft Genome Sequence of Pseudomonas aeruginosa Strain PA14-UM.
Distribution of Burkholderia cepacia complex species isolated from industrial processes and contaminated products in Argentina.
Molecular mechanisms of Cl- transport in fishes: New insights and their evolutionary context.
Transitioning from paediatric to adult services with cystic fibrosis or bronchiectasis: What is the impact on engagement and health outcomes?
Protease FRET Reporters Targeting Neutrophil Extracellular Traps.
An ex vivo cystic fibrosis model recapitulates key clinical aspects of chronic Staphylococcus aureus infection.
Airway epithelial stem cell chimerism in cystic fibrosis lung transplant recipients.

Osteoclastogenesis and sphingosine-1-phosphate secretion from human osteoclast precursor monocytes are modulated by the cystic fibrosis transmembrane conductance regulator.
Atypical Cystic Fibrosis: Diagnosis at the Age of 57 Years.
Multilevel Models of Therapeutic Response in the Lungs
Risk factors and clinical course of children with cystic fibrosis colonized with Staphylococcus aureus.
Insights for the Growth of Stenotrophomonas maltophilia in a Diabetic Patient with Long-Term Antibiotic Use: A Case Study.
Linaclotide, lubiprostone and tenapanor improve gut fluidity and alkalinity in CFTR-deficient and F508del mutant mice via NHE3 inhibition.
Impaired Ratio of Unsaturated to Saturated Non-Esterified Fatty Acids in Saliva from Patients with Cystic Fibrosis.
Bile Acid Signal Molecules Associate Temporally with Respiratory Inflammation and Microbiome Signatures in Clinically Stable Cystic Fibrosis Patients.
Factors influencing the acquisition and eradication of early Pseudomonas aeruginosa infection in cystic fibrosis.
Targeted Nanotherapeutics for Respiratory Diseases: Cancer, Fibrosis, and Coronavirus.
Severe pulmonary alveolar proteinosis with respiratory failure treated by intrapulmonary percussive ventilation.
Research advances in molecular mechanisms underlying the pathogenesis of cystic fibrosis: From technical improvement to clinical applications (Review).
Burkholderia gladioli infection in a pediatric patient with cystic fibrosis: the clinical challenges of an emergent pathogen.
P.F508del editing in cells from cystic fibrosis patients.
Mining Public Mass Spectrometry Data to Characterize the Diversity and Ubiquity of P. aeruginosa Specialized Metabolites.
Preclinical Studies of a Rare CF-Causing Mutation in the Second Nucleotide Binding Domain (c.3700A>G) Show Robust Functional Rescue in Primary Nasal Cultures by Novel CFTR Modulators.
Therapeutic potential for coxibs-nitric oxide releasing hybrids in cystic fibrosis.
Successful biodegradable stent insertion in an infant with severe bronchomalacia and cystic fibrosis.
The virtual CF clinic: Implications for sputum microbiology.
SHORT-TERM EFFECTS OF APPROPRIATE EMPIRICAL ANTIMICROBIAL TREATMENT WITH CEFTOLOZANE/TAZOBACTAM IN A SWINE MODEL OF NOSOCOMIAL PNEUMONIA.
Targeted deubiquitination rescues distinct trafficking-deficient ion channelopathies.
Longitudinal changes in exercise capacity among adult cystic fibrosis patients.
Overview of Atypical Diabetes.
The efficacy of Elexacaftor/Tezacaftor/Ivacaftor in patients with cystic fibrosis and advanced lung disease.
Burkholderia vietnamiensis causing a non-lactational breast abscess in a non-cystic fibrosis patient in Tamil Nadu, India.
Repeatability of the 6-min walk test in non-cystic fibrosis bronchiectasis.
Autologous fibrin sealant co-delivered with antibiotics is a robust method for topical antibiotic treatment after sinus surgery.
A systematic review of self-care measures for adolescents with health needs.
Fluoroquinolone resistance in Achromobacter spp.: substitutions in QRDRs of GyrA, GyrB, ParC and ParE and implication of the RND efflux system AxyEF-OprN.
Citrullination as a novel posttranslational modification of matrix metalloproteinases.
Photodynamic therapy to control microbial biofilms.
Sub-nanoliter metabolomics via mass spectrometry to characterize volume-limited samples.
“Summer hypokalemia” as an initial presentation of cystic fibrosis in a morbidly obese African American adult: case report.
Extracellular adenosine 5′-triphosphate in pulmonary disorders.
Early acquisition and conversion of Pseudomonas aeruginosa in Hispanic youth with cystic fibrosis in the United States.
Gaming Console Home-Based Exercise for Adults with Cystic Fibrosis: Study Protocol.
Hyperpolarized 129Xe MR Imaging of Lung Function in Healthy Volunteers and Subjects With Pulmonary Disease
DPI-Tobra-Kind Cyclops® in Children With Cystic Fibrosis
New Insights in Laboratory Testing for COVID-19 Patients: Looking for the Role and Predictive Value of Human epididymis secretory protein 4 (HE4) and the Innate Immunity of the Oral Cavity and Respiratory Tract.
Risk factors for respiratory Aspergillus fumigatus in German Cystic Fibrosis patients and impact on lung function.
Cell-based non-invasive prenatal diagnosis in a pregnancy at risk of cystic fibrosis.
Multi-stimuli controlled release of a transmembrane chloride ion carrier from a sulfonium-linked procarrier.
[Myelinosomes: A new pathway of protein quality control].
Lipoxin A4 promotes reduction and antibiotic efficacy against Pseudomonas aeruginosa biofilm.
In vitro activity of ceftolozane-tazobactam and ceftazidime-avibactam against Pseudomonas aeruginosa isolated from patients with cystic fibrosis.
Copper and Copper/Zinc Ratio in a Series of Cystic Fibrosis Patients.
Classical Drug Digitoxin Inhibits Influenza Cytokine Storm, With Implications for Covid-19 Therapy.
Molecular Docking and QSAR Studies as Computational Tools Exploring the Rescue Ability of F508del CFTR Correctors.
NGS-based expanded carrier screening for genetic disorders in North Indian population reveals unexpected results – a pilot study.
LC-MS detection of antibiotic agents in sputum from persons with cystic fibrosis.
Acid ceramidase rescues cystic fibrosis mice from pulmonary infections.
Sputum microbiota in adults with CF associates with response to inhaled tobramycin.
Machine learning evaluates improvement in sinus computed tomography opacification with CFTR modulator therapy.
Chest computed tomography outcomes in a randomized clinical trial in cystic fibrosis: Lessons learned from the first ataluren phase 3 study.
CF Bronchodilation
Effects of AD on VI in Patients With CF
Organoid based personalized medicine: from bench to bedside.
Factors influencing medical practitioner participation in population carrier screening for cystic fibrosis.
Cystic Fibrosis Lung Function Decline after Within-Host Evolution Increases Virulence of Infecting Pseudomonas Aeruginosa.
New strategies of physical activity assessment in cystic fibrosis: a pilot study.
Treatment of human airway epithelial Calu-3 cells with a peptide-nucleic acid (PNA) targeting the microRNA miR-101-3p is associated with increased expression of the cystic fibrosis Transmembrane Conductance Regulator () gene.
Cystic fibrosis: physiopathology and the latest pharmacological treatments.
Use of dornase alfa in the paediatric intensive care unit: current literature and a national cross-sectional survey.
Upregulation of CFTR Protects against Palmitate-Induced Endothelial Dysfunction by Enhancing Autophagic Flux.
Modulated Response of Aspergillus fumigatus and Stenotrophomonas maltophilia to Antimicrobial Agents in Polymicrobial Biofilm.
Stem Cells, Cell Therapies, and Bioengineering in Lung Biology and Disease 2019.
Inhaled dry powder alginate oligosaccharide in cystic fibrosis: a randomised, double-blind, placebo-controlled, crossover phase 2b study.
The Changing Face of Cystic Fibrosis and Its Implications for Screening.
Why Do We Screen Newborn Infants for Cystic Fibrosis?
Evaluation of Autonomic Modulation of Lung Function and Heart Rate in Children with Cystic Fibrosis.
Effects of music therapy as an adjunct to chest physiotherapy in children with cystic fibrosis: A randomized controlled trial.
Mitigating the Impact of Socioeconomic Status on Listing for Lung Transplantation in Cystic Fibrosis.
STeP IT UP CF: STimulating ImProved Health And Well-being In CysTic Fibrosis
New Antibiotics for the Treatment of Acute Bacterial Skin and Soft Tissue Infections in Pediatrics.
Pulmonary Delivery of Biological Drugs.
Potential Contributions of Anaerobes in Cystic Fibrosis Airways.
Efficacy and Safety of the CFTR Potentiator Icenticaftor (QBW251) in COPD: Results from a Phase 2 Randomized Trial.
Pseudomonas aeruginosa Type III Secretion System Virulotypes and Their Association with Clinical Features of Cystic Fibrosis Patients.
Liposomes Loaded With Phosphatidylinositol 5-Phosphate Improve the Antimicrobial Response to Pseudomonas aeruginosa in Impaired Macrophages From Cystic Fibrosis Patients and Limit Airway Inflammatory Response.
Ocular Surface Potential Difference Measured in Human Subjects to Study Ocular Surface Ion Transport.
Primary lung carcinoma in cystic fibrosis: A case report and literature review.
Pneumocystis jirovecii in Patients With Cystic Fibrosis: A Review.
The Clinicopathological and Imaging Characteristics of Primary Hepatic Carcinosarcoma and a Review of the Literature.
Newborn Screening for Cystic Fibrosis: Over the Hump, Still Need to Fine-Tune It.
EX18_21 large duplication: A novel pathogenic mutation of the CFTR gene for cystic fibrosis.
Pseudoxanthomonas winnipegensis sp. nov., derived from human clinical materials and recovered from cystic fibrosis and other patient types in Canada, and emendation of Pseudoxanthomonas spadix Young et al. 2007.
Oxygen transport and utilisation during exercise in cystic fibrosis: Contributors to exercise intolerance.
Are patients with lung cystic fibrosis at increased risk of severe and fatal COVID-19? Interleukin 6 as a predictor of COVID-19 outcomes.
Are patients with lung cystic fibrosis at increased risk of severe and fatal COVID-19? Interleukin 6 as a predictor of COVID-19 outcomes. Authors’ reply.
Treating systemic issues in families affected by cystic fibrosis: A solution-focused approach.
Streamlined Treatment of Pulmonary Exacerbations in Pediatrics (Pilot)
Use of medical marijuana in cystic fibrosis patients.
Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids.
Feasibility study for supporting medication adherence for adults with cystic fibrosis: mixed-methods process evaluation.
Gene Loss and Acquisition in Lineages of Pseudomonas aeruginosa Evolving in Cystic Fibrosis Patient Airways.
The first five-year evaluation of cystic fibrosis neonatal screening program in São Paulo State, Brazil.
Clinical impact of respiratory virus in pulmonary exacerbations of children with Cystic Fibrosis.
Getting Near to “Closing the gap” in the Pediatric Age-group for First Personalized Treatment of Cystic Fibrosis.
Strategies for cystic fibrosis transmembrane conductance regulator inhibition: from molecular mechanisms to treatment for secretory diarrhoeas.
Implementation of a Non-invasive Version of the Imaging β-adrenergic-dependent Sweat Secretion Test

New Markers of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function in Sweat
Efficacy of a New Sweat Collection Support: Impact on Neonatal Screening and Early Treatment of Cystic Fibrosis
Response of Pseudomonas aeruginosa to the innate immune system-derived oxidants hypochlorous acid and hypothiocyanous acid.
Mutant CFTR Drives TWIST1 mediated epithelial-mesenchymal transition.
How precision medicine paved the way to the first cystic fibrosis drug.
A Giant Thymic Cyst Accompanied by Acute Mediastinitis.
Learning to breathe with Tai Chi online – qualitative data from a randomized controlled feasibility study of patients with cystic fibrosis.
Sickle cell trait newborn screen results: disclosure and management.
Epidemiology, diagnosis & treatment of non-tuberculous mycobacterial diseases.
Nutrition and Markers of Disease Severity in Patients With Bronchiectasis.
Airway Mucins Inhibit Oxidative and Non-Oxidative Bacterial Killing by Human Neutrophils.
Mitochondrial Stress Responses and “Mito-Inflammation” in Cystic Fibrosis.
Temperate Bacteriophages (Prophages) in Pseudomonas aeruginosa Isolates Belonging to the International Cystic Fibrosis Clone (CC274).
Comparative Evolutionary Patterns of Burkholderia cenocepacia and B. multivorans During Chronic Co-infection of a Cystic Fibrosis Patient Lung.
Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis.
Implementing a tracking system for confirmatory diagnostic results after positive newborn screening for cystic fibrosis-implications for process quality and patient care.
Exercise Intolerance in Cystic Fibrosis: Importance of Skeletal Muscle.
Derivation of Airway Basal Stem Cells from Human Pluripotent Stem Cells.
The NHS virtual gym boosting fitness and social connections for cystic fibrosis patients.
Identification of AAV serotypes for lung gene therapy in human embryonic stem cell-derived lung organoids.
High-level antibiotic tolerance of a clinically-isolated Enterococcus faecalis strain.
Phenotypic and transcriptomic analysis of seven clinical Stenotrophomonas maltophilia isolates identify a small set of shared and commonly regulated genes involved in biofilm lifestyle.
The odorant receptor OR2W3 on airway smooth muscle evokes bronchodilation via a cooperative chemosensory tradeoff between TMEM16A and CFTR.
Managing Cystic Fibrosis in Polish Healthcare.
Pseudomonas aeruginosa isolates from cystic fibrosis patients induce neutrophil extracellular traps with different morphologies that could correlate with their disease severity.
Complete Genome Sequence of Burkholderia cenocepacia K56-2, an Opportunistic Pathogen.
Author Correction: Allele specific repair of splicing mutations in cystic fibrosis through AsCas12a genome editing.
1-BENZYLSPIRO[PIPERIDINE-4,1′-PYRIDO[3,4-b]indole] ‘co-potentiators’ for minimal function CFTR mutants.
Interdisciplinary Approach for the Medical Management of Gestational Gigantomastia.
Omics-based tracking of Pseudomonas aeruginosa persistence in “eradicated” CF patients.
Extended Screening for Cystic Fibrosis-related Liver Disease Including Elastography in Children and Adolescents.
Carbapenems drive the collateral resistance to ceftaroline in cystic fibrosis patients with MRSA.
Analysis of rearrangements of the CFTR gene in patients from Turkey with CFTR-related disorders: frequent exon 2 deletion.
TACKLING ANTIMICROBIAL RESISTANCE (AMR) – IN VITRO EFFECT OF SODIUM CHLORIDE ON ANTIBIOTIC SUSCEPTIBILITY IN CLINICAL PSEUDOMONAS AERUGINOSA ISOLATED FROM PATIENTS WITH CYSTIC FIBROSIS (CF).
In vitro assessment of CSA-131 and CSA-131 poloxamer form for the treatment of Stenotrophomonas maltophilia infections in cystic fibrosis.
Ivacaftor in People With Cystic Fibrosis and a 3849+10kb C →T or D1152H Residual Function Mutation.
Comparison between two newborn screening strategies for cystic fibrosis in Argentina: IRT/IRT vs. IRT/PAP.
miRNA repertoires of cystic fibrosis ex vivo models highlight miR-181a and miR-101 that regulate WISP1 expression.
Prevalence of CBAVD in azoospermic men carrying pathogenic CFTR mutations – evaluated in a cohort of 639 non-vasectomized azoospermic men.
Laparoscopic bile duct resection with lymph node dissection for gallbladder cancer diagnosed after laparoscopic cholecystectomy.
The German National Registry of Primary Immunodeficiencies (2012-2017).
Lipidome Alterations Induced by Cystic Fibrosis, CFTR Mutation, and Lung Function.
LocusFocus: Web-based colocalization for the annotation and functional follow-up of GWAS.
Biodistribution and elimination pathways of PEGylated recombinant human deoxyribonuclease I after pulmonary delivery in mice.
Ph 1/2 Study Evaluating Safety and Tolerability of Inhaled AP-PA02 in Subjects With Chronic Pseudomonas Aeruginosa Lung Infections and Cystic Fibrosis
A Study to Assess the Effect of ELX/TEZ/IVA on Glucose Tolerance in Participants With Cystic Fibrosis (CF)
Which score is better in association with exercise capacity and health status in non-cystic fibrosis bronchiectasis patients?
The evolution of virulence in Pseudomonas aeruginosa during chronic wound infection.
Protein kinase CK2 and ion channels (Review).
Evaluation of both exonic and intronic variants for effects on RNA splicing allows for accurate assessment of the effectiveness of precision therapies.
Glucocorticoids and serum- and glucocorticoid-inducible kinase 1 are potent regulators of CFTR in the native intestine: implications for stress-induced diarrhea.
Role of unfolded proteins in lung disease.
Long-term use of tube feeding in children with cystic fibrosis: results from two Belgian CF centers.
Utility of Mid-Upper Arm Circumference in Diagnosing Malnutrition in Children With Cystic Fibrosis.
Health and social effects of the COVID-19 pandemic on patients with cystic fibrosis.
Therapeutic Inhaled Sphingosine for Treating Lung Infection in a Mouse Model of Critical Illness.
Thymosin alpha 1 exerts beneficial extrapulmonary effects in cystic fibrosis.
Lipolytic enzymes inhibitors: A new way for antibacterial drugs discovery.
Does synbiotic supplementation affect the quality of life in children with cystic fibrosis? A pilot randomized controlled clinical trial.
Initial Evaluation of Prospective and Parallel Assessments of Cystic Fibrosis Newborn Screening Protocols in Eastern Andalusia: IRT/IRT versus IRT/PAP/IRT.
Newborn Screening: Current Status in Alberta, Canada.
History of Newborn Screening for Cystic Fibrosis-The Early Years.
Newborn Screening for CF across the Globe-Where Is It Worthwhile?
Inconclusive Diagnosis after Newborn Screening for Cystic Fibrosis.
The Role of Extended CFTR Gene Sequencing in Newborn Screening for Cystic Fibrosis.
Processing Newborn Bloodspot Screening Results for CF.
Psychological Impact of NBS for CF.
Pancreatitis-Associated Protein in Neonatal Screening for Cystic Fibrosis: Strengths and Weaknesses.
Newborn Screening for Cystic Fibrosis in Russia: A Catalyst for Improved Care.
Constructing a Bioethical Framework to Evaluate and Optimise Newborn Bloodspot Screening for Cystic Fibrosis.
Performance of a Three-Tier (IRT-DNA-IRT) Cystic Fibrosis Screening Algorithm in British Columbia.
It All Depends What You Count-The Importance of Definitions in Evaluation of CF Screening Performance.
Combination of Selective PARP3 and PARP16 Inhibitory Analogues of Latonduine A Corrects F508del-CFTR Trafficking.
The Balance between the Safety of Mother, Fetus, and Newborn Undergoing Cystic Fibrosis Transmembrane Conductance Regulator Treatments during Pregnancy.
Multiple Reaction Monitoring Mass Spectrometry for the Drug Monitoring of Ivacaftor, Tezacaftor, and Elexacaftor Treatment Response in Cystic Fibrosis: A High-Throughput Method.
Drug treatments for managing cystic fibrosis-related diabetes.
Characterization of Novel Lytic Bacteriophages of Achromobacter marplantensis Isolated from a Pneumonia Patient.
Serum-Based Proteomics Profiling in Adult Patients with Cystic Fibrosis.
Use of monoclonal antibodies for allergic bronchopulmonary aspergillosis in patients with asthma and cystic fibrosis: literature review.
Material Properties of Interfacial Films of Mucoid and Nonmucoid Pseudomonas aeruginosa Isolates.
Cystic Fibrosis: A Pocket Guide.
The Small RNA ErsA Plays a Role in the Regulatory Network of Pseudomonas aeruginosa Pathogenicity in Airway Infections.
COMPLEX ORPHAN PATHOLOGY: COMORBIDITY OF MUCOVISCIDOSIS AND CONGENITAL DYSFUNCTION OF ADRENAL GLANDS CORTEX (REFERENCES REVIEW AND OWN RESEARCH).
Biological applications of synthetic anion transporters.
Multiple breath washout quality control in the clinical setting.
[Probiotics: benefits and harms].
Nutritional Risk Index: A Predictive Metric for Mortality Following Lung Transplant.
Ambient air pollution and respiratory bacterial infections, a troubling association: epidemiology, underlying mechanisms, and future challenges.
Lung function over the life course of paediatric and adult patients with cystic fibrosis from a large multi-centre registry.
Early diagnosis effects the prognosis in children with atypical wheeze.
The Unique Challenges and Lessons Imparted by the Cystic Fibrosis Community in the Time of COVID-19 Pandemic.
Cystic fibrosis transmembrane conductance regulator (CFTR) regulates embryonic organizer formation during zebrafish early embryogenesis.
Patient and Family Participation in Clinical Guidelines Development: The Cystic Fibrosis Foundation Experience.
Modification of the Pseudomonas aeruginosa toxin 2-heptyl-1-hydroxyquinolin-4(1H)-one and other secondary metabolites by methyltransferases from mycobacteria.
Clinical evaluation of three chromogenic media for the isolation of Staphylococcus aureus in respiratory samples in patients with cystic fibrosis.
Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study.
Prevalence and Economic Burden of Respiratory Diseases in Central Asia and Russia: A Systematic Review.
Influence of Cystic Fibrosis-Related Diabetes on Mental Health in Adults: A Single-Center Study.
Communicating cystic fibrosis newborn screening results to parents.
Exploring the reproductive decision support needs and preferences of women with cystic fibrosis.

Impact of novel CFTR modulator on sinonasal quality of life in adult patients with cystic fibrosis.
Cystic fibrosis: On the cusp of cure?
A Phase 3 Randomized, Double-Blind Placebo-Controlled Trial to Evaluate the Efficacy and Safety of Remdesivir (GS-5734™) Treatment of COVID-19 in an Outpatient Setting
Decreased mRNA and protein stability of W1282X limits response to modulator therapy.
Taskforce recommends coordinated effort to improve clinical research conduct and find highly effective CFTR-directed treatment for rare mutations.
Theranostics by testing CFTR modulators in patient-derived materials: The current status and a proposal for subjects with rare CFTR mutations.
Important steps in the journey to highly effective CFTR modulator access for people with CF.
Transcript from Stuart Elborn’s address at the ECFC award in June 2019.
Six-Minute Walk Test: Clinical Role, Technique, Coding, and Reimbursement.
CRISPR/Cas9 genome editing tool: A Promising Tool for Therapeutic Applications on respiratory diseases.
[Neonatal cystic fibrosis screening: Analysis and differences in immunoreactive trypsin levels in newborns with a positive screen].
Extended-criteria uncontrolled DCD donor for a fragile recipient: A case report about a challenging yet successful lung transplantation.
A chronic strain of the cystic fibrosis pathogen Pandoraea pulmonicola expresses a heterogenous hypo-acylated lipid A.
Mucin secretion in cystic fibrosis – a systematic review.
Real World Experience of Pseudomonas aeruginosa Eradication at an Urban Pediatric Cystic Fibrosis Center.
A Novel Mouse Model of Aminoglycoside-Induced Hyperacusis and Tinnitus.
The current state of immunization against Gram-negative bacteria in children: a review of the literature.
Effect of apical chloride concentration on the measurement of responses to CFTR modulation in airway epithelia cultured from nasal brushings.
State of the Art in Exocrine Pancreatic Insufficiency.
Histological changes in odontogenic parakeratinized keratocysts treated with marsupialization followed by enucleation.
Glued in lipids: Lipointoxication in cystic fibrosis.
Cost-Effectiveness of Ivacaftor Therapy for Treatment of Cystic Fibrosis Patients With the G551D Gating Mutation.
Protocol for Project Fizzyo, an analytic longitudinal observational cohort study of physiotherapy for children and young people with cystic fibrosis, with interrupted time-series design.
The Antibacterial and Anti-biofilm Activity of Metal Complexes Incorporating 3,6,9-Trioxaundecanedioate and 1,10-Phenanthroline Ligands in Clinical Isolates of Pseudomonas Aeruginosa from Irish Cystic Fibrosis Patients.
Prospective longitudinal association between repeated multiple breath washout measurements and computed tomography scores in children with cystic fibrosis.
Focusing on the penultimate step: increasing early lung transplant discussion in cystic fibrosis clinic to prepare patients for referral.
Pseudomonas aeruginosa Volatilome Characteristics and Adaptations in Chronic Cystic Fibrosis Lung Infections.
The Association of Grip Strength, Body Mass Index, and Lung Function in Youth with Cystic Fibrosis.
Toll like Receptor signalling by Prevotella histicola activates alternative NF-κB signalling in Cystic Fibrosis bronchial epithelial cells compared to P. aeruginosa.
Lung Clearance Index to Track Acute Respiratory Events in School-age Children with Cystic Fibrosis.
The Enemy of my Enemy: Bacterial Competition in the Cystic Fibrosis Lung.
FREQMAX provides an alternative approach for determining high-resolution allele frequency thresholds in carrier screening.
Oral Glucose Tolerance Testing Using Candy for Cystic Fibrosis
Testing Drug Efficacy in Cystic Fibrosis Through N-of-1 Trials
Ivacaftor in Infants Aged 4 to <12 Months With Cystic Fibrosis and a Gating Mutation: Results of a 2-Part Phase 3 Clinical Trial.
Does cystic fibrosis constitute an advantage in COVID-19 infection?
Candida thrombophlebitis in children: a systematic review of the literature.
Tobacco smoke exposure limits the therapeutic benefit of tezacaftor/ivacaftor in pediatric patients with cystic fibrosis.
A Cohort Study of Sleep Quality in Adult Patients with Acute Pulmonary Exacerbations of Cystic Fibrosis.
Laparoscopic surgery in patients with cystic fibrosis: A systematic review.
Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial.
Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive CFTR genetics and sweat test.
Micro-rheological properties of lung homogenates correlate with infection severity in a mouse model of Pseudomonas aeruginosa lung infection.
The 34th Annual North American Cystic Fibrosis Conference, October 7-23, 2020.
[Therapy of cystic fibrosis – new drugs give hope].
THE PHOSPHODIESTERASE INHIBITOR ENSIFENTRINE REDUCES PRODUCTION OF PRO-INFLAMMATORY MEDIATORS IN WELL-DIFFERENTIATED BRONCHIAL EPITHELIAL CELLS BY INHIBITING PDE4.
CFTR Modulator Therapy Enhances Peripheral Blood Monocyte Contributions to Immune Responses in People With Cystic Fibrosis.
Multi-Omics Approaches: The Key to Improving Respiratory Health in People With Cystic Fibrosis?
Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis.
The Probable, Possible, and Novel Functions of ERp29.
High Occurrence of Bacterial Competition Among Clinically Documented Opportunistic Pathogens Including Achromobacter xylosoxidans in Cystic Fibrosis.
Functional Profiling of CFTR-Directed Therapeutics Using Pediatric Patient-Derived Nasal Epithelial Cell Models.
Identification of Compounds That Promote Readthrough of Premature Termination Codons in the CFTR.
Are patients with lung cystic fibrosis at increased risk for severe and fatal COVID-19? Interleukin-6 as a predictor of COVID-19 outcome.
Live imaging and quantitative analysis of Aspergillus fumigatus growth and morphology during inter-microbial interaction with Pseudomonas aeruginosa.
Combined Single Lung and Liver Transplantation in a Cystic Fibrosis Patient With Previous Contralateral Pneumonectomy: A Case Report.
Safety and efficacy of lenabasum in a phase 2 randomized, placebo-controlled trial in adults with cystic fibrosis.
Cystic Fibrosis in the PICU-More of a Zebra Than a Horse.
Prolonged infusion of beta-lactam antibiotics for Gram-negative infections: rationale and evidence base.
Are cystic fibrosis mutation carriers a potentially highly vulnerable group to COVID-19?
Impulse oscillometry and spirometry measurements relative to personal best values at the time of acute exacerbations of cystic fibrosis in adults.
Inhibitory activities of curzerenone, curdione, furanodienone, curcumol and germacrone on Ca2+-activated chloride channels.
Sit-to-stand test in children with bronchiectasis: Does it measure functional exercise capacity?
A case of reversible toxic optic neuropathy from tacrolimus (FK506).
Use of Hyaluronic Acid (HA) in Chronic Airway Diseases.
Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.
A systematic review of patient-reported outcome measures (PROMs) in cystic fibrosis.
Diagnostic Value of Sputum Cultures in Children under 2 Years of Age with Chronic Suppurative Lung Diseases.
Alleviation of depression-like behavior in a cystic fibrosis mouse model by Hdac6 depletion.
Intravenous versus oral antibiotics for eradication of Pseudomonas aeruginosa in cystic fibrosis (TORPEDO-CF): a randomised controlled trial.
SELF-BREATHE RCT for Chronic Breathlessness
Antimicrobial Peptide Exposure Selects for Resistant and Fit Stenotrophomonas maltophilia Mutants That Show Cross-Resistance to Antibiotics.
Mutations of the cystic fibrosis transmembrane conductance regulator gene in males with congenital bilateral absence of the vas deferens: Reproductive implications and genetic counseling (Review).
Membrane phospholipid composition of Pseudomonas aeruginosa grown in a cystic fibrosis mucus-mimicking medium.
Nebulizer Care and Inhalation Technique in Children with Cystic Fibrosis.
The Potential Role and Regulatory Mechanisms of MUC5AC in Chronic Obstructive Pulmonary Disease.
Clinical Presentation of the c.3844T>C (p.Trp1282Arg, W1282R) Variant in Russian Cystic Fibrosis Patients.
High-Intensity Interval Training Is Effective at Increasing Exercise Endurance Capacity and Is Well Tolerated by Adults with Cystic Fibrosis.
Sex Steroids Induce Membrane Stress Responses and Virulence Properties in Pseudomonas aeruginosa.
The first report on immunoglobulins A, E, G and M levels in cystic fibrosis patients in Saudi Arabia.
Immune transcriptomes of highly exposed SARS-CoV-2 asymptomatic seropositive versus seronegative individuals from the Ischgl community.
Stakeholder feedback informs investigations for chronic rhinosinusitis in cystic fibrosis.
Prophylactic anti-staphylococcal antibiotics for cystic fibrosis.
Factors Contributing to Vitamin D Status at Hospital Admission for Pulmonary Exacerbation in Adults With Cystic Fibrosis.
WNT-β-catenin signalling – a versatile player in kidney injury and repair.
Skin-Interfaced Microfluidic Systems that Combine Hard and Soft Materials for Demanding Applications in Sweat Capture and Analysis.
A 96-well format microvascularized human lung-on-a-chip platform for microphysiological modeling of fibrotic diseases.
Scedosporium Cell Wall: From Carbohydrate-Containing Structures to Host-Pathogen Interactions.
Bronchiectasis with secondary pulmonary infection in a child: A case report.
Abnormal glucose tolerance in a pediatric cystic fibrosis cohort: Trends in clinical outcomes and associated factors in the preceding years.
Neutrophil elastase promotes macrophage cell adhesion and cytokine production through the integrin-Src kinases pathway.
The Role of Specialized Pro-Resolving Mediators in Cystic Fibrosis Airways Disease.
Combined Bacteriophage and Antibiotic Treatment Prevents Pseudomonas aeruginosa Infection of Wild Type and cftr- Epithelial Cells.
Predicting Antibiotic-Associated Virulence of Pseudomonas aeruginosa Using an ex vivo Lung Biofilm Model.
Mycobacterium abscessus Infection after Breast Lipotransfer: A Report of 2 Cases.
A TLR2-Activating Fraction From Mycobacterium abscessus Rough Variant Demonstrates Vaccine and Diagnostic Potential.
Physical activity for cystic fibrosis: perceptions of people with cystic fibrosis, parents and healthcare professionals.
Antibacterial and antibiofilm activities of ceragenins against Achromobacter species isolated from cystic fibrosis patients.
Epithelial Barrier Properties of the 16HBE14o- Human Bronchial Epithelial Cell Culture Model.
Effect of highly effective modulator therapy on quality of life in adults with cystic fibrosis.

Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-deficient piglets.
Delayed Presentation of Meconium Ileus in an Infant With Cystic Fibrosis.
The different anion transport capability of prodiginine- and tambjamine-like molecules.
The Pseudomonas aeruginosa protease LasB directly activates IL-1β.
A Data-Driven Approach to Carrier Screening for Common Recessive Diseases.
Using next-generation sequencing of microRNAs to identify host and/or pathogen nucleic acid signatures in samples from children with biliary atresia – a pilot study.
Tracking the genome of four Pseudomonas aeruginosa isolates that have a defective Las quorum-sensing system, but are still virulent.
The first cases of genetically confirmed congenital diarrhea with chloride loss in Slovakia.
Positive orientation and posttraumatic growth in mothers of children with cystic fibrosis – mediating role of coping strategies.
Rheological analysis of sputum from patients with chronic bronchial diseases.
Consequences of CRISPR-Cas9-Mediated CFTR Knockout in Human Macrophages.
Fostering Innovation in the Treatment of Chronic Polymicrobial Cystic Fibrosis-Associated Infections Exploring Aspartic Acid and Succinic Acid as Ciprofloxacin Adjuvants.
[Management of Primary Ciliary Dyskinesia].
Phytomedicines (medicines derived from plants) for sickle cell disease.
Personalized Theratyping Trial
Choice of Differentiation Media Significantly Impacts Cell Lineage and Response to CFTR Modulators in Fully Differentiated Primary Cultures of Cystic Fibrosis Human Airway Epithelial Cells.
A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous for F508del or heterozygous for the F508del-CFTR mutation and a residual function mutation.
[Lungtransplantation in Sweden – over 1 200 patients transplanted since 1990].
Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor for ≥24 Weeks in People With CF and ≥1 F508del Allele: Interim Results of an Open-Label Phase Three Clinical Trial.
Iron metabolism in Pseudomonas aeruginosa biofilm and the involved iron-targeted anti-biofilm strategies.
Oral antibiotic prescribing patterns for treatment of pulmonary exacerbations in two large pediatric CF centers.
Cystic Fibrosis Year in Review 2019: Section 2 Pulmonary disease and infections.
Colistin dry powder inhalation with the Twincer™: An effective and more patient friendly alternative to nebulization.
Discovery of novel VX-809 hybrid derivatives as F508del-CFTR correctors by molecular modeling, chemical synthesis and biological assays.
Mucosal humoral immunity in cystic fibrosis – a tangled web of failed proteostasis, infection and adaptive immunity.
Capsaicin-Loaded Chitosan Nanocapsules for wtCFTR-mRNA Delivery to a Cystic Fibrosis Cell Line.
Aquagenic keratoderma and cystic fibrosis screening.
Predictors of massive haemoptysis after a first episode of mild-to-moderate haemoptysis in patients with cystic fibrosis.
Experiences of family members of children with cystic fibrosis under the light of Callista Roy.
The Lung Life of a Cystic Fibrosis Patient: A Patient and Physician Perspective.
Long-Term Ivacaftor in People Aged 6 Years and Older with Cystic Fibrosis with Ivacaftor-Responsive Mutations.
Airway microbiota is associated with the severity of non-CF bronchiectasis.
Pulmonary exacerbations in adults with cystic fibrosis – a grown-up issue in a changing CF landscape.
Of onions and men: Report of cavitary community acquired pneumonia due to Burkholderia cepacia complex in an immunocompetent patient and review of the literature.
Oral Glutathione and Growth in Cystic Fibrosis: A Multi-Center, Randomized, Placebo-Controlled, Double-Blind Trial.
Rates of depression and anxiety in Italian patients with cystic fibrosis and parent caregivers: Implementation of the Mental Health Guidelines.
Does newborn screening improve early lung function in cystic fibrosis?
Infant spirometry as a predictor of lung function at early childhood in cystic fibrosis patients.
The coughing body: etiquettes, techniques, sonographies and spaces.
In silico prediction and validation of potential therapeutic genes in pancreatic β-cells associated with type 2 diabetes.
Review: Pathogenesis of cholestatic liver diseases.
Characterization of the genomically encoded fosfomycin resistance enzyme from Mycobacterium abscessus.
Cystic Fibrosis and COVID-19: Care Considerations.
Isolation and first draft genome sequence of a linezolid-dependent Staphylococcus aureus clinical strain.
Hypoglycaemia in cystic fibrosis during an extended oral glucose tolerance test.
Reliability of impulse oscillometry parameters in healthy children and in children with cystic fibrosis.
Physical Function Measured Prior to Lung Transplantation Is Associated With Posttransplant Patient Outcomes.
Trends in nontuberculous mycobacteria infection in children and young people with cystic fibrosis.
Low rates of macrolide-resistant Mycobacterium avium complex in cystic fibrosis despite chronic azithromycin therapy.
Targeting UBC9-mediated protein hyper-SUMOylation in cystic cholangiocytes halts polycystic liver disease in experimental models.
LncRNA and transcriptomic analysis of fetal membrane reveal potential targets involved in oligohydramnios.
Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients.
Effect of Endoscope Sinus Surgery on Pulmonary Function in Cystic Fibrosis Patients: A Meta-Analysis.
Tissue Doppler and speckle tracking echocardiography assessment of left ventricular function in children with cystic fibrosis.
Sputum versus nasopharyngeal samples for the molecular diagnosis of respiratory viral infection in cystic fibrosis: A pilot study.
Global reprogramming of virulence and antibiotic resistance in Pseudomonas aeruginosa by a single nucleotide polymorphism in elongation factor, fusA1.
Differential response to biologics in a patient with severe asthma and ABPA: a role for dupilumab?
Stability of serum precipitins to Aspergillus fumigatus for the diagnosis of allergic bronchopulmonary aspergillosis.
Scoring tools to monitor risk of disease progression in patients with cystic fibrosis.
Analysis of airway microbiota in adults from a Brazilian cystic fibrosis center.
Breathing Easier: Molecular Therapeutics for Cystic Fibrosis.
Optimization of anti-microbials in the treatment of Cystic Fibrosis Pulmonary Exacerbations:  II. Therapies for Allergic Bronchopulmonary Aspergillosis (ABPA).
Identification, Structure-Activity Relationship, and Biological Characterization of 2,3,4,5-Tetrahydro-1H-pyrido[4,3-b]indoles as a Novel Class of CFTR Potentiators.
FOXL1 Regulates Lung Fibroblast Function via Multiple Mechanisms.
Epidemiology of Burkholderia Infections in People with Cystic Fibrosis in Canada between 2000 – 2017.
Dissecting the antibacterial activity of oxadiazolone-core derivatives against Mycobacterium abscessus.
Protease inhibitors elicit anti-inflammatory effects in CF mice with Pseudomonas aeruginosa acute lung infection.
Challenging barriers to an option for improved provision of enteral nutrition.
A B cell-dependent pathway drives chronic lung allograft rejection after ischemia-reperfusion injury in mice.
Impact of KLF4 on Cell Proliferation and Epithelial Differentiation in the Context of Cystic Fibrosis.
Promise and Potential Peril with Lumacaftor for the Trafficking Defective Type 2 Long QT Syndrome-Causative Variants, p.G604S, p.N633S, and p.R685P, Using Patient-Specific Re-Engineered Cardiomyocytes.
Drug Development for Cystic Fibrosis.
Changing landscape: psychological care in the era of cystic fibrosis transmembrane conductance regulator modulators.
Sexual and reproductive health in cystic fibrosis.
The changing landscape of cystic fibrosis: new therapies, challenges and a global pandemic.
Correction: Improved residual fat malabsorption and growth in children with cystic fibrosis treated with a novel oral structured lipid supplement: A randomized controlled trial.
An Observational Long-Term Safety Surveillance of Participants from Corbus Sponsored Lenabasum Pivotal Clinical Trials
The Anti-Pseudomonal Peptide D-BMAP18 Is Active in Cystic Fibrosis Sputum and Displays Anti-Inflammatory In Vitro Activity.
Antibiotic Treatment Of Staphylococcus Aureus In Stable People With CF
Advances in Ca2+ modulation of gastrointestinal anion secretion and its dysregulation in digestive disorders (Review).
Hyaluronan is abundant in COVID-19 respiratory secretions.
Editorial: Special Issue on “Therapeutic Approaches for Cystic Fibrosis”.
Screen-Printed Sensor for Low-Cost Chloride Analysis in Sweat for Rapid Diagnosis and Monitoring of Cystic Fibrosis.
Ionocytes and CFTR Chloride Channel Expression in Normal and Cystic Fibrosis Nasal and Bronchial Epithelial Cells.
Electrochemical Detection of Pyocyanin as a Biomarker for Pseudomonas aeruginosa: A Focused Review.
Sinister sinusitis.
Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity.
Rescue of common exon skipping mutations in Cystic Fibrosis with modified U1 snRNAs.
Carbon monoxide-releasing molecules inhibit the cystic fibrosis transmembrane conductance regulator Cl- channel.
Models of Palliative Care Delivery for Individuals with Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Consensus Guidelines.
Survey and Electronic Health Record-Based Medication Use Agreement in Children with Cystic Fibrosis: A Retrospective Cross-Sectional Study.
Extracellular DNA in sputum is associated with pulmonary function and hospitalization in patients with cystic fibrosis.
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.
The accuracy of four commercial broth microdilution tests in the determination of the minimum inhibitory concentration of colistin.
Delayed glucose peak and elevated 1-hour glucose on the oral glucose tolerance test identify youth with cystic fibrosis with lower oral disposition index.
Acute hyperosmolar hyperglycaemic state in cystic fibrosis-related diabetes caused by glucocorticoid and itraconazole interaction.
Qualitative evaluation of MRI features in aneurysmal bone cysts after percutaneous sclerotherapy.
Sperm retrieval and intracytoplasmic sperm injection outcomes in men with cystic fibrosis disease versus congenital bilateral absence of the vas deferens.
Kwashiorkor-like dermatosis: a rare presentation of cystic fibrosis.
Appearance of Pancreatic Sufficiency and Discontinuation of Pancreatic Enzyme Replacement Therapy (PERT) in Children with Cystic Fibrosis on Ivacaftor.

Pathways in the Pathophysiology of Coronavirus 19 Lung Disease Accessible to Prevention and Treatment.
Sars-CoV-2 infection in patients with cystic fibrosis. An overwiew.
Efficacy of Simeox Airway Clearance Therapy in Children With Cystic Fibrosis
Home Airway Clearance in CF Patients
Preliminary report of In vitro and In vivo Effectiveness of Dornase alfa on SARS-CoV-2 infection.
Ancillary Home Airway Clearance in CF Patients ( HomeCareSIMEOX )
Laying the Foundation for Cystic Fibrosis Therapy: An Interview with Michael Boyle.
Preserving Multidisciplinary Care Model and Patient Safety During Reopening of Ambulatory Cystic Fibrosis Clinic for Nonurgent Care: A Hybrid Telehealth Model.
Monitoring early stage lung disease in cystic fibrosis.
Two-Step Testing for Clostridioides Difficile is Inadequate in Differentiating Infection From Colonization in Children.
A Survey of Pediatric Competencies in Entry-Level Physical Therapy Programs in Australia.
Lung immunoglobulin A immunity dysregulation in cystic fibrosis.
Epidemiology of Pseudomonas aeruginosa in cystic fibrosis patients in Iran: A systematic review and meta-analysis.
A New Model of Chronic Mycobacterium abscessus Lung Infection in Immunocompetent Mice.
Transition to adult care in cystic fibrosis: The challenges and the structure.
A systematic cochrane review of probiotics for people with cystic fibrosis.
Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients.
Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression.
Living and dying with cystic fibrosis.
Genomics-driven discovery of a novel glutarimide antibiotic from Burkholderia gladioli reveals an unusual polyketide synthase chain release mechanism.
Exhaled volatile organic compounds analysis in clinical pediatrics: a systematic review.
Carriers of a single CFTR mutation are asymptomatic: an evolving dogma?
Infants with Congenital Disorders Identified Through Newborn Screening – United States, 2015-2017.
Neutrophil count in sputum is associated with increased sputum glucose and sputum L-lactate in cystic fibrosis.
An in vitro model for assessing drug transport in cystic fibrosis treatment: Characterisation of the CuFi-1 cell line.
Posaconazole-Induced Hypertension Masquerading as Congenital Adrenal Hyperplasia in a Child with Cystic Fibrosis.
Immune transcriptomes of highly exposed SARS-CoV-2 asymptomatic seropositive versus seronegative individuals from the Ischgl community.
MexXY RND pump of Pseudomonas aeruginosa PA7 effluxes bi-anionic β-lactams carbenicillin and sulbenicillin when it partners with the outer membrane factor OprA but not with OprM.
Incidence of nephrotoxicity with Prolonged aminoglycoside exposure in patients with cystic fibrosis.
Persistent Recovery of Pancreatic Function in Patients with Cystic Fibrosis after Ivacaftor.
Coumaronochromone as antibacterial and carbonic anhydrase inhibitors from Aerva persica (Burm.f.) Merr.: experimental and first-principles approaches.
A simple preservation method for the storage of Aspergillus fumigatus and Scedosporium apiospermum filamentous fungi isolated from the sputum of patients with cystic fibrosis (CF).
A Phase 3b, Randomized, Double blind, Controlled Study Evaluating the Efficacy and Safety of VX-445/Tezacaftor/Ivacaftor in Cystic Fibrosis Subjects, Homozygous for F508del
A Study Evaluating the Long-term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis (CF) Subjects 6 Years and Older and F/MF Genotypes
MR Imaging of Lung in the Follow-up Assessment of Cystic Fibrosis
TRPV4 and purinergic receptor signalling pathways are separately linked in airway epithelia to CFTR and TMEM16A chloride channels.
Targeting Nonsense: Optimization of 1,2,4-Oxadiazole TRIDs to Rescue CFTR Expression and Functionality in Cystic Fibrosis Cell Model Systems.
Novel ANO1 Inhibitor from Mallotus apelta Extract Exerts Anticancer Activity through Downregulation of ANO1.
Characterization of the Burkholderia cenocepacia J2315 Surface-Exposed Immunoproteome.
Factors influencing clinical trial participation for adult and pediatric patients with cystic fibrosis.
Effects of Innovative Aerobic Exercise Training in Cystic Fibrosis
How can we relieve gastrointestinal symptoms in people with cystic fibrosis? An international qualitative survey.
Noninvasive Ventilation in Cystic Fibrosis: Clinical Indications and Outcomes in a Large UK Adult Cystic Fibrosis Center.
Differential effects of the cystic fibrosis lung inflammatory environment on mesenchymal stromal cells.
Clinical and microbiological monitoring of Cystic Fibrosis patients, three years of follow-up via Tele-Medicine: an empirical research.
Mucinous cystic adenoma with inflammatory cell infiltration around the splenic artery mimicking pancreatic cancer: a case report.
Bronchiectasis Associated with Electronic Cigarette Use: A Case Series.
Effects of a long-term exercise program on motor performance in children and adolescents with CF.
Azole-Resistant Aspergillus fumigatus Among Danish Cystic Fibrosis Patients: Increasing Prevalence and Dominance of TR34/L98H.
Mechanism-Based Personalized Medicine for Cystic Fibrosis by Suppressing Pseudo Exon Inclusion.
Rheumatoid arthritis in a patient with cystic fibrosis: challenging treatment options.
Molar mass effect in food and health.
Genistein-Calcitriol Mitigates Hyperosmotic Stress-Induced TonEBP, CFTR Dysfunction, VDR Degradation and Inflammation in Dry Eye Disease.
Patients with Cystic Fibrosis and Advanced Lung Disease Benefit from Lumacaftor/Ivacaftor Treatment.
Evolutionary genomics of niche-specific adaptation to the cystic fibrosis lung in Pseudomonas aeruginosa.
Current development of CFTR potentiators in the last decade.
CFTR mutation compromises spermatogenesis by enhancing miR-15b maturation and suppressing its regulatory target CDC25A†.
[Appendiceal diverticulosis, a potential mimic of low grade appendiceal mucinous neoplasm: analysis of 20 cases].
A tale of two pancreases: exocrine pathology and endocrine dysfunction.
Paediatric nasal irrigation: The “fencing” method.
Vitamin E supplementation in people with cystic fibrosis.
The impact of segmentation on whole-lung functional MRI quantification: Repeatability and reproducibility from multiple human observers and an artificial neural network.
Aerosolized lancovutide in adolescents (≥12 years) and adults with cystic fibrosis – a randomized trial.
Airway Inflammation and Host Responses in the Era of CFTR Modulators.
Treatment of pulmonary exacerbations in cystic fibrosis.
Light at the End of Tunnel-Managing Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis.
Risk of malignancy in Caroli disease and syndrome: A systematic review.
Cystic Fibrosis and COVID-19.
Patterns of Health Insurance Coverage and Lung Disease Progression in Adolescents and Young Adults with Cystic Fibrosis.
Allergic Bronchopulmonary Aspergillosis in Children with Cystic Fibrosis: An Update on the Newest Diagnostic Tools and Therapeutic Approaches.
Recommended shielding against COVID-19 impacts physical activity levels in adults with cystic fibrosis.
A Phylogeny-Informed Proteomics Approach for Species Identification within the Burkholderia cepacia complex.
Interferon-gamma-activated macrophages infected with Burkholderia cenocepacia process and present bacterial antigens to T-cells by class I and II major histocompatibility complex molecules.
Immune response of polarized cystic fibrosis airway epithelial cells infected with Influenza A virus.
Transition From Pediatric to Adult Care for Young Adults With Chronic Respiratory Disease.
Using Near-Peer Teaching to Address Concepts of Cystic Fibrosis in Undergraduate Medical Learners.
Th17 associated cytokines in sputum samples from patients with cystic fibrosis.
[Cystic fibrosis : A systemic problem].
Impact of Pharmacy Technicians as part of an Integrated Health-System Pharmacy Team on Improvement of Medication Access in the Care of Cystic Fibrosis Patients.
Sleep Screening for Cystic Fibrosis Patients: A Survey of Cystic Fibrosis Programs.
Do Pulmonary and Extrapulmonary Features Differ Among Cystic Fibrosis, Primary Ciliary Dyskinesia and Healthy Children?
G551D Mutation Impairs Protein Kinase A (PKA)-dependent Activation of CFTR Channel that can be Restored by Novel Gain-Of-Function (GOF) Mutations.
Evaluation of ELX/TEZ/IVA in Cystic Fibrosis (CF) Subjects 2 Through 5 Years
Hepatitis B in Cystic Fibrosis and Latent Tuberculosis Respectively
Worsening pulmonary outcomes during sex reassignment therapy in a transgender female with cystic fibrosis (CF) and asthma/allergic bronchopulmonary aspergillosis: a case report.
Accelerated Approval or Risk Reduction? How Response Biomarkers Advance Therapeutics through Clinical Trials in Cystic Fibrosis.
Analysis of hospital management of chronic respiratory diseases in light of the “Maps of Health Needs” project in Poland.
Erratum: Cardiopulmonary Exercise Testing Provides Additional Prognostic Information in Cystic Fibrosis.
The effects of taurine supplementation on obesity, blood pressure and lipid profile: A meta-analysis of randomized controlled trials.
Optimization of nitric oxide donors for investigating biofilm dispersal response in Pseudomonas aeruginosa clinical isolates.
Transparency and diversity in cystic fibrosis research.
Transparency and diversity in cystic fibrosis research.
Transparency and diversity in cystic fibrosis research – Authors’ reply.
Relationship Between Estrogen Treatment and Skeletal Health in Women With Cystic Fibrosis.
Comparison of Meal-Time Dosing of Insulin in Cystic Fibrosis Related Diabetes
Short-Chain Fatty Acids Promote Mycobacterium avium subsp. hominissuis Growth in Nutrient-Limited Environments and Influence Susceptibility to Antibiotics.
From Mouse to Man and Back: Closing the Correlation Gap between Imaging and Histopathology for Lung Diseases.
Cystic fibrosis newborn screening: the importance of bloodspot sample quality.
Cyst growth in ADPKD is prevented by pharmacological and genetic inhibition of TMEM16A in vivo.
Crushing pancreatic enzymes with enteral feeds in an extremely premature infant with cystic fibrosis-a novel and effective technique.

The role of biochemical testing in cystic fibrosis.
Three-dimensional magnetic resonance imaging ultrashort echo-time cones for assessing lung density in pediatric patients.
Projecting the impact of delayed access to elexacaftor/tezacaftor/ivacaftor for people with Cystic Fibrosis.
Ivacaftor Modifies Cystic Fibrosis Neutrophil Phenotype in Subjects with R117H Residual Function CFTR Mutations.
Correction to: Physiologically Based Pharmacokinetic Modeling of CFTR Modulation in People with Cystic Fibrosis Transitioning from Mono or Dual Regimens to Triple-Combination Elexacaftor/Tezacaftor/Ivacaftor.
O-Methylation of the glycopeptidolipid acyl chain defines surface hydrophobicity of Mycobacterium abscessus and macrophage invasion.
Activity of airway antimicrobial peptides against cystic fibrosis pathogens.
Sleep disorders are distinctively associated with exercise intolerance and sedentary behavior in children with cystic fibrosis.
[Clinical features of cystic fibrosis associated allergic bronchopulmonary aspergillosis in children].
Risk factor identification in cystic fibrosis by flexible hierarchical joint models.
BPI-ANCA is expressed in the airways of cystic fibrosis patients and correlates to platelet numbers and Pseudomonas aeruginosa colonization.
Phenotypic and molecular characteristics of CF patients carrying the I1234V mutation.
Impact of SARS-CoV-2 infection in patients with cystic fibrosis in Spain: Incidence and results of the national CF-COVID19-Spain survey.
Not Quite the Bully in the Schoolyard: Staphylococcus aureus Can Survive and Co-exist with Pseudomonas aeruginosa in the Cystic Fibrosis Lung.
Benign postcricoid hypertrophy: Case report and review of the literature.
Ivacaftor improves lung disease in patients with advanced CF carrying CFTR mutations that confer residual function.
Chronic rhinosinusitis in COPD: A prevalent but unrecognized comorbidity impacting health related quality of life.
Autophagy Augmentation to Alleviate Immune Response Dysfunction, and Resolve Respiratory and COVID-19 Exacerbations.
Fatal respiratory infection due to ST308 VIM-1-producing Pseudomonas aeruginosa in a lung transplant recipient: case report and review of the literature.
Cardiorespiratory fitness on a treadmill in an adult cystic fibrosis population.
Flagellin shifts 3D bronchospheres towards mucus hyperproduction.
Novel Anti-Inflammatory Approaches for Cystic Fibrosis Lung Disease: Identification of Molecular Targets and Design of Innovative Therapies.
The Resolution Approach to Cystic Fibrosis Inflammation.
Lactose-Induced Chronic Diarrhea Results From Abnormal Luminal Microbial Fermentation and Disorder of Ion Transport in the Colon.
CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Human Lung Microvascular Endothelial Cells Controls Oxidative Stress, Reactive Oxygen-Mediated Cell Signaling and Inflammatory Responses.
The value of nasal nitric oxide measurement in the diagnosis of primary ciliary dyskinesia.
“Reactance inversion” at low frequencies in a child undergoing treatment of a cystic fibrosis exacerbation.
Role of Cystic Fibrosis Bronchial Epithelium in Neutrophil Chemotaxis.
Deletion of cftr Leads to an Excessive Neutrophilic Response and Defective Tissue Repair in a Zebrafish Model of Sterile Inflammation.
Recent advances in phenotypic drug discovery.
An Open Label Phase 2 Trial of Metformin for Improving CFTR Function in CFRD
A Clinical Study to Investigate Safety, Tolerability and Distribution of CHF 6333 After One or After Repeated Inhalation in Patients With Cystic Fibrosis (CF) and in Patients With Non Cystic Fibrosis (NCFB) Bronchiectasis
NETwork of Linoleic Acid Supplementation in Cystic Fibrosis
CFTR Depletion Confers Hypersusceptibility to Mycobacterium fortuitum in a Zebrafish Model.
Approaches for Genetic Discoveries in the Skin Commensal and Pathogenic Malassezia Yeasts.
Contribution of Short Chain Fatty Acids to the Growth of Pseudomonas aeruginosa in Rhinosinusitis.
Senescence and autophagy in usual interstitial pneumonia of different etiology.
Manuka honey versus saline sinus irrigation in the treatment of cystic fibrosis-associated chronic rhinosinusitis: A randomized pilot trial.
Allosteric folding correction of F508del and rare CFTR mutants by elexacaftor-tezacaftor-ivacaftor (Trikafta) combination.
The Ubiquitin System and A20: Implications in Health and Disease.
Evaluation of Short Antibiotic Combination Courses Followed by Aerosols in Cystic Fibrosis
Dornase alfa and rate of lung function decline in European patients with cystic fibrosis: A retrospective registry cohort study.
How good is end of life care for patients dying with cystic fibrosis?
The psychosocial burden of inflammatory bowel disease in adolescents and young adults.
Physical exercise as a tool to minimize the consequences of the Covid-19 quarantine: an overview for cystic fibrosis.
Why Airway Gland Secretions Are Abnormally Sticky in Cystic Fibrosis, and Why Not Much Can Be Done About It.
Sleep-disordered breathing in cystic fibrosis.
Explaining the sex effect on survival in cystic fibrosis: a joint modeling study of UK registry data.
Retropharyngeal abscess in a post-lung transplant cystic fibrosis patient with prior cervical fusion: a case study.
Viral vector manufacturing: quality attributes of rAAV used in clinical development.
The Impact of Insulin Therapy on Protein Turnover in Pre-Diabetic Cystic Fibrosis Patients
Prognostic Value of Functional Exercise Test (EFX) in Cystic Fibrosis
Proteomic Identification Reveals the Role of Ciliary Extracellular-Like Vesicle in Cardiovascular Function.
Cystic Fibrosis Year in Review 2019: Section 1 CFTR Modulators.
Physical activity in children and adolescents with cystic fibrosis: a systematic review and meta-analysis.
Cortisol regulates insulin-like growth-factor binding protein (igfbp) gene expression in Atlantic salmon parr.
Naturally Derived Membrane Lipids Impact Nanoparticle-Based Messenger RNA Delivery.
Autophagy markers as mediators of lung injury-implication for therapeutic intervention.
What metabolic, osmotic and molecular stress responses tell us about extreme ambient heatwave impacts in fish at low salinities: The case of European seabass, Dicentrarchus labrax.
SARS-CoV-2 seroprevalence in a Belgian cohort of patients with cystic fibrosis.
From Ivacaftor to Triple Combination: A Systematic Review of Efficacy and Safety of CFTR Modulators in People with Cystic Fibrosis.
CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis.
Vulnerable Child Syndrome and Newborn Screening Carrier Results for Cystic Fibrosis or Sickle Cell.
Cystic fibrosis in low and middle-income countries (LMIC): A view from four different regions of the world.
Obesity and ethnicity alter gene expression in skin.
Area Under the Curve Achievement of Once Daily Tobramycin in Children with Cystic Fibrosis during Clinical Care.
Under nonlimiting iron conditions pyocyanin is a major antifungal molecule, and differences between prototypic Pseudomonas aeruginosa strains.
H1N1 Influenza Virus in Patients With Cystic Fibrosis: A Literature Review Examining Both Disease Entities and Their Association in Light of the 2009 Pandemic.
Rapid and robust evolution of collateral sensitivity in Pseudomonas aeruginosa antibiotic-resistant mutants.
Cystic Fibrosis Related Diabetes – An Update.
Selectively targeting key inflammatory pathways in cystic fibrosis.
New Directions in Pulmonary Gene Therapy.
Trophic cooperation promotes bacterial survival of Staphylococcus aureus and Pseudomonas aeruginosa.
[Thoracic imaging in cystic fibrosis : Radiography].
LONGITUDINAL SURVEILLANCE AND COMBINATION ANTIMICROBIAL SUSCEPTIBILITY TESTING OF MULTIDRUG-RESISTANT ACHROMOBACTER SPP. FROM CYSTIC FIBROSIS PATIENTS.
Achromobacter Infections and Treatment Options.
What effective ways of motivation, support and technologies help people with cystic fibrosis improve and sustain adherence to treatment?
The Innate Lymphoid System Is a Critical Player in the Manifestation of Mucoinflammatory Airway Disease in Mice.
Simple binding of protein kinase A prior to phosphorylation allows CFTR anion channels to be opened by nucleotides.
An enzymatic toolkit for selective proteolysis, detection, and visualization of mucin-domain glycoproteins.
Structural insights into the gating mechanism of human SLC26A9 mediated by its C-terminal sequence.
Complex interplay between autophagy and oxidative stress in the development of pulmonary disease.
A Pilot Study of a Low Glycemic Load Diet in Adults With Cystic Fibrosis
The implementation of an aminoglycoside induced ototoxicity algorithm for people with cystic fibrosis.
ENaC regulation by phospholipids and DGK explained through mathematical modeling.
StatPearls
StatPearls
Lysosomal Reacidification Ameliorates Vinyl Carbamate-Induced Toxicity and Disruption on Lysosomal pH.
Long-term safety study of colistimethate sodium (Colobreathe®): Findings from the UK Cystic Fibrosis Registry.
Pharmacokinetics in Patients with Cystic Fibrosis: A Systematic Review of Data Published Between 1999 and 2019.
Pharmacotherapeutic management of bronchial infections in adults: non-cystic fibrosis bronchiectasis and chronic obstructive pulmonary disease.
Effect of Concomitant Azithromycin and Tobramycin Use on Cystic Fibrosis Pulmonary Exacerbation Treatment.
A novel HNF1B mutation p.R177Q in autosomal dominant tubulointerstitial kidney disease and maturity-onset diabetes of the young type 5: A pedigree-based case report.
Isolation of CFTR and TMEM16A inhibitors from Neorautanenia mitis (A. Rich) Verdcourt: Potential lead compounds for treatment of secretory diarrhea.
Orbital Myocysticercosis different Presentation and Management in Eastern Nepal.
Encephalopathy in cystic fibrosis.
Concerns regarding the safety of azithromycin in pregnancy – relevance for women with cystic fibrosis.
Decreased survival in cystic fibrosis patients with a positive screen for depression.
Changes in symptom scores as a potential clinical endpoint for studies of cystic fibrosis pulmonary exacerbation treatment.

Inhibition of Alk signaling promotes the induction of human salivary gland-derived organoids.
Effect of hypertonic saline on mucociliary clearance and clinical outcomes in chronic bronchitis.
The Gut Microbiota and Respiratory Diseases: New Evidence.
Ceftaroline versus Vancomycin for the Treatment of Acute Pulmonary Exacerbations in Pediatric Patients with Cystic Fibrosis.
Recipient Age Impacts Long-Term Survival in Adult Cystic Fibrosis Subjects after Lung Transplantation.
Towards the Development of AgoKirs: New Pharmacological Activators to Study Kir2.x Channel and Target Cardiac Disease.
Treatment of Nontuberculous Mycobacterial Pulmonary Disease: An Official ATS/ERS/ESCMID/IDSA Clinical Practice Guideline.
Choline in cystic fibrosis: relations to pancreas insufficiency, enterohepatic cycle, PEMT and intestinal microbiota.
Quantity not sufficient rates and delays in sweat testing in US infants with cystic fibrosis.
Management of superior mesenteric venous thrombus in cystic fibrosis related liver disease.
Attitudes of pain and opioids prescription practices in U.S. cystic fibrosis centers.
Early IL-2 treatment of mice with Pseudomonas aeruginosa pneumonia induced PMN-dominating response and reduced lung pathology.
A Phase 3b, Randomized, Placebo-Controlled Study Evaluating the Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Subjects 6 Through 11 Years of Age Who Are Heterozygous fo…
Delafloxacin – A Novel fluoroquinolone for the treatment of ciprofloxacin-resistant pseudomonas aeruginosa in patients with cystic fibrosis (CF).
Discovery and biosynthesis of bolagladins: unusual lipodepsipeptides from Burkholderia gladioli clinical isolates.
Microbiota-derived metabolites as diagnostic markers for respiratory fungal infections.
Alterocin, an antibiofilm protein secreted by Pseudoalteromonas sp. 3J6.
Tissue oxygenation in peripheral muscles and functional capacity in cystic fibrosis: A cross-sectional study.
Trikafta and Psychopathology in Cystic Fibrosis: A Case Report.
Investigating the Effect of Nano-Curcumin on the Expression of Biofilm Regulatory Genes of Pseudomonas aeruginosa.
Targeting IgG Autoantibodies for Improved Cytotoxicity of Bactericidal Permeability Increasing Protein in Cystic Fibrosis.
Rhinovirus Infection Drives Complex Host Airway Molecular Responses in Children With Cystic Fibrosis.
Heat acclimation improves sweat gland function and lowers sweat sodium concentration in an adult with cystic fibrosis.
Clinical and genetic characterization of patients with cystic fibrosis and functional assessment of the chloride channel with the pathogenic variant c.831G> A (p.Trp277*), described for the first time.
Targeting the Heme Oxygenase 1/Carbon Monoxide Pathway to Resolve Lung Hyper-Inflammation and Restore a Regulated Immune Response in Cystic Fibrosis.
Cystic fibrosis drug approved for patients aged 6-11 years worked well in clinical practice.
A putative enoyl-CoA hydratase contributes to biofilm formation and the antibiotic tolerance of Achromobacter xylosoxidans.
Increased intracellular Cl- concentration improves airway epithelial migration by activating the RhoA/ROCK Pathway.
Gene therapy for respiratory diseases; progress and a changing context.
Vardenafil increases intracellular accumulation of the most prevalent mutant CFTR in human bronchial epithelial cells.
Current Knowledge and Future Directions in Developing Strategies to Combat Pseudomonas aeruginosa Infection.
Abnormal n-6 fatty acid metabolism in cystic fibrosis contributes to pulmonary symptoms.
Vitamin K Supplementation to Reduce Deficiencies in Adults With Cystic Fibrosis
Correction of Airway Stem Cells: Genome Editing Approaches for the Treatment of Cystic Fibrosis.
Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants.
Cystic fibrosis – Ten promising therapeutic approaches in the current era of care.
ENaC inhibition in cystic fibrosis: potential role in the new era of CFTR modulator therapies.
Hyperglycaemia in CF adversely affects BK channel function critical for mucus clearance.
Genomic, transcriptomic, and protein landscape profile of CFTR and cystic fibrosis.
Physiologically Based Pharmacokinetic Modeling of CFTR Modulation in People with Cystic Fibrosis Transitioning from Mono or Dual Regimens to Triple-Combination Elexacaftor/Tezacaftor/Ivacaftor.
Airway cholinergic history modifies mucus secretion properties to subsequent cholinergic challenge in diminished chloride and bicarbonate conditions.
Cefoperazone sodium liposomal formulation to mitigate P.aeruginosa biofilm in Cystic fibrosis infection: A QbD approach.
Citrullination of extracellular histone H3.1 reduces antibacterial activity and exacerbates its proteolytic degradation.
Reading between the Lines: Utilizing RNA-Seq Data for Global Analysis of sRNAs in Staphylococcus aureus.
How much protein function needs to be restored in cystic fibrosis?
The coronavirus pandemic has forced rapid changes in care protocols for cystic fibrosis.
The gender gap in cystic fibrosis.
Bacteria-eating viruses could provide a route to stability in cystic fibrosis.
Gene therapy could offer an inclusive cure for cystic fibrosis.
A simple test could extend cystic-fibrosis treatments to those left behind.
Cystic fibrosis drugs target the malformed proteins at the root of the disease.
Cystic fibrosis.
Research round-up: Cystic fibrosis.
In utero intervention to stem the damage of cystic fibrosis.
[Magnetic resonance imaging of the lungs in cystic fibrosis].
Potential role of CFTR in bisphenol A-induced malignant transformation of prostate cells via mitochondrial apoptosis.
Returning to school in the midst of the COVID-19 pandemic for children with cystic fibrosis.
Steam disinfection of toothbrushes from patients with cystic fibrosis (CF): Evidence-based recommendations.
Acidic Submucosal Gland pH and Elevated Protein Concentration Produce Abnormal Cystic Fibrosis Mucus.
Hormonal Contraception Effects On Pulmonary Function In Adolescents With Cystic Fibrosis.
Organoids as a personalized medicine tool for ultra-rare mutations in cystic fibrosis: The case of S955P and 1717-2A > G.
Aspergillus Is Inhibited by Pseudomonas aeruginosa Volatiles.
Expanding access to CFTR modulators for rare mutations: The utility of n-of-1 trials.
Spelunking in Sputum: Single Cell RNA Sequencing Sheds New Insights into Cystic Fibrosis.
External and Genetic Conditions Determining Male Infertility.
Cigarette smoke induces endoplasmic reticulum stress and suppresses efferocytosis through the activation of RhoA.
Self-eating: friend or foe? The emerging role of autophagy in fibrotic diseases.
RNA helicase p68 inhibits the transcription and post-transcription of Pkd1 in ADPKD.
Key role of pediatricians and disease for influenza vaccination in children with high-risk chronic diseases.
Cellular Mechanism Underlying Oxytocin-Stimulated Cl- Secretion in Rat Cauda Epididymal Epithelium.
Gut microbiome a promising target for management of respiratory diseases.
Association of Inhaled Antibiotics in Addition to Standard IV Therapy and Outcomes of Pediatric Inpatient Pulmonary Exacerbations.
Cell-Based Therapeutic Approaches for Cystic Fibrosis.
[Bronchiectasis in Turkey: Under the light of national publications].
Will airway gene therapy for cystic fibrosis improve lung function? New imaging technologies can help us find out.
Palliative care and advances in cystic fibrosis: where now?
Allergic bronchopulmonary aspergillosis.
Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model.
Primary and secondary diseases of the perinephric space: an approach to imaging diagnosis with emphasis on MRI.
The evolutionary trajectories of P. aeruginosa in biofilm and planktonic growth modes exposed to ciprofloxacin: beyond selection of antibiotic resistance.
Progress of Clinical Application for Ex Vivo Lung Perfusion (EVLP) in Lung Transplantation.
Inhibition of CFTR-mediated intestinal chloride secretion by a fungus-derived arthropsolide A: Mechanism of action and anti-diarrheal efficacy.
Inhaled Dry Powder Antibiotics in Patients with Non-Cystic Fibrosis Bronchiectasis: Efficacy and Safety in a Real-Life Study.
Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis.
Screening for the Most Common Mutations of CFTR Gene among Egyptian Children with Difficult-to-Treat Asthma.
Molecular Heterogeneity in Cystic Fibrosis.
Differential Toll like receptor expression in cystic fibrosis patients’ airways during rhinovirus infection.
A Compound Heterozygous Mutation in the Ciliary Gene TTC21B Causes Nephronophthisis Type 12.
Adaptation and Survival of Burkholderia cepacia and B. contaminans During Long-Term Incubation in Saline Solutions Containing Benzalkonium Chloride.
Transport properties in CFTR-/- knockout piglets suggest normal airway surface liquid pH and enhanced amiloride-sensitive Na+ absorption.
Novel frameshift variant of the CFTR gene: S511Lfs*2 from phenotype to molecular predictions.
Infection prevention and control in cystic fibrosis: One size fits all?
A First in Human Study to Evaluate the Safety, Tolerability and Pharmacokinetics of Single and Multiple Ascending Doses of Inhaled ETD002 in Healthy Subjects
Cystic fibrosis related diabetes: Revisiting the OGTT and alternate screening tests.
Prospective cohort study of ototoxicity in persons with cystic fibrosis following a single course of intravenous tobramycin.
Bilateral Acute Cardioembolic Limb Ischemia After Coronavirus Disease 2019 Pneumonia in a Lung Transplant Recipient: A Case Report.
A systematic review of the clinical and genetic characteristics of Chinese patients with cystic fibrosis.
Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease.
Motor repertoire is age-inadequate in infants with cystic fibrosis.
Lubiprostone protects esophageal mucosa from acid injury in porcine esophagus.

Impaired Renal HCO3 – Excretion in Cystic Fibrosis.
Sulfhemoglobinemia associated with meconium ileus in cystic fibrosis.
Promoting Emotional Wellness in Children with CF, Part II: Mental Health Assessment and Intervention.
Pharmacokinetic and Pharmacodynamic Analysis of Ceftaroline in Children and Adolescents With Cystic Fibrosis
Pulmonary function testing in children’s interstitial lung disease.
Gene of the month: GLIS1-3.
Spatiotemporal Distribution of Pseudomonas aeruginosa Alkyl Quinolones under Metabolic and Competitive Stress.
Lethal mutations with fluctuating heterozygous effect: the lethal force of effective dominance.
Low iron-induced small RNA BrrF regulates central metabolism and oxidative stress responses in Burkholderia cenocepacia.
AcrAB-TolC Inhibition by Peptide-Conjugated Phosphorodiamidate Morpholino Oligomers Restores Antibiotic Activity in Vitro and in Vivo.
Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis.
The Respiratory Microbiome in Cystic Fibrosis: Compartment Patterns and Clinical Relationships in Early Stage Disease.
Expression and Roles of Antimicrobial Peptides in Innate Defense of Airway Mucosa: Potential Implication in Cystic Fibrosis.
Mechanisms of action of a web-based intervention with health professional support to increase nebulizer adherence in adults with Cystic Fibrosis: a qualitative interview study.
Effectiveness of a multistep Pseudomonas aeruginosa eradication treatment protocol in children with cystic fibrosis in Brazil.
Differential adaptability between reference strains and clinical isolates of Pseudomonas aeruginosa into the lung epithelium intracellular lifestyle.
A Phase III, Multicentre, Randomised, Placebo-Controlled, Double Blind Study of the Incidence of Recurring Pulmonary Exacerbations in Cystic Fibrosis Patients using Two Different Doses of Inhaled N…
Identification of the P. aeruginosa O17 and O15 O-Specific Antigen Biosynthesis Loci Reveals an ABC Transporter-Dependent Synthesis Pathway and Mechanisms of Genetic Diversity.
Sweat chloride assay by inductively coupled plasma mass spectrometry: a confirmation test for cystic fibrosis diagnosis.
Inhaled Liposomal Antimicrobial Delivery in Lung Infections.
A functional genomics approach to investigate the differentiation of iPSCs into lung epithelium at air-liquid interface.
Rats Race to Keep Pace in the Growing CF Model Space.
Advances in anion transport and supramolecular medicinal chemistry.
EnVision CF Multicenter Study of Glucose Tolerance in Cystic Fibrosis
Nebuliser hygiene in cystic fibrosis: evidence-based recommendations.
High-Dose Inhaled Nitric Oxide as Adjunct Therapy in Cystic Fibrosis Targeting Burkholderia multivorans.
[Emerging bacteria in cystic fibrosis and non-cystic fibrosis bronchiectasis from a microbiologist’s perspective].
Ivacaftor or lumacaftor/ivacaftor treatment does not alter the core CF airway epithelial gene response to rhinovirus.
Comparison of Organoid Swelling and in vivoBiomarkers of CFTR Function to Determine Effects of Lumacaftor-ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation.
Membrane Lipids and CFTR: The Ying/Yang of Efficient Ceramide Metabolism.
The impact of ivacaftor on sinonasal pathology in S1251N-mediated cystic fibrosis patients.
An Overview of the Treatment of Less Common Non-Lactose Fermenting Gram Negative Bacteria.
Mutations of the CFTR gene and novel variants in Turkish patients with cystic fibrosis: 24-years experience.
Intervention in Chronic Pediatric Patients and Their Families.
Assessment of Quality of Life Among Patients After Lung Transplantation: A Single-Center Study.
Evaluating assumptions of definition-based pulmonary exacerbation endpoints in cystic fibrosis clinical trials.
Strategies for dose reduction with specific clinical indications during computed tomography.
The outcome of pregnancy in women with cystic fibrosis: a UK population based descriptive study.
The SPLUNC1-βENaC complex prevents Burkholderia cenocepacia invasion in normal airway epithelia.
Sodium Status and Replacement in Children and Adults Living with Cystic Fibrosis: A Narrative Review.
Inflammasome inhibition under physiological and pharmacological conditions.
Emerging Technologies for Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Restoration in all People with CF.
Patient engagement to prioritise CF research: Inclusive or selective?
Transjugular intrahepatic portosystemic shunt in non-cirrhotic portal hypertension related to cystic fibrosis in a lung transplant patient.
K162E – A rare and uncategorized CFTR variant causing cystic fibrosis.
CFTR modulator therapy for cystic fibrosis caused by the rare c.3700A>G mutation.
Predicting the course of nutrition and lung disease in infants and children with cystic fibrosis.
Improved outcomes in cystic fibrosis using modified Re-Education of Airway Clearance Technique (REACT) programme.
Kv7 Channels in Lung Diseases.
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil.
Palliative care and cystic fibrosis: Opportunities for growth.
Sustained recovery of exocrine pancreatic function in a teenager with cystic fibrosis treated with ivacaftor.
Clinical Outcomes Associated with Burkholderia cepacia Complex Infection in Patients with Cystic Fibrosis.
Macrophage dysfunction in cystic fibrosis: Nature or nurture?
R560S: A class II CFTR mutation that is not rescued by current modulators.
Positive clinical response to ivacaftor treatment in an individual with the CFTR genotype F508del/V456A.
A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes.
A high prevalence of genetic polymorphisms in idiopathic and alcohol-associated chronic pancreatitis patients in Ireland.
Dispersal of Epithelium-Associated Pseudomonas aeruginosa Biofilms.
DNA Methylation Epigenetically Regulates Gene Expression in Burkholderia cenocepacia and Controls Biofilm Formation, Cell Aggregation, and Motility.
Cystic fibrosis diagnosed by state newborn screening: Or is it?
Mode of nitric oxide delivery affects antibacterial action.
Autophagy and Others Respiratory Diseases.
Antibiotic adjuvant therapy for pulmonary infection in cystic fibrosis.
Dietary interventions for phenylketonuria.
Users’ Experiences of a Mobile Health Self-Management Approach for the Treatment of Cystic Fibrosis: Mixed Methods Study.
Distinctive lipid signatures of bronchial epithelial cells associated with cystic fibrosis drugs, including Trikafta.
Antibiotic Adverse Reactions in Adult Cystic Fibrosis Patients.
A Randomized, Double-Blind, Parallel Group, Placebo-Controlled Study Investigating the Optimal Dose Regimen, Efficacy, and Safety of Adding Oral Cysteamine in Adult Patients with Cystic Fibrosis (C…
Role of Tobramycin in the Induction and Maintenance of Viable but Non-Culturable Pseudomonas aeruginosa in an In Vitro Biofilm Model.
Deoxyribonucleases and Their Applications in Biomedicine.
Sphingolipids and plasma membrane hydrolases in human primary bronchial cells during differentiation and their altered patterns in cystic fibrosis.
Predictors of long-term employment among patients with cystic fibrosis undergoing lung transplantation.
Duplex real-time PCR assay for the simultaneous detection of Achromobacter xylosoxidans and Achromobacter spp.
SARS-CoV-2 (COVID-19) and Cystic Fibrosis.
Hypoglycemia and Islet Dysfunction following Oral Glucose Tolerance Testing in Pancreatic Insufficient Cystic Fibrosis.
Non-Tuberculous Mycobacterial Diseases in Children.
Unilateral upper lung-field pulmonary fibrosis radiologically consistent with pleuroparenchymal fibroelastosis after thoracic surgery: Clinical and radiological courses with autopsy findings.
Pseudomonas aeruginosa PA14 enhances the efficacy of norfloxacin against Staphylococcus aureus Newman biofilms.
Setting a new standard in cystic fibrosis newborn screening illustrates controversial issues as new data emerge.
Association between habitual physical activity (HPA) and sleep quality in patients with cystic fibrosis.
PDGFA gene rs9690350 polymorphism increases biliary atresia risk in Chinese children.
Combining bacteriophages with cefiderocol and meropenem/vaborbactam to treat a pan-drug resistant Achromobacter species infection in a pediatric cystic fibrosis patient.
Urinary System and Renal Involvement in Children With Cystic Fibrosis.
Molecular characterization of Mycobacterium abscessus subspecies isolated from patients attending an Italian Cystic Fibrosis Centre.
Network-based characterization of disease-disease relationships in terms of drugs and therapeutic targets.
Promoting Emotional Wellness in Children with CF, Part I: Child And Family Resilience.
GCK-MODY in a child with cystic fibrosis: the doubt of the treatment plan.
Non-contrast Lung Perfusion Mapping Applied for New Insights in Cystic Fibrosis
Rational use of mucoactive medications to treat pediatric airway disease.
Diffusion and Characterization of Pseudomonas aeruginosa Aminoglycoside Resistance in an Italian Regional Cystic Fibrosis Centre.
Incorporating Patient and Caregiver Feedback into Lung Transplant Referral Guidelines for Individuals with Cystic Fibrosis – Preliminary Findings from a Novel Paradigm.
Discordant courses of COVID-19 in a cohabiting couple of lung transplant recipients.
Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR.
Cystic fibrosis-related diabetes: an update on pathophysiology, diagnosis, and treatment.
Bugs, breathing and blood pressure: Microbiota-gut-brain axis signalling in cardiorespiratory control in health and disease.
Screening for hearing loss in adults with CF: does it make sense?
Cholestasis in Benign Recurrent Intrahepatic Cholestasis 2.
You are my death: the shattered temporalities of zombie time.
High Prevalence of Triazole-Resistant Aspergillus fumigatus sensu stricto in an Argentinean Cohort of Patients with Cystic Fibrosis.

Steps Toward Cell Therapy for Cystic Fibrosis.
Integration of Mental Health Screening and Treatment into Cystic Fibrosis Clinics: Evaluation of Initial Implementation in 84 Programs Across the United States.
Impact of Socioeconomic Position on Access to the US Lung Transplant Waiting List in a Matched Cystic Fibrosis Cohort.
Investigating REPAIRv2 as a Tool to Edit CFTR mRNA with Premature Stop Codons.
Practitioner Due Diligence: Real-World Lumacaftor/Ivacaftor Use.
Therapeutic Drug Monitoring of Inhaled Tobramycin in a Post-Hematopoietic Cell Transplant Patient With Bronchiolitis Obliterans and End-Stage Renal Disease.
Airway complications after lung transplantation: benefit of a conservative bronchoscopy strategy.
Inhalable nano-composite microparticles with enhanced dissolution and superior aerosol performance.
Retinal Whitening After Lung Transplant for Cystic Fibrosis.
Is Endoscopic Sinus Surgery Beneficial Post Lung Transplant in Cystic Fibrosis Patients?
Implementation Strategies for Depression and Anxiety Screening in a Pediatric Cystic Fibrosis Center: A Quality Improvement Project.
Clinical Effectiveness of Lumacaftor/Ivacaftor in Cystic Fibrosis Patients Homozygous for F508del-CFTR.
Untargeted Metagenomic Investigation of the Airway Microbiome of Cystic Fibrosis Patients with Moderate-Severe Lung Disease.
Role of Curcumin and (-)-Epigallocatechin-3-O-Gallate in Bladder Cancer Treatment: A Review.
Objective Measures of Vest Therapy Adherence Among Pediatric Subjects With Cystic Fibrosis.
Treatment of nontuberculous mycobacterial pulmonary disease: an official ATS/ERS/ESCMID/IDSA clinical practice guideline.
Parallel Analysis of Cystic Fibrosis Sputum and Saliva Reveals Overlapping Communities and an Opportunity for Sample Decontamination.
Epithelial vectorial ion transport in cystic fibrosis: Dysfunction, measurement, and pharmacotherapy to target the primary deficit.
Pulmonary Aspergillosis: An Evolving Challenge for Diagnosis and Treatment.
Mucosal bacterial vaccines in clinical practice – a novel approach to an old problem?
Vav3 Mediates Pseudomonas aeruginosa Adhesion to the Cystic Fibrosis Airway Epithelium.
Using NPT to Evaluate Providing PPC as ELNEC-PPC WBT for Nurses
Clinical and Genotypical Features of False-Negative Patients in 26 Years of Cystic Fibrosis Neonatal Screening in Tuscany, Italy.
Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions.
Cystic Fibrosis: Overview of the Current Development Trends and Innovative Therapeutic Strategies.
KLF4 Acts as a wt-CFTR Suppressor through an AKT-Mediated Pathway.
Pro con debates in clinical medicine infection prevention and control in cystic fibrosis: One size fits all? The argument in favour.
Scnn1b-transgenic BALB/c mice as a model of Pseudomonas aeruginosa infections of the cystic fibrosis lung.
Maintenance tobramycin primarily affects untargeted bacteria in the CF sputum microbiome.
Apolipoprotein E polymorphism and vitamin K status in cystic fibrosis patients not supplemented with vitamin K.
The histone-like protein AlgP regulon is distinct in mucoid and nonmucoid Pseudomonas aeruginosa and does not include alginate biosynthesis genes.
Mental health and cystic fibrosis: Time to move from secondary prevention to predictive medicine.
Polyethylene Glycol and Intestinal Inflammation in Cystic Fibrosis
Pharmacokinetics of Omadacycline in Cystic Fibrosis
Looping of upstream cis-regulatory elements is required for CFTR expression in human airway epithelial cells.
Urine lipoarabinomannan as a marker for low-risk of NTM infection in the CF airway.
Methods for Extraction and Detection of Pf Bacteriophage DNA from the Sputum of Patients with Cystic Fibrosis.
Paracellular bicarbonate flux across human cystic fibrosis airway epithelia tempers changes in airway surface liquid pH.
Treatment of Nontuberculous Mycobacterial Pulmonary Disease: An Official ATS/ERS/ESCMID/IDSA Clinical Practice Guideline: Executive Summary.
Characterization of the Mechanism of Action of RDR01752, a Novel Corrector of F508del-CFTR.
High-refined carbohydrate diet leads to polycystic ovary syndrome-like features and reduced ovarian reserve in female rats.
[Cystic fibrosis and computed tomography of the lungs].
Ubiquitination of Disease-Causing CFTR Variants in a Microsome-Based Assay.
The positive effect of selective prostaglandin E2 receptor EP2 and EP4 blockade on cystogenesis in vitro is counteracted by increased kidney inflammation in vivo.
Evaluation of airway and circulating inflammatory biomarkers for cystic fibrosis drug development.
BAL inflammatory markers can predict pulmonary exacerbations in children with cystic fibrosis.
Dry powder aerosol containing muco-inert particles for excipient enhanced growth pulmonary drug delivery.
Inhaled antibiotics therapy for stable non-cystic fibrosis bronchiectasis: a meta-analysis.
Quantification of muco-obstructive lung disease variability in mice via laboratory X-ray velocimetry.
Altered iron metabolism in cystic fibrosis macrophages: the impact of CFTR modulators and implications for Pseudomonas aeruginosa survival.
A Case of Persistent Muscle Cramps in an American Football Player With Cystic Fibrosis.
Hematopoietic stem cell transplantation for people with sickle cell disease.
Hemoglobin A1c and fructosamine correlate in a patient with sickle cell disease and diabetes on chronic transfusion therapy.
Multidrug-resistant bacteria in lung transplantation.
Editorial for “Echo Time-Dependence of Observed Lung T1 in Patients With Cystic Fibrosis and Correlation With Clinical Metrics”.
COVID-19 network: the response of an Italian reference Institute to research challenges about a new pandemia.
Macrocyclic Transmembrane Anion Transporters via a One-Pot Condensation Reaction.
Cystic fibrosis: NHS England strikes deal to offer triple combination treatment.
Echo Time-Dependence of Observed Lung T1 in Patients With Cystic Fibrosis and Correlation With Clinical Metrics.
Preoperative blood transfusions for sickle cell disease.
Activated L-Arginine/Nitric Oxide Pathway in Pediatric Cystic Fibrosis and Its Association with Pancreatic Insufficiency, Liver Involvement and Nourishment: An Overview and New Results.
COVID-19 meets Cystic Fibrosis: for better or worse?
The changing demography of the cystic fibrosis population: forecasting future numbers of adults in the UK.
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer.
CFTR Regulates the Proliferation, Migration and Invasion of Cervical Cancer Cells by Inhibiting the NF-κB Signalling Pathway.
The Use of Exaggerative Language in News Articles About Cystic Fibrosis Therapies : Exaggerative Language Describing Cystic Fibrosis Therapies.
The Dance of Cystic Fibrosis: Experiences of Living with Cystic Fibrosis as an Adult.
Novel Therapies for Treatment of Resistant and Refractory Nontuberculous Mycobacterial Infections in Patients with Cystic Fibrosis.
Enhancing Care for Individuals with Advanced Cystic Fibrosis Lung Disease.
Evaluation of the psychological status of mothers of children with cystic fibrosis and the relationship between children’s clinical status.
Does Pseudomonas aeruginosa Colonization Affect Exercise Capacity in CF?
JCF Year in Review.
Fibrocyte accumulation in the lungs of cystic fibrosis patients.
No detection of SARS-CoV-2 in cystic fibrosis patients at the Regional (Lazio) Reference Center for CF in Italy.
Effects of Controlled Voluntary Increase in the Ventilatory Demand on Respiratory System Resistance in Healthy and Non-Cystic Fibrosis Bronchiectasis Subjects: A Cross-Sectional Study.
Religious coping and psychosocial adjustment in patients with cystic fibrosis.
Comparison of Vancomycin Pharmacokinetics in Cystic Fibrosis Patients Pre and Post-lung Transplant.
Spontaneously Occurring Small-Colony Variants of Staphylococcus aureus Show Enhanced Clearance by THP-1 Macrophages.
First Case of Brucella Pneumonia in a Lung Transplant Patient: Case Report and Review of the Literature.
Characterization of Ancestral Origin of Cystic Fibrosis of Patients with New Reported Mutations in CFTR.
Chronic rhinosinusitis in patients with cystic fibrosis-Current management and new treatments.
Cystic Fibrosis Remote Monitoring System
Matrix-assisted laser desorption/ionization time-of-flight MS for the accurate identification of Burkholderia cepacia complex and Burkholderia gladioli in the clinical microbiology laboratory.
P. aeruginosa Infected 3D Co-Culture of Bronchial Epithelial Cells and Macrophages at Air-Liquid Interface for Preclinical Evaluation of Anti-Infectives.
Ileocecal Valve Removal Reverses the Constipating Effects of Loperamide in Rats.
The Emergence of Elastography for Cystic Fibrosis Liver Disease.
Disrupted local innervation results in less VIP expression in CF mice tissues.
Acid-base disturbances in dehydrated patients with cystic fibrosis : four case reports with review of literature.
Comparison of Multiple Breath Washout and Spirometry in Children with Primary Ciliary Dyskinesia and Cystic Fibrosis and Healthy Controls.
Risk factors for nontuberculous mycobacterial isolation in patients with cystic fibrosis: a meta-analysis.
Evidence of Small Airways Disease and the Immediate Effects of Lumacaftor/Ivacaftor in Children with Cystic Fibrosis.
Single Cell Transcriptional Archetypes of Airway Inflammation in Cystic Fibrosis.
Magnetic Resonance Imaging and Neuro-Psychological Testing In Cystic Fibrosis
Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function.
Novel Imaging Techniques for Cystic Fibrosis Lung Disease.
Technical validity and usability of a novel smartphone connected spirometry device for pediatric patients with asthma and cystic fibrosis.
Tezacaftor/ivacaftor in people with cystic fibrosis who stopped lumacaftor/ivacaftor due to respiratory adverse events.
Lumacaftor/ivacaftor improves liver cholesterol metabolism but does not influence hypocholesterolemia in patients with cystic fibrosis.
Complete Genome Sequence of Pseudomonas aeruginosa Strain AA2 (LMG 27630), an Early Isolate Recovered from the Airway of a German Cystic Fibrosis Patient.
Luteolin reduces fluid hypersecretion by inhibiting TMEM16A in interleukin-4 treated Calu-3 airway epithelial cells.

Recurrent hypertrophic pulmonary osteoarthropathy in an adult with bronchiectasis.
Ion transport mechanisms for smoke inhalation-injured airway epithelial barrier.
Periodontal condition and periodontal risk assessment in adult patients with cystic fibrosis.
Survey of Patients with Cystic Fibrosis and Caregivers Decisions Regarding CFTR Modulators.
The Remaining Barriers to Normalcy in CF: Advances in Assessment of CF Lung Disease.
Lipopolymers and lipids from lung surfactants in association with N-acetyl-l-cysteine: characterization and cytotoxicity.
Cystic Fibrosis-Related Pancreatic Cysts Decrease in Size and Number Upon Treatment With Cystic Fibrosis Transmembrane Conductance Regulator Modulators.
Pediatric lung transplantation: Dynamics of the microbiome and bronchiolitis obliterans in cystic fibrosis.
A fly model of cystic fibrosis.
The Natural Polypeptides as Significant Elastase Inhibitors.
Impact of Coexistence Phenotype Between Staphylococcus aureus and Pseudomonas aeruginosa Isolates on Clinical Outcomes Among Cystic Fibrosis Patients.
Cystic Fibrosis: Fighting Together Against Coronavirus Infection.
Management and outcomes of Burkholderia cepacia complex bacteremia in patients without cystic fibrosis: a retrospective observational study.
Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR.
Understanding the interplay between factors that influence bone mineral density in CF.
Development and Implementation of Clinical Outcome Measures for Automated Collection Within Specialty Pharmacy Practice.
Cardiovascular Risk and Cardiovascular Health Behaviours in the Transition from Childhood to Adulthood.
Differential Chloride Secretory Capacity in Transepithelial Ion Transport Properties in Chronic Rhinosinusitis.
Lung clearance index-triggered intervention in children with cystic fibrosis – A randomised pilot study.
High-Resolution Longitudinal Dynamics of the Cystic Fibrosis Sputum Microbiome and Metabolome through Antibiotic Therapy.
Genotypic and Phenotypic Diversity of Staphylococcus aureus Isolates from Cystic Fibrosis Patient Lung Infections and Their Interactions with Pseudomonas aeruginosa.
Decades of cough: delayed recognition of atypical cystic fibrosis in an adult patient.
FACTORS ASSOCIATED TO QUALITY OF LIFE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS.
A Phase 3, 2-Arm, Open-label Study to Evaluate the Safety and Pharmacodynamics of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Init…
Associations between Pretend Play, Psychological Functioning and Coping Strategies in Pediatric Chronic Diseases: A Cross-Illness Study.
Single Lung Transplantation With Concomitant Cardiac Surgery in a Patient With Cystic Fibrosis: A Case Report.
Diagnostic performance and role of the contrast enema for low intestinal obstruction in neonates.
Cytoskeleton regulators CAPZA2 and INF2 associate with CFTR to control its plasma membrane levels under EPAC1 activation.
CFTR deficiency causes cardiac dysplasia during zebrafish embryogenesis and is associated with dilated cardiomyopathy.
Fetal Meconium Peritonitis – Prenatal Findings and Postnatal Outcome: A Case Series, Systematic Review, and Meta-Analysis.
CRISPR Genome Editing Technology and its Application in Genetic Diseases: A Review.
CFTR modulator therapies – Effect on life expectancy in people with cystic fibrosis.
Influenza B outbreak at an adult cystic fibrosis centre – Clinical impact and factors influencing spread.
In vitro 3D culture lung model from expanded primary cystic fibrosis human airway cells.
Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline.
Molecular biology of BPIFB1 and its advances in disease.
Metabolomic Description of Ivacaftor Elevating Polymyxin B Mediated Antibacterial Activity in Cystic Fibrosis Pseudomonas aeruginosa.
Interventions for treating intrahepatic cholestasis in people with sickle cell disease.
Neuropsychological Outcomes Following Pediatric Lung Transplantation.
Clinical features related to hospital expenses for non-cystic fibrosis bronchiectasis in China.
Newborn screening for galactosaemia.
Impact of myalgic encephalomyelitis on treatment of comorbidities: A lived experience.
Effects of CFTR modulators on pharmacokinetics of tobramycin during acute pulmonary exacerbations in the pediatric cystic fibrosis population.
Recombinant Acid Ceramidase Reduces Inflammation and Infection in Cystic Fibrosis.
SAD and MAD of Inhaled AR-501 in Health Adults and P. Aeruginosa Infected Cystic Fibrosis Subjects
Antimicrobial resistance: Concerns of healthcare providers and people with CF.
Haplotype analysis of the CFTR gene on normal and mutant CFTR genes.
Parotitis due to Burkholderia cepacia Infection after Autologous Hematopoietic Stem Cell Transplantation.
MiRNA Expression Profile in the Airways is Altered during Pulmonary Exacerbation in Children with Cystic Fibrosis-A Preliminary Report.
Efficacy and safety of aerosolized intra-tracheal dornase alfa administration in patients with SARS-CoV-2-induced acute respiratory distress syndrome (ARDS): a structured summary of a study protocol for a randomised controlled trial.
In silico integrative analysis predicts relevant properties of exotoxin-derived peptides for the design of vaccines against Pseudomonas aeruginosa.
Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging.
Acrodermatitis Enteropathica as a Presentation of Cystic Fibrosis in an Infant.
Enigmatic variations: the many facets of CFTR function in the heart.
Unusual presentation of CF in an infant.
Cell surface remodeling of Mycobacterium abscessus under cystic fibrosis airway growth conditions.
Data driven decision making to characterize clinical personas of parents of children with cystic fibrosis: a mixed methods study.
Clinical service delivery of non-invasive prenatal diagnosis (NIPD) by relative haplotype dosage (RHDO) for single gene disorders.
Phenotypic characterization of trimeric autotransporter adhesin-defective bcaC mutant of Burkholderia cenocepacia: cross-talk towards the histidine kinase BCAM0218.
Epithelial Vasopressin Type-2 Receptors Regulate Myofibroblasts by a YAP-CCN2-Dependent Mechanism in Polycystic Kidney Disease.
CFTR targeted therapies: recent advances in cystic fibrosis and possibilities in other diseases of the airways.
[Revolution in the treatment of cystic fibrosis].
[Allergic bronchopulmonary aspergillosis].
Adherence and barriers to general and respiratory exercises in Cystic Fibrosis.
A Case Report of Cystic Fibrosis Complicated by Burkholderia Cepacia and Cutaneous Vasculitis.
A Phase 2, Randomized, Double-blind Study to Evaluate the Efficacy and Safety of VX-561 in Subjects Aged 18 Years and Older With Cystic Fibrosis
Cystic fibrosis-related diabetes: The unmet need.
Novel Administration of Clofazimine for the Treatment of Mycobacterium avium Infection.
Peripheral type squamous cell carcinoma of the lung: clinicopathologic characteristics in comparison to the central type.
Changing clinical characteristics of non-cystic fibrosis bronchiectasis in children.
Newborn screening alone insufficient to improve pulmonary outcomes for cystic fibrosis.
Cystic Fibrosis mutation W19X in Tunisia: Second case identified.
Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations.
Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment.
UPR modulation of host immunity by P. aeruginosa in cystic fibrosis.
Real-life experience with high-frequency chest wall oscillation vest therapy in adults with non-cystic fibrosis bronchiectasis.
Navigating School Reentry in Lung Transplant Recipients With Cystic Fibrosis.
Characterization of clinical and genetic spectrum of Chinese patients with cystic fibrosis.
Immunotyping of clinically divergent p.Phe508del homozygous monozygous cystic fibrosis twins.
The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis.
Comparing effects of low glycemic index/high-fat, high-calorie diet and high-fat, high-calorie diet on cytokine levels of patients with cystic fibrosis: A randomized controlled clinical trial.
Association of IVS6A GATT polymorphism of CFTR gene with cystic fibrosis: first study in CF and normal Tunisian population.
Effect of acupuncture on chronic bronchitis: A protocol for systematic review and meta-analysis.
Impact at school age of early chronic methicillin-sensitive Staphylococcus aureus infection in children with cystic fibrosis.
The role of inflammation in cystic fibrosis pulmonary exacerbations.
Pseudomonas aeruginosa and children with cystic fibrosis.
Pseudomonas aeruginosa strain sharing in early infection among children with cystic fibrosis.
Lysophosphatidic Acid Increases Maturation of Brush Borders and SGLT1 activity in MYO5B-deficient Mice, a Model of Microvillus Inclusion Disease.
Clinimetric evaluation of muscle function tests for individuals with cystic fibrosis: A systematic review.
Defective BACH1/HO-1 regulatory circuits in cystic fibrosis bronchial epithelial cells.
Tracking of Infused Mesenchymal Stem Cells in Injured Pulmonary Tissue in Atm-Deficient Mice.
Development of an Innovative, Carrier-Based Dry Powder Inhalation Formulation Containing Spray-Dried Meloxicam Potassium to Improve the In Vitro and In Silico Aerodynamic Properties.
An 8 week open-label interventional multicenter study to explore the lung clearance index as endpoint for clinical trials in cystic fibrosis patients ≥8 years of age, chronically infected with Pseudomonas aeruginosa.
The Impact of Airway Complications on Survival Among Lung Transplant Recipients.
Comparison of Functional Free-Breathing Pulmonary 1H and Hyperpolarized 129Xe Magnetic Resonance Imaging in Pediatric Cystic Fibrosis.
Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review.
Dietary Macronutrient Distribution and Nutrition Outcomes in Persons with Cystic Fibrosis: An Evidence Analysis Center Systematic Review.
Visuomotor Reaction Time and Dynamic Balance in Children with Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis: A Case-Control Study.
Infections Within the First Month After Pediatric Lung Transplantation: Epidemiology and Impact on Outcomes.
Childhood rare lung disease in the 21st century: “-omics” technology advances accelerating discovery.

Comparative In Vitro Activities of Meropenem in Combination with Colistin, Levofloxacin, or Chloramphenicol Against Achromobacter xylosoxidans Strains Isolated from Patients with Cystic Fibrosis.
Possible metabolic switch between environmental and pathogenic Pseudomonas aeruginosa strains: 1H NMR based metabolomics study.
Evaluation of a New Culture Protocol for Enhancing Fungal Detection Rates in Respiratory Samples of Cystic Fibrosis Patients.
Appropriate age at solid introduction: is EFSA extending the individualised window on a sounding scientific evidence?
Healthcare reassessment in a pandemics time: challenges for CF.
Cystic fibrosis drug trial design in the era of CFTR modulators associated with substantial clinical benefit: stakeholders’ consensus view.
Fungal lung disease.
A Core Genome Multilocus Sequence Typing Scheme for Pseudomonas aeruginosa.
Resolvin D1 Reduces Lung Infection and Inflammation Activating Resolution in Cystic Fibrosis.
Characterization of Burkholderia cepacia Complex Core Genome and the Underlying Recombination and Positive Selection.
Ultrasound features of immature ovarian teratomas: Case series and review of literature.
Effect of the COVID-19 pandemic on anxiety among children with cystic fibrosis and their mothers.
Hyaluronic Acid for the Treatment of Airway Diseases in Children: Little Evidence for Few Indications.
Elevated exopolysaccharide levels in Pseudomonas aeruginosa flagellar mutants have implications for biofilm growth and chronic infections.
Myeloid CFTR loss-of-function causes persistent neutrophilic inflammation in cystic fibrosis.
Building global development strategies for cf therapeutics during a transitional cftr modulator era.
Benchmarking service provision, scope of practice, and skill mix for physiotherapists in adult cystic fibrosis care delivery.
The Association of Antibiotic Duration With Successful Treatment of Community-Acquired Pneumonia in Children.
Caffeine Accelerates Cystic Kidney Disease in a Pkd1-Deficient Mouse Model
Human Sperm Capacitation Involves the Regulation of the Tyr-Phosphorylation Level of the Anion Exchanger 1 (AE1).
Review of Potential Pseudomonas Weaponry, Relevant to the Pseudomonas-Aspergillus Interplay, for the Mycology Community.
Utility of Second-Look Endoscopy with Debridement After Pediatric Functional Endoscopic Sinus Surgery in Patients with Cystic Fibrosis.
Palliative care in cystic fibrosis.
TREATMENT ADHERENCE AMONG CHILDREN AND ADOLESCENTS IN A CYSTIC FIBROSIS REFERENCE CENTER.
Standard versus biofilm antimicrobial susceptibility testing to guide antibiotic therapy in cystic fibrosis.
Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis.
Clonally Related Viable Nontuberculous Mycobacteria in Gastric Juice and Sputum in People with Cystic Fibrosis.
Reply to: Clonally Related Viable Nontuberculous Mycobacteria in Gastric Juice and Sputum in People with Cystic Fibrosis.
The role of rhinovirus infections in young children with cystic fibrosis.
Molecular Diagnosis and Genetic Counseling of Cystic Fibrosis and Related Disorders: New Challenges.
The Effect of Sodium Bicarbonate, a Beneficial Adjuvant Molecule in Cystic Fibrosis, on Bronchial Epithelial Cells Expressing a Wild-Type or Mutant CFTR Channel.
Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study.
Airway microbial diversity is decreased in young children with cystic fibrosis compared to healthy controls but improved with CFTR modulation.
Assessing the Performance of Dried-Blood-Spot DNA Extraction Methods in Next Generation Sequencing.
Gastrointestinal malignancy in cystic fibrosis.
Laboratory reporting on the clinical spectrum of CFTR p.Arg117His: Still room for improvement.
Eligibility of CFTR modulators for the adult-diagnosed cystic fibrosis population.
Impact of Clinical, Unit-Specific Guidelines on Dornase Alfa Use in Critically Ill Pediatric Patients Without Cystic Fibrosis.
Cystic fibrosis knowledge and practice among primary care physicians in southwest region, Saudi Arabia.
Bacterial pathogens from lower respiratory tract infections: A study from Western Rajasthan.
Pseudomonas aeruginosa antimicrobial susceptibility test (AST) results and pulmonary exacerbation treatment responses in cystic fibrosis.
Comment on “Effect of one-year lumacaftor-ivacaftor treatment on glucose tolerance abnormalities in cystic fibrosis patients”.
Pseudomonas aeruginosa biofilm eradication via nitric oxide-releasing cyclodextrins.
Azithromycin and ciprofloxacin have a chloroquine-like effect on respiratory epithelial cells.
Nebulized in-line endotracheal dornase alfa and albuterol administered to mechanically ventilated COVID-19 patients: A case series.
Tobacco smoke exposure in pediatric cystic fibrosis: A qualitative study of clinician and caregiver perspectives on smoking cessation.
Inhalation delivery technology for genome-editing of respiratory diseases.
Pharmacological approaches for targeting cystic fibrosis nonsense mutations.
Long term clinical outcome of cystic fibrosis pediatric patients presenting with meconium ileus.
Management of initial colonisations with Burkholderia species in France, with retrospective analysis in five cystic fibrosis Centres: a pilot study.
Cystic Fibrosis Presenting as Pseudo-Bartter Syndrome: An Important Diagnosis that is Missed!
Clinical effects of the three CFTR potentiator treatments curcumin, genistein and ivacaftor in patients with the CFTR-S1251N gating mutation.
CRISPRi-mediated functional analysis of lung disease-associated loci at non-coding regions.
Release mechanisms and molecular interactions of Pseudomonas aeruginosa extracellular DNA.
EFFECT OF POLYETHYLENE GLYCOL TREATMENT ON INTESTINAL INFLAMMATION ASSOCIATED WITH CYSTIC FIBROSIS IN CHILDREN
Intrinsic Abnormalities of Cystic Fibrosis Airway Connective Tissue Revealed by an In Vitro 3D Stromal Model.
Pharmacological modulation of mitochondrial calcium uniporter controls lung inflammation in cystic fibrosis.
[Abdominal imaging in cystic fibrosis].
Prevention of severe respiratory syncytial virus infection in the pediatric population in Mexico: position of a group of experts.
[News drugs and evolution towards personalized treatment for cystic fibrosis].
Vitamin K supplementation for cystic fibrosis.
Acceptance and Commitment Therapy vs. Supportive Psychotherapy With Cystic Fibrosis Patients
StatPearls
Combined Effect of CFTR Protein Modulator Drugs and Exercise in Cystic Fibrosis
Biochemical and computational study of an alginate lyase produced by Pseudomonas aeruginosa strain S21.
Ceftolozane/tazobactam for pulmonary exacerbation in a 63-year-old cystic fibrosis patient with renal insufficiency and an elevated MIC to Pseudomonas aeruginosa.
Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing.
Human Nasal Epithelial Organoids for Therapeutic Development in Cystic Fibrosis.
Gene Therapy for Cystic Fibrosis: Progress and Challenges of Genome Editing.
The Virulence Potential of Livestock-Associated Methicillin-Resistant Staphylococcus aureus Cultured from the Airways of Cystic Fibrosis Patients.
A Novel Inducible Prophage from Burkholderia Vietnamiensis G4 is Widely Distributed across the Species and Has Lytic Activity against Pathogenic Burkholderia.
Short-term effects of Lumacaftor/Ivacaftor (Orkambi™) on exertional symptoms, exercise performance, and ventilatory responses in adults with cystic fibrosis.
Metabolomics profiling of tobacco exposure in children with cystic fibrosis.
Development of the gut microbiota in early life: The impact of cystic fibrosis and antibiotic treatment.
TMEM16A deficiency: a potentially fatal neonatal disease resulting from impaired chloride currents.
Cardiac and Pulmonary Disorders and the Nervous System.
Inactivation of Rho GTPases by Burkholderia cenocepacia Induces a WASH-Mediated Actin Polymerization that Delays Phagosome Maturation.
Provision of information on transplantation to cystic fibrosis patients and their relatives: Overview of French practices and recommendations.
Disruption of the extracellular polymeric network of Pseudomonas aeruginosa biofilms by alginate lyase enhances pathogen eradication by antibiotics.
A longitudinal assessment of non-invasive biomarkers to diagnose and predict cystic fibrosis-associated liver disease.
National Newborn Screening for cystic fibrosis in the Republic of Ireland: genetic data from the first 6.5 years.
Carrier frequency of CFTR variants in the non-Caucasian populations by genome aggregation database (gnomAD)-based analysis.
Circulating CRP and calprotectin to diagnose CF pulmonary exacerbations.
Bacterial Community Interactions During Chronic Respiratory Disease.
Phage Therapy Application to Counteract Pseudomonas aeruginosa Infection in Cystic Fibrosis Zebrafish Embryos.
Metabolic Alkalosis and Cystic Fibrosis: A Case Report.
Haemoptysis management in cystic fibrosis: A case report and treatment pathway.
Single Lung Transplant vs Double Lung Transplant: A Single-Center Experience With Particular Consideration for Idiopathic Pulmonary Arterial Hypertension.
Oral prevalence and antifungal susceptibility of Candida species in cystic fibrosis patients.
Outcomes of pulmonary Mycobacterium abscessus infection.
Susceptibility of the Mycobacterium abscessus complex to drying: Implications for nebulizer hygiene in patients with cystic fibrosis.
Motor dysfunction of the gut in cystic fibrosis.
Testicular pain following initiation of elexacaftor/tezacaftor/ivacaftor in males with cystic fibrosis.
Extracorporeal Photopheresis as Induction Therapy after Lung Transplantation for Cystic Fibrosis: Interim Analysis.
The Effect of the Cystic Fibrosis Care Center on Lung Transplant Outcomes.
Donation after Euthanasia (DCD-V) Results in Excellent Long-Term Outcome after Lung Transplantation, Equal to Donation after Brain Death (DBD) and Circulatory Death (DCD-III).
Clinical Outcomes after Pediatric Lung Transplantation at Cystic Fibrosis Care Centers.
A Bumpy Road: Work Integration of People with Cystic Fibrosis after Lung Transplantation.
Prolonged Survival Following Lung Transplantation in the Absence of Conventional Immunosuppression.
“Awake” Extracorporeal Membrane Oxygenation as a Bridge to Lung Transplant.

Ten Years of ECP for CLAD: A Real Life Experience.
Successful Pseudomonas aeruginosa Eradication Improves Outcomes after Lung Transplantation.
Association between MRSA Colonization and Chronic Lung Allograft Dysfunction in Lung Transplantation for Cystic Fibrosis.
Utility of Comprehensive Assessment for Hepatic Fibrosis in Lung Transplant Candidates.
Use of Cyclosporine Leads to Poorer Outcomes in Patients with Cystic Fibrosis Following Lung Transplant.
Tacrolimus Time in Therapeutic Range and Level Variability are Not Associated with Acute Cellular Rejection in Lung Transplant Recipients.
Relationship between Phenotypic Characteristics from the Donors, Predictive Biomarkers from the Donor Grafts and the Development of Primary Graft Dysfunction in Lung Transplant Recipients.
Surface Antigens on Plasma Extracellular Vesicles of Cystic Fibrosis Patients Treated by Extracorporeal Photopheresis as Induction Therapy after Lung Transplantation: Preliminary Results of a Pilot Randomized Trial.
CF Lung Transplant Survival by Surrogate Markers for Socioeconomic Status.
Preoperative Trajectories in Cystic Fibrosis and Their Association with Short and Long Term Survival after Lung Transplantation.
Incorporating Body Composition into Lung Transplant Assessment Enhances the Detection of Malnutrition.
Outcome of Marginal Donors in Lung Transplantation, Stretching the Limits.
Exceptional LAS Requests in Eurotransplant: Analysis of an 8-year Effort to Improve Lung Allocation for Precarious Patients.
Early and Midterm Results of Lung Transplantation for Cystic Fibrosis with Donors over 65 Years Old.
Improved Survival after Repeat Lung Transplantation at Cystic Fibrosis Care Centers.
Shifting Paradigms Regarding the Role of Nontuberculous Mycobacteria in Patients with Cystic Fibrosis Advanced Lung Disease.
Discrepancy between Lung Function Measurements at Home and in the Hospital in Children with Asthma and CF.
Characterization of Respiratory Pathogens in Contemporary Lung Transplant Recipients.
Meeting Cystic Fibrosis Patient Needs in Transition to Lung Transplant.
Lung Transplantation after Allogeneic Stem Cell Transplantation: A Single-Centre Experience.
A Novel Pseudomonas aeruginosa Virulence Factor Predicts CLAD and Death after Lung Transplantation.
Decreased Immune Activation in Cystic Fibrosis Airway Epithelium Following Lung Transplant.
Increasing Relative Abundance of Pseudomonads Predicts Chronic Rejection after Lung Transplant.
The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery.
Lessons learnt from MyCyFAPP Project: Effect of cystic fibrosis factors and inherent-to-food properties on lipid digestion in foods.
Control the platelets, control the disease: A novel cystic fibrosis hypothesis.
Easy measurement of health related quality of life in patients with cystic fibrosis by the COPD assessment test (CAT) – A pilot study.
Quantitative phase imaging to study transmembrane water fluxes regulated by CFTR and AQP3 in living human airway epithelial CFBE cells and CHO cells.
Neonatal screening programme for CF: results from the Irish Comparative Outcomes Study (ICOS).
[Ileocolonic intussusception in an adolescent patient with cystic fibrosis].
PREMO Study: to Investigate Port REMoval Outcomes
Sleep disturbance and sleep insufficiency in primary caregivers and their children with cystic fibrosis.
Nontypeable Haemophilus influenzae Responds to Virus-Infected Cells with a Significant Increase in Type IV Pilus Expression.
Segniliparus rugosus from the sputum of a child with cystic fibrosis in Ecuador: challenges in bacterial identification.
CFTR promotes malignant glioma development via up-regulation of Akt/Bcl2-mediated anti-apoptosis pathway.
Pathophysiology of Hyperechogenic Bowel in Congenitally Human Cytomegalovirus Infected Fetuses.
A helper-dependent adenoviral vector rescues CFTR to wild type functional levels in CF epithelial cells harbouring class I mutations.
ILC2 Lung-Homing in Cystic Fibrosis Patients: Functional Involvement of CCR6 and Impact on Respiratory Failure.
Population pharmacokinetics of ceftazidime in critically ill children: impact of cystic fibrosis.
MRI Evaluation of Regional Lung Tidal Volumes in Severe Neonatal Bronchopulmonary Dysplasia.
[Dynamics of indicators of vitamin D status in children with cystic fibrosis of the Moscow region for 2016-2018].
Molecular characterization of methicillin-resistant Staphylococcus aureus from chronically colonized cystic fibrosis pediatric patients in Brazil.
Global assessment of the integrated stress response in CF patient-derived airway and intestinal tissues.
Pseudomonas aeruginosa Isolates From a Cohort of Mexican Children With Cystic Fibrosis Show Adaptation to a Chronic Phenotype.
Three-dimensional Ultrashort Echotime Magnetic Resonance Imaging for Combined Morphologic and Ventilation Imaging in Pediatric Patients With Pulmonary Disease.
Exercise testing for children with cystic fibrosis: A systematic review.
Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.
Identification of a Mutation in the Novel Compound Heterozygous CFTR in a Chinese Family with Cystic Fibrosis.
Portal Hypertension and Systemic Endothelial Function
Females with cystic fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males.
A 16-year retrospective study on fungal prevalence and diversity in patients with Cystic Fibrosis- Candida dubliniensis was associated with a decline in lung function.
50 Years Ago in TheJournalofPediatrics: Massive Hemoptysis in Patients with Cystic Fibrosis: When the Problem Is Solved with Prevention.
Distal Intestinal Obstruction Syndrome resolved by dissolution with Coca-Cola® via colonoscopy in a cystic fibrosis patient.
NICE guidance on diagnosis and management of cystic fibrosis.
The Aspergillus fumigatus secretome alters the proteome of Pseudomonas aeruginosa to stimulate bacterial growth: implications for co-infection .
A Phase 3, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor in Subjects Aged 6 Years and Older With Cystic Fibrosis, Homozy…
Antifungal Activity of Antimicrobial Peptides and Proteins against Aspergillus fumigatus.
Overcoming Immunological Challenges to Helper-Dependent Adenoviral Vector-Mediated Long-Term CFTR Expression in Mouse Airways.
An observational study of the lung clearance index throughout childhood in cystic fibrosis: Early years matter.
Working towards consensus in the management of pediatric chronic rhinosinusitis in cystic fibrosis.
Integrating latent classes in the Bayesian shared parameter joint model of longitudinal and survival outcomes.
IVS8-5T Allele of CFTR is the Risk Factor in Chronic Pancreatitis, Especially in Idiopathic Chronic Pancreatitis.
Extracellular DNA release, quorum sensing, and PrrF1/F2 small RNAs are key players in Pseudomonas aeruginosa tobramycin-enhanced biofilm formation.
Correctors modify the bicarbonate permeability of F508del-CFTR.
The Pediatric Sinus Staging System: A Computed Tomography-Based Approach to Grading Pediatric Sinus Disease.
Limitations of regional ventilation inhomogeneity indices in children with cystic fibrosis.
The intestinal virome in children with cystic fibrosis differs from healthy controls.
Clinical characteristics of rare CFTR mutations causing cystic fibrosis in Polish population.
Cystic Fibrosis-related Liver Disease is Associated With Increased Disease Burden and Endocrine Comorbidities.
Effectivity of Pancreatic Enzyme Replacement Therapy in Malnourished Children.
Treatment of human T-cell acute lymphoblastic leukemia cells with CFTR inhibitor CFTRinh-172.
Correction to: Assessment of stigma in patients with cystic fibrosis.
Chronic cough in adults.
Pharmacophore-Based Design of New Chemical Scaffolds as Translational Readthrough-Inducing Drugs (TRIDs).
Assessing Human Airway Epithelial Progenitor Cells for Cystic Fibrosis Cell Therapy.
Analysis of CFTR Mutation Spectrum in Ethnic Russian Cystic Fibrosis Patients.
Multivariate joint modeling to identify markers of growth and lung function decline that predict cystic fibrosis pulmonary exacerbation onset.
How do cystic fibrosis patients experience parenthood? A systematic review.
NtrBC Regulates Invasiveness and Virulence of Pseudomonas aeruginosa During High-Density Infection.
Ceftolozane/Tazobactam for Treating Children With Exacerbations of Cystic Fibrosis Due to Pseudomonas aeruginosa: A Review of Available Data.
TNFα and IL-17 Alkalinize Airway Surface Liquid through CFTR and Pendrin.
ERp29 as a regulator of Insulin biosynthesis.
Functional rescue of an ABCB11 mutant by ivacaftor: a new targeted pharmacotherapy approach in bile salt export pump deficiency.
Identification of FDA-approved antivirulence drugs targeting the Pseudomonas aeruginosa quorum sensing effector protein PqsE.
Mycobacterium abscessus clearance by neutrophils is independent of autophagy.
A novel combination of CYP51A mutations confers pan-azole resistance in Aspergillus fumigatus.
Cystic Fibrosis Patients Infected With Epidemic Pseudomonas aeruginosa Strains Have Unique Microbial Communities.
Changes in Mucociliary Clearance Over Time in Children with Cystic Fibrosis.
Small Molecule Channels Harness Membrane Potential to Concentrate Potassium in trk1Δtrk2Δ Yeast.
Dissecting the cellular specificity of smoking effects and reconstructing lineages in the human airway epithelium.
Laboratory biomarkers for lung disease severity and progression in cystic fibrosis.
Cystic fibrosis-associated liver disease in children.
Prospective evaluation of vascular changes in acute respiratory infections in children with cystic fibrosis.
Plasma pharmacokinetics of ceftolozane/tazobactam in pediatric patients with cystic fibrosis.
Pulmonary fibrosis secondary to COVID-19: a call to arms?
A Pilot Study of Mindfulness-Based Cognitive Therapy to Improve Well-Being for Health Professionals Providing Chronic Disease Care.
Soy Isoflavones and Gastrointestinal Health.
Transcriptomic and Proteostasis Networks of CFTR and the Development of Small Molecule Modulators for the Treatment of Cystic Fibrosis Lung Disease.
The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor.
Volumetric quantification of lung MR signal intensities using ultrashort TE as an automated score in cystic fibrosis.

Delivery of genome-editing biomacromolecules for treatment of lung genetic disorders.
A broad-spectrum antibacterial natural product from the cystic fibrosis isolate, Pantoea agglomerans Tx10.
An Innovative Lipidomic Workflow to Investigate the Lipid Profile in a Cystic Fibrosis Cell Line.
Breaking the diagnosis of cystic fibrosis to parents: A process not a one-off event.
The O2-independent pathway of ubiquinone biosynthesis is essential for denitrification in Pseudomonas aeruginosa.
Tablet and web-based audiometry to screen for hearing loss in adults with cystic fibrosis.
Longitudinal Associations of the Cystic Fibrosis Airway Microbiome and Volatile Metabolites: A Case Study.
Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas aeruginosa in cystic fibrosis.
Flexible link functions in a joint hierarchical Gaussian process model.
The Microbiome in Cystic Fibrosis Pulmonary Disease.
Bronchial Infection due to Pseudomonas Aeruginosa in Patients with Cystic Fibrosis Diagnosed in Neonatal Screening.
Optimal management of acute kidney injury in critically ill patients with invasive fungal infections being treated with liposomal amphotericin B.
Probiotics may be considered for children and adults with cystic fibrosis.
CFTR variant testing: a technical standard of the American College of Medical Genetics and Genomics (ACMG).
[Radicality of maxillary sinus surgery and size of the maxillary sinus ostium].
The Cystic Fibrosis Impact Questionnaire: qualitative development and cognitive evaluation of a new patient-reported outcome instrument to assess the life impacts of cystic fibrosis.
Air care: an ‘aerography’ of breath, buildings and bugs in the cystic fibrosis clinic.
Immunomodulation in Cystic Fibrosis: Why and How?
Synthesis and Therapeutic Applications of Iminosugars in Cystic Fibrosis.
Exposure of Mycobacterium abscessus to Environmental Stress and Clinically Used Antibiotics Reveals Common Proteome Response among Pathogenic Mycobacteria.
Morbidity and mortality in carriers of the cystic fibrosis mutation CFTR Phe508del in the general population.
Development of bacterial resistance during treatment with topical gentamicin for chronic rhinosinusitis in patients with cystic fibrosis and primary ciliary dyskinesis. Retrospective case series.
Improved Fmoc-solid-phase peptide synthesis of an extracellular loop of CFTR for antibody selection by the phage display technology.
Highly Efficient Gene Editing of Cystic Fibrosis Patient-Derived Airway Basal Cells Results in Functional CFTR Correction.
COVID-19 in lung-transplanted and cystic fibrosis patients: Be careful.
Xenon-129 Lung Magnetic Resonance Imaging: Study of Healthy Volunteers and Participants With Pulmonary Disease
The role of essential fatty acids in cystic fibrosis and normalizing effect of fenretinide.
Factors associated with severe lung disease in an adult population with cystic fibrosis: a single center experience.
Intestinal epithelial potassium channels and CFTR chloride channels activated in ErbB tyrosine kinase inhibitor diarrhea.
Comparison of the Efficacy of Comprehensive Respiratory Physiotherapy in Children With Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis
Novel bioinformatics quality control metric for next-generation sequencing experiments in the clinical context.
Phenotypes of Chronic Rhinosinusitis.
Combined Lung-Liver and Delayed Kidney Transplantation for Cystic Fibrosis Clinical Approach and Outcome: A Case Report.
Prevalence of constipation in cystic fibrosis patients: a systematic review of observational studies.
Genetic Variation Near chrXq22-q23 Is Linked to Emotional Functioning in Cystic Fibrosis.
COVID-19: A message of hope from a young girl with severe cystic fibrosis.
Comparison of microstructural alterations in the proximal aorta between aortic stenosis and regurgitation.
Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor.
Experience with Parent Follow-Up for Communication Outcomes after Newborn Screening Identifies Carrier Status.
Anti-inflammatory effects of lenabasum, a cannabinoid receptor type 2 agonist, on macrophages from cystic fibrosis.
Can lumacaftor-ivacaftor reverse glucose-tolerance abnormalities in cystic fibrosis?
Imaging of genetically-mediated pancreatitis.
Effectiveness and Safety of Omalizumab in Patients with Allergic Bronchopulmonary Aspergillosis Complicated by Chronic Bacterial Infection in the Airways.
The Detection of Bile Acids in the Lungs of Paediatric Cystic Fibrosis Patients Is Associated with Altered Inflammatory Patterns.
Bioorthogonal non-canonical amino acid tagging reveals translationally active subpopulations of the cystic fibrosis lung microbiota.
Oral-lung microbiome interactions in lung diseases.
Structural Consequences of the 1,2,3-Triazole as an Amide Bioisostere in Analogs of Cystic Fibrosis Drugs VX-809 and VX-770.
Genetic potential and height velocity during childhood and adolescence do not fully account for shorter stature in cystic fibrosis.
Antibiofilm activity of antifungal drugs, including the novel drug olorofim, against Lomentospora prolificans.
Cystic Fibrosis Transmembrane Conductance Regulator-dependent bicarbonate entry controls rat cardiomyocyte ATP release via pannexin1 through mitochondrial signalling and caspase activation.
Regulation of TMEM16A by CK2 and Its Role in Cellular Proliferation.
End-of-Life Care in Cystic Fibrosis: Comparing Provider Practices Based on Lung Transplant Candidacy.
Royal Society of Medicine Cystic Fibrosis Symposium 2019.
Average rate of lung function decline in adults with cystic fibrosis in the United Kingdom: Data from the UK CF registry.
ICER releases evidence report on elexacaftor/tezacaftor/ivacaftor for cystic fibrosis.
Introduction to Cystic Fibrosis for Mental Health Care Coordinators and Providers: Collaborating to Promote Wellness.
Improved residual fat malabsorption and growth in children with cystic fibrosis treated with a novel oral structured lipid supplement: A randomized controlled trial.
Palliative care skills in CF: Perspectives of adults with CF, caregivers, and CF care team members.
The Italian External Quality Assessment Program for Cystic Fibrosis Sweat Chloride Test: Does Active Participation Improve the Quality?
Antibiotic resistance in Pseudomonas aeruginosa and adaptation to complex dynamic environments.
Effect of Postprandial Insulin Administration of Faster-acting Insulin Analogue Versus Pre-prandial Administration of Acting-insulin Analogue in Cystic Fibrosis Related Diabetes
A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis.
An organoid model to assay the role of CFTR in the human epididymis epithelium.
Gene expression in human liver fibrosis associated with Echinococcus granulosus sensu lato.
Is extracorporeal membrane oxygenation withdrawal a safe option after double-lung transplantation?
A Phase 2a, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate Safety, Tolerability, and Effect of Inhaled SNSP113 in Adult Subjects with Cystic Fibrosis
Impact of Discontinuing Chronic Therapies in People With Cystic Fibrosis on Highly Effective CFTR Modulator Therapy
ACR Appropriateness Criteria® Hemoptysis.
Keep cystic fibrosis patients out of the hospital.
Manifestations of pulmonary aspergillosis in pediatrics.
Primary immunodeficiency disorders in children with Non-Cystic Fibrosis Bronchiectasis.
iPSC-Derived Intestinal Organoids from Cystic Fibrosis Patients Acquire CFTR Activity upon TALEN-Mediated Repair of the p.F508del Mutation.
Synergistic interactions of cadmium-free quantum dots embedded in a photosensitised polymer surface: efficient killing of multidrug-resistant strains at low ambient light levels.
Patient-Reported Outcome Measures in Cystic Fibrosis: Protocol for a Systematic Review.
Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update.
Novel Therapy of Bicarbonate, Glutathione and Ascorbic Acid Improves Cystic Fibrosis Mucus Transport.
General Asymmetric Synthesis of Densely Functionalized Pyrrolidines via Endo-Selective [3+2] Cycloaddition of β-Quaternary-Substituted Nitroalkenes and Azomethine Ylides.
OPTION 2: A Trial to Assess the Safety and Efficacy of MS1819 in Enteric Capsules in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis
Clinically relevant epithelial lining fluid concentrations of meropenem with ciprofloxacin provide synergistic killing and resistance suppression of hypermutable Pseudomonas aeruginosa in a dynamic biofilm model.
CFAP300: Mutations in Slavic Patients with Primary Ciliary Dyskinesia and a Role in Ciliary Dynein Arms Trafficking.
What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?
PROCEEDINGS OF THE 17th ITALIAN CONVENTION OF FFC INVESTIGATORS IN CYSTIC FIBROSIS : VERONA 14th-16th NOVEMBER 2019 Every year, the Italian Cystic Fibrosis Research Foundation (FFC) brings together all its funded researchers from across Italy and beyond, in a Convention where results from FFC projects are shared and debated. These projects are either newly funded, on-going or recently concluded research. The Proceedings of the 17th Italian Convention of FFC Investigators in Cystic Fibrosis report the results of the concluded research projects. Correspondence: Dr. F. Malvezzi (flaminia.malvezzi@fibrosicisticaricerca.it).
Achromobacter xylosoxidans cellular pathology is correlated with activation of a type III secretion system.
Clinical experience with carrier screening in a general population: support for a comprehensive pan-ethnic approach.
Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations.
Study of ARO-ENaC in Healthy Volunteers and in Patients With Cystic Fibrosis
What it takes to implement regular longitudinal multiple breath washout tests in infants with cystic fibrosis.
Regulation of Cl- Electrolyte Permeability in Epithelia by Active Traditional Chinese Medicine.
Rare cause of upper bowel obstruction arising in a 17-year-old boy with cystic fibrosis: Superior mesenteric artery (Wilkie’s) syndrome.
Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.
Sleep-disordered breathing and markers of morbidity in children and adolescents with cystic fibrosis.
Cystic fibrosis 2019: Year in review.
The relationship between the structure and toxicity of aminoglycoside antibiotics.
Generation of two human induced pluripotent stem cell lines derived from two juvenile nephronophthisis patients with NPHP1 deletion.
Evaluation of Droplet Digital Polymerase Chain Reaction (ddPCR) for the Absolute Quantification of Aspergillus species in the Human Airway.
Staphylococcus aureus Pathogenicity in Cystic Fibrosis Patients-Results from an Observational Prospective Multicenter Study Concerning Virulence Genes, Phylogeny, and Gene Plasticity.
The true panel of cystic fibrosis mutations in the Sicilian population.
Inhaled mannitol for cystic fibrosis.
Extracorporeal membrane oxygenation for grade 3 primary graft dysfunction after lung transplantation: Long-term outcomes.
Formative evaluation of a dashboard to support coproduction of healthcare services in cystic fibrosis.

The role of innate immunity in the long-term outcome of lung transplantation.
The pig as a model for immunology research.
Influence of pancreatic status, CFTR mutations, Staphylococcus aureus and/or Pseudomonas aeruginosa infection/colonization on lung function in cystic fibrosis during a 2-year follow-up period.
Genetic testing costs and compliance with clinical best practices.
A Feasibility Study of Urgent Implementation of Cystic Fibrosis Multidisciplinary Telemedicine Clinic in the Face of COVID-19 Pandemic: Single-Center Experience.
Repeatability of transient elastography in children.
STaph Aureus Resistance-Treat Early and Repeat (STAR-TER)
Drugs and Lactation Database (LactMed)
Measuring airway clearance outcomes in bronchiectasis: a review.
Disruption of Cross-Feeding Inhibits Pathogen Growth in the Sputa of Patients with Cystic Fibrosis.
Pilot study for cystic fibrosis neonatal screening: the Cuban experience.
Oscillating devices for airway clearance in people with cystic fibrosis.
Esophageal Motility Disorders Associated With Death or Allograft Dysfunction After Lung Transplantation? Results of a Retrospective Monocentric Study.
19F MRI of Ventilation in Subjects With Cystic Fibrosis Undergoing Treatment for Pulmonary Exacerbation
Drosophila as a model for studying cystic fibrosis pathophysiology of the gastrointestinal system.
Commensal Oral Rothia mucilaginosa Produces Enterobactin, a Metal-Chelating Siderophore.
2019 Year in Review: Aerosol Therapy.
The Role of Noninvasive Ventilation in Cystic Fibrosis: A Cochrane Review Summary With Commentary.
The association between serum albumin/prealbumin level and disease severity in non-CF bronchiectasis.
Are We Missing the Opportunity to Measure Muscle Mass on Computed Tomography Thorax?
Environmental fungal sensitisation associates with poorer clinical outcomes in COPD.
Allelic polymorphism shapes community function in evolving Pseudomonas aeruginosa populations.
Exercise and Cystic Fibrosis.
Gene therapy for haemophilia.
Three-dimensional Ultrashort Echo Time MRI for Functional Lung Imaging in Cystic Fibrosis.
Palliative drug treatments for breathlessness in cystic fibrosis.
Molecular mechanisms of action of naringenin in chronic airway diseases.
Ivacaftor decreases monocyte sensitivity to interferon-γ in people with cystic fibrosis.
Omics in CF – Where are we now?
Secondary Pulmonary Hypertension Among Patients Qualified for Lung Transplantation: Single-Center Study.
Combination antifungal therapy for Scedosporium species in cystic fibrosis.
Targeted Killing of Pseudomonas aeruginosa by Pyocin G Occurs via the Hemin Transporter Hur.
A Study Evaluating the Long-Term Safety of Elexacaftor Combination Therapy
A Phase 3 Randomized, Double-blind, Placebo-controlled Study to Evaluate the Safety and Efficacy of MEDI8897, a Monoclonal Antibody With an Extended Half-life Against Respiratory Syncytial Virus, i…
Comparison of MycoPrep and the new MYCO-TB kit: rapid and efficient digestion and decontamination of respiratory specimens for the detection of Mycobacteria.
[Computed tomography in children with cystic fibrosis: The role of an expiratory protocol].
Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis.
The specialist as primary care provider in CF.
Assessing the impact of cystic fibrosis on the antipyretic response of ibuprofen in children: physiologically-based modeling as a candle in the dark.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitylation as a Novel Pharmaceutical Target for Cystic Fibrosis.
Redesign of the Australian Cystic Fibrosis Data Registry: A multidisciplinary collaboration.
Expression of Cl- channels/transporters in nasal polyps.
A new future for patients with cystic fibrosis.
Cystic Fibrosis, CFTR, and Colorectal Cancer.
Mechanical Properties of Human Bronchial Epithelial Cells Expressing Wt- and Mutant CFTR.
Salivary Cytokines and Airways Disease Severity in Patients with Cystic Fibrosis.
Whole genome sequencing of Nontuberculous Mycobacterium (NTM) isolates from sputum specimens of co-habiting patients with NTM pulmonary disease and NTM isolates from their environment.
Penetrance is a critical parameter for assessing the disease liability of CFTR variants.
Dyslipidemia is not associated with the development of glucose intolerance or diabetes in cystic fibrosis.
Cell free DNA from respiratory pathogens is detectable in the blood plasma of Cystic Fibrosis patients.
The Contribution of Membrane Vesicles to Bacterial Pathogenicity in Cystic Fibrosis Infections and Healthcare Associated Pneumonia.
Rhinovirus Infection Is Associated With Airway Epithelial Cell Necrosis and Inflammation via Interleukin-1 in Young Children With Cystic Fibrosis.
Bilateral Pneumonia in a Patient with Chronic Bronchiectasis Caused by Achromobacter xylosoxidans Subspecies denitrificans.
Discovery of Cephalosporin-3´-Diazeniumdiolates that show Dual Antibacterial and Antibiofilm Effects against Pseudomonas aeruginosa Clinical Cystic Fibrosis Isolates and Efficacy in a Murine Respiratory Infection Model.
A Phase 1 / 2 Study to Evaluate the Safety, Tolerability, and Pharmacokinetics of PTI-808 in Healthy Adult Subjects and in Adults with Cystic Fibrosis
Chloride – The Underrated Ion in Nociceptors.
Microbial clues lead to a diagnosis of cystic fibrosis in late adulthood.
Chemical, Metabolic, and Cellular Characterization of a FtsZ Inhibitor Effective Against Burkholderia cenocepacia.
Progress in Model Systems of Cystic Fibrosis Mucosal Inflammation to Understand Aberrant Neutrophil Activity.
The effectiveness of a mobile high-frequency chest wall oscillation (HFCWO) device for airway clearance.
The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.
JCF – progress in 2018.
Innate Immune Signaling Contributes to Tubular Cell Senescence in the Glis2 Knockout Mouse Model of Nephronophthisis.
Dried blood spot-based metabolomic profiling in adults with cystic fibrosis.
CYSTIC FIBROSIS AS A CAUSE OF MALABSORPTION AND INCREASED REQUIREMENT OF L-THYROXINE.
Highly selective and sensitive online measurement of trace exhaled HCN by acetone-assisted negative photoionization time-of-flight mass spectrometry with in-source CID.
Endobronchial valve treatment of persistent alveolopleural fistulae in a patient with cystic fibrosis and empyema.
The bidirectional relationship between CFTR and lipids.
Evaluation of a five-year predicted survival model for cystic fibrosis in later time periods.
A novel hemizygous loss-of-function mutation in ADGRG2 causes male infertility with congenital bilateral absence of the vas deferens.
Fast chemical force microscopy demonstrates that glycopeptidolipids define nanodomains of varying hydrophobicity on mycobacteria.
StatPearls
StatPearls
Negative chronotropic and inotropic effects of lubiprostone on iPS cell-derived cardiomyocytes via activation of CFTR.
Serum KL-6 levels in Pulmonary Langerhans’ Cell Histiocytosis.
Integrin-β1 is required for the renal cystogenesis caused by ciliary defects.
Assessment of Selected Parameters of Liver Fibrosis and Inflammation in Patients with Diagnosed Cystic Fibrosis.
Encapsulated Methionine γ-Lyase: Application in Enzyme Prodrug Therapy of Pseudomonas aeruginosa Infection.
High-intensity interval training accelerates oxygen uptake kinetics and improves exercise tolerance for individuals with cystic fibrosis.
Can Point Shear Wave Elastography Be Used as an Early Indicator of Involvement?: Evaluation of the Pancreas and Liver in Children With Cystic Fibrosis.
Extracellular matrix, integrins, and focal adhesion signaling in polycystic kidney disease.
Overlooking the landscape of palliative care in cystic fibrosis.
Age-Dependent Progression in Lung Pathophysiology can be Prevented by Restoring Fatty Acid and Ceramide Imbalance in Cystic Fibrosis.
Decreased airway epithelial ion transport was associated with the severity of the respiratory syncytial virus infection and complications in infants.
Asymptomatic case of Covid-19 in an infant with cystic fibrosis.
Real-World Outcomes Among Patients with Cystic Fibrosis Treated with Ivacaftor: 2012-2016 Experience.
Outcomes of Children With Cystic Fibrosis Admitted to PICUs.
Celiac Disease in Patients with Cystic Fibrosis on Ivacaftor: A Case Series.
Impact of COVID-19 on people with cystic fibrosis.
CT score and correlation with lung function and microbiology of adult patients with cystic fibrosis with predominant I1234V genotype in Qatar.
Blood co-expression modules identify potential modifier genes of diabetes and lung function in cystic fibrosis.
Influence of Dominance and Drift on Lethal Mutations in Human Populations.
Lung transplantation as standard of care for advanced cystic fibrosis lung disease.
Spectrum of chronic lung allograft pathology in a mouse minor-mismatched orthotopic lung transplant model.
Burkholderia cepacia Complex Bacteria: a Feared Contamination Risk in Water-Based Pharmaceutical Products.
Genomic analysis of an Irish population of Mycobacterium abscessus complex collected between 2006 and 2017.
Electronic health record phenotypes associated with genetically regulated expression of CFTR and application to cystic fibrosis.
Prenatal diagnosis of rare genetic conditions at a tertiary care hospital in Karachi.
Lung compartment analysis assessed from N2 multiple-breath washout in children with cystic fibrosis.
[Mycobacterium abscessus complex representatives in patients with bronchopulmonary pathology: prevalence, peculiarities of cultivation and identification.]

Susceptibility of Staphylococcus aureus (MSSA & MRSA) to drying: Implications for nebuliser hygiene in patients with cystic fibrosis (CF).
The preclinical discovery and development of the combination of ivacaftor + tezacaftor used to treat cystic fibrosis.
Real-world evidence in cystic fibrosis modulator development: Establishing a path forward.
Resting energy expenditure in cystic fibrosis patients decreases after lung transplantation, which improves applicability of prediction equations for energy requirement.
The prevalence of viral infections in children with cystic fibrosis in a tertiary care center in Saudi Arabia.
Cooperation or Tension? Dyadic Coping in Cystic Fibrosis.
Inhaled tigecycline is effective against Mycobacterium abscessus in vitro and in vivo.
Thinking inside the box: nebulizer care, safe storage, and risk of infection in cystic fibrosis.
Optimization of western blotting for the detection of proteins of different molecular weights.
Gene therapy for cystic fibrosis paved the way for the use of AAV in gene therapy.
The assessment of pancreatic exocrine function in patients with inoperable pancreatic cancer: In need of a new gold-standard.
Regulation of Virulence by Two-component Systems in Pathogenic Burkholderia.
An Infant with Severe Anemia and Hypoalbuminemia.
The “Tango” of Problem Formulation: A patient/researcher reflection on an Action Design Research journey.
Target-Based Design of Promysalin Analogues Identifies a New Putative Binding Cleft in Succinate Dehydrogenase.
Treatment of allergic bronchopulmonary aspergillosis: from evidence to practice.
Chloride channels regulate differentiation and barrier functions of the mammalian airway.
Molecular Diagnostics of Cystic Fibrosis in Serbia: Our Approach to Meet the Diagnostic Challenges.
Improved Prognosis in Cystic Fibrosis: Consideration for Intensive Care During the COVID-19 Pandemic.
Regression of Peritubular Capillaries Coincides with Angiogenesis and Renal Cyst Growth in Experimental Polycystic Kidney Disease.
Ataluren/ivacaftor combination therapy: Two N-of-1 trials in cystic fibrosis patients with nonsense mutations.
Cystic fibrosis transmembrane conductance regulator function, not TAS2R38 gene haplotypes, predict sinus surgery in children and young adults with cystic fibrosis.
Cellular mechanisms underlying carbon monoxide stimulated anion secretion in rat epididymal epithelium.
Development of a database for the rapid and accurate routine identification of Schromobacter species by matrix-assisted laser desorption/ionization-time-of-flight mass spectrometry (MALDI-TOF MS).
Management of Sinusitis in the Cystic Fibrosis Patient.
The Impact of the CFTR Gene Discovery on Cystic Fibrosis Diagnosis, Counseling, and Preventive Therapy.
Cystic Fibrosis from Childhood to Adulthood: What Is New in Imaging Assessment?
CFTR gets together.
Monoacylglycerol Form of Omega-3s Improves Its Bioavailability in Humans Compared to Other Forms.
Pharmacological Inhibition and Activation of the Ca2+ Activated Cl- Channel TMEM16A.
Age and environmental exposures influence the fecal bacteriome of young children with cystic fibrosis.
Role of Stenotrophomonas maltophilia isolation in patients with non CF bronchiectasis.
Omega-3 fatty acid supplementation for cystic fibrosis.
Putting CHIP(s) on the Table: Introducing Nitrosothiols into the Arena of CFTR Modulation.
Caring for gender diverse youth with cystic fibrosis.
Bronchodilator Responsiveness in Children with Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis.
Functional Characterization of Clinical Isolates of the Opportunistic Fungal Pathogen Aspergillus nidulans.
Etiology, Clinical, Radiological, and Microbiological Profile of Patients with Non-cystic Fibrosis Bronchiectasis at a Tertiary Care Hospital of Pakistan.
Initiation of triple therapy maintenance treatment among patients with COPD.
Molecular and epidemiological analysis of a Burkholderia cepacia sepsis outbreak from a tertiary care hospital in Bangladesh.
The study of CFTR modulators in the very young.
Chitosan Nanocomplexes for the Delivery of ENaC Antisense Oligonucleotides to Airway Epithelial Cells.
A Peptide Nucleic Acid (PNA) Masking the miR-145-5p Binding Site of the 3’UTR of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) mRNA Enhances CFTR Expression in Calu-3 Cells.
Influences of environmental exposures on individuals living with cystic fibrosis.
Initiating Self-Administration of Medicines for inpatients with cystic fibrosis.
Personalised medicine for non-classic cystic fibrosis resulting from rare CFTR mutations.
RhlR-Regulated Acyl-Homoserine Lactone Quorum Sensing in a Cystic Fibrosis Isolate of Pseudomonas aeruginosa.
Increased extracellular vesicles mediate inflammatory signalling in cystic fibrosis.
Elexacaftor-Tezacaftor-Ivacaftor: The First Triple-Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulating Therapy.
Phenotypical Characteristics of the Black Yeast Exophiala dermatitidis Are Affected by Pseudomonas aeruginosa in an Artificial Sputum Medium Mimicking Cystic Fibrosis-Like Conditions.
Epidemiology of E. coli in Cystic Fibrosis Airways Demonstrates the Capacity for Persistent Infection but Not Patient-Patient Transmission.
Resveratrol restores intracellular transport in cystic fibrosis epithelial cells.
Role of Genetic Factors in the Development of Lung Disease
A New Optical Sweat Test Method Based on a Citric Acid-derived Multi-halide Sensor
Sildenafil Exercise: Role of PDE5 Inhibition
Updates on bone health in children with gastrointestinal diseases.
Impact of Bacterial Toxins in the Lungs.
Functional and Pharmacological Characterization of the Rare CFTR Mutation W361R.
Recent progress in structural studies on TMEM16A channel.
Effects of high intensity interval training on exercise capacity in people with chronic pulmonary conditions: a narrative review.
Oral ethinyl estradiol treatment in women with cystic fibrosis is associated with lower bone mineral density.
Emerging Cystic Fibrosis Transmembrane Conductance Regulator Modulators as New Drugs for Cystic Fibrosis: A Portrait of in Vitro Pharmacology and Clinical Translation.
Exploring the relationship between FEV1 loss and recovery and aminoglycoside pharmacokinetics in adult patients with cystic fibrosis: implications for clinical dosing strategies.
Middle ear disease in CF? It’s not just about the sinuses anymore!
A Study of AeroVanc for the Treatment of MRSA Infection in CF Patients
Cystic Fibrosis and Gut Dysmotility: The Effect of Polyethylene Glycol (PEG) on Intestinal Transit
Antioxidant Micronutrients and Essential Fatty Acids Supplementation on Cystic Fibrosis Outcomes: A Systematic Review.
Skin wipe test: A simple, inexpensive, and fast approach in the diagnosis of cystic fibrosis.
The anti-inflammatory potential of selected plant-derived compounds in respiratory diseases.
Interventions for preventing silent cerebral infarcts in people with sickle cell disease.
Non-CF bronchiectasis: Orphan disease no longer.
Treatments for priapism in boys and men with sickle cell disease.
Treatment of Cystic Fibrosis Patients Homozygous for F508del with Lumacaftor-Ivacaftor (Orkambi®) Restores Defective CFTR Channel Function in Circulating Mononuclear Cells.
Recent Strategic Advances in CFTR Drug Discovery: An Overview.
Leveraging early markers of cystic fibrosis structural lung disease to improve outcomes.
Diagnostic markers of allergic bronchopulmonary aspergillosis in patients with severe asthma.
Cystic fibrosis in Turkey.
Association between faecal pH and fat absorption in children with cystic fibrosis on a controlled diet and enzyme supplements dose.
[Screening audiological control in children of different ages after taking ototoxic medications].
TAS2R38 is a novel modifier gene in patients with cystic fibrosis.
Regional Structure-function in Cystic Fibrosis Lung Disease Using Hyperpolarized 129Xe and Ultrashort Echo Magnetic Resonance Imaging.
Correcting CFTR: New Gene Editing Strategies for Rescuing CFTR Function Ex Vivo.
Peptides as surface coatings of nanoparticles that penetrate human cystic fibrosis sputum and uniformly distribute in vivo following pulmonary delivery.
Three-axis accelerometer system for comparison of gait parameters in children with cystic fibrosis and healthy peers.
Abstracts from the 25th Italian Congress of Cystic Fibrosis and the 15th National Congress of Cystic Fibrosis Italian Society : Assago, Milan. 10 – 12 October 2019.
TMEM16A: An Alternative Approach to Restoring Airway Anion Secretion in Cystic Fibrosis?
Cathelicidin-inspired antimicrobial peptides as novel antifungal compounds.
Improvements in frailty contribute to substantial improvements in quality of life after lung transplantation in patients with cystic fibrosis.
Allergic bronchopulmonary aspergillosis without asthma or cystic fibrosis.
Lung function and microbiota diversity in cystic fibrosis.
Antibiotic treatment for Burkholderia cepacia complex in people with cystic fibrosis experiencing a pulmonary exacerbation.
β2 adrenoceptor signaling regulates ion transport in 16HBE14o- human airway epithelial cells.
Pulmonary Aspergillosis: What the Generalist Needs to Know.
A Phase 2 Open Label Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics of Multiple Dose Levels of Subcutaneously Administered ELX-02 in Patients with Cystic Fibrosis…
Place of Death for Individuals with Chronic Lung Disease: Trends and Associated Factors from 2003 to 2017 in the United States.
Glibenclamide inhibits BK polyomavirus infection in kidney cells through CFTR blockade.
The Iron-chelator, N,N’-bis (2-hydroxybenzyl) Ethylenediamine-N,N’-Diacetic acid is an Effective Colistin Adjunct against Clinical Strains of Biofilm-Dwelling Pseudomonas aeruginosa.
Pulmonary Pathogens Adapt to Immune Signaling Metabolites in the Airway.
Hardy-Weinberg Equilibrium in the Large Scale Genomic Sequencing Era.
Semaglutide in Cystic Fibrosis-Related Diabetes.

Long-term effects of ivacaftor on nonpulmonary outcomes in individuals with cystic fibrosis, heterozygous for a S1251N mutation.
Extracellular Vesicle-Mediated siRNA Delivery, Protein Delivery, and CFTR Complementation in Well-Differentiated Human Airway Epithelial Cells.
Exposure of Aspergillus fumigatus to Atorvastatin Leads to Altered Membrane Permeability and Induction of an Oxidative Stress Response.
Optical Nanosensors for in vivo Physiological Chloride Detection for Monitoring Cystic Fibrosis Treatment.
Interventions for preventing and managing advanced liver disease in cystic fibrosis.
The CFTR variant profile of Hispanic patients with cystic fibrosis: Impact on access to effective screening, diagnosis, and personalized medicine.
Physical therapies for postural abnormalities in people with cystic fibrosis.
Respiratory viruses: What is their role in acute exacerbations in children with cystic fibrosis?
A Phase 2, Randomized, Double-blind, Controlled Study to Evaluate the Safety and Efficacy of VX-121 Combination Therapy in Subjects Aged 18 Years and Older With Cystic Fibrosis
Feasibility of a Mobile Medication Plan Application in CF Patient Care
Developing e-Health Systems to Improve Growth and Nutrition in CF
Phase-Ib/IIa study to investigate safety, tolerability, pharmacokinetics and pharmacodynamics of orally inhaled multiple doses of POL6014 in patients with Cystic Fibrosis
Generation of an induced pluripotent stem cell line (MHHi018-A) from a patient with Cystic Fibrosis carrying p.Asn1303Lys (N1303K) mutation.
Lung clearance index evaluation in detecting nocturnal hypoxemia in cystic fibrosis patients: Toward a new diagnostic tool.
Utility and validity of dynamic chest radiography in cystic fibrosis (dynamic CF): an observational, non-controlled, non-randomised, single-centre, prospective study.
Confidence and skills for cystic fibrosis end-of-life care.
Complete Genome Sequences of Clinical Pandoraea fibrosis Isolates.
Who’s at The Door? – Surface Contamination of Door Frames in a Single-Bedded In-Patient Adult Cystic Fibrosis (CF) Unit.
STAPHYLOCOCCUS AUREUS ENTEROTOXINS IN PEOPLE WITH CYSTIC FIBROSIS (CF).
Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity.
Differences in cystic fibrosis-associated Burkholderia spp. bacteria metabolomes after exposure to the antibiotic trimethoprim.
Maturity-onset diabetes of the young: Different diabetes in an infant with cystic fibrosis.
[Protective effect of Yishen Tongluo Recipe against benzo(a)pyrene-induced sperm DNA methylation changes in male rats].
[Correlation between CFTR 5T polymorphisms and the risk of congenital bilateral absence of the vas deferens].
Importance of Nebulizer Drying for Patients With Cystic Fibrosis.
A Phase 3 Randomized, Double-blind, Placebo-controlled, Multi-center Study to Evaluate the Efficacy and Safety of Pimodivir in Combination With the Standard-of-care Treatment in Adolescent, Adult, …
Project UPLIFT to Reduce Anxiety and Depression in CF Patients
Surfing motility is a complex adaptation dependent on the stringent stress response in Pseudomonas aeruginosa LESB58.
Trikafta in Cystic Fibrosis Patients
Effects of SIMEOX on Airway Clearance in Cystic Fibrosis
Antibiofilm and mucolytic action of nitric oxide delivered via gas or macromolecular donor using in vitro and ex vivo models.
Fis Contributes to Resistance of Pseudomonas aeruginosa to Ciprofloxacin by Regulating Pyocin synthesis.
Genetic testing in male infertility – reassessing screening thresholds.
Ethnogeographic and inter-individual variability of human ABC transporters.
Mechanisms for Vascular Dysfunction and Exercise Tolerance in CF
IV Colistin for Pulmonary Exacerbations: Improving Safety and Efficacy
A Study to Evaluate Safety and Efficacy of Subcutaneous Administration of Anakinra in Patients With CF
Effect of Losartan in Cystic Fibrosis (CF)-NIH Grant #133240
Beta-cell Response to Incretin Hormones in Cystic Fibrosis
Effect of one-year lumacaftor-ivacaftor treatment on glucose tolerance abnormalities in cystic fibrosis patients.
Microbicidal activity of N-chlorotaurine can be enhanced in the presence of lung epithelial cells.
Vitamin D Deficiency and Radiological Findings in Adult Non-Cystic Fibrosis Bronchiectasis.
Newborn Screening for Cystic Fibrosis in Mersin Province: Yearly Assessment of the National Program.
Comparison of a handheld turbine spirometer to conventional spirometry in children with cystic fibrosis.
Sensor Augmented Pump (SAP) Therapy for Inpatient CFRD Management
Total bacterial load, inflammation, and structural lung disease in paediatric cystic fibrosis.
Comparative whole genome phylogeny of animal, environmental, and human strains confirms the genogroup organization and diversity of the Stenotrophomonas maltophilia complex.
Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease.
Modulator treatment for people with cystic fibrosis: moving in the right direction.
Intradialytic parenteral nutrition improves nutritional status in a complex cystic fibrosis patient with redo double lung transplant and end-stage renal disease.
Analysis of volatile metabolites from in vitro biofilms of Pseudomonas aeruginosa with thin-film microextraction by thermal desorption gas chromatography-mass spectrometry.
Cryptides Identified in Human Apolipoprotein B as New Weapons to Fight Antibiotic Resistance in Cystic Fibrosis Disease.
Evaluation of secondhand smoke effects on CFTR function in vivo.
Comparison of the outcomes of biliary atresia with cystic degeneration and isolated biliary atresia: A matched-pair analysis.
The origin of extracellular DNA in bacterial biofilm infections in vivo.
Functional Role of Basolateral ClC-2 Channels in the Regulation of Duodenal Anion Secretion in Mice.
Antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis.
Persistent Stridor in a 10-year-old Patient with Cystic Fibrosis.
Study Of Effects Of Oral CREON Capsules In Adult Participants With Exocrine Pancreatic Insufficiency Not Due To Cystic Fibrosis, Chronic Pancreatitis, Pancreatectomy, Or Pancreatic Cancer
Epigenetic Modification of CFTR in Head and Neck Cancer.
Plasma Levels of the Bioactive Sphingolipid Metabolite S1P in Adult Cystic Fibrosis Patients: Potential Target for Immunonutrition?
Dilation of the cystic duct confluence in laparoscopic common bile duct exploration and stone extraction in patients with secondary choledocholithiasis.
An investigation on parenting stress of children with cystic fibrosis.
Pharmacokinetic/pharmacodynamic adequacy of polymyxin B against extensively drug-resistant gram-negative bacteria in critically ill, general ward and cystic fibrosis patient populations.
Contextual Flexibility in Pseudomonas aeruginosa Central Carbon Metabolism during Growth in Single Carbon Sources.
TMEM16A drives renal cyst growth by augmenting Ca2+ signaling in M1 cells.
Cystic Fibrosis: A Simple and Customized Strategy for Genetic Screening Able to Detect Over 90% of Identified Mutated Alleles in Brazilian Newborns.
Psychological interventions for people with hemophilia.
Randomized, Double-Blind, Phase 3B Trial to Evaluate the Safety and Efficacy of 2 Treatment Regimens of Aztreonam 75 mg Powder and Solvent for Nebulizer Solution / Aztreonam for Inhalation Solution…
Measurement of Multi Ion Transport through Human Bronchial Epithelial Cell Line Provides an Insight into the Mechanism of Defective Water Transport in Cystic Fibrosis.
Leukocyte adhesion defect: Where do we stand circa 2019?
Screening practices for nontuberculous mycobacteria at us cystic fibrosis centers.
Evaluation of Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are 6 Years of Age and Older
MCP-1 promotes detrimental cardiac physiology, pulmonary edema, and death in the cpk model of polycystic kidney disease.
Longitudinal Relationships Between Depression and Chronic Illness in Adolescents: An Integrative Review.
It Is a Life Journey: A Roadmap of Teens With Chronic Diseases in Transitioning to Independence.
Meta-analysis Reveals Potential Influence of Oxidative Stress on the Airway Microbiomes of Cystic Fibrosis Patients.
Adults with cystic fibrosis: spiritual coping with lifelong disease.
Current and future diagnosis of cystic fibrosis: Performance and limitations.
Genetic diagnosis in practice: From cystic fibrosis to CFTR-related disorders.
Genetic counseling for cystic fibrosis: A basic model with new challenges.
Newborn screening for CF in France: An exemplary national experience.
Genetics of cystic fibrosis: Basics.
Genomically-guided therapies: A new era for cystic fibrosis.
Conclusion and Prospects: Genetics of cystic fibrosis – an agenda for the next ten years.
CFTR gene variants, epidemiology and molecular pathology.
Antibiotic Treatment and Age Are Associated With Staphylococcus aureus Carriage Profiles During Persistence in the Airways of Cystic Fibrosis Patients.
Domino heart transplant following heart-lung transplantation: a systematic review and meta-analysis.
Platelets: inflammatory effector cells in the conflagration of cystic fibrosis lung disease.
Lung function determinants and mortality of children and adolescents with cystic fibrosis in South Africa 2007-2016.
Thymidine-auxotrophic Staphylococcus aureus small-colony variant bacteremia in a patient with cystic fibrosis.
Glucose Tolerance in Patients with Cystic Fibrosis – Results from the German Cystic Fibrosis Registry.
Markers of Osteoporosis in Cystic Fibrosis
An open-label study to assess the safety, pharmacokinetics and pharmacodynamics of inhaled PC945 in adult Cystic Fibrosis (CF) patients with persistent pulmonary Aspergillus fumigatus infection.
A Randomized, Double-Blind, Placebo-Controlled, Dose-finding Study to Evaluate the Efficacy and Safety of Aerosolized Moli1901 in Adolescents (12 Years of Age or Older) and Adults with Cystic Fibrosis
A Novel Genetically Encoded Single Use Sensory Cellular Test System Measures Bicarbonate Concentration Changes in Living Cells.
Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy: A Review for the Otolaryngologist.
Effectiveness and cost-effectiveness of guided Internet- and mobile-based CBT for adolescents and young adults with chronic somatic conditions and comorbid depression and anxiety symptoms (youthCOACHCD): study protocol for a multicentre randomized controlled trial.
Intrinsic alterations in peripheral neutrophils from cystic fibrosis newborn piglets.
Health-related quality-of-life in children with cystic fibrosis aged 5-years and associations with health outcomes.

Update on SLC6A14 in lung and gastrointestinal physiology and physiopathology: focus on cystic fibrosis.
Neurotoxicity in adult patients with cystic fibrosis using polymyxin B for acute pulmonary exacerbations.
Infection prevention and chronic disease management in cystic fibrosis and noncystic fibrosis bronchiectasis.
Impaired Airway Epithelial Barrier Integrity in Response to Stenotrophomonas maltophilia Proteases, Novel Insights Using Cystic Fibrosis Bronchial Epithelial Cell Secretomics.
Pseudomonas Quinolone Signal molecule PQS behaves like a B Class inhibitor at the IQ site of mitochondrial complex I.
Lung T1 mapping magnetic resonance imaging in the assessment of pulmonary disease in children with cystic fibrosis: a pilot study.
A molecular mechanism for how sigma factor AlgT and transcriptional regulator AmrZ inhibit twitching motility in Pseudomonas aeruginosa.
A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-659 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation…
Combined Use of CFTR Correctors in LGMD2D Myotubes Improves Sarcoglycan Complex Recovery.
‘Go for it, dream big, work hard and persist’: A message to the next generation of CF leaders in recognition of International Women’s Day 2020.
Clinical features and management of children with primary ciliary dyskinesia in England.
The Cross-Talk Between Gut Microbiota and Lungs in Common Lung Diseases.
The dual phosphodiesterase 3/4 inhibitor RPL554 stimulates rare class III and IV CFTR mutants.
Airway hyperresponsiveness, remodeling and inflammation in infants with wheeze.
Acid exposure disrupts mucus secretion and impairs mucociliary transport in neonatal piglet airways.
A Phase 3, Randomized, Double-blind, Controlled Study Evaluating the Efficacy and Safety of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation…
An Exploratory Phase 2, 2-part, Randomized, Double blind, Placebo controlled Study With a Long term, Open label Period to Explore the Impact of Lumacaftor/Ivacaftor on Disease Progression in Subjec…
CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine.
Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators – an international survey.
Cytokinetic bridge triggers de novo lumen formation in vivo.
Author Correction: A randomised controlled trial of rosuvastatin for the prevention of aminoglycoside-induced kidney toxicity in children with cystic fibrosis.
Synthesis and Evaluation of Airway-Targeted PLGA-PEG Nanoparticles for Drug Delivery in Obstructive Lung Diseases.
Synthesis and Evaluation of Dendrimers for Autophagy Augmentation and Alleviation of Obstructive Lung Diseases.
Pf Bacteriophage and Their Impact on Pseudomonas Virulence, Mammalian Immunity, and Chronic Infections.
Ivacaftor for the treatment of cystic fibrosis in children under six years of age.
Phase 2 Combination Study With Escalating Doses of MS1819-SD on Top of a Stable Dose of PPEs
[Focus on cystic fibrosis].
[Family support upon immediate cystic fibrosis announcement].
Lung transplantation for cystic fibrosis.
[An essential tool in the management of cystic fibrosis in childhood].
[Cystic fibrosis, the transition to adult centres].
[Cystic fibrosis, what follow-up action for adults in 2019?]
[Cystic fibrosis, support and end of life of patients].
[Cystic fibrosis, growing and living better through sport].
Ebselen attenuates tobramycin-induced ototoxicity in mice.
Treating allergic bronchopulmonary aspergillosis with short-term prednisone and itraconazole in cystic fibrosis.
Culture of Small Colony Variant of Pseudomonas aeruginosa and Quantitation of its Alginate.
The biofilm-associated bacterial infections unrelated to indwelling devices.
Trend of sweat chloride values in a cohort of patients carrying CFTR mutations of varying clinical consequence: Is there a risk of increasing sweat chloride over time?
[SLC6A14, a modifier gene in cystic fibrosis].
A Systematized review of experiences of individuals in Arnett’s emerging adulthood stage who live with or are at-risk for genetic conditions.
Clinical, genetic and microbiological characterization of pediatric patients with cystic fibrosis in a public Hospital in Ecuador.
A qualitative study of design stakeholders’ views of developing and implementing a registry-based learning health system.
Selective Binding of HSC70 and its Co-Chaperones to Structural Hotspots on CFTR.
The master regulators Myc and p53 cellular signaling and functions in polycystic kidney disease.
[Cystic fibrosis and pregnancy:outcome, prognostic factors and obstetrical management].
The social life of microbes in chronic infection.
Sleep disorders in cystic fibrosis: A systematic review and meta-analysis.
Viral Vectors, Animal Models, and Cellular Targets for Gene Therapy of Cystic Fibrosis Lung Disease.
Advances in the use of cell penetrating peptides for respiratory drug delivery.
Changes in the parent cystic fibrosis questionnaire-revised (CFQ-R) with respiratory symptoms in preschool children with cystic fibrosis.
Structural determinants of long term functional outcomes in young children with cystic fibrosis.
Re-Imagining Cystic Fibrosis Care: Next Generation Thinking.
New method for rapid and dynamic quantification of elastase activity on sputum neutrophils from patients with cystic fibrosis using flow cytometry.
Outcomes and Safety of Outpatient Parenteral Antimicrobial Therapy in Select Children with Cystic Fibrosis.
Weight Gain and Growth After Fundoplication in the Pediatric Patient: A Case Report in the Pediatric Patient With Cystic Fibrosis and Literature Review.
Synthesis and structure-activity relationship of aminoarylthiazole derivatives as potential potentiators of the chloride transport defect in cystic fibrosis.
Effectivity of Pancreatic Enzyme Replacement Therapy in Malnourished Children.
Cystic Fibrosis-related Liver Disease is Associated With Increased Disease Burden and Endocrine Comorbidities.
MRI Detects Chronic Rhinosinusitis in Infants and Preschool Children with Cystic Fibrosis.
Risk factors for bronchiolitis hospitalization in infants: A French nationwide retrospective cohort study over four consecutive seasons (2009-2013).
Myeloperoxidase inhibition decreases morbidity and oxidative stress in mice with cystic fibrosis-like lung inflammation.
Antibacterial and Antivirulence Activity of Glucocorticoid PYED-1 against Stenotrophomonas maltophilia.
Burkholderia cenocepacia H111 Produces a Water-Insoluble Exopolysaccharide in Biofilm: Structural Determination and Molecular Modelling.
Role of phosphodiesterase-4 inhibitors in chronic obstructive pulmonary disease.
Lung cancer in a CF patient: combination of bad luck or is there more to say?
Genome-wide analysis of MicroRNA-messenger RNA interactome in ex-vivo gill filaments, Anguilla japonica.
Lung transplant referral practice patterns: a survey of cystic fibrosis physicians and general pulmonologists.
Secretin-Stimulated Magnetic Resonance Imaging Reveals Variable Diagnostic Accuracy According to Etiology in Pancreatic Disease.
Aetiological agents for pulmonary exacerbations in children with cystic fibrosis: An observational study from a tertiary care centre in northern India.
A Study Evaluating the Long Term Safety and Efficacy of VX-659 Combination Therapy
A Study to Evaluate the Safety and Efficacy of Long Term Treatment With VX-661 in Combination With Ivacaftor in Subjects With Cystic Fibrosis Who Have an F508del-CFTR Mutation
Effect of Chronic Incretin-based Therapy in Cystic Fibrosis
A Study Evaluating the Efficacy and Safety of VX-445/Tezacaftor/Ivacaftor in Cystic Fibrosis Subjects, Homozygous for F508del
Two Unanticipated Pregnancies While on Cystic Fibrosis Gene-Specific Drug Therapy.
Collaborative Cross Mice Yield Genetic Modifiers for Pseudomonas aeruginosa Infection in Human Lung Disease.
IRE1α kinase-mediated unconventional protein secretion rescues misfolded CFTR and pendrin.
Discovery of a picomolar potency pharmacological corrector of the mutant CFTR chloride channel.
Predictive value of computed tomography scoring systems evolution in adults with cystic fibrosis.
Outcome according to subspecies following lung transplantation in cystic fibrosis paediatric patients infected with mycobacterium abscessus.
Airway submucosal glands from cystic fibrosis swine suffer from abnormal ion transport across the serous acini, collecting duct and ciliated duct.
Cost of precision medicine at a referral center for cystic fibrosis.
Finding the relevance of antimicrobial stewardship for cystic fibrosis.
Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis-causing mutation.
Changes in the R-region interactions depend on phosphorylation and contribute to PKA and PKC regulation of the cystic fibrosis transmembrane conductance regulator chloride channel.
Physiological mechanisms determining eccrine sweat composition.
Transcutaneous electrical nerve stimulation (TENS) for pain management in sickle cell disease.
A Phase 1 IV Gallium Study for Patients With Cystic Fibrosis Who Have NTM (ABATE Study)
Heart Rate Variability in Children and Adolescents With Cystic Fibrosis
Is prolonged incubation required for optimal recovery of Burkholderia cepacia complex in sputum from cystic fibrosis patients? Data versus dogma.
Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease.
Glucose ingestion in cystic fibrosis induces severe redox imbalance: A potential role in diabetes.
Some young adults with cystic fibrosis-related diabetes may safely stop insulin without any adverse clinical sequelae.
Evaluation of Cellular Responses for the Diagnosis of Allergic Bronchopulmonary Mycosis: A Preliminary Study in Cystic Fibrosis Patients.
Pseudomonas aeruginosa Modulates the Antiviral Response of Bronchial Epithelial Cells.
Influenza A Virus Pre-Infection Exacerbates Pseudomonas aeruginosa-Mediated Lung Damage Through Increased MMP-9 Expression, Decreased Elafin Production and Tissue Resilience.
TGF-β1 Augments the Apical Membrane Abundance of Lemur Tyrosine Kinase 2 to Inhibit CFTR-Mediated Chloride Transport in Human Bronchial Epithelia.
Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis.
Major Aspects of Burkholderia gladioli and Burkholderia cepacia Infections in Children.
Amikacin nomogram for treatment of adult cystic fibrosis exacerbations based on an external evaluation of a population pharmacokinetic model.

Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomography.
Plasma Ceramides and Sphingomyelins of Pediatric Patients Increase in Primary Ciliary Dyskinesia but Decrease in Cystic Fibrosis.
A double-blind, placebo controlled, multicentre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled Promixin® (colistimethate sodium) in the treatment of sub…
Glucose abnormalities detected by continuous glucose monitoring are common in young children with Cystic Fibrosis.
Host responses to mucosal biofilms in the lung and gut.
DNA Methylation Changes in Cystic Fibrosis: Cause or Consequence?
Effect of Sex Differences on Computed Tomography Findings in Adults With Cystic Fibrosis: A Multicenter Study.
Characterization of Pseudomonas lytic phages and their application as a cocktail with antibiotics in controlling Pseudomonas aeruginosa.
Timing of hypertonic saline inhalation for cystic fibrosis.
Respiratory mycobiome and suggestion of inter-kingdom network during acute pulmonary exacerbation in cystic fibrosis.
Successful treatment of allergic bronchopulmonary aspergillosis with posaconazole in a child with cystic fibrosis: Case report and review of the literature.
Deviations of body functions and structure, activity limitations, and participation restrictions of the International Classification of Functioning, Disability, and Health model in children with cystic fibrosis and non-cystic fibrosis bronchiectasis.
Coping with Cystic Fibrosis in the Republic of Macedonia-Parent Perspective.
Contraceptive use among women with cystic fibrosis: A pilot study linking reproductive health questions to the Cystic Fibrosis Foundation National Patient Registry.
Impact of gastrointestinal physiology on drug absorption in special populations – An UNGAP review.
A Phase 3b Open-label Study Evaluating the Safety of Elexacaftor/Tezacaftor/Ivacaftor Combination Therapy in Cystic Fibrosis Subjects
PKA phosphorylation potentiates CFTR gating by relieving auto-inhibition on the stimulatory C terminus of the regulatory domain.
Disease modification and biomarker development in Parkinson disease: Revision or reconstruction?
Global chemical effects of the microbiome include new bile-acid conjugations.
Cystic fibrosis: a rare disease emerging in China.
Uncommon clinical presentation of cystic fibrosis in a patient homozygous for a rare CFTR mutation: a case report.
In vitro activity of Fosfomycin against mucoid and non-mucoid Pseudomonas aeruginosa strains.
Measuring and Improving Coproduction Using coopeRATE
Comparative Genomics and Evolutionary Analysis of RNA-Binding Proteins of Burkholderia cenocepacia J2315 and Other Members of the B. cepacia Complex.
Small Molecule Anion Carriers Correct Abnormal Airway Surface Liquid Properties in Cystic Fibrosis Airway Epithelia.
Alpha 1-Antitrypsin Deficiency: A Disorder of Proteostasis-Mediated Protein Folding and Trafficking Pathways.
Misunderstandings, misperceptions, and missed opportunities: Perspectives on adherence barriers from people with CF, caregivers, and CF team members.
Staphylococcal lung abscess in a child with cystic fibrosis: Case report & review of literature.
Salivary biomarkers in the context of gingival inflammation in children with cystic fibrosis.
Comparison of International Growth Standards for Assessing Nutritional Status in Cystic Fibrosis: The GreeCF Study.
A Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy
Anti-Infectives Restore ORKAMBI® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa.
Complete versus Limited Endoscopic Sinus Surgery for Chronic Rhinosinusitis in Adults with Cystic Fibrosis.
Real-life evaluation of clinical outcomes in patients undergoing treatment for non-tuberculous mycobacteria lung disease: A ten-year cohort study.
Hepatic manifestations of cystic fibrosis.
Noncanonical Wnt planar cell polarity signaling in lung development and disease.
Burkholderia cenocepacia-host cell contact controls the transcription activity of the trimeric autotransporter adhesin BCAM2418 gene.
Telehealth Cognitive Behavioral Stress Management for Adults With Cystic Fibrosis
Efficacy and Safety of Inhaled Nitric Oxide (NO) in Cystic Fibrosis (CF) Patients
Small Circulating RNA as Molecular Markers of Lung Disease in Cystic Fibrosis
50 Years Ago in TheJournalofPediatrics: Sodium Concentration in Unstimulated Parotid Saliva and on Oral Mucosa in Normal Subjects and Patients with Cystic Fibrosis.
XBP1S Regulates MUC5B in a Promoter Variant-Dependent Pathway in Idiopathic Pulmonary Fibrosis Airway Epithelia.
New insights to guide patient care: the bidirectional relationship between male infertility and male health.
CF: There’s an app for that!
First case of cystic fibrosis in Greenland – diagnosed by neonatal screening.
Adult outcomes of childhood bronchiectasis.
Technology-enabled Patient Support System for Self-management of CF
Peptide Nucleic Acids for MicroRNA Targeting.
Barriers and facilitators to physical activity among children, adolescents, and young adults with cystic fibrosis: a systematic review and thematic synthesis of qualitative research.
Characteristics of the Airway Microbiome of Cystic Fibrosis Patients.
Tobacco smoke exposure and socioeconomic factors are independent predictors of pulmonary decline in pediatric cystic fibrosis.
Pancreatic cystosis in cystic fibrosis: Sometimes a bike ride can help you decide.
Influence of pancreatic status on circulating plasma sterols in patients with cystic fibrosis.
[Dietary intake of children with cystic fibrosis].
Graph-based description of tertiary lymphoid organs at single-cell level.
Perceptions towards physical activity in adult lung transplant recipients with cystic fibrosis.
Viral strategies predisposing to respiratory bacterial superinfections.
Phenotypic characterization and comparison of Phe508del and cystic fibrosis transmembrane conductance regulator (CFTR) knockout rat models of cystic fibrosis generated by CRISPR/Cas9 gene editing.
CRISPR-Based Adenine Editors Correct Nonsense Mutations in a Cystic Fibrosis Organoid Biobank.
Postinfectious Bronchiolitis Obliterans in Children: Diagnostic Workup and Therapeutic Options: A Workshop Report.
European Position Paper on Rhinosinusitis and Nasal Polyps 2020.
Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study.
Targeting different binding sites in the CFTR structures allows to synergistically potentiate channel activity.
A Study to Evaluate the Safety and Efficacy of Long-term Treatment With TEZ/IVA in CF Subjects With an F508del CFTR Mutation
Study to Evaluate the Safety of CB-280 in Patients With Cystic Fibrosis
A randomised, double-blind, dose finding study of inhaled alginate oligosaccharide (OligoG) vs placebo in patients with Cystic Fibrosis (CF).
Preventing Depression and Anxiety: A Cystic Fibrosis-Specific Cognitive Behavioral Therapy Intervention
A double-blind, placebo controlled, multi-centre, clinical trial to investigate the efficacy and safety of 12 months of therapy with inhaled colistimethate sodium in the treatment of subjects with …
Etiology and characteristics of patients with bronchiectasis in Taiwan: a cohort study from 2002 to 2016.
Antibacterial activity of iron oxide, iron nitride, and tobramycin conjugated nanoparticles against Pseudomonas aeruginosa biofilms.
Hypermutator Pseudomonas aeruginosa exploits multiple genetic pathways to develop multidrug resistance during long-term infections in the airways of cystic fibrosis patients.
Effectiveness of chest physiotherapy and pulmonary rehabilitation in patients with non-cystic fibrosis bronchiectasis: a narrative review.
Derivation of induced pluripotent stem cells from ferret somatic cells.
Effect of Inspiratory Maneuvers on Lung Deposition of Tobramycin Inhalation Powder: A Modeling Study.
In vivo demonstration of Pseudomonas aeruginosa biofilms as independent pharmacological microcompartments.
Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA.
Role of transcription factor hepatocyte nuclear factor-1β in polycystic kidney disease.
Is out-patient based treatment of bronchiectasis exacerbations in children comparable to inpatient based treatment?
CFTR regulation of aquaporin-mediated water transport.
Heterogeneous Liver on Research Ultrasound Identifies Children with Cystic Fibrosis at High Risk of Advanced Liver Disease: Interim Results of a Prospective Observational Case-Controlled Study.
Lymphocyte responses to Mycobacterium tuberculosis and Mycobacterium bovis are similar between BCG-vaccinated patients with cystic fibrosis and healthy controls.
Predictive effects of low birth weight and small for gestational age status on respiratory and nutritional outcomes in cystic fibrosis.
Targeting defective pulmonary innate immunity – A new therapeutic option?
An unusual appearance of a serous ovarian cyst coexisting with endometriosis: A case report.
At the forefront of cystic fibrosis Basic Science research: 16th ECFS Basic Science Conference.
The cell division protein FtsZ as a cellular target to hit cystic fibrosis pathogens.
CFTR Related Pancreatitis Study
PPIs and Fat Absorption in CF and EPI
Standardized Treatment of Pulmonary Exacerbations II
The Effect of Proprioceptive Neuromuscular Facilitation (PNF) Technique for Children With Chronic Pulmonary Diseases.
Gut Imaging for Function & Transit in Cystic Fibrosis Study 2
Precise Targeting of miRNA Sites Restores CFTR Activity in CF Bronchial Epithelial Cells.
Ablation of IL-33 Suppresses Th2 Responses but Is Accompanied by Sustained Mucus Obstruction in the Scnn1b Transgenic Mouse Model.
Essential oils against bacterial isolates from cystic fibrosis patients by means of antimicrobial and unsupervised machine learning approaches.
Effect of short-term inhalation of warm saline atomised gas on patients with non-cystic fibrosis bronchiectasis.
Inactivation of Apoptosis Antagonizing Transcription Factor in tubular epithelial cells induces accumulation of DNA damage and nephronophthisis.
HMGB1 is increased in adolescents with polycystic ovary syndrome (PCOS) and decreases after treatment with myo-inositol (MYO) in combination with alpha-lipoic acid (ALA).
The year’s new drugs and biologics 2019.
Respiratory viral infections in Western Australians with cystic fibrosis.
Evaluation of increased arterial stiffness in pediatric patients with cystic fibrosis by augmentation index and pulse wave velocity analysis.