Pending

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.
Airway profile of bioactive lipids predicts early progression of lung disease in cystic fibrosis.
Discovery of a novel chalcone derivative inhibiting CFTR chloride channel via AMPK activation and its anti-diarrheal application.
Making the invisible visible: the availability and desirability of adherence data in routine CF care- findings from a national questionnaire survey.
Triple Therapy for Cystic Fibrosis with a Phe508del CFTR Mutation.
Triple Therapy for Cystic Fibrosis with a Phe508del CFTR Mutation. Reply.
Ion Channels in The Pathogenesis of Endometriosis: A Cutting-Edge Point of View.
Eradication of Pseudomonas aeruginosa in cystic fibrosis patients with inhalation of dry powder tobramycin.
Unilateral temporary diaphragmatic paralysis secondary to bronchial artery embolization in a girl with cystic fibrosis and massive hemoptysis: a case report.
Determinants of Exercise Capacity Assessed With the Modified Shuttle Test in Individuals With Cystic Fibrosis.
Human Metapneumovirus Infection in Hospitalized Children.
Bacterial dominance is due to effective utilisation of secondary metabolites produced by competitors.
Atraumatic splenic rupture was attributed to intra-cystic haemorrhage and hypersplenism in a patient with cirrhosis and portal hypertension: A case report.
Targeting neutrophils using novel drug delivery systems in chronic respiratory diseases.
Does Circadian Rhythm affect the Pharmacokinetics of Once-Daily Tobramycin in Adults with Cystic Fibrosis?
A Clinical Trial to Compare the Efficacy and Safety of 1-week Treatment of Intravenous N-acetylcysteine (NAC) 600 mg Twice Daily, Ambroxol Hydrochloride 30 mg Twice Daily and Placebo as Expectorant Therapies in Adult Chinese Patients With Respiratory Tract Diseases and Abnormal Mucus Secretions
A Phase 2 Study to Evaluate the Safety, Tolerability, PK and PD in Cystic Fibrosis Patients With at Least 1 G542X Allele
Non-pulmonary Contributors of Exercise Intolerance in Patients With Cystic Fibrosis
A Phase 2 Study to Evaluate the Safety, Tolerability, PK and PD of ELX-02 in Cystic Fibrosis Patients With G542X Allele
Collaboration is key: identification of a novel cystic fibrosis mutation in three patients of South Asian descent.
Influence of lung transplantation on the essential fatty acid profile in cystic fibrosis.
Minimal change in structural, functional and inflammatory markers of lung disease in newborn screened infants with cystic fibrosis at one year.
Pseudomonas aeruginosa antibody response in cystic fibrosis decreases rapidly following lung transplantation.
Variable cellular ivacaftor concentrations in people with cystic fibrosis on modulator therapy.
Circulating biomarkers of antioxidant status and oxidative stress in people with cystic fibrosis: A systematic review and meta-analysis.
Towards automated in vivo tracheal mucociliary transport measurement: detecting and tracking particle movement in synchrotron phase-contrast X-ray images.
A randomised, double-blind, placebo-controlled and parallel group trial to evaluate efficacy and safety of twice daily inhaled doses of BI 1265162 delivered by Respimat® inhaler as add-on therapy t…
Virtual Care in CF (VIRTUAL-CF) Study
Enhancing the Expression of CFTR Using Antisense Molecules against MicroRNA miR-145-5p.
Reply to Finotti et al.: Enhancing the Expression of CFTR Using Antisense Molecules against MicroRNA miR-145-5p.
Nontuberculous mycobacterial infections in a Brazilian pediatric population: a seven-year survey.
Centralised versus outreach models of cystic fibrosis care should be tailored to the needs of the individual patient.
Evaluation of a radiographer-led peripherally inserted central catheter insertion service.
The Effects of the Anti-aging Protein Klotho on Mucociliary Clearance.
Assessing and responding to stress related to pulmonary function testing in cystic fibrosis through quality improvement.
Topical Pilocarpine Formulation for diagnosis of Cystic Fibrosis.
Optimized plant compound with potent anti-biofilm activity across gram-negative species.
Exendin-4 restores airway mucus homeostasis through the GLP1R-PKA-PPARγ-FOXA2-phosphatase signaling.
A comparative study of the pancreas in pediatric patients with cystic fibrosis and healthy children using two-dimensional shear wave elastography.
Microbial contamination of home nebulizers in children with cystic fibrosis and clinical implication on the number of pulmonary exacerbations.
Neuropeptide regulation of secretion and inflammation in human airway gland serous cells.
Cystic fibrosis patients at risk for disease progression marked by decline in FEV1% predicted: development of the cystic fibrosis risk of disease progression score.
Incidence and prevalence of common respiratory pathogens before and after implementation of the Cystic Fibrosis Foundation Infection Prevention and Control Guideline.
Nebuliser cleaning and disinfection practice in the home among patients with cystic fibrosis.
Clinical relevance of Scedosporium spp. and Exophiala dermatitidis in patients with cystic fibrosis: A nationwide study.
Hyperpolarized Imaging for New Treatments
A Dose-finding Study of Inhaled OligoG vs Placebo in Patients With Cystic Fibrosis

A Phase 2b Randomised, Placebo Controlled Study of OligoG in Patients With Cystic Fibrosis
Vasoactive Intestinal Peptide Derived From Liver Mesenchymal Cells Mediates Tight Junction Assembly in Mouse Intrahepatic Bile Ducts.
Alginate-lyase Guided Silver Nanocomposites for Eradicating Pseudomonas Aeruginosa from lung.
mSphere of Influence: a Community To Study Communities.
Genetics of the congenital absence of the vas deferens.
Genotype patterns for mutations of the cystic fibrosis transmembrane conductance regulator gene: a retrospective descriptive study from Saudi Arabia.
Impact of hypertonic saline nebulisation combined with oscillatory positive expiratory pressure on sputum expectoration and related symptoms in cystic fibrosis: a randomised crossover trial.
Elicitation interview study to identify salient beliefs about exercise participation in adults with cystic fibrosis.
Differing Pulmonary Structural Abnormalities Detected on Pulmonary MR Imaging in Cystic Fibrosis Patients with Varying Pancreatic Function.
A randomised controlled trial of rosuvastatin for the prevention of aminoglycoside-induced kidney toxicity in children with cystic fibrosis.
Genetic Diagnosis and Sperm Retrieval Outcomes for Chinese Patients with Congenital Bilateral Absence of Vas Deferens.
Antibody response against Pseudomonas aeruginosa and its relationship with immune mediators in the upper and lower airways of cystic fibrosis patients.
Palliative Care Needs of Individuals with Cystic Fibrosis: Perspectives of Multiple Stakeholders.
Activity of antibiotics against Pseudomonas aeruginosa in an in vitro model of biofilms in the context of cystic fibrosis : influence of the culture medium.
Azithromycin inhibits constitutive airway epithelial sodium channel activation in vitro and modulates downstream pathogenesis in vivo.
Sodium antiporters of Pseudomonas aeruginosa in challenging conditions: effects on growth, biofilm formation, and swarming motility.
Study on the association between TGF-β1 and liver fibrosis in patients with hepatic cystic echinococcosis.
Maternal Genetic Disorders and Fetal Development.
Challenges facing microRNA therapeutics for cystic fibrosis lung disease.
Is Phosphatidylinositol 3- kinase (PI3K) a Villain in Cystic Fibrosis?
Alpha-1 Antitrypsin-A Target for MicroRNA-Based Therapeutic Development for Cystic Fibrosis.
Neutrophil Adaptations upon Recruitment to the Lung: New Concepts and Implications for Homeostasis and Disease.
What Spanish consensus meant for the treatment of Pseudomonas aeruginosa infection in patients with cystic fibrosis in the initial lung infection?
Applying whole-genome sequencing in relation to phenotype and outcomes in siblings with cystic fibrosis.
In-vivo crystals reveal critical features of the interaction between CFTR and the PDZ2 domain of Na+/H+ exchange cofactor NHERF1.
Succinate links mitochondria to deadly bacteria in cystic fibrosis.
Pseudomonas aeruginosa Alginate Benefits Staphylococcus aureus?
Whole Gene Sequencing of CFTR Reveals a High Prevalence of the Intronic Variant c.3874-4522A>G in Cystic Fibrosis.
A Study in Healthy Men to Test the Influence of BI 1323495 on the Amount of the Medicines Rosuvastatin and Dabigatran in the Blood
Organoid Study R334W
A Study to Evaluate the Safety, Pharmacokinetics, and Pharmacodynamics of Ivacaftor in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age and Have a CFTR Gating Mutation
Resting Energy Expenditure in Patients With Cystic Fibrosis: A Pilot Study
A Phase 3 Study Evaluating the Pharmacokinetics, Safety, and Tolerability of VX-445/TEZ/IVA Triple Combination Therapy in Cystic Fibrosis Subjects 6 Through 11 Years of Age
A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous or Heterozygous for the F508del Mutation
ppGpp ribosome dimerization model for bacterial persister formation and resuscitation.
Bronchiectasis in rheumatoid arthritis. A clinical appraisial.
Pulmonary Cystic Disease and Its Mimics.
High Dietary Protein Does Not Alter Renal Prostanoids and Other Oxylipins in Normal Mice or in Those with Inherited Kidney Disease.
Opportunities for machine learning to transform care for people with cystic fibrosis.
Systematic comparison of the protein-protein interaction databases from a user’s perspective.
Estimation of GFR in Patients With Cystic Fibrosis: A Cross-Sectional Study.
Clinical characteristics and genetic analysis of cystic fibrosis transmembrane conductance reseptor- related disease.
Expanded carrier screening for preconception reproductive risk assessment: Prevalence of carrier status in a Mexican population.
ActivOnline: Physical Activity in Cystic Fibrosis Trial UK
The current status and further prospects for lung magnetic resonance imaging in pediatric radiology.
Glycosylation changes in inflammatory diseases.
Downregulation of CFTR Is Involved in the Formation of Hypertrophic Scars.
Elexacaftor/tezacaftor/ivacaftor (Trikafta) for cystic fibrosis.
Inhibition of Sphingolipid Synthesis as a Phenotype-Modifying Therapy in Cystic Fibrosis.
Study to Evaluate the Safety & Tolerability of MRT5005 Administered by Nebulization in Adults With Cystic Fibrosis
Desaturation during exercise is not a sufficient mechanism for prediction of osteoporosis in non-cystic fibrosis bronchiectasis.
Interactions between the gut microbiome and host gene regulation in cystic fibrosis.
Cystic fibrosis heterozygosity: Carrier state or haploinsufficiency?
Physical Activity Level and Perception of Exercise in Cystic Fibrosis.
Effects of Signal Disruption Depends on the Substrate Preference of the Lactonase.
Impact of Pseudomonas aeruginosa Isolation on Mortality and Outcomes in an Outpatient Chronic Obstructive Pulmonary Disease Cohort.
Impaired counterregulatory responses to hypoglycaemia following oral glucose in adults with cystic fibrosis.
Prevalence and Risk Factors for Iron Deficiency in Adults With Cystic Fibrosis.
Regulatory SNP rs5743417 impairs constitutive expression of human β-defensin 1 and has high frequency in Africans and Afro-Americans.
Intravital microscopic optical coherence tomography imaging to assess mucus mobilizing interventions for muco-obstructive lung disease in mice.
Evaluation of the ReX-C System, in Patients Receiving CFTR Modulators for the Treatment of Cystic Fibrosis (CF).
Safety and Tolerability Study of Allogeneic Mesenchymal Stem Cell Infusion in Adults With Cystic Fibrosis
An atypical acute exacerbation of COPD due to Aspergillus fumigatus.
Nutritional rehabilitation for children with cystic fibrosis: Single center study.
Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.
Effect of Amoxicillin in combination with Imipenem-Relebactam against Mycobacterium abscessus.
Two years of newborn screening for cystic fibrosis in Turkey: Çukurova experience.
The RNFT2/IL3Rα axis regulates IL3 signaling and innate immunity.
Effects of low glycemic index/high-fat, high-calorie diet on glycemic control and lipid profiles of children and adolescence with cystic fibrosis: A randomized double-blind controlled clinical trial.
Thermic Effect of Feeding in Cystic Fibrosis
Regulation effects of naringin on diesel particulate matter-induced abnormal airway surface liquid secretion.
Efficacy and safety of ataluren in patients with nonsense-mutation cystic fibrosis not receiving chronic inhaled aminoglycosides: The international, randomized, double-blind, placebo-controlled Ataluren Confirmatory Trial in Cystic Fibrosis (ACT CF).
Hospitalization rates among patients with cystic fibrosis using pancreatic enzyme replacement therapy.
CFTR deletion affects mouse osteoblasts in a gender-specific manner.
Intraclonal competitive fitness of longitudinal cystic fibrosis Pseudomonas aeruginosa airway isolates in liquid cultures.
Brown Bowel Syndrome: A Multi-institutional Case Series.
Project 2 Airway Potential Hydrogen (pH) in Asthma
Connexin Genotypes in Cystic Fibrosis
Prednisone in Cystic Fibrosis Pulmonary Exacerbations
Enteral nutrition in cystic fibrosis
Overweight, obesity and significant weight gain in adult patients with cystic fibrosis association with lung function and cardiometabolic risk factors.
Identification of Mycobacterium porcinum in patients with cystic Fibrosis: Pathogen or contaminant?
Glycemic control and FEV1 recovery during pulmonary exacerbations in pediatric cystic fibrosis-related diabetes.
Plasma microRNA levels in male and female children with cystic fibrosis.
Salinity-dependent expression of ncc2 in opercular epithelium and gill of mummichog (Fundulus heteroclitus).
Outcome of fetal echogenic bowel: a systematic review and meta-analysis.
Nanograss sensor for selective detection of Pseudomonas aeruginosa by pyocyanin identification in airway samples.
A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long term Treatment With VX 661 in Combination With Ivacaftor in Subjects Aged 12 Years and Older With Cystic Fibrosis, …
A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-659 Combination Therapy in Subjects With Cystic Fibrosis Who Are Homozygous or Heterozygous for the F508del Mutation
Investigating the Temporal Relationships between Symptoms and Nebuliser Adherence in People with Cystic Fibrosis: A Series of N-of-1 Observations.
Hypermutation as an Evolutionary Mechanism for Achromobacter xylosoxidans in Cystic Fibrosis Lung Infection.
Abnormal glucose tolerance and the 50-gram glucose challenge test in Cystic fibrosis.
What biologists want from their chloride reporters – a conversation between chemists and biologists.
A SYK/SHC1 pathway regulates the amount of CFTR in the plasma membrane.
A Novel Pathogenic Variant of the CFTR Gene in a Patient with Cystic Fibrosis Phenotype-c.4096A > T.
Recurrent Metabolic Alkalosis in a Cystic Fibrosis Patient: Coexistence with Congenital Chloride Diarrhea.
Clinical Outcomes Associated With Escherichia coli Infections in Adults With Cystic Fibrosis: A Cohort Study.
Clinical communication preferences in cystic fibrosis and strategies to optimize care.
New Treatment for Cystic Fibrosis.
Rectal Prolapse in Children: An Update to Causes, Clinical Presentation, and Management.

Pancreatoscopy-directed Electrohydraulic Lithotripsy in a Pediatric Patient With Pancreas Divisum and Cystic Fibrosis.
Disease-relevant mutations alter amino acid co-evolution networks in the second nucleotide binding domain of CFTR.
Associations between health literacy and patient outcomes in adolescents and young adults with cystic fibrosis.
A Therapy for Most with Cystic Fibrosis.
Dextran Microparticulate Inhalable Dry Powder for the Treatment of Cystic Fibrosis and Mucopolysaccharidosis.
Prevalence of hypermutator isolates of Achromobacter spp. from cystic fibrosis patients.
Procalcitonin predicts the severity of cystic fibrosis pulmonary exacerbations and readmissions in adult patients: a prospective cohort study.
Advances in the diagnosis and management of pulmonary aspergillosis.
Development of mutant human immunoreactive trypsinogen 1 (IRT1) and mutant human immunoreactive trypsinogen 2 (IRT2) for use in immunoassays.
Study Assessing the Safety, Tolerability, Pharmacokinetics, Food Effect, and Drug-Drug Interactions of PTI-801 in Healthy Volunteers, and Safety, Tolerability, and Pharmacokinetics of PTI-801 in Subjects With Cystic Fibrosis
Steps Ahead: Optimising Physical Activity and Health in Adults With Cystic Fibrosis Using Fitness Trackers and Personalised Goal-based Text Messaging Support
Lumacaftor/ivacaftor therapy is associated with reduced hepatic steatosis in cystic fibrosis patients.
A Phase I, dose-escalating study of safety and pharmacokinetics of inhaled dry powder vancomycin (AeroVanc) in volunteers and patients with cystic fibrosis: a new approach to therapy for methicillin-resistant Staphylococcus aureus.
Treatment with Lycopodium clavatum 200dH Intensifies Kidney and Liver Injury in Mice Infected with Toxoplasma gondii.
Potentially lethal cystic fibrosis gene variant in the orangutan.
Clinical Utility of the 1-minute Sit to Stand Test as a Measure of Submaximal Exercise Tolerance in Patients With Cystic Fibrosis During Acute Pulmonary Exacerbation
Organizing pneumonia secondary to Exophiala dermatitidis in cystic fibrosis: A case report.
KMBARC registry: protocol for a multicentre observational cohort study on non-cystic fibrosis bronchiectasis in Korea.
Culture-enriched metagenomic sequencing enables in-depth profiling of the cystic fibrosis lung microbiota.
A human ciliopathy reveals essential functions for NEK10 in airway mucociliary clearance.
Oxidative and inflammatory effects of pulmonary rehabilitation in patients with bronchiectasis. A prospective, randomized study.
Probiotics for people with cystic fibrosis.
A Study to Evaluate the Safety of Long-term Ivacaftor Treatment in Subjects With Cystic Fibrosis Who Are Less Than 24 Months of Age at Treatment Initiation and Have an Approved Ivacaftor-Responsive Mutation
Long-term Safety of Lumacaftor/Ivacaftor in Subjects With Cystic Fibrosis Who Are Homozygous for F508del and 12 to <24 Months of Age at Treatment Initiation
The Family of Chloride Channel Regulator, Calcium-activated Proteins in the Feline Respiratory Tract: A Comparative Perspective on Airway Diseases in Man and Animal Models.
[Impact of the transition period from childhood to adulthood in cystic fibrosis].
A systematic Cochrane Review of antioxidant supplementation lung disease for cystic fibrosis.
Volatile organic compound breath signatures of children with cystic fibrosis by real-time SESI-HRMS.
UNDERWEIGHT PATIENTS WITH CYSTIC FIBROSIS HAVE ACCEPTABLE SURVIVAL AFTER LUNG TRANSPLANTATION: A UNOS REGISTRY STUDY.
A Randomized, Double-Blind, Placebo-Controlled, Parallel-Group, Multi-Center Study to Assess the Efficacy, Safety and Tolerability, and Pharmacokinetics of INS1007 Administered Once Daily for 24 We…
A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Gating or…
A Multicenter, Randomized, Double-Blind, Placebo-Controlled Phase 2 Trial to Evaluate Efficacy and Safety of Lenabasum in Cystic Fibrosis
A Study to Explore the Impact of Lumacaftor/Ivacaftor on Disease Progression in Subjects Aged 2 Through 5 Years With Cystic Fibrosis, Homozygous for F508del
Short-term Effects of Physiotherapy on LCI
A Phase 2 Study to Evaluate Efficacy and Safety of VX-561 in Subjects Aged 18 Years and Older With Cystic Fibrosis
Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy
A Study to Compare US Marketed Pancrelipase Drug Product With Drug Product Manufactured With a Modernized Process at an Alternate Manufacturing Site, in Participants With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis.
Testing the Effect of Adding Chronic Oral Azithromycin to Inhaled Tobramycin in People With CF
Evaluation of the Safety, Usability and Efficacy of the ReX-C® System in Management of Medication Adherence, in Patients Receiving Kalydeco, Orkambi and/or Symdeko for the Treatment of Cystic Fibrosis (CF).
Cystic Fibrosis and Gut Dysmotility: The Effect of Polyethylene Glycol (PEG) on Intestinal Transit
Safety and Pharmacokinetic Study of Lumacaftor/Ivacaftor in Subjects 1 to Less Than 2 Years of Age With Cystic Fibrosis, Homozygous for F508del
Measuring and Improving Coproduction Using coopeRATE
Study of LAU-7b in the Treatment of Cystic Fibrosis in Adults
A Dose-finding Study of Inhaled OligoG vs Placebo in Patients With Cystic Fibrosis
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
A Phase 2 Study of ABBV-3067 Alone and in Combination With ABBV-2222
Impact of Bronchial Drainage Technique by the Medical Device Simeox® on Respiratory Function and Symptoms in Adult Patients With Cystic Fibrosis
Colonisation of Staphylococcus aureus in patients with Nasal Polyposis.
Role of Genetic Factors in the Development of Lung Disease
A 4-week Study to Test Different Doses of BI 1265162 in Adolescents and Adults With Cystic Fibrosis Using the Respimat® Inhaler – BALANCE – CF™1
Lung function changes before and after pulmonary exacerbation antimicrobial treatment in cystic fibrosis.
Biomarkers for diagnosis of Wilson’s disease.
Hyperactivity of Mek in TNS1 knockouts leads to potential treatments for cystic kidney diseases.
Deciphering the role of protein kinase CK2 in the maturation/stability of CFTR F508del.
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…
Forskolin-induced swelling of intestinal organoids correlates with disease severity in adults with cystic fibrosis and homozygous F508del mutations.
Evaluation of Copeptin during Pulmonary Exacerbation in Cystic Fibrosis.
Pancreatic Enzyme Replacement Therapy: A Concise Review.
Characterising burden of treatment in cystic fibrosis to identify priority areas for clinical trials.
Genetic variation in CFTR and modifier loci may modulate cystic fibrosis disease severity.
Nutrition in cystic fibrosis: From the past to the present and into the future.
Cystic fibrosis precision therapeutics: Emerging considerations.
Intranasal Peptide-Based FpvA-KLH Conjugate Vaccine Protects Mice From Pseudomonas aeruginosa Acute Murine Pneumonia.
Bilateral lung transplantation in cystic fibrosis with hepatitis C infection – a study of two cases.
A new role for heat shock factor 27 in the pathophysiology of Clostridium difficile toxin B.
Cystic fibrosis in Turkey: First data from the national registry.
Erratum: Profile Of Tezacaftor/Ivacaftor Combination And Its Potential In The Treatment Of Cystic Fibrosis [Erratum].
Computational Analysis of Energy Landscapes Reveals Dynamic Features that Contribute to Binding of Inhibitors to CFTR-Associated Ligand.
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
Whole body periodic acceleration in normal and reduced mucociliary clearance of conscious sheep.
Development of a library of thiophene-based drug-like LEGO molecules: evaluation of their anion binding, transport properties and cytotoxicity.
Pregnancy after lung and heart-lung transplantation: a French multicentre retrospective study of 39 pregnancies.
Drug allergy.
Profile of tezacaftor/ivacaftor combination and its potential in the treatment of cystic fibrosis.
Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
Cigarette smoke promotes HIV infection of primary bronchial epithelium and additively suppresses CFTR function.
Variable agreement between wearable heart rate monitors during exercise in cystic fibrosis.
Synthesis of β-d-galactopyranoside-Presenting Glycoclusters, Investigation of Their Interactions with Pseudomonas aeruginosa Lectin A (PA-IL) and Evaluation of Their Anti-Adhesion Potential.
Interplay between host-microbe and microbe-microbe interactions in cystic fibrosis.
Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell disease.
Genomic analyses of Burkholderia cenocepacia reveal multiple species with differential host-adaptation to plants and humans.
The implications of dysglycaemia on aerobic exercise and ventilatory function in cystic fibrosis.
Cystic fibrosis: triple therapy shows promising results.
Allergic bronchopulmonary aspergillosis.
Isolation of Enterobacteriaceae in airway samples is associated with worse outcome in preschool children with cystic fibrosis.
Availability of Zinc Impacts Interactions Between Streptococcus sanguinis and Pseudomonas aeruginosa in Co-culture.
Nasal polyps and rhinosinusitis.
Learning representations of microbe-metabolite interactions.
Characterization of the proteomic response of A549 cells following sequential exposure to Aspergillus fumigatus and Pseudomonas aeruginosa.
Adhesive and Cohesive Peel Force Measurement of Human Airway Mucus.
Variation of Burkholderia cenocepacia cell wall morphology and mechanical properties during cystic fibrosis lung infection, assessed by atomic force microscopy.
Telehealth spirometry for children with cystic fibrosis.
Rectal Prolapse in Children: An Update to Causes, Clinical Presentation, and Management.
Management of Australian Adults with Bronchiectasis in Tertiary Care: Evidence-Based or Access-Driven?
A phase 1/1b study of PUR1900, an inhaled formulation of itraconazole, in healthy volunteers and asthmatics to study safety, tolerability and pharmacokinetics.
Pandoraea sputorum Bacteremia In A Patient Who Had Undergone Allogeneic Liver Transplantation Plus Immunosuppressive Therapy: A Case Report.
Cystic fibrosis bone disease: Pathophysiology, assessment and prognostic implications.
A Randomized, Double-Blind, Placebo-Controlled Phase II Study to Evaluate the Efficacy and Safety of SPX-101 Inhalation Solution in Subjects with Cystic Fibrosis (HOPE-1 STUDY: HYDRATION FOR OPTIMA…
Hypoglycemia in cystic fibrosis: Prevalence, impact and treatment.
Endocrine complications after solid-organ transplant in cystic fibrosis.

Antimicrobial resistance in cystic fibrosis: A Delphi approach to defining best practices.
Malaria chemoprophylaxis in sickle cell disease.
Mabellini: a genome-wide database for understanding the structural proteome and evaluating prospective antimicrobial targets of the emerging pathogen Mycobacterium abscessus.
Cystic fibrosis newborn screening in Denmark: Experience from the first 2 years.
Continuous glucose monitoring in cystic fibrosis – A practical guide.
Cystic fibrosis related diabetes: Medical management.
Introduction: Endocrinology in Cystic Fibrosis.
CFTR processing, trafficking and interactions.
Puberty in cystic fibrosis.
Radiological difference between new sputum-positive and sputum-negative pulmonary tuberculosis.
Cephalosporin nitric oxide-donor prodrug DEA-C3D disperses biofilms formed by clinical cystic fibrosis isolates of Pseudomonas aeruginosa.
N-Acetylcysteine Instillation During Bronchoscopy for Patients Requiring Non-Cystic Fibrosis-Related Mucus Secretion Clearance: A Review of Clinical Effectiveness and Guidelines
Male reproductive health in cystic fibrosis.
Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis.
Cystic fibrosis related diabetes: Nutrition and growth considerations.
Energy balance and obesity in individuals with cystic fibrosis.
Cystic fibrosis bone disease treatment: Current knowledge and future directions.
Adrenal function in cystic fibrosis.
Vitamin D deficiency and its treatment in cystic fibrosis.
Growth failure and treatment in cystic fibrosis.
Female reproductive health in cystic fibrosis.
An Ancient CFTR Ortholog Informs Molecular Evolution in ABC Transporters.
Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.
Cystic fibrosis related diabetes in Europe: Prevalence, risk factors and outcome; Olesen et al.
Infection prevention and control in patients with cystic fibrosis (CF): Results from a survey in 35 German CF treatment centers.
EnVisioning the future: Endocrinology in cystic fibrosis.
Regeneration of airway epithelial cells to study rare cell states in cystic fibrosis.
Control of Ion Transport by Tmem16a Expressed in Murine Intestine.
Importance of SLC26 Transmembrane Anion Exchangers in Sperm Post-testicular Maturation and Fertilization Potential.
Dietary Phosphate Restriction Attenuates Polycystic Kidney Disease in Mice.
Ceftazidime-avibactam for the treatment of multidrug resistant Burkholderia cepacia complex in a pediatric cystic fibrosis patient.
Glycoprotein A as a biomarker of pulmonary infection and inflammation in children with cystic fibrosis.
Starting early: An international focus on mental health in children living with cystic fibrosis.
Activating alternative chloride channels to treat CF: Friends or Foes?: Report on the Meeting of the Basic Science Working Group in Dubrovnik, Croatia.
Intraoperative extracorporeal membrane oxygenation for lung transplantation in cystic fibrosis patients: Predictors and impact on outcome.
Longitudinal changes in lung function following initiation of lumacaftor/ivacaftor combination.
Occurrence, outcomes and predictors of portal hypertension in cystic fibrosis: A longitudinal prospective birth cohort study.
Mutation-specific dual potentiators maximize rescue of CFTR gating mutants.
Test-retest reliability, minimal detectable change and minimal clinically important differences in modified shuttle walk test in children and adolescents with cystic fibrosis.
Exploring the implications of different approaches to estimate centre-level adherence using objective adherence data in an adult cystic fibrosis centre – a retrospective observational study.
Regarding the article entitled “A smartphone application for reporting symptoms in adults with cystic fibrosis improves the detection of exacerbations: Results of a randomised controlled trial”.
Inhalational Drug Delivery in Pulmonary Aspergillosis.
Transitory pancreatic insufficiency in cystic fibrosis children.
Cystic fibrosis in black African children in South Africa: a case control study.
Fenretinide favorably affects mucins (MUC5AC/MUC5B) and fatty acid imbalance in a manner mimicking CFTR-induced correction.
Endocrine Complications of Cystic Fibrosis: A Multisystem Disease of the Endocrine Organs.
Cystic Fibrosis Diagnosis in Newborns, Children, and Adults.
Immune and Cytokine Dysfunction in Cystic Fibrosis.
Differences in clinical outcomes of paediatric cystic fibrosis patients with and without meconium ileus.
Improved detection of CFTR variants by targeted next-generation sequencing in male infertility: a case series.
Risk factors for progression of structural lung disease in school-age children with cystic fibrosis.
The CYSMA web server: an example of integrative tool for in silico analysis of missense variants identified in Mendelian disorders.
Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues.
Realizing the Dream of Molecularly Targeted Therapies for Cystic Fibrosis.
Is meconium ileus associated with worse outcomes in cystic fibrosis?
PEGylated mucus-penetrating nanocrystals for lung delivery of a new FtsZ inhibitor against Burkholderia cenocepacia infection.
Clinical significance of Pseudomonas aeruginosa 2-alkyl-4-quinolone quorum-sensing signal molecules for long-term outcomes in adults with cystic fibrosis.
Reclassification of CLSI criteria for ciprofloxacin and levofloxacin susceptibility against Pseudomonas aeruginosa: Implications for patients with cystic fibrosis(CF).
Herbal components of Japanese Kampo medicines exert laxative actions in colonic epithelium cells via activation of BK and CFTR channels.
Microarray profiling identifies extracellular circulating miRNAs dysregulated in cystic fibrosis.
Interactions of the effector ExoU from Pseudomonas aeruginosa with short-chain phosphatidylinositides provide insights into ExoU targeting to host membranes.
Identification of CFTR variants in Latino patients with cystic fibrosis from the Dominican Republic and Puerto Rico.
Alternative chloride transport pathways as pharmacological targets for the treatment of cystic fibrosis.
Risk factors for development of aminoglycoside resistance among gram-negative rods.
Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis.
Pulmonary Outcomes Associated with Long-Term Azithromycin Therapy in Cystic Fibrosis.
Direct-from-blood RNA sequencing identifies the cause of post-bronchoscopy fever.
Acute Pulmonary Exacerbations in Cystic Fibrosis.
A Phase IIa, Randomized, Double-blind, Placebo-controlled, Parallel Group Study to Assess the Safety and Efficacy of 28 Day Oral Administration of BAY 85-8501 in Patients with non-Cystic Fibrosis B…
Treating the Airway Consequences of Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction.
Quantification of MRI T2-weighted High Signal Volume in Cystic Fibrosis: A Pilot Study.
Use of MRI to Measure Bronchial Inflammation in Cystic Fibrosis.
Comparative Effectiveness of Vancomycin Versus Linezolid for the Treatment of Acute Pulmonary Exacerbations of Cystic Fibrosis.
Nontuberculous Mycobacteria in Cystic Fibrosis.
Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis.
Pulmonary Complications of Cystic Fibrosis.
The Extra-pulmonary Effects of CFTR Modulators in Cystic Fibrosis.
An Open-Label, Rollover Study to Evaluate the Long Term Safety and Efficacy of VX 770 in Subjects with Cystic Fibrosis
Effect of salinity and temperature on the expression of genes involved in branchial ion transport processes in European sea bass.
Impact of genetic testing and family health history of cystic fibrosis in the early prenatal diagnosis and prevention of a new case of genetic disorder.
Slowing ribosome velocity restores folding and function of mutant CFTR.
Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Lumacaftor in Combination With Ivacaftor
Complete Genome Sequence of the Multidrug-Resistant Pseudomonas aeruginosa Endemic Houston-1 Strain, Isolated from a Pediatric Patient with Cystic Fibrosis and Assembled Using Oxford Nanopore and Illumina Sequencing.
Distal intestinal obstruction syndrome: a diagnostic and therapeutic challenge in cystic fibrosis.
Pseudomonas aeruginosa-Derived Volatile Sulfur Compounds Promote Distal Aspergillus fumigatus Growth and a Synergistic Pathogen-Pathogen Interaction That Increases Pathogenicity in Co-infection.
Total oxidant and antioxidant status and paraoxonase 1 levels of children with non-cystic fibrosis bronchiectasis.
Plumbagin Prevents Secretory Diarrhea by Inhibiting CaCC and CFTR Channel Activities.
Infrared biomarkers of impaired cystic fibrosis transmembrane regulator protein biogenesis.
Aberrant MicroRNAomics in Pulmonary Complications: Implications in Lung Health and Diseases.
Cystic fibrosis drugs to be available on NHS in England within 30 days.
Design of N-Terminal Derivatives from a Novel Dermaseptin Exhibiting Broad-Spectrum Antimicrobial Activity against Isolates from Cystic Fibrosis Patients.
The KDEL Receptor has a role in the Biogenesis and Trafficking of the Epithelial Sodium Channel (ENaC).
Spray-drying of inhalable, multifunctional formulations for the treatment of biofilms formed in cystic fibrosis.
Initial Regional Evaluation of Cystic Fibrosis Newborn Screening Program: Data from Mediterranean Coast of Turkey.
The importance of effective registries in pulmonary diseases and how to optimize their output.
The anion transporter SLC26A9 localizes to tight junctions and is degraded by the proteasome when co-expressed with F508del-CFTR.
Inhaled Mucoactive Particles with Tailored Architecture for Enhanced Aerodynamicity, Stability and Efficacy.
Introduction to Purinergic Signaling.
CF Bronchodilation
Rational particle design to overcome pulmonary barriers for obstructive lung diseases therapy.

LiverTox: Clinical and Research Information on Drug-Induced Liver Injury
Pneumonia due to Pandoraea Apista after evacuation of traumatic intracranial hematomas:a case report and literature review.
Medical interventions for chronic rhinosinusitis in cystic fibrosis.
LiverTox: Clinical and Research Information on Drug-Induced Liver Injury
Immunoreactive trypsinogen levels in newborn screened infants with an inconclusive diagnosis of cystic fibrosis.
High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening.
Patienthèque of Finisterian (South of Brittany) Children With Cystic Fibrosis in the Time of Precision Medicine
Role of ion channels in gastrointestinal cancer.
A Phase 2 Study to Evaluate the Safety, Tolerability, PK and PD of ELX-02 in Cystic Fibrosis Patients With G542X Allele
Ketosis Ameliorates Renal Cyst Growth in Polycystic Kidney Disease.
[Relationship of Cystic Fibrosis Transmembrane Conductance Regulator Expression with Clinical Features and Prognosis in Patients with Acute Leukemia].
Hamartomas and midline anomalies in association with infantile hemangiomas, PHACE, and LUMBAR syndromes.
Cystic fibrosis-related diabetes: the missing evidence.
Lumacaftor/ivacaftor for cystic fibrosis.
The Immune-Modulator Pidotimod Affects the Metabolic Profile of Exhaled Breath Condensate in Bronchiectatic Patients: A Metabolomics Pilot Study.
Therapeutic endobronchial resection of a benign tumor in a patient with cystic fibrosis.
Uncovering the structure and function of Pseudomonas aeruginosa periplasmic proteins by an in silico approach.
Tezacaftor/ivacaftor for cystic fibrosis.
Parenting Concerns in Patients With Cystic Fibrosis (MucoPar)
Azithromycin is the answer in paediatric respiratory medicine, but what was the question?
Pro Con Debate – Infection prevention and control in cystic fibrosis: One size fits all The argument against.
Orphan Drug Pricing and Costs: A Case Study of Kalydeco and Orkambi.
Basal Serum Cortisol and Testosterone/Cortisol Ratio Are Related to Rate of Na+ Lost During Exercise in Elite Soccer Players.
Targeting Proteases in Cystic Fibrosis Lung Disease: Paradigms, Progress, and Potential.
Adapting to the Airways: Metabolic Requirements of Pseudomonas aeruginosa during the Infection of Cystic Fibrosis Patients.
Tezacaftor and ivacaftor for the treatment of cystic fibrosis.
Complete Genome Sequence of Stenotrophomonas Phage Pokken.
[Insurability and history of cancer].
Evaluation of active neutrophil elastase in sputum of bronchiectasis and cystic fibrosis patients: A comparison among different techniques.
Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?
Long-term non-invasive ventilation in cystic fibrosis: A bridge too far?
Dispersion of endoplasmic reticulum-associated compartments by 4-phenyl butyric acid in yeast cells.
Gastroesophageal reflux in cystic fibrosis across the age spectrum.
Renal involvement in cystic fibrosis: is it a contraindication to transplant?
Early Detection of Cystic Fibrosis Acute Pulmonary Exacerbations by Exhaled Breath Condensate Metabolomics.
Quantitative real-time PCR assay for the rapid identification of the intrinsically multidrug-resistant bacterial pathogen Stenotrophomonas maltophilia.
Factors affecting the growth of infants diagnosed with cystic fibrosis by newborn screening.
Tedizolid is a promising antimicrobial option for the treatment of Staphylococcus aureus infections in cystic fibrosis patients.
Pseudomonas aeruginosa colonization in the upper and lower airways of a child with cystic fibrosis: a father’s meticulous approach to successful eradication.
StatPearls
StatPearls
StatPearls
Inflammatory Myofibroblastic Tumor After Lung Transplant-A Rare and Aggressive Complication: A Case Report.
Frequency of Cystic Fibrosis Transmembrane Conductance Regulator Variants in Individuals Evaluated for Primary Ciliary Dyskinesia.
Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
Improved culture detection of Staphylococcus aureus from sputum of patients with cystic fibrosis (CF).
Losartan Rescues Inflammation-Related Mucociliary Dysfunction in Relevant Models of Cystic Fibrosis.
Structure of Pseudomonas aeruginosa ribosomes from an aminoglycoside-resistant clinical isolate.
In vitro Activities of β-Lactam-β-Lactamase Inhibitor Antimicrobial Agents Against Cystic Fibrosis Respiratory Pathogens.
A Phase 2 Study to Evaluate the Safety, Tolerability, PK and PD in Cystic Fibrosis Patients With at Least 1 G542X Allele
Physiology of sweat gland function: The roles of sweating and sweat composition in human health.
Infection with Prevotella nigrescens induces TLR2 signalling and low levels of p65 mediated inflammation in Cystic Fibrosis bronchial epithelial cells.
Ovarian Metabolic activity in Dehydroepiandrosterone-Induced Polycystic Ovary in Wistar rats Treated with Aspirin.
Improving the phenotype risk score as a scalable approach to identifying patients with Mendelian disease.
Oral administration of Lactobacillus acidophilus alleviates exacerbations in Pseudomonas aeruginosa and Staphylococcus aureus pulmonary infections.
State of the art in cystic fibrosis pharmacology-Optimization of antimicrobials in the treatment of cystic fibrosis pulmonary exacerbations: I. Anti-methicillin-resistant Staphylococcus aureus (MRSA) antibiotics.
A negative screening of rare genetic variants in the ADIPOQ and STATH genes in cystic fibrosis.
Non-invasive ventilation versus oxygen therapy in cystic fibrosis: Long-term effects – Reply.
Non-invasive ventilation versus oxygen therapy in cystic fibrosis: Long-term effects.
Cl- channels regulate lipid droplet formation via Rab8a expression during adipocyte differentiation.
Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis.
Osteopenia and Osteoporosis in Patients with Bronchiectasis: Association with Respiratory Parameters, Body Composition, Muscle Strength and Bone Remodeling Biomarkers.
Quantifying Long-term Changes in Lung Function and Exacerbations after Initiation of Azithromycin in Cystic Fibrosis.
Inhaled nitric oxide for treating pain crises in people with sickle cell disease.
Changes in Airway Microbiome and Inflammation with Ivacaftor Treatment in Patients with Cystic Fibrosis and the G551D Mutation.
Bile Acids, Microbiota, and Cystic Fibrosis: Channeling Intestinal FXR Signals.
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
Reducing Health Care Disparities in Sickle Cell Disease: A Review.
Health care provider’s experiences, practices, and recommendations for interventions and screening of cystic fibrosis patients with disordered eating: A qualitative analysis.
Putting CHIP(S) on the Table – Introducing Nitrosothiols (SNOs) into the Arena of CFTR Modulation.
N-acetylcysteine for Management of Distal Intestinal Obstruction Syndrome.
A Dose-finding Study of Inhaled OligoG vs Placebo in Patients With Cystic Fibrosis
A novel high-throughput molecular counting method with single base-pair resolution enables accurate single-gene NIPT.
Anaphylaxis and anaphylactoid reactions associated with the insertion of peripherally inserted central catheters: A multiyear comparative retrospective cohort study.
Two novel and correlated CF-causing insertions in the (TG)mTn tract of the CFTR gene.
Secondhand Smoke Exposure and Serum Trypsinogen in Cystic Fibrosis Carriers.
Inhalational Anesthetics Induce Neuronal Protein Aggregation and Affect ER Trafficking.
Antibiotics for treating osteomyelitis in people with sickle cell disease.
Prevalence and characterization of chronic rhinosinusitis in patients with non-cystic fibrosis bronchiectasis at a tertiary care center in the United States.
Vitamin D and Prebiotics for Intestinal Health in Cystic Fibrosis
19F MRI of Ventilation in Subjects With Cystic Fibrosis Undergoing Treatment for Pulmonary Exacerbation
Adiponectin Expression Is Modulated by Long-Term Physical Activity in Adult Patients Affected by Cystic Fibrosis.
The renoprotective effect of concomitant fosfomycin in the treatment of pulmonary exacerbations in cystic fibrosis.
Approaching two decades of cystic fibrosis research in Qatar: a historical perspective and future directions.
Ceftazidime plasma concentration in a patient with cystic fibrosis treated with ceftazidime/avibactam plus trimethoprim/sulfametoxazole for Bulkholderia cepacia reacutization.
Feasibility and Acceptability of a Telephone-Based Chaplaincy Intervention to Decrease Parental Spiritual Struggle.
Increased expression of anion transporter SLC26A9 delays diabetes onset in cystic fibrosis.
Antioxidant supplementation for lung disease in cystic fibrosis.
Of Slide Rules and Stethoscopes: AI and the Future of Doctoring.
A Phase 3, Open-label, Rollover Study to Evaluate the Safety and Efficacy of Long-term Treatment With Tezacaftor in Combination With Ivacaftor in Subjects With Cystic Fibrosis Aged 6 Years and Olde…
Acceptance and Commitment Therapy vs. Supportive Psychotherapy With Cystic Fibrosis Patients
Pseudomonas aeruginosa Quorum Sensing Molecule Alters Skeletal Muscle Protein Homeostasis by Perturbing the Antioxidant Defense System.
A patient’s experience of cystic fibrosis care.
Characterization of poxtA, a novel phenicol-oxazolidinone-tetracycline resistance gene from an MRSA of clinical origin.
Cystic fibrosis lung disease and bronchiectasis.
Progress in understanding the molecular pathology and microbiology of cystic fibrosis.
Bridging the Gap between Scientific Advancement and Real-World Application: Pediatric Genetic Counseling for Common Syndromes and Single-Gene Disorders.
The future of cystic fibrosis care: a global perspective.
Pharmacological reversal of renal cysts from secretion to absorption suggests a potential therapeutic strategy for managing polycystic kidney disease.
Clinical care for cystic fibrosis: preparing for the future now.

Stem Cells, Cell Therapies, and Bioengineering in Lung Biology and Diseases 2017. An Official American Thoracic Society Workshop Report.
The C-terminal dimerization domain of the respiratory mucin MUC5B functions in mucin stability and intracellular packaging before secretion.
Efficacy and safety of oral sildenafil in cystic fibrosis children with mild to moderate lung disease.
ABC Family Transporters.
Role of Genetic Factors in the Development of Lung Disease
Commentaries on Viewpoint: Use aerobic energy expenditure instead of oxygen uptake to quantify exercise intensity and predict endurance performance.
Adherence to long-term therapies in cystic fibrosis: a French cross-sectional study linking prescribing, dispensing, and hospitalization data.
Pathology of Echinococcosis: A Morphologic and Immunohistochemical Study on 138 Specimens With Focus on the Differential Diagnosis Between Cystic and Alveolar Echinococcosis.
New Drug Indications for Pediatric Type 2 Diabetes, Cystic Fibrosis, and Hospital-Acquired and Ventilator-Associated Bacterial Pneumonia.
A Retrospective Cohort Study of Growth in the First Two Years of Life in Preterm Infants with Cystic Fibrosis.
Long-Term Inhaled Antipseudomonal Antibiotics in Cystic Fibrosis.
Prevention of malnutrition in cystic fibrosis.
Prevention of osteoporosis in cystic fibrosis.
Association between fat-soluble nutrient status and auditory and visual related potentials in newly diagnosed non-screened infants with cystic fibrosis: A case-control study.
Area Deprivation as a Risk Factor for Methicillin-resistant Staphylococcus aureus Infection in Pediatric Cystic Fibrosis.
Retrospective Study on the Association of Biomarkers With Real-world Outcomes of Omalizumab-treated Patients With Allergic Asthma.
Enzyme-encapsulating polymeric nanoparticles: A potential adjunctive therapy in Pseudomonas aeruginosa biofilm-associated infection treatment.
Adjusting for time-varying confounders in survival analysis using structural nested cumulative survival time models.
Increased water intake reduces long-term renal and cardiovascular disease progression in experimental polycystic kidney disease.
Nasal nitric oxide levels in primary ciliary dyskinesia, cystic fibrosis and healthy children.
Course of lung function in children with cystic fibrosis in their first 3 years of life.
A rare clinical association: Barth syndrome and cystic fibrosis.
Risk factors associated with pulmonary exacerbations in pediatric patients with cystic fibrosis.
Regulation of CFTR Bicarbonate Channel Activity by WNK1: Implications for Pancreatitis and CFTR-related disorders.
Waardenburg Syndrome Type IV De Novo SOX10 Variant Causing Chronic Intestinal Pseudo-Obstruction.
Exposure to Bile Leads to the Emergence of Adaptive Signaling Variants in the Opportunistic Pathogen Pseudomonas aeruginosa.
Clinical outcomes for cystic fibrosis patients with Pseudomonas aeruginosa cross-infections.
Intestinal organoids in infants and children.
Technique and outcome of Domino Liver Transplantation from patients with Maple Syrup Urine Disease: Expanding the donor pool for Live Donor Liver Transplantation.
Coordinate regulation of ELF5 and EHF at the chr11p13 CF modifier region.
[Polygenic risk prediction of common diseases: from epidemiology to clinical application].
SPINK1, PRSS1, CTRC, and CFTR Genotypes Influence Disease Onset and Clinical Outcomes in Chronic Pancreatitis.
US Patient-Centered Research Priorities and Roadmap for Bronchiectasis.
Noninvasive Prenatal Diagnosis for Cystic Fibrosis: Implementation, Uptake, Outcome, and Implications.
Molecular and Microbial Microenvironments in Chronically Diseased Lungs Associated with Cystic Fibrosis.
Short-term consequences of F508del-CFTR thermal instability on CFTR-dependent transepithelial currents in human airway epithelial cells.
Pyoverdine-Dependent Virulence of Pseudomonas aeruginosa Isolates From Cystic Fibrosis Patients.
Genomic and phenotypic comparison of environmental and patient-derived isolates of Pseudomonas aeruginosa suggest that antimicrobial resistance is rare within the environment.
Non-ribosomal Peptide Synthetase Gene Clusters in the Human Pathogenic Fungus Scedosporium apiospermum.
Lung Inflammatory Environments Differentially Alter Mesenchymal Stromal Cell Behavior.
The Effect of 2-Thiocyanatopyridine Derivative 11026103 on Burkholderia Cenocepacia: Resistance Mechanisms and Systemic Impact.
Validity and Reliability of Pediatric Nutrition Screening Tools for Hospital, Outpatient, and Community Settings: A 2018 Evidence Analysis Center Systematic Review.
Practice variation of genetic counselor engagement in the cystic fibrosis newborn screen-positive diagnostic resolution process.
Efficacy of Glutathione for Patients With Cystic Fibrosis: A Meta-analysis of Randomized-Controlled Studies.
Epidemiology of bronchiectasis in the UK: Findings from the British lung foundation’s ‘Respiratory health of the nation’ project.
Face Masks Reduce the Release of Pseudomonas aeruginosa Cough Aerosols When Worn for Clinically Relevant Periods.
Does partitioning the subcomponents of the ventilatory equivalent for carbon dioxide slope provide evidence that ventilatory efficiency is retained in cystic fibrosis?
Evaluation of hospitalization data for the CFFPR-PHIS linked data set.
The identification of loci that encode potentially active compounds against drug resistant pathogens amidst a decreasing pool of novel antibiotics.
Cigarette Smoke Exposure Induces Retrograde Trafficking of CFTR to the Endoplasmic Reticulum.
RNA-based qPCR as a tool to quantify and to characterize dual-species biofilms.
The open door policy – An important and under-recognised activity of the adult CF centre.
Dysglycaemia in CF – Why are investigating it, what should we measure and how should we treat it?
Guidance for computed tomography (CT) imaging of the lungs for patients with cystic fibrosis (CF) in research studies.
Variations in prenatal screening in a US federal healthcare system: Same coverage, different options.
Home Airway Clearance in CF Patients
Management of Exocrine Pancreatic Insufficiency in Children.
The Prevalence of Pseudomonas Aeruginosa Infection Over a Ten-Year Period in Children with Cystic Fibrosis
Efficacy and Safety Curcuma zadoaria L. to inactivate the hydatid cyst protoscoleces.
Minimal detectable change in six-minute walk test in children and adolescents with cystic fibrosis.
Upper versus lower airway microbiome and metagenome in children with cystic fibrosis and their correlation with lung inflammation.
Modeling of Chest Physiotherapy Using Impedance Measurements (PHYSIOMOD)
Ancillary Home Airway Clearance in CF Patients ( HomeCareSIMEOX )
Social complexity negatively influences lung function in cystic fibrosis after transfer to adult care.
Splenectomy for people with thalassaemia major or intermedia.
Influence of sex gap on muscle strength and functional mobility in patients with cystic fibrosis.
A critical review of definitions used to describe Pseudomonas aeruginosa microbiological status in patients with cystic fibrosis for application in clinical trials.
NHS England explores funding options for cystic fibrosis drug.
Targeting airway inflammation in cystic fibrosis.
Micronutrient intake in children with cystic fibrosis in Sydney, Australia.
A Metacognitive Intervention of Narrative Imagery for young people with cystic fibrosis: A feasibility study.
VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.
ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in Cystic Fibrosis.
Effects of AD on VI in Patients With CF
Unwarranted use of intravenous aminoglycosides at UK paediatric cystic fibrosis centres.
A smartphone application for reporting symptoms in adults with cystic fibrosis improves the detection of exacerbations: Results of a randomised controlled trial.
A Novel Tool to Evaluate the Accuracy of Predicting Survival and Guiding Lung Transplantation in Cystic Fibrosis.
Cystic Fibrosis Mutation Spectrum in North Macedonia: A Step Toward Personalized Therapy.
Multilocus Sequence Typing of Staphylococcus aureus.
Methicillin-Resistant Staphylococcus aureus Infection and Treatment Options.
Estimated Ventricular Size, Asthma Severity and Exacerbations: The SARP III Cohort.
Estimated Ventricular Size, Asthma Severity and Exacerbations: The SARP III Cohort.
Proximal small bowel obstruction in a patient with cystic fibrosis: a case report.
Bone Health in Children and Youth with Cystic Fibrosis: A Systematic Review and Meta-Analysis of Matched Cohort Studies.
Scotland strikes deal to offer cystic fibrosis drugs.
Validation of the “Good2Go”: the first French-language transition readiness questionnaire.
An Unusual Lung Mass of Heterotopic Pancreatic Tissue in a Neonate With an Elevated Immunoreactive Trypsinogen on Newborn Screen.
Bronchoscopic instillation of DNase to manage refractory lobar atelectasis in a lung cancer patient.
Assessing the Perceptions of Inspiratory Muscle Training in Children With Cystic Fibrosis and Their Multidisciplinary Team: Mixed-Methods Study.
Clinical Adoption of mHealth Technology to Support Pediatric Cystic Fibrosis Care in Sweden: Qualitative Case Study.
Evaluation of VX 445/TEZ/IVA in Cystic Fibrosis Subjects 6 Through 11 Years of Age
Evaluating High Flow Humidification Therapy in Patients With Cystic Fibrosis
A Study to Explore the Impact of Lumacaftor/Ivacaftor on Disease Progression in Subjects Aged 2 Through 5 Years With Cystic Fibrosis, Homozygous for F508del
Maternal Smoking Induces Acquired CFTR Dysfunction in Neonatal Rats.
G-CSF and GM-CSF Modify Neutrophil Functions at Concentrations found in Cystic Fibrosis.
Creating genetic reports that are understood by nonspecialists: a case study.
Comparative Analysis of Peptidoglycans From Pseudomonas aeruginosa Isolates Recovered From Chronic and Acute Infections.
Diagnostic imaging in adult non-cystic fibrosis bronchiectasis.
[Complex care, high cost, and loss of income: frequent issues for families of children and adolescents with rare health conditions].
Predictive value of genomic screening: cross-sectional study of cystic fibrosis in 50,788 electronic health records.

Prevalence of Bactericidal/Permeability-Increasing Protein Autoantibodies in Cystic Fibrosis Patients: Systematic Review and Meta-Analysis.
Characterization of Cystic Fibrosis Airway Smooth Muscle Cell Proliferative and Contractile Activities.
Achromobacter spp. healthcare associated infections in the French West Indies: a longitudinal study from 2006 to 2016.
Single dose escalation studies with inhaled POL6014, a potent novel selective reversible inhibitor of human neutrophil elastase, in healthy volunteers and subjects with cystic fibrosis.
Pharmacokinetics of colistin after nebulization or intravenous administration of colistin methanesulphonate (Colimycin®) to cystic fibrosis patients.
Human cystic fibrosis monocyte derived macrophages display no defect in acidification of phagolysosomes when measured by optical nanosensors.
Conceptual Model of Biofilm Antibiotic Tolerance that Integrates Phenomena of Diffusion, Metabolism, Gene Expression, and Physiology.
Prevention of drug-related complications in cystic fibrosis.
Survey of current treatment practices for venous thromboembolism in patients with cystic fibrosis.
Cytological appearance of pancreatic cystosis on fine-needle aspiration.
Hypersensitivity pneumonitis in a cystic fibrosis patient.
Efficacy of Simeox Airway Clearance Therapy in Children With Cystic Fibrosis
Airway disease on chest computed tomography of preschool children with cystic fibrosis is associated with school-age bronchiectasis.
Rapidly Progressive Cystic Lung Disease.
Magnesium for treating sickle cell disease.
Lung clearance index to detect the efficacy of Aztreonam lysine inhalation in patients with cystic fibrosis and near normal spirometry – A single-centre feasibility study.
A European ECMM-ESCMID survey on goals and practices for mycobiota characterization using Next Generation Sequencing.
Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis.
A Phase 3, Open-label Study Evaluating the Long-term Safety and Efficacy of VX-445 Combination Therapy in Subjects With Cystic Fibrosis Who Are Heterozygous for the F508del Mutation and a Gating or…
The impact of chest computed tomography and chest radiography on clinical management of cystic fibrosis lung disease.
Acid sphingomyelinase regulates TH 2-cytokine release and bronchial asthma.
A physiologically-motivated model of cystic fibrosis liquid and solute transport dynamics across primary human nasal epithelia.
Phase-Ib/IIa study to investigate safety, tolerability, pharmacokinetics and pharmacodynamics of orally inhaled multiple doses of POL6014 in patients with Cystic Fibrosis
Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis-data from the European cystic fibrosis society patient registry.
In vitro evolution of Pseudomonas aeruginosa AA2 biofilms in the presence of cystic fibrosis lung microbiome members.
Enhanced osmoregulatory ability marks the smoltification period in developing chum salmon (Oncorhynchus keta).
The pathobiology of polycystic kidney disease from a metabolic viewpoint.
Toothbrushes may convey bacteria to the cystic fibrosis lower airways.
Exome sequencing of Saudi Arabian patients with ADPKD.
Systemic versus tissue-level prolactin signaling in a teleost during a tidal cycle.
[Ion transporters in the lungs. Use as therapeutic targets].
Duration of intravenous antibiotic therapy in people with cystic fibrosis.
Eradication of Pseudomonas aeruginosa with inhaled colistin in adults with non-cystic fibrosis bronchiectasis.
Viral respiratory tract infections in young children with cystic fibrosis: a prospective full-year seasonal study.
Phosphorylation-dependent modulation of CFTR macromolecular signalling complex activity by cigarette smoke condensate in airway epithelia.
Whole-exome sequencing provides insights into monogenic disease prevalence in Northwest Russia.
Once-daily versus multiple-daily dosing with intravenous aminoglycosides for cystic fibrosis.
MR Imaging of Lung in the Follow-up Assessment of Cystic Fibrosis
Reduced expression of the Ion channel CFTR contributes to airspace enlargement as a consequence of aging and in response to cigarette smoke in mice.
Isolation and identification of Pandoraea spp. From bronchoalveolar lavage of cystic fibrosis patients in Iran.
Feasibility of a New Model for Exercise Prescription in Cystic Fibrosis
Ensifentrine (RPL554): an investigational PDE3/4 inhibitor for the treatment of COPD.
EFSUMB Gastrointestinal Ultrasound (GIUS) Task Force Group: Celiac sprue and other rare gastrointestinal diseases ultrasound features.
Children with bronchiectasis have poorer lung function than those with cystic fibrosis and do not receive the same standard of care.
Deciphering multivalent glycocluster-lectin interactions through AFM characterization of the self-assembled nanostructures.
Case Report of Nursing Care for a Pregnant Woman With Cystic Fibrosis.
Salsalate, but not metformin or canagliflozin, slows kidney cyst growth in an adult-onset mouse model of polycystic kidney disease.
Nutritional assessment in adults with cystic fibrosis.
Generation of a CFTR knock-in reporter cell line (MHHi006-A-1) from a human induced pluripotent stem cell line.
Associations between demographic characteristics and unmet supportive care needs in adults with cystic fibrosis.
Addressing the burden of illness in adults with cystic fibrosis with screening and triage: An early intervention model of palliative care.
Evaluation of TD test for analysis of persistence or tolerance in clinical isolates of Staphylococcus aureus.
A comorbidity of CF in need of our attention and activity: Attention Deficit Hyperactivity Disorder!
Prenatal genetic testing for cystic fibrosis: a systematic review of clinical effectiveness and an ethics review.
Current status of fertility and family formation in men with cystic fibrosis.
The bridging bronchus: A comprehensive review of a rare, potentially life-threatening congenital airway anomaly associated with cardiovascular defects.
Caregiver burden in children with cystic fibrosis and primary ciliary dyskinesia.
Impact of a Reduction in Susceptibility Testing for Pseudomonas aeruginosa in a Cystic Fibrosis Program.
Screening for Cystic Fibrosis-Related Diabetes and Pre-diabetes: Evaluating 1,5-Anhydroglucitol, Fructosamine, Glycated Albumin, and Hemoglobin A1c.
Impaired intestinal farnesoid X receptor signaling in cystic fibrosis mice.
GH-IGF1 Axis in Children with Cystic Fibrosis.
Associations between circulating inflammatory markers, diabetes type and complications in youth.
Congenital hepatic fibrosis and coexistent retinal macular degeneration: A case report.
Impact of clonally-related Burkholderia contaminans strains in two patients attending an Italian cystic fibrosis centre: a case report.
Loss of incretin effect contributes to postprandial hyperglycaemia in cystic fibrosis-related diabetes.
New insights into the immunoproteome of B. cenocepacia J2315 using serum samples from cystic fibrosis patients.
A Case Report of Intraoperative Coagulopathy Secondary to Chronic Vitamin K Deficiency.
A Metabolome- and Metagenome-Wide Association Network Reveals Microbial Natural Products and Microbial Biotransformation Products from the Human Microbiota.
Major depression and psychiatric comorbidity in Turkish children and adolescents with cystic fibrosis.
Timing of choledochal cyst perforation.
Effect of Lumacaftor/Ivacaftor on Pulmonary Exacerbation Rates in Members with Cystic Fibrosis in a Medicaid Population.
Inactivation of Tsc2 in Abcg2 Lineage Derived Cells Drives the Appearance of Polycystic Lesions and Fibrosis in the Adult Kidney.
Off the street phasing (OTSP): no hassle haplotype phasing for molecular PGD applications.
Amikacin liposome inhalation suspension for chronic Pseudomonas aeruginosa infection in cystic fibrosis.
Psychological predictors of nutritional adherence in adolescents with cystic fibrosis.
Adapting the James Lind Alliance priority setting process to better support patient participation: an example from cystic fibrosis.
Prevalence and Antibiotic Resistance of Stenotrophomonas maltophilia in Respiratory Tract Samples: A 10-Year Epidemiological Snapshot.
Specific Inhibition of the NLRP3 Inflammasome as an Anti-Inflammatory Strategy in Cystic Fibrosis.
Antimicrobial Treatment of Staphylococcus aureus in Patients With Cystic Fibrosis.
The Emergence of Rare Clinical Aspergillus Species in Qatar: Molecular Characterization and Antifungal Susceptibility Profiles.
In silico Analysis Reveals Distribution of Quorum Sensing Genes and Consistent Presence of LuxR Solos in the Pandoraea Species.
The presence of Aspergillus fumigatus is associated with worse respiratory quality of life in cystic fibrosis.
Biochemistry of very-long-chain and long-chain ceramides in cystic fibrosis and other diseases: The importance of side chain.
Surgical management of lumbar disc herniation in children and adolescents.
The association of pediatric cystic fibrosis-related diabetes screening on clinical outcomes by center: A CF patient registry study.
Deciphering the Ecology of Cystic Fibrosis Bacterial Communities: Towards Systems-Level Integration.
Predictors of Malnutrition in Children with Cystic Fibrosis.
Liver Ultrasound Patterns in Children With Cystic Fibrosis Correlate With Noninvasive Tests of Liver Disease.
Prevention of transmission of Mycobacterium abscessus among patients with cystic fibrosis.
Opportunities to Improve Utilization of Palliative Care among Adults with Cystic Fibrosis: A Systematic Review.
Integrating Clinical Ultrasound into Screening for Cystic Fibrosis Liver Disease: Approach with Caution and Optimism.
Comparative study of immune responses elicited by outer membrane vesicles of different Pseudomonas aeruginosa strains.
Phosphatidylcholine Passes by Paracellular Transport to the Apical Side of the Polarized Biliary Tumor Cell Line Mz-ChA-1.
Fluoroquinolones for the Treatment of Other Respiratory Tract Infections: A Review of Clinical Effectiveness, Cost-Effectiveness, and Guidelines
Incidental high-grade dysplasia of the cystic duct margin: case report and literature review.
Efficacy and safety of tobramycin inhalation powder in bronchiectasis patients with P. aeruginosa infection: Design of a dose-finding study (iBEST-1).
Basic and translational science advances in congenital diaphragmatic hernia.
What’s BEST for children with non-cystic fibrosis bronchiectasis?
Efficacy of oral amoxicillin-clavulanate or azithromycin for non-severe respiratory exacerbations in children with bronchiectasis (BEST-1): a multicentre, three-arm, double-blind, randomised placebo-controlled trial.
Discrete choice experiment to evaluate preferences of patients with cystic fibrosis among alternative treatment-related health outcomes: a protocol.

A high prevalence of chronic gastrointestinal symptoms in adults with cystic fibrosis is detected using tools already validated in other GI disorders.
To establish a treatment for GI symptoms of cystic fibrosis, it is necessary to develop a symptom evaluation tool.
To treat or not to treat: dysglycaemia in cystic fibrosis.
Cystic Fibrosis: The Sense of Smell.
StatPearls
StatPearls
Total pancreatectomy with islet autotransplantation in a pancreatic-sufficient cystic fibrosis patient.
Automated glycemic control with the bionic pancreas in cystic fibrosis-related diabetes: A pilot study.
Luminescent nanosensors for ratiometric monitoring of three-dimensional oxygen gradients in lab and clinical Pseudomonas aeruginosa biofilms.
Metabolite Profiling of the Antisense Oligonucleotide Eluforsen Using Liquid Chromatography-Mass Spectrometry.
CNS imaging studies in cystic fibrosis patients presenting with sudden neurological events.
Singing as an adjunct therapy for children and adults with cystic fibrosis.
c.753_754delAG, a novel CFTR mutation found in a Chinese patient with cystic fibrosis: A case report and review of the literature.
Controlled evaluation of a transition clinic for Dutch young people with cystic fibrosis.
Treatment of dental and orthodontic complications in thalassaemia.
Feedback of airborne bacterial consortia to haze pollution with different PM2.5 levels in typical mountainous terrain of Jinan, China.
Measuring and Improving Coproduction Using coopeRATE
Burkholderia cepacia Complex Species Differ in the Frequency of Variation of the Lipopolysaccharide O-Antigen Expression During Cystic Fibrosis Chronic Respiratory Infection.
Anti-PcrV titers in non-cystic fibrosis patients with Pseudomonas aeruginosa respiratory tract infection.
A 4-week Study to Test Different Doses of BI 1265162 in Adolescents and Adults With Cystic Fibrosis Using the Respimat® Inhaler
Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System.
Emerging non-genetic therapies for sickle cell disease.
Training dogs to differentiate Pseudomonas aeruginosa from other cystic fibrosis bacterial pathogens: not to be sniffed at?
Measures of Cystic Fibrosis Airway Microbiota during Periods of Clinical Stability.
Robot-assisted radical prostatectomy due to a primary carcinoid prostatic tumour in a three-year-old child: a case report.
Iterative Optimization of the Cyclic Peptide SFTI-1 Yields Potent Inhibitors of Neutrophil Proteinase 3.
Taste Receptors Regulation in CF Patients
Clinical Utility of the 1-minute Sit to Stand Test as a Measure of Submaximal Exercise Tolerance in Patients With Cystic Fibrosis During Acute Pulmonary Exacerbation
A Latent Class Approach to Modeling Trajectories of Health Care Cost in Pediatric Cystic Fibrosis.
Inhaled Corticosteroids for Cystic Fibrosis: A Review of Clinical Effectiveness
Patient-reported outcomes in patients with cystic fibrosis with a G551D mutation on ivacaftor treatment: results from a cross-sectional study.
Health-resource use and quality of life in children with bronchiectasis: a multi-center pilot cohort study.
A paper-based length of stain analytical device for naked eye (readout-free) detection of cystic fibrosis.
P.TYR1381X: Are We Facing a New Mutation of CFTR in a Patient With Severe Cystic Fibrosis?
Cystic fibrosis drugs are rejected for NHS use in Scotland.
Mouse pancreatic ductal organoid culture as a relevant model to study exocrine pancreatic ion secretion.
Bilateral lobar lung transplantation for cystic fibrosis – first in Poland.
Brazilian consensus on non-cystic fibrosis bronchiectasis.
Animal models for cystic fibrosis: A systematic search and mapping review of the literature – Part 1: genetic models.
Disassembling the complexity of mucus barriers to develop a fast screening tool for early drug discovery.
Portal Hypertension and Systemic Endothelial Function
Advancing the GI frontier for patients with CF.
Dysregulated insulin in pancreatic insufficient cystic fibrosis with post-prandial hypoglycemia.
Population pharmacokinetics of amikacin in patients with pediatric cystic fibrosis.
Bronchiectasis in India: results from the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) and Respiratory Research Network of India Registry.
The CFTR Corrector, VX-809 (Lumacaftor), Rescues ABCA4 Trafficking Mutants: a Potential Treatment for Stargardt Disease.
Intermittent colonisation with Methicillin-Resistant Staphylococcal aureus can be eradicated from the Airways of Adults with Cystic Fibrosis.
Omalizumab for asthma and allergic bronchopulmonary aspergillosis in adults with cystic fibrosis.
Parasitic pneumonia caused by Paragonimus spp. in a wild Royal Bengal Tiger, Mysuru, South India.
The efficacy and safety of inhaled antibiotics for the treatment of bronchiectasis in adults: a systematic review and meta-analysis.
Long-term macrolide antibiotics for the treatment of bronchiectasis in adults: an individual participant data meta-analysis.
Contemporary management of bronchiectasis in children.
Homozygous Deletion of the CFTR Gene Caused by Interstitial Maternal Isodisomy in a Peruvian Child with Cystic Fibrosis.
Hsp70 and DNAJA2 limit CFTR levels through degradation.
Successful Pregnancy of a Patient with Cystic Fibrosis Genotype F508del/ F508del and Progressed Pulmonary Destruction on lumacaftor/ivacaftor.
A randomised, double-blind, placebo-controlled and parallel group trial to evaluate efficacy and safety of twice daily inhaled doses of BI 1265162 delivered by Respimat® inhaler as add-on therapy t…
Benzyloxycarbonyl-proline-prolinal (ZPP): Dual complementary roles for neutrophil inhibition.
Distribution and outcomes of infection of Mycobacterium avium complex species in cystic fibrosis.
Cystic mesenteric lymphangioma: A case report.
A Novel in situ Approach to Studying Pancreatic Ducts in Mice.
A putative enoyl-CoA hydratase contributes to biofilm formation and the antibiotic tolerance of Achromobacter xylosoxidans.
Abdominal Wall Clear Cell Carcinoma: Case Report of a Rare Event with Potential Diagnostic Difficulties.
Immunopathology of Airway Surface Liquid Dehydration Disease.
On the self-triggering Cox model for recurrent event data.
Inspiration for the Future: The Role of Inspiratory Muscle Training in Cystic Fibrosis.
[Prenatal diagnosis and postnatal outcome of isolated intra-abdominal calcifications: a 10-year experience from a referral fetal medicine center].
Prevention of chronic infection with Pseudomonas aeruginosa infection in cystic fibrosis.
Inhalable combination powder formulations of phage and ciprofloxacin for P. aeruginosa respiratory infections.
STaph Aureus Resistance-Treat Early and Repeat (STAR-TER)
Protein and lipid interactions – Modulating CFTR trafficking and rescue.
Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies.
Nebulized hypertonic saline in noncystic fibrosis bronchiectasis: a comprehensive review.
Niclosamide repurposed for the treatment of inflammatory airway disease.
Cystic Fibrosis Gene Mutation Frequency Among a Group of Suspected Children in King Hussein Medical Center.
Allele specific repair of splicing mutations in cystic fibrosis through AsCas12a genome editing.
Home videos of cystic fibrosis patients using tobramycin inhalation powder: Relation of flow and cough.
Cystic fibrosis transmembrane conductance regulator modulates enteric cholinergic activities and is abnormally expressed in the enteric ganglia of patients with slow transit constipation.
Burkholderia multivorans Exhibits Antibiotic Collateral Sensitivity.
DPI-Tobra-Kind Cyclops® in Children With Cystic Fibrosis
The prevalence of Aspergillus fumigatus in early cystic fibrosis disease is underestimated by culture-based diagnostic methods.
Developing agency in the transition to self-management of cystic fibrosis in young people.
Gq activity- and β-arrestin-1 scaffolding-mediated ADGRG2/CFTR coupling are required for male fertility.
Alginate/Chitosan Particle-Based Drug Delivery Systems for Pulmonary Applications.
Potentiation of aminoglycoside lethality by C4-dicarboxylates requires RpoN in antibiotic tolerant Pseudomonas aeruginosa.
Review of the British Thoracic Society Winter Meeting 2018, 5-7 December 2018, London, UK.
Clinical evaluation of the dental hard tissues in an adult population with cystic fibrosis.
Regulation of Pseudomonas aeruginosa-Mediated Neutrophil Extracellular Traps.
The German National Registry of Primary Immunodeficiencies (2012-2017).
The outcomes of 80 lung transplants in a single center from Saudi Arabia.
Losartan and Inflammation in Cystic Fibrosis
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…
Short-term CFTR inhibition reduces islet area in C57BL/6 mice.
Biatrial Remodeling in Patients with Cystic Fibrosis Running Title: Atrial Function in Cystic Fibrosis.
Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).
[Italian Cystic Fibrosis Registry (ICFR). Report 2015-2016].
Melioidosis in travelers: An analysis of Dutch melioidosis registry data 1985-2018.
Impact of total body weight on rate of acute kidney injury in patients treated with piperacillin-tazobactam and vancomycin.
Distal intestinal obstruction syndrome: an important differential diagnosis for abdominal pain in patients with cystic fibrosis.
Pseudomonas aeruginosa Increases the Sensitivity of Biofilm-Grown Staphylococcus aureus to Membrane-Targeting Antiseptics and Antibiotics.
Clinical Use of Colistin in Biofilm-Associated Infections.

Genetically diverse Pseudomonas aeruginosa populations display similar transcriptomic profiles in a cystic fibrosis explanted lung.
Composition of airway bacterial community correlates with chest HRCT in adults with bronchiectasis.
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…
Sildenafil Exercise: Role of PDE5 Inhibition
Particle coating alters mucociliary transit in excised rat trachea: A synchrotron X-ray imaging study.
Case report of synchronous post-lung transplant colon cancers in the era of colorectal cancer screening recommendations in cystic fibrosis: screening “too early” before it’s too late.
Motor Performance in South African Children with Cystic Fibrosis.
An investigation into biomarkers for the diagnosis of ABPA and aspergillus disease in cystic fibrosis.
Histopathological and biochemical assessment of liver damage in albino Wistar rats treated with cytotoxic platinum compounds in combination with 5-fluorouracil.
Crystal deposition triggers tubule dilation that accelerates cystogenesis in polycystic kidney disease.
Use of continuous infusion ceftolozane-tazobactam with therapeutic drug monitoring in a patient with cystic fibrosis.
Risk factors for adverse outcome in infancy in meconium ileus cystic fibrosis infants: A multicentre Italian study.
An aging population of patients with cystic fibrosis undergoes lung transplantation: An analysis of the ISHLT Thoracic Transplant Registry.
Pseudomonas aeruginosa populations in the cystic fibrosis lung lose susceptibility to newly applied β-lactams within 3 days.
Characterization of Tn6349, a novel mosaic transposon carrying poxtA, cfr and other resistance determinants, inserted in the chromosome of an ST5-MRSA-II strain of clinical origin.
DNA Methylation Changes in Regional Lung Macrophages Are Associated with Metabolic Differences.
Call for Changes in Lung Allocation to Reduce Transplant Waitlist Mortality for Cystic Fibrosis.
A novel mutation (-195C>A) in the promoter region of CFTR gene is associated with Chinese Congenital Bilateral Absence of Vas Deferens (CBAVD).
Steric Inhibition of 5′ UTR Regulatory Elements Results in Upregulation of Human CFTR.
Airway Surface Liquid and Impaired Antiviral Defense in Cystic Fibrosis.
In Vitro Synergism of Colistin and N-acetylcysteine against Stenotrophomonas maltophilia.
Targeting Vitamin D Deficiency to Limit Exacerbations in Respiratory Diseases: Utopia or Strategy With Potential?
SENSITIZATION TO ASPERGILLUS FUMIGATUS IN FIBROCYSTICS.
Exposure of Pseudomonas aeruginosa to bactericidal hypochlorous acid during neutrophil phagocytosis is compromised in cystic fibrosis.
Molecular analysis and antimicrobial resistance pattern of distinct strains of Pseudomonas aeruginosa isolated from cystic fibrosis patients in Iran.
Novel CT Features of an Abdominal Gossypiboma in a Female Dog.
A Retrospective Claims Analysis of Dual Bronchodilator Fixed-Dose Combination Versus Bronchodilator Monotherapy in Patients with Chronic Obstructive Pulmonary Disease.
Image-based β-adrenergic sweat rate assay captures minimal cystic fibrosis transmembrane conductance regulator function.
Ursodeoxycholic acid inhibits ENaC and Na/K pump activity to restore airway surface liquid height in cystic fibrosis bronchial epithelial cells.
Current Practices and Potential Nanotechnology Perspectives for Pain Related to Cystic Fibrosis.
Association of vitamin D status with lung function measurements in children and adolescents with cystic fibrosis.
Isolation of Candida auris from cystic fibrosis patient, Greece, April 2019.
Overlap of Genetic Risk Between Interstitial Lung Abnormalities and Idiopathic Pulmonary Fibrosis.
Hospital Readmission of Adolescents and Young Adults With Complex Chronic Disease.
Cryo-EM structures and functional characterization of murine Slc26a9 reveal mechanism of uncoupled chloride transport.
Non-CF Bronchiectasis as a Possible Indicator of a Primary Immunodeficiency: Diagnosis, Clinical Course, and Quality of Life in a Pediatric Cohort.
Best practice for using ports and non-coring needles.
Extract from Coriolus versicolor fungus partially prevents endotoxin tolerance development by maintaining febrile response and increasing IL-6 generation.
Nasal potential difference in suspected cystic fibrosis patients with 5T polymorphism.
Draft genome and description of Chryseobacterium phocaeense sp. nov.: a new bacterial species isolated from the sputum of a cystic fibrosis patient.
Niflumic Acid Reverses Airway Mucus Excess and Improves Survival in the Rat Model of Steroid-Induced Pneumocystis Pneumonia.
Comment on: In vitro activity of seven β-lactams including ceftolozane/tazobactam and ceftazidime/avibactam against Burkholderia cepacia complex, Burkholderia gladioli and other non-fermentative Gram-negative bacilli isolated from cystic fibrosis patients.
Simplification of CF-related Diabetes Screening at Home
Should isolated Pseudo-Bartter syndrome be considered a CFTR-related disorder of infancy?
Randomized controlled study of aerosolized hypertonic xylitol versus hypertonic saline in hospitalized patients with pulmonary exacerbation of cystic fibrosis.
Enteral tube feeding for cystic fibrosis.
An overview on chemical structures as ΔF508-CFTR correctors.
Australian adults with bronchiectasis: The first report from the Australian Bronchiectasis Registry.
Healthcare transition for adolescents and young adults with long-term conditions: Qualitative study of patients, parents and healthcare professionals’ experiences.
Positive Newborn Screening for Cystic Fibrosis, What to Do Next?
Inhibition of calpain 1 restores plasma membrane stability to pharmacologically rescued Phe508del-CFTR variant.
Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis versus gluten-mediated inhibition in celiac disease.
Microgallbladder: Self-Remitting Acute Cholecystitis-Like Condition Unique to Patients with Cystic Fibrosis.
Making precision medicine personal for cystic fibrosis.
Ivacaftor Is Associated with Reduced Lung Infection by Key Cystic Fibrosis Pathogens: A Cohort Study Using National Registry Data.
Endothelial Dysfunction in Cystic Fibrosis: Role of Oxidative Stress.
Pharmacokinetic/pharmacodynamic analysis of weight- and height-scaled tobramycin dosage regimens for patients with cystic fibrosis.
Establishment and equilibrium levels of deleterious mutations in large populations.
Furocoumarins as multi-target agents in the treatment of cystic fibrosis.
Hepatic enzyme ALT as a marker of glucose abnormality in men with cystic fibrosis.
A Phase 3 Efficacy and Safety Study of PTC124 as an Oral Treatment for Nonsense-Mutation-Mediated Cystic Fibrosis
Side lying during nebulisation can significantly improve apical deposition in healthy adults and adults with mild cystic fibrosis lung disease: a randomised crossover trial.
Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.
Patient-derived pancreas-on-a-chip to model cystic fibrosis-related disorders.
HDAC6 depletion improves cystic fibrosis mouse airway responses to bacterial challenge.
What is the recommended amikacin dosing for cystic fibrosis patients with acute pulmonary exacerbations?
A Phase 3, Randomized, Double-Blind, Placebo-Controlled, Parallel Group Study to Evaluate the Efficacy and Safety of VX 770 in Subjects with Cystic Fibrosis and the G551D Mutation
A Phase 3, 2 Part, Randomized, Double-Blind, Placebo Controlled, Parallel Group Study to Evaluate the Pharmacokinetics, Efficacy and Safety of VX 770 in Subjects Aged 6 to 11 Years with Cystic Fibr…
Assessing cell-specific effects of genetic variations using tRNA microarrays.
CFTRgene variants:A predisposition factor to aquagenic palmoplantar keratoderma.
First clinical trials of novel ENaC targeting therapy, SPX-101, in healthy volunteers and adults with cystic fibrosis.
Chronic rhinosinusitis in unified airway disease: surfactant proteins as mediators of respiratory immunity.
When Is Forgetting Not Forgetting? A Discursive Analysis of Differences in Forgetting Talk Between Adults With Cystic Fibrosis With Different Levels of Adherence to Nebulizer Treatments.
Lung Transplantation for Cystic Fibrosis and Non-cystic Fibrosis Bronchiectasis: A Single-Center Experience.
Supersonic shear-wave elastography and APRI for the detection and staging of liver disease in pediatric cystic fibrosis.
Self-reported alcohol use in the cystic fibrosis community.
Speeding up access to new drugs for CF: Considerations for clinical trial design and delivery.
Patient-reported outcomes of dual bronchodilator fixed-dose combination versus bronchodilator monotherapy in individuals with COPD.
How does Pseudomonas aeruginosa affect the progression of bronchiectasis?
A cystic fibrosis child with lung function decline.
Beyond cystic fibrosis transmembrane conductance regulator therapy: a perspective on gene therapy and small molecule treatment for cystic fibrosis.
Health-related Quality of Life of Macedonian Families Experiencing Cystic Fibrosis in Pediatric Practice.
Role of Dietary Supplements in the Management of Parkinson’s Disease.
Comparable Bioavailability and Disposition of Pefloxacin in Patients with Cystic Fibrosis and Healthy Volunteers Assessed via Population Pharmacokinetics.
The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report.
F508del disturbs the dynamics of the nucleotide binding domains of CFTR before and after ATP hydrolysis.
Hydrogels Embedded With Melittin and Tobramycin Are Effective Against Pseudomonas aeruginosa Biofilms in an Animal Wound Model.
Caffeine boosts Ataluren’s readthrough activity.
Transient Elastography in the Evaluation of Cystic Fibrosis-Associated Liver Disease: Systematic Review and Meta-analysis.
Enhancement of ciliary beat amplitude by carbocisteine in ciliated human nasal epithelial cells.
Vitamin K and cystic fibrosis: A gordian knot that deserves our attention.
Impact of pharmacy services on cystic fibrosis transmembrane conductance regulator modulator prescribing at a pediatric cystic fibrosis center.
STeP IT UP CF: STimulating ImProved Health And Well-being In CysTic Fibrosis
Amyloidosis in cystic fibrosis.
Diagnostic value of chest ultrasound in children with cystic fibrosis – Pilot study.
Integrating Supportive Care in Cystic Fibrosis
Implementation of a Non-invasive Version of the Imaging β-adrenergic-dependent Sweat Secretion Test
Cost-effectiveness of home mechanical ventilation in children living in a developing country.
Effects of Exercise Intervention Program on Bone Mineral Accretion in Children and Adolescents with Cystic Fibrosis: A Randomized Controlled Trial.
Synthesis and evaluation of tetrahydropyrazolopyridine inhibitors of anion exchange protein SLC26A4 (pendrin).

Development of inhibitors against Mycobacterium abscessus tRNA (m1G37) methyltransferase (TrmD) using fragment-based approaches.
[Allergies to non-betalactam antibiotics: a challenge in practice].
Strategies against Nonsense: Oxadiazoles as Translational Readthrough-Inducing Drugs (TRIDs).
Anti-quorum sensing and anti-biofilm activity of 5-hydroxymethylfurfural against Pseudomonas aeruginosa PAO1: Insights from in vitro, in vivo and in silico studies.
Influence of the lung microbiome on antibiotic susceptibility of cystic fibrosis pathogens.
Genomic analysis identifies novel Pseudomonas aeruginosa resistance genes under selection during inhaled aztreonam therapy in vivo.
Antibacterial and transfection activities of nebulized formulations incorporating long n-alkyl chain silver N-heterocyclic carbene complexes.
Targeting DNAJB9, a novel ER luminal co-chaperone, to rescue ΔF508-CFTR.
Outpatient Parenteral Antimicrobial Treatment for Non-Cystic Fibrosis Bronchiectasis Exacerbations: A Prospective Multicentre Observational Cohort Study.
Optimization of dosing regimens of intravenous colistin in patients with cystic fibrosis: What data are required?
Lawn Summer Nights kicks off fundraising for cystic fibrosis
Impact of Bronchial Drainage Technique by the Medical Device Simeox® on Respiratory Function and Symptoms in Adult Patients With Cystic Fibrosis
Seasonal respiratory virus testing in management of adult cystic fibrosis patients.
Taskforce recommends coordinated effort to improve clinical research conduct and find highly effective CFTR-directed treatment for rare mutations.
Indoor hospital air and the impact of ventilation on bioaerosols: a systematic review.
High Dose Cholecalciferol Supplementation In Adults With Cystic Fibrosis.
CD8+ T cells modulate autosomal dominant polycystic kidney disease progression.
Pregnancy outcome in women with cystic fibrosis and poor pulmonary function.
Interventions for treating neuropathic pain in people with sickle cell disease.
Clinicopathologic features of rheumatoid nodules: a retrospective analysis.
FDG PET/CT of Gardnerella vaginalis Infection.
Type 1 Autoimmune Pancreatitis with Imaging Appearance Similar to That of Malignant Cystic Tumor.
Ventilation efficiency to exercise in patients with cystic fibrosis.
Diagnosing Allergic Bronchopulmonary Aspergillosis: A Review.
Gut Imaging for Function & Transit in Cystic Fibrosis Study 2
[Value of sweat conductivity testing in the diagnosis of cystic fibrosis in children].
Functional and metabolic impairment in cigarette smoke-exposed macrophages is tied to oxidative stress.
Inhaled corticosteroids for cystic fibrosis.
Personalized exercise training in chronic lung diseases.
Primary cilia disruption differentially affects the infiltrating and resident macrophage compartment in the liver.
CFTR is not a gluten lover either.
Long-Term CFTR Modulators and Changes in Hemoglobin.
Mild cystic fibrosis in carriers of two nonsense mutations – a case of genetic compensation response?
Extra-respiratory comorbidities and transplantation in the French cystic fibrosis registry.
Symptom Burden and Unmet Existential Needs in Adults With Cystic Fibrosis.
Inhibition of CFTR-mediated intestinal chloride secretion as potential therapy for bile acid diarrhea.
Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders.
Effect of changes in tidal volume on multiple breath washout outcomes.
Missense mutation within cystic fibrosis transmembrane conductance regulator (CFTR) gene is associated with selected parameters of the frozen-thawed sperm in Holstein-Friesian bulls.
Characterisation of the Major Extracellular Proteases of Stenotrophomoas maltophilia and Their Effects on Pulmonary Antiproteases.
Dissociation of systemic and mucosal autoimmunity in cystic fibrosis.
CFTR regulates B cell activation and lymphoid follicle development.
Azithromycin Polarizes Macrophages to an M2 Phenotype via Inhibition of the STAT1 and NF-κB Signaling Pathways.
Normal Calcium-Activated Anion Secretion in a Mouse Selectively Lacking TMEM16A in Intestinal Epithelium.
[Intestinal microbiota, nutrients and probiotics viewed from the «gut-lung» axis].
Update on the current modalities used to screen high risk youth for prediabetes and/or type 2 diabetes mellitus.
Fertility of women with cystic fibrosis: a French survey.
Predictors of pulmonary exacerbation treatment in cystic fibrosis.
Real-Time, Semi-Automated Fluorescent Measurement of the Airway Surface Liquid pH of Primary Human Airway Epithelial Cells.
Gastrointestinal, Pancreatic, and Hepatic Manifestations of Cystic Fibrosis in the Newborn.
Computer and mobile technology interventions to promote medication adherence and disease management in people with thalassemia.
Using qualitative methods to inform the design of a decision aid for people with advanced cystic fibrosis: The InformedChoices CF patient decision aid.
A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11 years with cystic fibrosis.
Transcriptome analysis of the distal small intestine of Cftr null mice.
Diversity of Achromobacter species recovered from patients with cystic fibrosis, in Argentina.
Variation in lung function and nutritional decline in cystic fibrosis by genotype: An analysis of the Canadian cystic fibrosis registry.
Antimicrobial stewardship impact on Pseudomonas aeruginosa susceptibility to meropenem at a tertiary pediatric institution.
Longitudinal associations between gratitude and depression 1 year later among adult cystic fibrosis patients.
Increased intracellular Cl- concentration mediates Trichomonas vaginalis-induced inflammation in the human vaginal epithelium.
Using a stated preference discrete choice experiment to assess societal value from the perspective of patients with rare diseases in Italy.
Sensors for detecting pulmonary diseases from exhaled breath.
Autosomal recessive diseases among the Israeli Arabs.
Long-Term Intrahost Evolution of Methicillin Resistant Staphylococcus aureus Among Cystic Fibrosis Patients With Respiratory Carriage.
Diffuse pulmonary ossification associated with fibrosing interstitial lung disease.
Identification of 99% of CFTR gene mutations in Bulgarian-, Bulgarian Turk-, and Roma cystic fibrosis patients.
Compartmentalized crosstalk of CFTR and TMEM16A (ANO1) through EPAC1 and ADCY1.
Understanding patient activation and adherence to nebuliser treatment in adults with cystic fibrosis: responses to the UK version of PAM-13 and a think aloud study.
Merkel Cell Polyomavirus DNA Detection in Respiratory Samples: Study of a Cohort of Patients Affected by Cystic Fibrosis.
Impact of Cystic Fibrosis on Unaffected Siblings: A Systematic Review.
Do current clinical trials in cystic fibrosis match the priorities of patients and clinicans? A systematic review.
Improvement in body composition following a supervised exercise-training program of adult patients with cystic fibrosis.
Stenotrophomonas maltophilia Encodes a VirB/D4 Type IV Secretion System That Modulates Apoptosis in Human Cells and Promotes Competition Against Heterologous Bacteria Including Pseudomonas aeruginosa.
A Method for Comprehensive Proteomic Analysis of Human Faecal Samples to Investigate Gut Dysbiosis in Patients with Cystic Fibrosis.
Assessing psychosocial risk in pediatric cystic fibrosis.
Long-Term Parenteral Nutrition in a Patient With Cystic Fibrosis During Pregnancy.
Palivizumab and Long-term Outcomes in Cystic Fibrosis.
Epidemiology of rare diseases detected by newborn screening in the Czech Republic.
PREVALENCE OF HEPATIC STEATOSIS AMONG CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS AND ITS ASSOCIATION WITH NUTRITIONAL STATUS.
Airway surface liquid has innate antiviral activity that is reduced in cystic fibrosis.
Prednisone in Cystic Fibrosis Pulmonary Exacerbations
Challenging barriers to an option for improved provision of enteral nutrition.
OMT for the Prevention and Management of Chronic Constipation and Distal Intestinal Obstructive Syndrome in Cystic Fibrosis: A Pilot Study.
Mesenteric Lift for Constipation in Cystic Fibrosis.
Common content between quality of life questionnaires for children with cystic fibrosis and the International Classification of Functionality, Disability and Health.
What do we know about travel for children with special health care needs? A review of the literature.
Exhaled nitric oxide in pediatric patients with respiratory disease.
Intravenous antibiotic use and exacerbation events in an adult cystic fibrosis centre: A prospective observational study.
A Phase 3 Extension Study of Ataluren (PTC124) in Subjects with Nonsense-Mutation-Mediated Cystic Fibrosis
Successful lung transplantation from a hepatitis C RNA-positive donor to a hepatitis C treatment-experienced recipient with cystic fibrosis.
The polysaccharide extracted from the biofilm of Burkholderia multivorans strain C1576 binds hydrophobic species and exhibits a compact 3D-structure.
Pancreatic ductal deletion of Hnf1b disrupts exocrine homeostasis, leads to pancreatitis and facilitates tumorigenesis.
Conversion of human and mouse fibroblasts into lung-like epithelial cells.
Structural identification of a hotspot on CFTR for potentiation.
Paediatric and adult bronchiectasis: Specific management with coexisting asthma, COPD, rheumatological disease and inflammatory bowel disease.
Cadmium at Human Dietary Levels Disturbed Homeostasis of Nutritional Metals in Lung (P24-055-19).
Impacts of Genistein on the Gut Microbiota of a Mouse Model of Cystic Fibrosis (P06-070-19).
Dietary Macronutrient Distribution and Nutrition-Related Outcomes in Cystic Fibrosis: A Systematic Review (P12-016-19).
Effect of Cystic Fibrosis Transmembrane Receptor Modulators on Anthropometric and Growth Parameters in Individuals with Cystic Fibrosis: A Systematic Review (OR36-08-19).
Cross-transmission is not the source of new Mycobacterium abscessus infections in a multi-centre cohort of cystic fibrosis patients.
Inhaled corticosteroids prescriptions increased in the ED for recurrent asthma exacerbations by automated electronic reminders in the ED.

Evaluation of Quality of Life in Terms of Sinonasal Symptoms in Children with Cystic Fibrosis.
Goldston syndrome with congenital hepatic fibrosis: A rare cause of neonatal cholestasis.
Intranasal Corticosteroids for the Management of Chronic Rhinosinusitis or Nasal Polyposis in Cystic Fibrosis: A Review of Clinical Effectiveness
Novel Population Pharmacokinetic Approach to Explain the Differences between Cystic Fibrosis Patients and Healthy Volunteers via Protein Binding.
Cystic fibrosis disease severity correlates with plasma levels of desmosine and isodesmosine, biomarkers of elastin degradation.
Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles.
Late diagnosis and poor nutrition in cystic fibrosis diagnosed before implementation of newborn screening.
Reduced exercise ventilatory efficiency in Cystic Fibrosis adults with normal to moderately impaired lung function.
Hepatocellular carcinoma in cystic fibrosis liver disease: a cautionary tale.
Mechanisms for Vascular Dysfunction and Exercise Tolerance in CF
New Markers of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function in Sweat
Catabolism of Nucleic Acids by a Cystic Fibrosis Pseudomonas aeruginosa Isolate: An Adaptive Pathway to Cystic Fibrosis Sputum Environment.
Targeting bacterial biofilm: a new LecA multivalent ligand with inhibitory activity.
Hypertrophic osteoarthropathy presenting with joint pain in cystic fibrosis.
Mucoid Pseudomonas aeruginosa alters sputum viscoelasticity in patients with non-cystic fibrosis bronchiectasis.
CFTR variants and renal abnormalities in males with congenital unilateral absence of the vas deferens (CUAVD): a systematic review and meta-analysis of observational studies.
Characterization of a bacteriophage with broad host range against strains of Pseudomonas aeruginosa isolated from domestic animals.
Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction.
[Quality criteria for the transition to adult care in French CF centers – results from the SAFETIM APP study?]
Peptide-based efflux pump inhibitors of the small multidrug resistance protein from Pseudomonas aeruginosa.
Altered Stool Microbiota of Infants with Cystic Fibrosis Shows a Reduction in Genera Associated with Immune Programming From Birth.
Giardiasis : An overview.
Mucoid Pseudomonas aeruginosa infection in a cat with severe chronic rhinosinusitis.
Potentiation of ABCA3 lipid transport function by ivacaftor and genistein.
Investigation of a Pandoraea apista cluster common to adult and paediatric cystic fibrosis patients attending two hospitals in the same city.
An Exploration Into Knowledge, Attitudes, and Beliefs Towards Risky Health Behaviours in a Paediatric Cystic Fibrosis Population.
Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I.
A novel method for infant multiple breath washout: First report in clinical practice.
Alterations of skeletal muscle bioenergetics in a mouse with F508del mutation leading to a cystic fibrosis-like condition.
Mini-gut: a promising model for drug development.
Respiratory Fungal Diseases in Adult Patients With Cystic Fibrosis.
Healthcare professionals’ perceptions of risky health behaviours: A qualitative evaluation of preventative measures for populations with cystic fibrosis.
A case report of successful eradication of new isolates of Burkholderia cenocepacia in a child with cystic fibrosis.
The First Report of Total Pancreatectomy and Islet Cell Autotransplantation for Pancreatic Cystosis in Patient With Cystic Fibrosis.
Non-invasive ventilation versus oxygen therapy in cystic fibrosis: A 12-month randomized trial.
A multi-scale model of gas transport in the lung to study heterogeneous lung ventilation during the multiple-breath washout test.
Pseudomonas aeruginosa eradication therapy and risk of acquiring Aspergillus in young children with cystic fibrosis.
Enhancing intrinsic motivation for physical activity among adolescents with cystic fibrosis: a qualitative study of the views of healthcare professionals.
Domain-interface dynamics of CFTR revealed by stabilizing nanobodies.
Mucosal-associated invariant T cells: new players in CF lung disease?
Proteomic Profiling to Identify Blood Biomarkers Predictive of Response to Azithromycin in Children and Adolescents with Cystic Fibrosis.
A non-randomised single centre cohort study, comparing standard and modified bowel preparations, in adults with cystic fibrosis requiring colonoscopy.
Roles of volume-regulatory anion channels, VSOR and Maxi-Cl, in apoptosis, cisplatin resistance, necrosis, ischemic cell death, stroke and myocardial infarction.
Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries.
Effect of Including Important Clinical Variables on Accuracy of the Lung Allocation Score for Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.
Identification of a novel large deletion and other copy number variations in the CFTR gene in patients with Cystic Fibrosis from a multiethnic population.
Overexpression of miRNA-25-3p inhibits Notch1 signaling and TGF-β-induced collagen expression in hepatic stellate cells.
Chronic rhino-sinusitis treatment in children with cystic fibrosis: A cross-sectional survey of pediatric pulmonologists and otolaryngologists.
Cystic Fibrosis-Related Diabetes: Pathophysiology and Therapeutic Challenges.
Editorial Focus: CFTR-dependent bicarbonate secretion by Calu-3 cells.
CFTR Expression Analysis for Subtyping of Human Pancreatic Cancer Organoids.
Exercise capacity in patients with cystic fibrosis vs. non-cystic fibrosis bronchiectasis.
Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis.
Exotoxin A-PLGA nanoconjugate vaccine against Pseudomonas aeruginosa infection: protectivity in murine model.
Cystic fibrosis presentation in del. F508 and p. Tyr109Glyfs compound heterozygote CFTR state: a case report.
Protein Misfolding Diseases and Therapeutic Approaches.
Regulating, measuring and modelling the viscoelasticity of bacterial biofilms.
Mature bony metaplasia in multinodular goiter: A case report.
Pseudo-Bartter syndrome in children with cystic fibrosis.
Enhanced avidity from a multivalent fluorescent antimicrobial peptide enables pathogen detection in a human lung model.
Hemoptysis in Pediatric Patients.
Randomized, double-blind, placebo-controlled, multicEnter Study comParing CIprofloxacin DPI 32.5 mg BID intermittently administered for 28 days on / 28 days off or 14 days on / 14 days off versus p…
Randomised cross-over trial evaluating the short-term effects of non-invasive ventilation as an adjunct to airway clearance techniques in adults with cystic fibrosis.
Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial.
Codon-usage frequency mediated SNPs selection in lasR gene of cystic fibrosis Pseudomonas aeruginosa isolates.
Study of a Cohort of Patients With Cystic Fibrosis and Isolation of Scedosporium spp.
Type I interferon induced by TLR2-TLR4-MyD88-TRIF-IRF3 controls Mycobacterium abscessus subsp. abscessus persistence in murine macrophages via nitric oxide.
Inhaled hypertonic saline for 3-6-year-olds with cystic fibrosis.
Lactation Consultation Sustains Breast Milk Intake in Infants with Cystic Fibrosis.
Liver Ultrasound Patterns in Children with Cystic Fibrosis Correlate with Non-Invasive Tests of Liver Disease.
New Algorithm for the Integration of Ultrasound into Cystic Fibrosis Liver Disease Screening.
Non-fasting bioelectrical impedance analysis in cystic fibrosis: Implications for clinical practice and research.
Abnormal pro-gly-pro pathway and airway neutrophilia in pediatric cystic fibrosis.
Iontophoresis burns: a peculiar cutaneous injury from a diagnostic sweat test for cystic fibrosis.
Focus on TRP channels in cystic fibrosis.
Non-fasting bioelectrical impedance analysis in cystic fibrosis: Implications for clinical practice and research.
Abnormal pro-gly-pro pathway and airway neutrophilia in pediatric cystic fibrosis.
Fatigue in childhood chronic disease.
Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand.
The gasotransmitter hydrogen sulfide inhibits transepithelial anion secretion of pregnant mouse endometrial epithelium.
Pharmacological analysis of CFTR variants of cystic fibrosis using stem cell-derived organoids.
Cystic fibrosis revisited.
“Switching partners”: Piperacillin-avibactam is a highly potent combination against multidrug-resistant Burkholderia cepacia Complex and Burkholderia gladioli Cystic Fibrosis isolates.
Cystic Fibrosis: Proteostatic correctors of CFTR trafficking and alternative therapeutic targets.
A preventive effect of the combination of albendazole and pomegranate peel aqueous extract treatment in cystic echinococcosis mice model: an alternative approach.
Temperate Bacteriophages from Chronic Pseudomonas aeruginosa Lung Infections Show Disease-Specific Changes in Host Range and Modulate Antimicrobial Susceptibility.
Atypical presentation of cystic fibrosis at 70 years of age.
Identifying the molecular target sites for CFTR potentiators GLPG1837 and VX-770.
Is the Potable Water System an Advantageous Preinfection Niche for Bacteria Colonizing the Cystic Fibrosis Lung?
Human epididymis protein 4 (HE4) protects against cystic pulmonary fibrosis associated-inflammation through inhibition of NF-κB and MAPK singnaling.
Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases.
Cigarette and ENDS preparations differentially regulate ion channels and mucociliary clearance in primary normal human bronchial 3D cultures.
Microbiological contamination of nebulizers used by cystic fibrosis patients: an underestimated problem.
Standardized Treatment of Pulmonary Exacerbations II
Outcomes of Lung Transplantation for Cystic Fibrosis in the Setting of Extensively Drug-Resistant Organisms.
Long-term outcome following laser therapy of benign cystic-solid thyroid nodules.
Phenotypic Stability of Staphylococcus Aureus Small Colony Variants (SCV) Isolates from Cystic Fibrosis (CF) Patients.
Clinical predictors of cystic fibrosis chronic rhinosinusitis severity.
Look at the wood and not at the tree: The Microbiome in Chronic Osxtructive Lung Disease and Cystic Fibrosis.
Influence of frequency and amplitude on the mucus viscoelasticity of the novel mechano-acoustic Frequencer™.