- Distinct Screening Approaches Uncover PA14_36820 and RecA as Negative Regulators of Biofilm Phenotypes in Pseudomonas aeruginosa PA14
- Effects of Lumacaftor-Ivacaftor on Airway Microbiota-Mycobiota and Inflammation in Patients with Cystic Fibrosis Appear To Be Linked to Pseudomonas aeruginosa Chronic Colonization
- High-Throughput Screen Reveals the Structure-Activity Relationship of the Antimicrobial Lasso Peptide Ubonodin
- AN 84-YEAR-OLD PATIENT WITH CAROLI SYNDROME: WHAT IS THE PROGNOSIS OF THIS CONDITION?
- Early and sustained improvements of lung clearance index from two to sixteen weeks of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis-a real world study
- CFTR High Expresser Cells in cystic fibrosis and intestinal diseases
- Outcomes of children with cystic fibrosis screen positive, inconclusive diagnosis/CFTR related metabolic syndrome
- Mycobacterium abscessus DosRS two-component system controls a species-specific regulon required for adaptation to hypoxia
- Normalizing salt content by mixing native human airway mucus samples normalizes sample rheology
- Evaluating the efficacy of inhaled amikacin as an adjunct to intravenous combination therapy (ceftazidime and amikacin) in pediatric cystic fibrosis pulmonary exacerbation
- PK-PD Evaluation of Inhaled Microparticles loaded with Ciprofloxacin-Copper complex in a Rat Model of Chronic Pseudomonas aeruginosa Lung Infection
- Autoimmunity in people with cystic fibrosis
- Cervical mucus quality in females with and without cystic fibrosis
- A paradigm shift in cystic fibrosis nutritional care: Clinicians’ views on the management of patients with overweight and obesity
- The Burkholderia contaminans prevalent phenotypes as possible markers of poor clinical outcomes in chronic lung infection of children with cystic fibrosis
- Systematic review and meta-analysis: Associations of vitamin D with pulmonary function in children and young people with cystic fibrosis
- Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study
- Perioperative Outcomes During Double-Lung Transplantation and Retransplantation in Cystic Fibrosis Patients: A Monocentric Cohort Study
- Metal complexes with valuable biomolecules produced by Pseudomonas aeruginosa : a review of the coordination properties of pyocyanin, pyochelin and pyoverdines
- Who Modifies the Modifiers: A High Resolution View of the Genetic Modifiers of Cystic Fibrosis
- Regulated cell death pathways in kidney disease
- Assessment of adaptive immune responses of dairy cows with Burkholderia contaminans -induced mastitis
- Cystic fibrosis transmembrane conductance regulator modulators attenuate platelet activation and aggregation in blood of healthy donors and COVID-19 patients
- Cystic Fibrosis Reprograms Airway Epithelial IL-33 Release and Licenses IL-33 Dependent Inflammation
- Ionocyte-Specific Regulation of CFTR
- BEAT CF pulmonary exacerbations core protocol for evaluating the management of pulmonary exacerbations in people with cystic fibrosis
- Enhanced Optimal Parameter-Based Nebulizer Design for Flow Analysis of Fluticasone Propionate
- CFTR function, pathology and pharmacology at single-molecule resolution
- Regarding the article entitled “Effect of elexacaftor/tezacaftor/ivacaftor on annual rate of lung function decline in people with cystic fibrosis”
- So You Want to Build Your Disease’s First Online Patient Registry: An Educational Guide for Patient Organizations Based on US and European Experience
- Effects of Lumacaftor/Ivacaftor on Cystic Fibrosis Disease Progression in Children 2 through 5 Years of Age Homozygous for F508del-CFTR: A Phase 2 Placebo-controlled Clinical Trial
- Increasing incidence rate of breast cancer in cystic fibrosis – relationship between pathogenesis, oncogenesis and prediction of the treatment effect in the context of worse clinical outcome and prognosis of cystic fibrosis due to estrogens
- Liver Assessment in Patients with Ataxia-Telangiectasia: Transient Elastography Detects Early Stages of Steatosis and Fibrosis
- Acute cholecystitis in cystic fibrosis patients after initiation of treatment with elexacaftor/tezacaftor/ivacaftor
- Drug Hypersensitivity Reactions in Patients with Cystic Fibrosis: Potential Value of the Lymphocyte Toxicity Assay to Assess Risk
- Ivacaftor: Five-year outcomes in the West of Scotland cystic fibrosis population
- Association of Oxygen Therapy with the Natural Disease Progression of Cystic Fibrosis: A Multi-State Model of the European Cystic Fibrosis Society Patient Registry
- Development of a Cystic Fibrosis Primary Palliative Care Intervention: Qualitative Analysis of Patient and Family Caregiver Preferences
- Cystic Lymphatic Malformation with Lymphedema Treated by Lymphaticovenular Anastomosis Combined with Ethanol Sclerotherapy
- Intussusception of the appendix in a young adult: an important differential diagnosis of abdominal pain in cystic fibrosis patients?
- Effects of postage on recovery of pathogens from cystic fibrosis sputum samples
- Safety and efficacy of a new supplementation protocol in patients with cystic fibrosis and vitamin D deficiency
- Iron bioavailability regulates Pseudomonasaeruginosa interspecies interactions through type VI secretion expression
- Proteome of airway surface liquid and mucus in newborn wildtype and cystic fibrosis piglets
- Down-expression of Foxj1 on airway epithelium with impaired cilia architecture in non-cystic fibrosis bronchiectasis implies disease severity
- The impact of elexacaftor/tezacaftor/ivacaftor on body composition in a small cohort of youth with cystic fibrosis
- In Utero Mapping and Development Role of CFTR in Lung and Gastrointestinal Tract of Cystic Fibrosis Patients
- Medical traumatic stress in cystic fibrosis: A qualitative analysis
- The STING/TBK1/IRF3/IFN type I pathway is defective in cystic fibrosis
- Concern over tobacco and marijuana perceptions and use among adolescents and young adults with cystic fibrosis
- Cystic fibrosis prevalence in the United States and participation in the Cystic Fibrosis Foundation Patient Registry in 2020
- Association between nebuliser therapies adherence and visit-to-visit variability of FEV1 in patients with cystic fibrosis
- Phase 3 Open-Label Clinical Trial of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged 2 Through 5 Years with Cystic Fibrosis and at Least One F508del Allele
- Genetic Modifiers of Cystic Fibrosis Lung Disease Severity: Whole Genome Analysis of 7,840 Patients
- Can percussive intrapulmonary ventilation improve the efficacy of physiotherapy in children with cystic fibrosis?
- Genome-engineering technologies for modeling and treatment of cystic fibrosis
- Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis
- Isolated abnormal FEF75% detects unsuspected bronchiolar obstruction in CF children
- Burkholderia cepacia infection associated with sickle cell disease: An uncommon entity
- Sweat conductivity for diagnosing cystic fibrosis after positive newborn screening: prospective, diagnostic test accuracy study
- Acute pancreatitis in pancreatic-insufficient cystic fibrosis patients treated with CFTR modulators
- Upper Airway Disease in Adults with Cystic Fibrosis in the Era of CFTR Modulators
- Feasibility of free-breathing 19 F MRI image acquisition to characterize ventilation defects in CF and healthy volunteers at wash-in
- Efficacy of Omadacycline-Containing Regimen in a Mouse Model of Pulmonary Mycobacteroides abscessus Disease
- FEED-Cystic Fibrosis (FEED-CF)
- Biomaterial therapeutic strategies for treatment of bacterial lung infections
- Adherence, reliability, and variability of home spirometry telemonitoring in cystic fibrosis
- “Just Move It . . . Move It”: A Multidisciplinary Motivational Approach to Improve Physical Activity in Children With Cystic Fibrosis
- Early Glucose abnormalities in cystic fibrosis – how best to measure them, and what is to be done about them?
- Oral care considerations for people with cystic fibrosis: a cross-sectional qualitative study
- CFTR Inhibitors Display In Vitro Antiviral Activity against SARS-CoV-2
- Effectiveness of Autogenic Drainage in Improving Pulmonary Function in Patients with Cystic Fibrosis
- Mycobacterium abscessus Infections in Cystic Fibrosis Individuals: A Review on Therapeutic Options
- Revisiting Host-Pathogen Interactions in Cystic Fibrosis Lungs in the Era of CFTR Modulators
- Concomitant Use of Elexacaftor/Tezacaftor/Ivacaftor and Etanercept in a Cystic Fibrosis Patient with Juvenile Idiopathic Arthritis
- Potentiometric Chloride Ion Biosensor for Cystic Fibrosis Diagnosis and Management: Modeling and Design
- Functional restoration of a CFTR splicing mutation through RNA delivery of CRISPR adenine base editor
- Advanced Cystic Fibrosis Lung Disease and Lung Transplantation in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulators
- Post-transplant outcomes among cystic fibrosis patients undergoing lung transplantation colonized by Burkholderia: A single center cohort study
- Influence of environmental factors and polymorphic loci rs6580502 of the SPINK1 gene, rs10273639 of the PRSS1 gene, rs213950 of the CFTR gene on the risk of developing acute alcoholic-alimentary pancreatitis
- The Cystic Fibrosis Upper and Lower Airway Metagenome
- Comprehensive semen examination in patients with pancreatic-sufficient and pancreatic-insufficient cystic fibrosis
- The impact of telehealth based care on paediatric cystic fibrosis outcomes
- Quality of anthropometric data measured in children and adolescents with cystic fibrosis: a scoping review
- Clinical and functional course of patients with cystic fibrosis treated with lumacaftor/ivacaftor at a children’s hospital. A case series
- Lung Function Decline in Cystic Fibrosis: Impact of Data Availability and Modeling Strategies on Clinical Interpretations
- Position paper: Models of post-transplant care for individuals with cystic fibrosis
- Regulatory T cell enhancement in adults with cystic fibrosis receiving Elexacaftor/Tezacaftor/Ivacaftor therapy
- Metabolism-based approaches for autosomal dominant polycystic kidney disease
- Inherited causes of exocrine pancreatic insufficiency in pediatric patients: clinical presentation and laboratory testing
- Genomic analysis of Staphylococcus aureus sequential isolates from lungs of patients with cystic fibrosis
- Canadian Cystic Fibrosis-related Diabetes Clinical Practice Survey: Analysis of Current Practices and Gaps in Clinical Care
- Incidence of transaminitis in adults with cystic fibrosis taking elexacaftor/tezacaftor/ivacaftor
- Influence on sputum microbiology with CFTR modulator (tezacaftor-ivacaftor) in adult patients with cystic fibrosis: Multicenter study
- Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis
- Safety of elexacaftor/tezacaftor/ivacaftor dose reduction: mechanistic exploration through physiologically-based pharmacokinetic modeling and a clinical case series
- Advances in inhaled antibiotics for management of respiratory tract infections
- Phage therapy in a lung transplant recipient with cystic fibrosis infected with multidrug-resistant Burkholderia multivorans
- SLC26A9 in airways and intestine: secretion or absorption?
- Sharing decisions on reproductive goals: A mixed-methods study of the views of women who have cystic fibrosis
- The two faces of pyocyanin – why and how to steer its production?
- Effect of budesonide on pulmonary activity of multidrug resistance-associated protein 1 assessed with PET imaging in rats
- High Deductible Insurance Plans Impart Economic Burden for People with Cystic Fibrosis
- Whole Exome Sequencing Identifies a Rare CFTR Mutation in Brothers With Anomalies of the Vas Deferens: a case study
- Early life growth trajectories in cystic fibrosis are associated with lung function at age six
- Investigating serum extracellular vesicles in Cystic Fibrosis
- Mechanisms of iron homeostasis in Pseudomonas aeruginosa and emerging therapeutics directed to disrupt this vital process
- De Novo Generation of Pulmonary Ionocytes from Normal and Cystic Fibrosis Human Induced Pluripotent Stem Cells
- Cystic fibrosis liver disease in children – A review of our current understanding
- Exploring the nature of perceived treatment burden: a study to compare treatment burden measures in adults with cystic fibrosis [version 1; peer review: 2 approved]
- Comparing the Efficacy and Safety of Nebulized Gentamicin plus amikacin versus Tobramycin in Patients with Cystic Fibrosis
- Nicotine aerosols diminish airway CFTR function and mucociliary clearance
- Association between Cystic Fibrosis exacerbations, lung function, T2 inflammation and microbiological colonization
- Risk Factors for Influenza-Induced Exacerbations and Mortality in Non-Cystic Fibrosis Bronchiectasis
- Elexacaftor/tezacaftor/ivacaftor projected survival and long-term health outcomes in people with cystic fibrosis homozygous for F508del
- Polymicrobial infections and antibiotic treatment patterns for cystic fibrosis pulmonary exacerbations
- Exhaled breath profiles to detect lung infection with Staphylococcus aureus in children with cystic fibrosis
- A systematic review to explore how exercise-based physiotherapy via telemedicine can promote health related benefits for people with cystic fibrosis
- Detection and identification of fungi in the lower airway of children with and without cystic fibrosis
- Comparative analysis of five type II TA systems identified in Pseudomonas aeruginosa reveals their contributions to persistence and intracellular survival
- High throughput determination of the biofilm prevention concentration for Pseudomonas aeruginosa biofilms using a synthetic cystic fibrosis sputum medium
- Safety and efficacy of vanzacaftor-tezacaftor-deutivacaftor in adults with cystic fibrosis: randomised, double-blind, controlled, phase 2 trials
- A new triple combination cystic fibrosis transmembrane regulator modulator
- The Impact of Highly Effective Cystic Fibrosis Transmembrane Conductance Regulator Modulators on the Health of Female Subjects With CF
- Prevalence of CFTR variants in PID patients with bronchiectasis – an important modifying co-factor
- Cystic fibrosis and primary ciliary dyskinesia: Similarities and differences
- Phylogenomics of nontuberculous mycobacteria respiratory infections in people with cystic fibrosis
- Home Spirometry in Children with Cystic Fibrosis
- Antibiotic Resistance in Patients with Cystic Fibrosis: Past, Present, and Future
- The Role of MMPs in the Era of CFTR Modulators: An Additional Target for Cystic Fibrosis Patients?
- Simultaneous Quantification of Ivacaftor, Tezacaftor, and Elexacaftor in Cystic Fibrosis Patients’ Plasma by a Novel LC-MS/MS Method
- Cystic Fibrosis: A Descriptive Analysis of Deaths in a Two-Decade Period in Brazil According to Age, Race, and Sex
- Efficacy of the Simeox® Airway Clearance Technology in the Homecare Treatment of Children with Clinically Stable Cystic Fibrosis: A Randomized Controlled Trial
- A Multicentre Italian Study on the Psychological Impact of an Inconclusive Cystic Fibrosis Diagnosis after Positive Neonatal Screening
- Nutritional Status and Circulating Levels of Fat-Soluble Vitamins in Cystic Fibrosis Patients: A Cohort Study and Evaluation of the Effect of CFTR Modulators
- CFTR -Related Metabolic Syndrome: Genetic Variants Increasing Pancreatitis Risk in the Pediatric Puerto Rican Population
- Effects of Elexacaftor/Tezacaftor/Ivacaftor on Cardiorespiratory Polygraphy Parameters and Respiratory Muscle Strength in Cystic Fibrosis Patients with Severe Lung Disease
- Myxinidin-Derived Peptide against Biofilms Caused by Cystic Fibrosis Emerging Pathogens
- Rescue of Rare CFTR Trafficking Mutants Highlights a Structural Location-Dependent Pattern for Correction
- Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages
- Vx-809, a CFTR Corrector, Acts through a General Mechanism of Protein Folding and on the Inflammatory Process
- Pregnancy in Cystic Fibrosis-Past, Present, and Future
- Dot Immunobinding Assay for the Rapid Serodetection of Scedosporium /Lomentospora in Cystic Fibrosis Patients
- Exposure of Aspergillus fumigatus to Klebsiella pneumoniae Culture Filtrate Inhibits Growth and Stimulates Gliotoxin Production
- Do Patients with Bronchiectasis Have an Increased Risk of Developing Lung Cancer? A Systematic Review
- Comparative Proteomic Analysis of Protein Patterns of Stenotrophomonas maltophilia in Biofilm and Planktonic Lifestyles
- Preparation and Characterization of Ibuprofen Containing Nano-Embedded-Microparticles for Pulmonary Delivery
- A universal stress protein upregulated by hypoxia has a role in Burkholderia cenocepacia intramacrophage survival: Implications for chronic infection in cystic fibrosis
- Inquilinus limosus Bacteremia in Lung Transplant Recipient after SARS-CoV-2 Infection
- To bead or not to bead: A review of Pseudomonas aeruginosa lung infection models for cystic fibrosis
- Shuttle Peptide Delivers Base Editor RNPs to Rhesus Monkey Airway Epithelial Cells In Vivo
- Infection dynamics of Stenotrophomonas maltophilia in patients with cystic fibrosis
- Distinct community structures of the fungal microbiome and respiratory health in adults with cystic fibrosis
- Cepacia syndrome in cystic fibrosis: a systematic review of the literature and possible new perspectives in treatment
- Bioenergetics of the polyphosphates accumulation in Pseudomonas aeruginosa via polyphosphate kinase activation by choline in a lung colonization model
- MDCT-based longitudinal automated airway and air trapping analysis in school-age children with mild cystic fibrosis lung disease
- Fluorinated tetrapodal anion transporters
- Classic Cystic Fibrosis Presentation in Two Middle Eastern Siblings with a rare CFTR mutation (c.80G>T)
- A rare case of vasculitis in cystic fibrosis: A clinical case
- Inhaled Mannitol on Mucociliary Clearance in Moderate to Severe Cystic Fibrosis
- Management of respiratory tract exacerbations in people with cystic fibrosis: Focus on imaging
- Clinical Characteristics and Outcomes of Cystic Fibrosisin Palestine: Cross Sectional Study
- “Find Ways to Work Parenting into Cystic Fibrosis”: A PhotoVoice Exploration of Being a Parent and Having CF
- Online activity – A beaming good initiative! Delivering alternative exercise opportunities for people with cystic fibrosis
- Cellular nitric oxide synthesis is affected by disorders in the interdependent [Formula: see text] and [Formula: see text] dynamics during cystic fibrosis disease
- External validity of machine learning-based prognostic scores for cystic fibrosis: A retrospective study using the UK and Canadian registries
- A novel direct adenosine monophosphate kinase activator ameliorates disease progression in preclinical models of Autosomal Dominant Polycystic Kidney Disease
- A conceptual framework to develop a patient-reported experience questionnaire on the cystic fibrosis journey in France: the ExPaParM collaborative study
- Comparative in vitro antipseudomonal activity of ceftolozane/tazobactam against Pseudomonas aeruginosa isolates from children with cystic fibrosis
- Impact of elexacaftor/tezacaftor/ivacaftor on bacterial cultures from people with cystic fibrosis
- Resilience and stability of the CF- intestinal and respiratory microbiome during nutritional and exercise intervention
- Re-examining baseline lung function recovery following IV-treated pulmonary exacerbations
- Bronchial artery embolization for hemoptysis in adult patients with cystic fibrosis: a single-center retrospective study
- Managing cystic fibrosis in children aged 6-11yrs: a critical review of elexacaftor/tezacaftor/ivacaftor combination therapy
- The Clinical Association between Aspergillus fumigatus and Respiratory Outcomes in Adolescents and Adults with Cystic Fibrosis
- Increase of liver stiffness and altered bile acid metabolism after triple CFTR modulator initiation in children and young adults with cystic fibrosis
- Osmolytes: Wonder molecules to combat protein misfolding against stress conditions
- Lessons from other fields of medicine, Part 2: Cystic fibrosis
- The French Compassionate Program of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant
- Genetics and epigenetics of chronic rhinosinusitis
- Unusual prophages in Mycobacterium abscessus genomes and strain variations in phage susceptibilities
- Bicarbonate Effects on Antibacterial Immunity and Mucus Glycobiology in the Cystic Fibrosis Lung: A Review With Selected Experimental Observations
- Understanding impact of δF508 and G551D CFTR mutations on CFTR/PKA-c interaction
- Posttranslational modifications of cyclic AMP-dependent protein kinase a affect its binding to the CFTR anion channel
- Lipid-driven CFTR clustering is impaired in cystic fibrosis and restored by corrector drugs
- Intra- and Inter-visit Repeatability of 129 Xenon Multiple-Breath Washout MRI in Children With Stable Cystic Fibrosis Lung Disease
- Targeting ATP12A, a Non-Gastric Proton Pump Alpha Subunit, for Idiopathic Pulmonary Fibrosis Treatment
- Identification of Potential Antimicrobial Targets of Pseudomonas aeruginosa Biofilms through a Novel Screening Approach
- Built environment factors predictive of early rapid lung function decline in cystic fibrosis
- Review of Tobramycin Dosing in Pediatric Patients With Cystic Fibrosis
- Changes in cystic fibrosis transmembrane conductance regulator protein expression prior to and during elexacaftor-tezacaftor-ivacaftor therapy
- Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome in a Child with Cystic Fibrosis
- Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics
- The impact of hypoxia on the ion channels in cystic fibrosis bronchial epithelial cells
- Lower Expression of CFTR Is Associated with Higher Mortality in a Meta-Analysis of Individuals with Colorectal Cancer
- The COPD-Associated Polymorphism Impairs the CFTR Function to Suppress Excessive IL-8 Production upon Environmental Pathogen Exposure
- Poly-L-Lysine to Fight Antibiotic Resistances of Pseudomonas aeruginosa
- Nutritional Care in Children with Cystic Fibrosis
- Gender differences in clinical characteristics of patients with non-cystic fibrosis bronchiectasis in different age groups in northern China
- ACUTE SOLUBLE FIBRE SUPPLEMENTATION HAS NO IMPACT ON REDUCING POST-PRANDIAL GLUCOSE EXCURSIONS IN ADULTS WITH CYSTIC FIBROSIS AND GLUCOSE INTOLERANCE
- The Effects of a Low Glycemic Load Diet on Dysglycemia and Body Composition in Adults With Cystic Fibrosis-Related Diabetes
- Mutation-class dependent signatures outweigh disease-associated processes in cystic fibrosis cells
- Pharmacogenetic actionability and medication prescribing in people with cystic fibrosis
- Comparison of Three Eradication Treatment Protocols for Pseudomonas Aeruginosa in Children and Adolescents with Cystic Fibrosis
- Suicide attempts in adolescents with cystic fibrosis on Elexacaftor/Tezacaftor/Ivacaftor therapy
- Risk of new onset and prevalent disease in chronic rhinosinusitis: a prospective cohort study
- Pharmacological inhibition of CFTR attenuates nonalcoholic steatohepatitis (NASH) progression in mice
- Future therapies for cystic fibrosis
- Patient care in cystic fibrosis centers: a real-world analysis in Brazil
- Pilot Study to Assess Safety and Efficacy of an Enteric Microgranule Formulation of Adrulipase in Patients With Exocrine Pancreatic Insufficiency (EPI) Due to Cystic Fibrosis (CF).
- Culture Result Discrepancy Between Laboratories for Nontuberculous Mycobacteria in People with Cystic Fibrosis
- Proteomic profiling of sweat in patients with cystic fibrosis provides new insights into epidermal homoeostasis
- Novel cystic fibrosis transmembrane conductance (CFTR) variant in a patient with Ghanaian parentage
- Risk factors and prognostic value of osteoporosis in hospitalized patients with bronchiectasis
- Manual therapies in cystic fibrosis care: a scoping review
- IMPROVEMENTS IN BODY MASS INDEX OF CHILDREN WITH CYSTIC FIBROSIS FOLLOWING IMPLEMENTATION OF A STANDARDIZED NUTRITIONAL ALGORITHM – A QUALITY IMPROVEMENT PROJECT
- Pseudomonas aeruginosa Siderophores Damage Lung Epithelial Cells and Promote Inflammation
- ABERRANT IMMUNE PROGRAMMING IN NEUTROPHILS IN CYSTIC FIBROSIS
- Acute polymicrobial airway infections: analysis in cystic fibrosis mice
- Challenges and opportunities in the development of novel antimicrobial therapeutics for cystic fibrosis
- Transcriptional profiling of Pseudomonas aeruginosa mature single- and dual-species biofilms in response to meropenem
- Identification of and discrimination between the Mycobacterium abscessus complex and Mycobacterium avium complex directly from sputum using quadruplex real-time PCR
- Frequency of quorum-sensing mutations in Pseudomonas aeruginosa strains isolated from different environments
- Diagnosis and Management of Cystic Fibrosis Exacerbations
- Clinical Relevance of Fungi in Cystic Fibrosis
- Airway epithelial cell-specific deletion of HMGB1 exaggerates inflammatory responses in mice with muco-obstructive airway disease
- Beyond the Local Basic Panel: Full CFTR Gene Analysis Identifies Novel CF Mutation Missed on Standard Testing in an Arabic Child
- Loss of CFTR function is associated with reduced bitter taste receptor-stimulated nitric oxide innate immune responses in nasal epithelial cells and macrophages
- Dry Powder Inhaler with the technical and practical obstacles, and forthcoming platform strategies
- Short-term structural and functional changes after airway clearance therapy in cystic fibrosis
- Characterization of CFTR mutations in people with cystic fibrosis and severe liver disease who are not eligible for CFTR modulators
- Effects of CFTR-modulator triple therapy on sinunasal symptoms in children and adults with cystic fibrosis
- Epidemiological Trends in Nontuberculous Mycobacterial Infection among People with Cystic Fibrosis in Germany
- Airway mucus in pulmonary diseases: Muco-adhesive and muco-penetrating particles to overcome the airway mucus barriers
- Digital technology for monitoring adherence to inhaled therapies in people with cystic fibrosis
- Safety, Tolerability, and Pharmacokinetics of ARCT-032 in Healthy Adult Subjects
- Development and evaluation of a height-based tobramycin initial dosing nomogram for the treatment of adult cystic fibrosis pulmonary exacerbations
- Active cycle of breathing technique for cystic fibrosis
- High-throughput functional assay in cystic fibrosis patient-derived organoids allows drug repurposing
- Patient Perspectives on the Use of Digital Technology to Help Manage Cystic Fibrosis
- Embolisation of a bronchial artery aneurysm to treat significant hemoptysis in cystic fibrosis: a case report
- Time to man up: Increased risk of testicular cancer among boys with cystic fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator Protein Modulators in Children and Adolescents with different CF Genotypes – Systematic Review and Meta-Analysis
- Use of methylene blue to treat vasoplegia syndrome in cystic fibrosis patients undergoing lung transplantation: A case series
- How Many Billions Is Enough? Prioritizing Profits Over Patients With Cystic Fibrosis
- Use of dornase alfa in pediatric patients without cystic fibrosis
- Optimization of CFTR gating through the evolution of its extracellular loops
- Menopause in people with cystic fibrosis
- Extracellular vesicles-derived miRNAs as mediators of pulmonary exacerbation in pediatric cystic fibrosis
- Elexacaftor/tezacaftor/ivacaftor Treatment Reduces Airway Inflammation in Cystic Fibrosis
- Vitamin D levels and their association with oxidative stress and inflammation markers in patients with cystic fibrosis
- Potential of mean force conformational energy maps for disaccharide linkages of the Burkholderia multivorans exopolysaccharide C1576 in aqueous solution
- The gut microbiome, short chain fatty acids, and related metabolites in cystic fibrosis patients with and without colonic adenomas
- Estrogen Patches for Adolescent and Young Adult Women With Cystic Fibrosis
- Efficacy of hypertonic saline versus isotonic saline among children with cystic fibrosis: A systematic review and meta-analysis
- The importance of prenatal diagnosis for the surgical strategy of giant cystic meconium peritonitis: A case report
- MEK1/2 inhibition decreases pro-inflammatory responses in macrophages from people with cystic fibrosis and mitigates severity of illness in experimental murine methicillin-resistant Staphylococcus aureus infection
- Cystic fibrosis rabbits develop spontaneous hepatobiliary lesions and CF-associated liver disease (CFLD)-like phenotypes
- Cost burden among the CF population in the United States: A focus on debt, food insecurity, housing and health services
- Diabetes is associated with increased burden of gastrointestinal symptoms in adults with cystic fibrosis
- An imaging pitfall: misdiagnosis of pulmonary embolism in a patient with advanced cystic fibrosis and bronchiectasis
- Alpha-1 antitrypsin limits neutrophil extracellular trap disruption of airway epithelial barrier function
- Real-time observation of functional specialization among phosphorylation sites in CFTR
- Airway clearance therapy in the school environment: Retrospective analysis of a cohort of pediatric patients with cystic fibrosis
- Correlation between the Brody score and lung function using an ultra-low-dose CT protocol without anesthesia in children with cystic fibrosis
- Antigen specificity and cross-reactivity drive functionally diverse anti-Aspergillus fumigatus T cell responses in cystic fibrosis
- Using metabolic potential within the airway microbiome as predictors of clinical state in persons with cystic fibrosis
- Case report: A case of SLC26A4 mutations causing pendred syndrome and non-cystic fibrosis bronchiectasis
- A conserved WXXE motif is an apical delivery determinant of ABC transporter C subfamily isoforms
- Antisense Oligonucleotide Therapeutics for Cystic Fibrosis: Recent Developments and Perspectives
- Transforming the Nutrition Care Model for Infants with Cystic Fibrosis: a qualitative study of clinicians’ perspectives
- Functional Tests to Resolve Unsolved Rare Diseases. Rares.
- Cystic Fibrosis
- Bariatric surgery in a patient with cystic fibrosis and diabetes: A case report
- Molecular dynamics study of Cl- permeation through cystic fibrosis transmembrane conductance regulator (CFTR)
- Antimicrobial Activity of Ceftazidime-Avibactam, Ceftolozane-Tazobactam, Cefiderocol, and Novel Darobactin Analogs against Multidrug-Resistant Pseudomonas aeruginosa Isolates from Pediatric and Adolescent Cystic Fibrosis Patients
- Transition from pediatric to adult care in chronic respiratory diseases: The cystic fibrosis model
- Progress and prospects toward supramolecular bioactive ion transporters
- Development of musculoskeletal deficits in children with cystic fibrosis in later childhood
- Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report
- TRACK-CF prospective cohort study: Understanding early cystic fibrosis lung disease
- Diversity and prevalence of type VI secretion system effectors in clinical Pseudomonas aeruginosa isolates
- Case report: Cystic fibrosis with kwashiorkor: A rare presentation in the era of universal newborn screening
- A case of mistaken identity: bile duct masquerading as gallbladder
- Therapeutic beta-lactam dosages and broad-spectrum antibiotics are associated with reductions in microbial richness and diversity in persons with cystic fibrosis
- Low Vitamin K Status In Adults With Cystic Fibrosis Is Associated With Reduced Body Mass Index, Insulin Secretion And Increased Pseudomonal Colonization
- Improved glucose tolerance after initiation of Elexacaftor / Tezacaftor / Ivacaftor in adults with cystic fibrosis
- Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on liver tests at a large single adult cystic fibrosis centre
- Bone health outcomes in post-lung transplant patients with cystic fibrosis
- Organoid Technology and Its Role for Theratyping Applications in Cystic Fibrosis
- Evaluation of the Efficiency of Random and Diblock Methacrylate-Based Amphiphilic Cationic Polymers against Major Bacterial Pathogens Associated with Cystic Fibrosis
- Molecular Profiling of Tissue Samples with Chronic Rejection from Patients with Chronic Lung Allograft Dysfunction: A Pilot Study in Cystic Fibrosis Patients
- Immunogenicity and Safety of the BNT162b2 COVID-19 Vaccine in Patients with Cystic Fibrosis with or without Lung Transplantation
- Phenotypic Alteration of an Established Human Airway Cell Line by Media Selection
- Comparison of Virulence-Factor-Encoding Genes and Genotype Distribution amongst Clinical Pseudomonas aeruginosa Strains
- Additive Potentiation of R334W-CFTR Function by Novel Small Molecules
- Lignin-Modifying Enzymes in Scedosporium Species
- Gut Dysbiosis in Children with Cystic Fibrosis: Development, Features and the Role of Gut-Lung Axis on Disease Progression
- Antibacterial Activity of a Natural Clay Mineral against Burkholderia cepacia Complex and Other Bacterial Pathogens Isolated from People with Cystic Fibrosis
- Can Bioactive Food Substances Contribute to Cystic Fibrosis-Related Cardiovascular Disease Prevention?
- Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine
- Effects of CFTR modulators on serum biomarkers of liver fibrosis in children with cystic fibrosis
- Codelivery of synergistic antimicrobials with polyelectrolyte nanocomplexes to treat bacterial biofilms and lung infections
- Induction of killing of Mycobacterium avium subsp. hominissuis in macrophages by cytokine stimulated innate-like lymphoid cells is negatively affected by the pathogen
- Community composition shapes microbial-specific phenotypes in a cystic fibrosis polymicrobial model system
- Gene, RNA, and ASO-based therapeutic approaches in Cystic Fibrosis
- Molecular Epidemiologic Investigation of Mycobacterium intracellulare subspecies chimaera Lung Infections at an Adult Cystic Fibrosis Program
- Acute Recurrent Pancreatitis in a Child With INS-Related Monogenic Diabetes and a Heterozygous Pathogenic CFTR Mutation
- Diagnostic agreement among experts assessing adults presenting with possible cystic fibrosis: need for improvement and implications for patient care
- Nasal epithelial cell culture fluorescence recovery after photobleaching predicts cystic fibrosis therapeutic response
- Home Based Rehabilitation in Children and Adolescents with Cystic Fibrosis: A Systematic Review with Meta-Analysis and Grade Recommendations
- Mycobacterium abscessus infection results in decrease of oxidative metabolism of lung airways cells and relaxation of the epithelial mucosal tight junctions
- Drug-drug interactions with CFTR modulator therapy in cystic fibrosis: Focus on Trikafta®/Kaftrio®
- Corrigendum to FEV1 Variability Helps Identify Patients with Cystic Fibrosis at Risk of Greater Loss of Lung Function [The Journal of Pediatrics (2016) 116-121]
- Is cystic fibrosis a risk factor for COVID-19 infection or related complications?
- Deficient Complement Opsonization Impairs Mycobacterium avium Killing by Neutrophils in Cystic Fibrosis
- Clinical outcome of individuals carrying 5T;TG12 in trans with CFTR variants with varying clinical consequences
- Molecular docking and simulation studies against nucleoside diphosphate kinase (NDK) of Pseudomonas aeruginosa with secondary metabolite identified by genome mining from paenibacillusehimensis
- A Case-Control Study of the Effects of Aspergillus Clinical Phenotypes on Pulmonary Functions in Patients with Cystic Fibrosis
- Nontuberculous Mycobacteria in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Regulator Modulators
- Clinical change two years from start of elexacaftor-tezacaftor-ivacaftor in severe cystic fibrosis
- A model-based economic analysis of the CFHealthHub intervention to support adherence to inhaled medications for people with cystic fibrosis in the UK
- AQP5, a second gene at play with CFTR in aquagenic palmoplantar keratoderma
- High rates of procedure-related pain and anxiety in children with cystic fibrosis
- Early developmental phenotypes in the cystic fibrosis sheep model
- Cystic fibrosis: a call for papers for ECFS 2023
- Anti-protease levels in cystic fibrosis are associated with lung function, recovery from pulmonary exacerbations and may be gender-related
- Use of 2,6-diaminopurine as a potent suppressor of UGA premature stop codons in cystic fibrosis
- Overcoming the burden of cystic fibrosis
- Cystic fibrosis: Infection prevention & control recommendations for universities, colleges and institutes of further and higher education – A practical guide
- French national diagnosis and care protocol (PNDS, protocole national de diagnostic et de soins): cystic lymphatic malformations
- Commentary on “Is Measuring Physical Literacy in School-Aged Children With Cystic Fibrosis or Congenital Heart Disease Needed?”
- PIMS Mimicking Cystic Fibrosis in Infants
- Can Echinococcus granulosus-Derived MicroRNAs be Biomarkers for Diagnosis and Follow-up of Cystic Echinococcosis Patients?
- The impact of CFTR modulators on bone health
- Effects of elexacaftor/tezacaftor/ivacaftor therapy in children with cystic fibrosis – a comprehensive assessment using lung clearance index, spirometry, and functional and structural lung MRI
- Lipidomic alterations in human saliva from cystic fibrosis patients
- Pseudomonas aeruginosa virulence attenuation by inhibiting siderophore functions
- French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version
- Cystic Fibrosis: Back to the Basics
- Role of inhaled antibiotics in the era of highly effective CFTR modulators
- In Vitro Activity of the Novel Antifungal Olorofim against Scedosporium and Lomentospora prolificans
- The Usefulness of Combining Noninvasive Methods for Early Identification and Potential Prevention of Cystic Fibrosis-Associated Liver Disease
- Airway bacterial community composition in persons with advanced cystic fibrosis lung disease
- Evaluation of a novel ELISA test using synthetic mycolic acid antigens for serodiagnosis of non-tuberculous mycobacterial (NTM) infections
- SARS-CoV-2 viral entry and replication is impaired in Cystic Fibrosis airways due to ACE2 downregulation
- SARS-CoV-2-Mediated Lung Edema and Replication Are Diminished by Cystic Fibrosis Transmembrane Conductance Regulator Modulators
- Mutual Effects of Single and Combined CFTR Modulators and Bacterial Infection in Cystic Fibrosis
- Bisphosphonates for osteoporosis in people with cystic fibrosis
- Diagnosing Cystic Fibrosis in Adults
- Microbial Epidemiology of the Cystic Fibrosis Airways: Past, Present, and Future
- Accurate, Sensitive, and Rapid Detection of Pseudomonas aeruginosa Based on CRISPR/Cas12b with One Fluid-Handling Step
- Expression of matrix metalloproteinases in human cystic echinococcosis
- Paranasal mucoceles in children without cystic fibrosis: A case report
- The Association of Field Test Outcomes with Peak Oxygen Uptake in Patients with Cystic Fibrosis: A Systematic Review
- Glycemia and β-cell function before and after elexacaftor/tezacaftor/ivacaftor in youth and adults with cystic fibrosis
- Cell signaling and regulation of CFTR expression in cystic fibrosis cells in the era of high efficiency modulator therapy
- CFTR-beyond the airways: Recent findings on the role of the CFTR channel in the pancreas, the intestine and the kidneys
- Suspected Autosomal Recessive Polycystic Kidney Disease but Cerebellar Vermis Hypoplasia, Oligophrenia Ataxia, Coloboma, and Hepatic Fibrosis (COACH) Syndrome in Retrospect, A Delayed Diagnosis Aided by Genotyping and Reverse Phenotyping: A Case Report and A Review of the Literature
- The CFHealthHub Learning Health System: Using Real-Time Adherence Data to Support a Community of Practice to Deliver Continuous Improvement in an Archetypal Long-Term Condition
- The Role of ABC Transporters in Skin Cells Exposed to UV Radiation
- Extracellular Vesicles’ Role in the Pathophysiology and as Biomarkers in Cystic Fibrosis and COPD
- Biofilm Formation by Staphylococcus aureus in the Specific Context of Cystic Fibrosis
- Nystatin is commonly prescribed as prophylaxis in children beyond the neonatal age
- Lung transplantation for cystic fibrosis and other bronchiectasies
- Thiopurines impair the apical plasma membrane expression of CFTR in pancreatic ductal cells via RAC1 inhibition
- Clinical management of pediatric patients with cystic fibrosis and autism spectrum disorder
- ABC-transporter CFTR folds with high fidelity through a modular, stepwise pathway
- Clinical relevance of low bone density in cystic fibrosis adult patients: A pilot study
- Association of Islet Amyloid Polypeptide to C-Peptide Ratio With Cystic Fibrosis-Related Diabetes: A Prospective Cross-sectional Study
- Benefits of secretion clearance with high frequency percussive ventilation in tracheostomized critically ill patients: a pilot study
- Global burden of nontuberculous mycobacteria in the cystic fibrosis population: a systematic review and meta-analysis
- Correlation between CFTR variants and outcomes of ART in patients with CAVD in Central China
- Targeting HuR-Vav3 mRNA interaction prevents Pseudomonas aeruginosa adhesion to the cystic fibrosis airway epithelium
- AtbFinder Diagnostic Test System Improves Optimal Selection of Antibiotic Therapy in Persons with Cystic Fibrosis
- Implementation of a comprehensive pharmacy-driven immunization care process model in a pediatric cystic fibrosis clinic
- Is there a multinational consensus of tobramycin prescribing and monitoring for cystic fibrosis? Survey of current therapeutic drug monitoring practices in USA/Canada, UK/Ireland, and Australia/New Zealand
- Amyloids and Amyloid-like Protein Aggregates in Food System: Challenges and New Perspectives
- Molecular targets for cystic fibrosis and therapeutic potential of monoclonal antibodies
- Exercise as airway clearance therapy (ExACT) in cystic fibrosis: a UK-based e-Delphi survey of patients, caregivers and health professionals
- Cystic Fibrosis-Related Gut Dysbiosis: A Systematic Review
- Moving the Dial on Airway Inflammation in Response to Trikafta® in Adolescents with Cystic Fibrosis
- “A systematic review of the colorectal microbiome in adult cystic fibrosis patients.”
- Pilot study to determine whether reduced-dose photon-counting detector chest computed tomography can reliably display Brody II score imaging findings for children with cystic fibrosis at radiation doses that approximate radiographs
- Evaluation of the safety of piperacillin-tazobactam extended infusion in pediatric cystic fibrosis patients
- Distribution and Characteristics of Bacteria Isolated from Cystic Fibrosis Patients with Pulmonary Exacerbation
- The Use of DXA for Early Detection of Pediatric Cystic Fibrosis related Bone Disease
- Prevalence, Risk Factors, and Sequelae of Asymptomatic Clostridioides difficile Colonization in Children with Cystic Fibrosis
- The coexistence of two rare diseases thought to use the same pathologic pathway: cystic fibrosis and Niemann-Pick disease
- A Phase 1 Study of VX-522 in Participants With Cystic Fibrosis (CF)
- Gradual Increase in Sweat Chloride Concentration Is Associated with a Higher Risk of CRMS/CFSPID to CF Reclassification
- Cystic fibrosis related diabetes (CFRD) in the era of modulators: A scoping review
- Long-term tezacaftor/ivacaftor safety and efficacy in people with cystic fibrosis and an F508del-CFTR mutation: 96-week, open-label extension of the EXTEND trial
- EFFECT OF ELEXACAFTOR/TEZACAFTOR/IVACAFTOR ON ANNUAL RATE OF LUNG FUNCTION DECLINE IN PEOPLE WITH CYSTIC FIBROSIS
- Transient diabetes insipidus after vasopressin discontinuation in cystic fibrosis with septic shock
- Editorial for “Intra-Visit and Inter-Visit Repeatability of 129 Xenon Multiple-Breath Washout MRI in Children With Stable Cystic Fibrosis Lung Disease”
- Fundamental and translational research in Cystic Fibrosis – why we still need it
- Safety and microbiological activity of phage therapy in persons with cystic fibrosis colonized with Pseudomonas aeruginosa: study protocol for a phase 1b/2, multicenter, randomized, double-blind, placebo-controlled trial
- Inter- and intravisit repeatability of free-breathing MRI in pediatric cystic fibrosis lung disease
- The role of CFTR channel in female infertility
- Diet and the gut-lung axis in cystic fibrosis – direct & indirect links
- Preliminary study of the exploration patients’ experiences of chronic respiratory experiences during the COVID-19 pandemic using interpretative phenomenological analysis
- Synthetic Strategies for Improving Solubility: Optimization of Novel Pyrazolo[1,5-a ]pyrimidine CFTR Activator That Ameliorates Dry Eye Disease
- Impaired distal colonic pH in adults with cystic fibrosis
- Pulmonary exacerbation inflammatory phenotypes in adults with cystic fibrosis
- Detecting clusters of high nontuberculous mycobacteria infection risk for persons with cystic fibrosis – An analysis of U.S. counties
- Comparison of mental health in individuals with primary ciliary dyskinesia, cystic fibrosis, and parent caregivers
- Alteration in glucocorticoids secretion and metabolism in patients affected by cystic fibrosis
- Inhaled Bacteriophage Therapy for Multi-Drug Resistant Achromobacter
- The Status of Carbapenem Resistance in Cystic Fibrosis: A Systematic Review and Meta-Analysis
- Non-pulmonary CFTR-related symptom improvement with ivacaftor in p.Phe508del/p.Arg117His (7T) cystic fibrosis
- Tartrate resistant acid phosphatase 5 (TRAP5) mediates immune cell recruitment in a murine model of pulmonary bacterial infection
- Design of crotoxin-based peptides with potentiator activity targeting the ΔF508NBD1 Cystic Fibrosis Transmembrane Conductance Regulator
- Subtractive sequence analysis aided druggable targets mining in Burkholderia cepacia complex and finding inhibitors through bioinformatics approach
- Tubular-specific expression of HIV protein Vpr leads to severe tubulointerstitial damage accompanied by progressive fibrosis and cystic development
- Evaluation of specificity and sensitivity of IRT/IRT protocol in the cystic fibrosis newborn screening program: 6-year experience of three tertiary centers
- Underreporting of patient-reported outcomes in cystic fibrosis randomized controlled trials using CONSORT-PRO and RoB 2.0
- Comparison of two pregnancies with and without elexacaftor-tezacaftor-ivacaftor in a woman with cystic fibrosis
- Combined multi-channel intraluminal impedance measurement and pHmetry in the detection of gastroesophageal reflux disease in children with cystic fibrosis
- Effect of elexacaftor-tezacaftor-ivacaftor modulator on lung structure in cystic fibrosis
- Structural Determinants of Indole-2-carboxamides: Identification of Lead Acetamides with Pan Antimycobacterial Activity
- Impact of elexacaftor/tezacaftor/ivacaftor on depression and anxiety in cystic fibrosis
- Genetic and Serum Screening for Alpha-1-Antitrypsin Deficiency in Adult Patients with Cystic Fibrosis: A Single-Center Experience
- Gain- and Loss-of-Function CFTR Alleles Are Associated with COVID-19 Clinical Outcomes
- Congenital Cystic Adenomatoid Malformation (CCAM) Type II: A Rare Case of Sudden Infant Death
- Inflammation and Infection in Cystic Fibrosis: Update for the Clinician
- Effects of Exercise Training on Peripheral Muscle Strength in Children and Adolescents with Cystic Fibrosis: A Meta-Analysis
- Acting on the CFTR Membrane-Spanning Domains Interface Rescues Some Misfolded Mutants
- SARS-CoV-2 Infection in Patients with Cystic Fibrosis: What We Know So Far
- Psychometric properties of the Cystic Fibrosis Eating Attitudes and Behaviours scale (CFEAB) in an adult population
- A phase I study assessing the safety and tolerability of allogeneic mesenchymal stem cell infusion in adults with cystic fibrosis
- Lived experiences of people with cystic fibrosis that were not eligible for elexacaftor-tezacaftor-ivacaftor (ETI): A qualitative study
- Longitudinal MRI Detects Onset and Progression of Chronic Rhinosinusitis from Infancy to School Age in Cystic Fibrosis
- CDK8 and CDK19 act redundantly to control the CFTR pathway in the intestinal epithelium
- Rescue by elexacaftor-tezacaftor-ivacaftor of the G1244E cystic fibrosis mutation’s stability and gating defects are dependent on cell background
- ISPAD Clinical Practice Consensus Guidelines 2022: Management of cystic fibrosis-related diabetes in children and adolescents
- The value of bronchocele attenuation in pulmonary computed tomography in assessment of allergic bronchopulmonary aspergillosis in the background of cystic fibrosis: A cross-sectional study
- Respiratory Viruses and Cystic Fibrosis
- Beyond the Lungs-Emerging Challenges in Adult Cystic Fibrosis Care
- Antimicrobial Strategies for Cystic Fibrosis
- Impact of Digital Technologies on Clinical Care for Adults with Cystic Fibrosis
- CFTR Modulators: Current Status and Evolving Knowledge
- Exercise and Airway Clearance Techniques in Cystic Fibrosis
- Effects of Breathing Exercises Combined With Endurance and Strength Training in Patients With Cystic Fibrosis.
- The BATTLE study: Effects of long-term tobramycin inhalation solution (TIS) once daily on exacerbation rate in patients with non-cystic fibrosis bronchiectasis. Study protocol of a double blind, randomized, placebo-controlled trial: study protocol
- Commentary: The experience of living with and managing cystic fibrosis related diabetes: a qualitative review
- The experience of living with and managing cystic fibrosis related diabetes: a qualitative review
- Gene transfection using branched cationic amphiphilic compounds for an aerosol administration in cystic fibrosis context
- Patient-derived cell models for personalized medicine approaches in cystic fibrosis
- Genetic revision of the Hungarian Cystic Fibrosis Registry
- High rates of anxiety detected in mothers of children with inconclusive cystic fibrosis screening results
- Surgical treatment of non-cystic fibrosis bronchiectasis in children and adolescents: A review
- Burkholderia vietnamiensis causing infections in noncystic fibrosis patients in a tertiary care hospital in Mexico
- Human papillomavirus prevalence, persistence and cervical dysplasia in females with cystic fibrosis
- Factors associated with receiving CF care and use of telehealth in 2020 among persons with Cystic Fibrosis in the United States
- Therapeutic advances in cystic fibrosis: from genetics to treatment personalized
- Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis
- Lung transplantation for end-stage cystic fibrosis before and after the accessibility of elexacaftor/tezacaftor/ivacaftor, Germany, 2012-2021
- Assessing the health risk of living near composting facilities on lung health, fungal and bacterial disease in cystic fibrosis: a UK CF Registry study
- Development of liquid culture media mimicking the conditions of sinuses and lungs in cystic fibrosis and health
- Antibiotic Tolerance and Treatment Outcomes in Cystic Fibrosis Methicillin-Resistant Staphylococcus aureus Infections
- Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis
- Psychosocial evaluation in adults with cystic fibrosis-related diabetes: A cross-sectional study
- Development of the CFQ-R-8D: Estimating Utilities From the Cystic Fibrosis Questionnaire-Revised
- CFTR pharmacological modulators: A great advance in cystic fibrosis management
- Linezolid resistance: detection of the cfr(B) gene in French clinical MRSA strains
- Pseudomonas aeruginosa aggregation and Psl expression in sputum is associated with antibiotic eradication failure in children with cystic fibrosis
- An ectopic enhancer restores CFTR expression through de novo chromatin looping
- Cystic fibrosis-related diabetes: An interdisciplinary diagnostic and therapeutic challenge
- Variation in Cystic Fibrosis Newborn Screening Algorithms in the United States
- Mental Health of Cystic Fibrosis Patients and the COVID-19 Pandemic in Poland: A Single-Center Comparative Study
- Cystic Fibrosis Patients with F508del/Minimal Function Genotype: Laboratory and Nutritional Evaluations after One Year of Elexacaftor/Tezacaftor/Ivacaftor Treatment
- Redefining Hypo- and Hyper-Responding Phenotypes of CFTR Mutants for Understanding and Therapy
- The influence of exocrine pancreatic function on the exposure and pharmacokinetics of ivacaftor in people with cystic fibrosis
- What Do Adults With Cystic Fibrosis Want From Their Doctors?
- Biofilm infection of a central venous port-catheter caused by Mycobacterium avium complex in an immunocompetent child with cystic fibrosis
- The inequity of targeted cystic fibrosis reproductive carrier screening tests in Australia
- Sepsis Due to Pandoraea sputorum Infection After Multiple Trauma in a Non-Cystic Fibrosis Patient: A Case Report from Southeast China
- Cancer incidence and prevalence in cystic fibrosis patients with and without a lung transplant in France
- A survey assessing the impact of COVID-19 and elexacaftor/tezacaftor/ifavacaftor on both physical and mental health in adults with cystic fibrosis
- Normal reference values for aerobic fitness in cystic fibrosis: a scoping review
- Physical activity and liver disease affect the fat-free mass in adolescents with cystic fibrosis
- Impact of Viral Respiratory Infections on Pulmonary Exacerbations in Children with Cystic Fibrosis
- Eradication of early MRSA infection in cystic fibrosis: a novel study design for the STAR-ter trial
- Whole blood RNA-seq demonstrates an increased host immune response in individuals with cystic fibrosis who develop nontuberculous mycobacterial pulmonary disease
- Loss of endothelial CFTR drives barrier failure and edema formation in lung infection and can be targeted by CFTR potentiation
- Cystic fibrosis transmembrane conductance regulator attenuates oxidative stress-induced injury in diabetic retinopathy rats
- Expression of CFTR, a hallmark gene of ionocytes, is downregulated in salivary glands of Sjögren’s syndrome patients
- Study to Determine the Efficacy&Safety of ARV-1801/ACG-701 for the Treatment of Cystic Fibrosis Pulmonary Exacerbations
- Clinician perspectives on assessing for disordered eating and body image disturbance in adolescents and young adults with cystic fibrosis
- Divergent dynamics of inflammatory mediators and multiplex PCRs during airway infection in cystic fibrosis patients and healthy controls: Serial upper airway sampling by nasal lavage
- S945L-CFTR molecular dynamics, functional characterization and tezacaftor/ivacaftor efficacy in vivo and in vitro in matched pediatric patient-derived cell models
- SIMPLIFYing cystic fibrosis treatment in a post-modulator era
- Restoring myocardial infarction-induced long-term memory impairment by targeting the cystic fibrosis transmembrane regulator
- Approaches to the management of haemoptysis in young people with cystic fibrosis
- Effects of long-term tobramycin inhalation solution (TIS) once daily on exacerbation rate in patients with non-cystic fibrosis bronchiectasis
- Prevalence and Characteristics of Cystic Fibrosis in Omani Children: A Multi-center Cross-sectional Study
- Exploring parental cystic fibrosis disclosure to well children
- Elexacaftor-Tezacaftor-Ivacaftor corrects monocyte microbicidal deficiency in cystic fibrosis
- Normalization of circulating neutrophil counts after 12 months of elexacaftor-tezacaftor-ivacaftor in patients with advanced cystic fibrosis
- Moonlighting chaperone activity of the enzyme PqsE contributes to RhlR-controlled virulence of Pseudomonas aeruginosa
- Diagnosis of cystic fibrosis in adults: Australian Cystic Fibrosis Data Registry data, 2000-2019
- The Effects of Antibiotic Combination Treatments on Pseudomonas aeruginosa Tolerance Evolution and Coexistence with Stenotrophomonas maltophilia
- Impact of the COVID-19 pandemic and associated lockdown measures on the management, health, and behavior of the cystic fibrosis population in France during 2020 (MUCONFIN)
- Manuka honey in combination with azithromycin shows potential for improved activity against Mycobacterium abscessus
- Role of Inhalational Aztreonam Lysine in Lower Airway Infections in Cystic Fibrosis: An Updated Literature Review
- Clinical characteristics and outcomes of incident cases of COVID-19 in unvaccinated adult cystic fibrosis patients in southern Brazil: a prospective cohort study conducted during the first year of the COVID-19 pandemic
- Acneiform Eruption Following Elexacaftor-Tezacaftor-Ivacaftor Treatment in Patients With Cystic Fibrosis
- Noninvasive prenatal screening for cystic fibrosis using circulating trophoblasts: Detection of the 50 most common disease-causing variants
- Diverticulitis and Diverticulosis of the Appendix: A Case Series
- Need to study simplification of gastrointestinal medication regimen in cystic fibrosis in the era of highly effective modulators
- German Newborn Screening for Cystic Fibrosis – Parental perspectives and suggestions for improvements
- Impact of Elexacaftor-Tezacaftor-Ivacaftor on Bacterial Colonization and Inflammatory Responses in Cystic Fibrosis
- Pulmonary drug delivery technology enables anakinra repurposing in cystic fibrosis
- The association of fecal calprotectin and respiratory exacerbation in cystic fibrosis patients
- Real-life efficacy and safety of elexacaftor/tezacaftor/ivacaftor on severe cystic fibrosis lung disease patients
- Congenital absence of the vas deferens with hypospadias or without hypospadias: Phenotypic findings and genetic considerations
- Inhaled Antibiotics in Non-cystic Fibrosis Bronchiectasis (NCFB): A Systematic Review of Efficacy and Limitations in Adult Patients
- The Bep gene cluster in Burkholderia cenocepacia H111 codes for a water-insoluble exopolysaccharide essential for biofilm formation
- Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis
- Aminoglycoside induced ototoxicity risk in the cystic fibrosis population: The utility of large-scale screening
- Cefiderocol resistance genomics in sequential chronic Pseudomonas aeruginosa isolates from cystic fibrosis patients
- How macronutrients and pancreatic enzyme supplements dose variability affect fat, protein and starch absorption in children with cystic fibrosis
- Anti-Aspergillus fumigatus IgG in patients with bronchiectasis and its relationship with clinical outcome
- Impact of N-Acetylcysteine and Antibiotics Against Single and Dual Species Biofilms of Pseudomonas aeruginosa and Achromobacter xylosoxidans
- Clinical and Genetic Characterisation of Cystic Fibrosis Patients in Latvia: A Twenty-Five-Year Experience
- Modifier Factors of Cystic Fibrosis Phenotypes: A Focus on Modifier Genes
- Conjunctival Fluid Secretion Impairment via CaCC-CFTR Dysfunction Is the Key Mechanism in Environmental Dry Eye
- New TMA (4,6,4′-Trimethyl angelicin) Analogues as Anti-Inflammatory Agents in the Treatment of Cystic Fibrosis Lung Disease
- The importance of aeroallergen sensitivity in children with cystic fibrosis
- A 3D stack-of-spirals approach for rapid hyperpolarized 129 Xe ventilation mapping in pediatric cystic fibrosis lung disease
- Permissive and nonpermissive channel closings in CFTR revealed by a factor graph inference algorithm
- Pediatric Pulmonology 2021 Year in Review: Rare and Diffuse Lung Disease
- Rectal organoid-guided CFTR modulator therapy restores lung function in a CF patient with the rare 1677delTA/R334W genotype
- Day 5 versus day 3 embryo biopsy for preimplantation genetic testing for monogenic/single gene defects
- Longitudinal Study of Therapeutic Adherence in a Cystic Fibrosis Unit: Identifying Potential Factors Associated with Medication Possession Ratio
- Airspace Enlargement with Fibrosis in a Young Heavy Smoker Mimicking Diffuse Cystic Lung Disease
- Culture independent markers of nontuberculous mycobacterial (NTM) lung infection and disease in the cystic fibrosis airway
- Clinical outcomes associated with Achromobacter species infection in people with cystic fibrosis
- Delayed Spontaneous Pneumothorax in a Previously Healthy Nonventilated COVID-19 Patient
- Antibody deficiencies with normal IgG in adults with Non-cystic fibrosis bronchiectasis or recurrent pneumonia: Cross-sectional study
- Cefiderocol to manage chronic, multi-drug-resistant Burkholderia cepacia complex infection in a patient with cystic fibrosis: a case report
- Real-world data on the efficacy and safety of tezacaftor-ivacaftor in adults living with cystic fibrosis homozygous for F508del and heterozygous for F508del and a residual function mutation
- REFINEMENT OF NEWBORN SCREENING FOR CYSTIC FIBROSIS WITH NEXT GENERATION SEQUENCING
- The Fatty Acid Imbalance of Cystic Fibrosis Exists at Birth Independent of Feeding, in Pig and Ferret Models
- Long-Term Use Of Inhaled Hypertonic Saline In Children And Adolescents With Cystic Fibrosis: Experience From A Tertiary Care Center In A Low And Middle Income Country
- Missed Cystic Fibrosis Newborn Screening Cases due to Immunoreactive Trypsinogen Levels below Program Cutoffs: A National Survey of Risk Factors
- Clinical efficacy of elexacaftor-tezacaftor-ivacaftor in an adolescent with homozygous G85E cystic fibrosis
- Nebulized levofloxacin for chronic Burkholderia cenocepacia pulmonary infection in cystic fibrosis: A case report
- Oxygen-induced hypercapnia: physiological mechanisms and clinical implications
- Identification of Novel F508del-CFTR traffic correctors among triazole derivatives
- Using Hygge to Promote Wellness and Coping With a Chronic Illness
- Cystic Fibrosis Screen Positive, Inconclusive Diagnosis Genotypes in People with Cystic Fibrosis from the U.S. Patient Registry
- Perceptions of airway gene therapy for cystic fibrosis
- Cystic fibrosis treatment: hope for all?
- Physical activity and exercise training in cystic fibrosis
- Steps Ahead: Optimising physical activity in adults with cystic fibrosis: A pilot randomised trial using wearable technology, goal setting and text message feedback
- Altered fecal microbiome and metabolome in adult patients with non-cystic fibrosis bronchiectasis
- Patient registry data highlights international differences in survival in cystic fibrosis
- Radiological Changes After Treatment With Triple Therapy in Cystic Fibrosis. Are Bronchiectasis Irreversible?
- Vasoactive intestinal peptide and cystic fibrosis transmembrane conductance regulator contribute to the transepithelial calcium transport across intestinal epithelium-like Caco-2 monolayer
- Survival of people with cystic fibrosis in Australia
- Bronchial artery diameter in massive hemoptysis in cystic fibrosis
- Prevalence and Risk Factors for Low Bone Mineral Density in Adults With Cystic Fibrosis
- Personalized medicine approaches in cystic fibrosis related pancreatitis
- Corneal Refractive Surgery Considerations in Patients with Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders
- Surface Dependent Inhibition of Mycobacterium abscessus by Diverse Pseudomonas aeruginosa Strains
- Boosting Lung Accumulation Of Gallium With Inhalable Nano-Embedded Microparticles For The Treatment Of Bacterial Pneumonia
- An unusual case of cystic fibrosis with pancytopenia due to copper deficiency and blindness caused by vitamin A deficiency: A case-report
- Successful pregnancy in a cystic fibrosis patient with a severe impairment of lung function receiving Elexacaftor-Tezacaftor-Ivacaftor
- Atypical Presentation of Cystic Fibrosis in An Infant
- Experience to date with CFTR modulators during pregnancy and breastfeeding in the British Columbia Cystic Fibrosis clinic
- High-quality read-based phasing of cystic fibrosis cohort informs genetic understanding of disease modification
- Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
- Drug treatment of cystic fibrosis
- The long-term effects of insulin use in incident cystic fibrosis-related diabetes: a target trial emulated using longitudinal national registry data
- Assessment of factors and interventions towards therapeutic adherence among persons with non-cystic fibrosis bronchiectasis
- Long-term effectiveness of dual CFTR modulator treatment of cystic fibrosis
- Synthesis and Biological Characterization of Fluorescent Cyclipostins and Cyclophostin Analogues: New Insights for the Diagnosis of Mycobacterial-Related Diseases
- Systems-Wide Dissection of Organic Acid Assimilation in Pseudomonas aeruginosa Reveals a Novel Path To Underground Metabolism
- CFTR modulates aquaporin-mediated glycerol permeability in mouse Sertoli cells
- Cystic Fibrosis-Related Diabetes: Clinical approach and knowledge gaps
- Distinct Long- and Short-Term Adaptive Mechanisms in Pseudomonas aeruginosa
- Inhaled anti-pseudomonal antibiotics for long-term therapy in cystic fibrosis
- COVID-19 Antibody Responses in Cystic Fibrosis
- Effect of Trikafta on bone density, body composition and exercise capacity in CF: a pilot study
- Effects of elexacaftor-tezacaftor-ivacaftor discontinuation in cystic fibrosis
- Quantitation of cystic fibrosis triple combination therapy, elexacaftor/tezacaftor/ivacaftor, in human plasma and cellular lysate
- European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance
- Chest magnetic resonance imaging in cystic fibrosis: technique and clinical benefits
- Fungal microbiota dynamics and its geographical, age and gender variability in patients with cystic fibrosis
- Contemporary cystic fibrosis incidence rates in Canada and the United States
- Screening by high-throughput sequencing for pathogenic variants in cystic fibrosis: Benefit of introducing personalized therapies
- MR Imaging of Germ Cell and Sex Cord Stromal Tumors
- Impact of COVID-19 on Lung Disease in People with Cystic Fibrosis: A 6-Month Follow-Up Study on Respiratory Outcomes
- The Efficacy of an N-Acetylcysteine-Antibiotic Combination Therapy on Achromobacter xylosoxidans in a Cystic Fibrosis Sputum/Lung Cell Model
- Effect of Dornase Alfa on the Lung Clearance Index in Children with Cystic Fibrosis: A Lesson from a Case Series
- Physical Activity and Quality of Life among Patients with Cystic Fibrosis
- Effectiveness of Physical Exercise Interventions on Pulmonary Function and Physical Fitness in Children and Adults with Cystic Fibrosis: A Systematic Review with Meta-Analysis
- “What Are the Applications for Remote Rehabilitation Management in Cystic Fibrosis?”: A Scoping Review Protocol
- Genetic Deletion of Mmp9 Does Not Reduce Airway Inflammation and Structural Lung Damage in Mice with Cystic Fibrosis-like Lung Disease
- Anti-Virulence Potential of a Chionodracine-Derived Peptide against Multidrug-Resistant Pseudomonas aeruginosa Clinical Isolates from Cystic Fibrosis Patients
- Clinical Advances in Cystic Fibrosis
- Role of Bronchial Artery Embolization as Early Treatment Option in Stable Cystic Fibrosis Patients with Sub-Massive Hemoptysis: Personal Experience and Literature Review
- Vitamin Status in Children with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutation
- Essential Fatty Acid Deficiency in Cystic Fibrosis Disease Progression: Role of Genotype and Sex
- Pulmonary Safety Profile of Esc Peptides and Esc-Peptide-Loaded Poly(lactide-co-glycolide) Nanoparticles: A Promising Therapeutic Approach for Local Treatment of Lung Infectious Diseases
- Probiotic knowledge of adults with cystic fibrosis is limited but is associated with probiotic use: A cross-sectional survey study
- The Value of the Patient Voice: A Review of Salt in My Soul by Mallory Smith
- Cystic Fibrosis
- The periplasmic chaperone Skp prevents misfolding of the secretory lipase A from Pseudomonas aeruginosa
- Cystic fibrosis with liver involvement in adults has a benign course. Results from a tertiary referral centre cohort
- Immunoreactive trypsinogen in healthy newborns and infants with cystic fibrosis
- Embedded Specialist Palliative Care in Cystic Fibrosis: Results of a Randomized Feasibility Clinical Trial
- Higher Mortality Rates Associated with Clostridioides difficile Infection in Hospitalized Children with Cystic Fibrosis
- Cystic fibrosis-related mortality trends in Mexico between 1999 – 2020
- Immunoglobulin A Vasculitis in a Child: Secondary to COVID-19 or Cystic Fibrosis?
- Clearing Up the Smoke: Physical and Mental Health Considerations Regarding Cannabis Use in Adolescents with Cystic Fibrosis
- Rounding out the pediatric CF care team: Including the profession of occupational therapy
- Case Report of a Pediatric Chinese Cystic Fibrosis Patient with the c.1521_1523delCTT/c.3874-4522A>G Genotype
- Epidemiology and management of nontuberculous mycobacterial disease in people with cystic fibrosis, the Netherlands
- Carbohydrate metabolism impairment in children and adolescents with cystic fibrosis
- Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials
- Toward a Broader Understanding of Cystic Fibrosis Epidemiology and Its Impact on Clinical Manifestations
- Genetics of Cystic Fibrosis: Clinical Implications
- Update on Innate and Adaptive Immunity in Cystic Fibrosis
- Novel Applications of Biomarkers and Personalized Medicine in Cystic Fibrosis
- Emerging Approaches to Monitor and Modify Care in the Era of Cystic Fibrosis Transmembrane Conductance Regulators
- The Impact of Highly Effective Modulator Therapy on Cystic Fibrosis Microbiology and Inflammation
- Novel Approaches to Multidrug-Resistant Infections in Cystic Fibrosis
- Update on Clinical Outcomes of Highly Effective Modulator Therapy
- Nontuberculous Mycobacterial Infections in Cystic Fibrosis
- Non-Modulator Therapies: Developing a Therapy for Every Cystic Fibrosis Patient
- Updates in Nutrition Management of Cystic Fibrosis in the Highly Effective Modulator Era
- Update in Advancing the Gastrointestinal Frontier in Cystic Fibrosis
- Transitions of Care in Cystic Fibrosis
- Endocrine Complications of Cystic Fibrosis
- Management of Mental Health in Cystic Fibrosis
- Family Planning and Reproductive Health in Cystic Fibrosis
- Update on Lung Transplantation for Cystic Fibrosis
- Cystic Fibrosis in the Era of Highly Effective CFTR Modulators
- Sinusitis, otitis media and diffuse bronchiectasis in both lungs
- The effect of N-acetylcysteine in patients with non-cystic fibrosis bronchiectasis (NINCFB): study protocol for a multicentre, double-blind, randomised, placebo-controlled trial
- Natural Products Lysobactin and Sorangicin A Show In Vitro Activity against Mycobacterium abscessus Complex
- Parallel Evolution of Pseudomonas aeruginosa during a Prolonged ICU-Infection Outbreak
- Persistent pleuritic chest pain in a patient with cystic fibrosis
- Management of pregnancy in cystic fibrosis
- Transition from paediatric to adult care in cystic fibrosis
- Toxoplasma gondii infection triggers ongoing inflammation mediated by increased intracellular Cl- concentration in airway epithelium
- Risk factors and outcomes of non-tuberculous mycobacteria infection in lung transplant recipients: A systematic review and meta-analysis
- Marginal association of fasting blood glucose with the risk of cystic fibrosis-related diabetes
- Salicylate attenuates gentamicin-induced ototoxicity and facilitates the recovery in the basilar papilla of the lizard Podarcis siculus
- Interplay between biofilm microenvironment and pathogenicity of Pseudomonas aeruginosa in cystic fibrosis lung chronic infection
- Significance and potential role of eosinophils in non-cystic fibrosis bronchiectasis
- Transcription factor FoxM1 promotes cyst growth in PKD1 mutant ADPKD
- The Effects of Virtual Reality Physiotherapy Interventions on Cardiopulmonary Function and Breathing Control in Cystic Fibrosis: A Systematic Review
- Rate of Lung Function Decline in People with Cystic Fibrosis Having a Residual Function Gene Mutation
- The relationship between weight and pulmonary outcomes in overweight and obese people with cystic fibrosis: A retrospective observational study
- An Unusual Case of Type 2 Fronto-Ethmoidal Mucopyocele
- The effect of Pseudomonas aeruginosa eradication regimens on chronic colonization and clinical outcomes in pediatric patients with cystic fibrosis
- Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis
- Development of novel therapeutics for all individuals with CF (the future goes on)
- Can Identifying Pulmonary Exacerbation Phenotypes Guide New Treatment Approaches for Cystic Fibrosis?
- Challenged Urine Bicarbonate Excretion as a Measure of Cystic Fibrosis Transmembrane Conductance Regulator Function in Cystic Fibrosis
- The Clinical Yield of Bronchoscopy in The Management of Cystic Fibrosis – A Retrospective Multicenter Study
- Outcome of elexacaftor/tezacaftor/ivacaftor therapy in patients with cystic fibrosis and solid organ transplantation
- A Case of Allergic Bronchopulmonary Aspergillosis With Bronchial Asthma
- Quick and wide-range taxonomical repertoire establishment of the cystic fibrosis lung microbiota by tandem mass spectrometry on sputum samples
- Serum levels of hormones regulating appetite in patients with cystic fibrosis – a single-center, cross-sectional study
- Efficacy of selective histone deacetylase 6 inhibition in mouse models of Pseudomonas aeruginosa infection: A new glimpse for reducing inflammation and infection in cystic fibrosis
- Chest radiography and computed tomography imaging in cystic fibrosis: current challenges and new perspectives
- Clinical findings of methicillin-resistant Staphylococcus aureus in cystic fibrosis
- Gene therapy for cystic fibrosis: Challenges and prospects
- Modulation of Biofilm Mechanics by DNA Structure and Cell Type
- Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders
- The In Vivo and In Vitro Assessment of Pyocins in Treating Pseudomonas aeruginosa Infections
- Antimicrobial Treatment on a Catheter-Related Bloodstream Infection (CRBSI) Case Due to Transition of a Multi-Drug-Resistant Ralstonia mannitolilytica from Commensal to Pathogen during Hospitalization
- Virtual Drug Repositioning as a Tool to Identify Natural Small Molecules That Synergize with Lumacaftor in F508del-CFTR Binding and Rescuing
- Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with CFTR Mutations Causing Pancreatic Insufficiency
- Complementary Dual Approach for In Silico Target Identification of Potential Pharmaceutical Compounds in Cystic Fibrosis
- Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease
- Kidney Cyst Lining Epithelial Cells Are Resistant to Low-Dose Cisplatin-Induced DNA Damage in a Preclinical Model of Autosomal Dominant Polycystic Kidney Disease
- Serratiopeptidase Affects the Physiology of Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients
- Drug Repurposing for Cystic Fibrosis: Identification of Drugs That Induce CFTR-Independent Fluid Secretion in Nasal Organoids
- Barriers for Sports and Exercise Participation and Corresponding Barrier Management in Cystic Fibrosis
- The Effectiveness of Ultraviolet-C (UV-C) Irradiation on the Viability of Airborne Pseudomonas aeruginosa
- Dynamic Perfluorinated Gas MRI Shows Improved Lung Ventilation in People with Cystic Fibrosis after Elexacaftor/Tezacaftor/Ivacaftor: An Observational Study
- Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium
- Quantifying Intracellular Viral Pathogen: Specimen Preparation, Visualization and Quantification of Multiple Immunofluorescent Signals in Fixed Human Airway Epithelium Cultured at Air-Liquid Interface
- Identification of Exhaled Metabolites in Children with Cystic Fibrosis
- Recovery of ΔF508-CFTR Function by Citrate
- Positive impact of insulin treatment on clinical trend in cystic fibrosis patients: a retrospective study
- Short Chain Fatty Acids: Fundamental mediators of the gut-lung axis and their involvement in pulmonary diseases
- Cftr deletion in mouse epithelial and immune cells differentially influence the intestinal microbiota
- 3D Single-Breath Chemical Shift Imaging Hyperpolarized Xe-129 MRI of Healthy, CF, IPF, and COPD Subjects
- Personalized Selection of a CFTR Modulator for a Patient with a Complex Allele [L467F;F508del]
- Small Colonies, Bigger Problems? New Evidence That Staphylococcus aureus Small Colony Variants Can Worsen Lung Inflammation in Cystic Fibrosis Rats
- Physiologically Based Pharmacokinetic Modeling To Guide Management of Drug Interactions between Elexacaftor-Tezacaftor-Ivacaftor and Antibiotics for the Treatment of Nontuberculous Mycobacteria
- Newly Discovered Cutting-Edge Triple Combination Cystic Fibrosis Therapy: A Systematic Review
- Use of systemic steroids during pulmonary exacerbations in children and adolescent with Cystic fibrosis
- Impact of cholesterol and Lumacaftor on the folding of CFTR helical hairpins
- Cystic Fibrosis: Highly Effective Targeted Therapeutics and the Impact on Sex and Racial Disparities
- Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI
- Parental Preferences about Policy Options Regarding Disclosure of Incidental Genetic Findings in Newborn Screening: Using Videos and the Internet to Educate and Obtain Input
- Update on cystic fibrosis : From neonatal screening to causal treatment
- The relationship between lung disease severity and the sputum proteome in cystic fibrosis
- Patient engagement in the development of CF-CBT: A cystic fibrosis-specific cognitive-behavioral intervention for adults
- A pathogenic variant of TULP3 causes renal and hepatic fibrocystic disease
- Ion channels as a therapeutic target for renal fibrosis
- The Impact of Elexacaftor/Ivacaftor/Tezacaftor on Cystic Fibrosis Patients Who Acquire COVID-19 Infection
- Myofibroblast depletion reduces kidney cyst growth and fibrosis in autosomal dominant polycystic kidney disease
- Changes in exercise endurance and inspiratory capacity after lumacaftor/ivacaftor therapy in cystic fibrosis
- Validity and reliability of the one-minute sit-to-stand test for the measurement of cardio-respiratory responses in children with cystic fibrosis. Authors’ reply
- Frequencies of pathogenic CFTR variants in Greek cystic fibrosis patients with allergic bronchopulmonary aspergillosis and Aspergillus fumigatus chronic colonization: A retrospective cohort study
- Narrowing the Differential Diagnosis of Cystic Lesions in Smokers with Expiratory CT Acquisition Using the Cyst-Airway Communication Hypothesis
- Serum kisspeptin and proopiomelanocortin in cystic fibrosis: a single study
- New concepts in antimicrobial resistance in cystic fibrosis respiratory infections
- Corrigendum for Update on the management of vitamins and minerals in cystic fibrosis. Nutr Clin Pract. 2022;37(5):1074-1087
- Effects of Telerehabilitation-based Exercises in Cystic Fibrosis
- Coronary artery disease in patients with cystic fibrosis – A case series and review of the literature
- Amikacin Liposomal Inhalation Suspension in the Treatment of Mycobacterium abscessus Lung Infection: A French Observational Experience
- Family case studies: absence of Pseudomonas aeruginosa transmission in bronchiectasis
- Nonsense codons suppression. An acute toxicity study of three optimized TRIDs in murine model, safety and tolerability evaluation
- Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor
- Unified Airway-Cystic Fibrosis
- Real life evaluation of the multi-organ effects of Lumacaftor/Ivacaftor on F508del homozygous cystic fibrosis patients
- Identification and characterization of bacteria isolated from patients with cystic fibrosis in Jordan
- Inhaled Corticosteroids in Adults with Non-cystic Fibrosis Bronchiectasis: From Bench to Bedside. A Narrative Review
- Molecular structures reveal synergistic rescue of Δ508 CFTR by Trikafta modulators
- Bicarbonate defective CFTR variants increase risk for chronic pancreatitis: A meta-analysis
- Novel Substituted Cyclopropyl Compounds as CFTR Modulators for Treating Cystic Fibrosis
- A single heterozygous nonsense mutation in the TTC21B gene causes adult-onset nephronophthisis 12: A case report and review of literature
- Effect of Ultraviolet-c (UVc) light and ozone on the survival of Mycobacterium abscessus complex organisms associated with cystic fibrosis
- Helping map the taxonomical position of the Nontuberculous Mycobacteria (NTM) in cystic fibrosis
- Allergic bronchopulmonary aspergillosis in association with rheumatic heart disease: report of three cases
- Assessment of Cystic Fibrosis Distribution Based on Air Pollution by Geographical Information System (GIS)
- Effect of Physical Activity Program on the Quality of Life of Children with Cystic Fibrosis at School Age: A Randomized Clinical Trail
- Production of CFTR Mutant Gene Model by Homologous Recombination System
- Statistical Evaluation of Metaproteomics and 16S rRNA Amplicon Sequencing Techniques for Study of Gut Microbiota Establishment in Infants with Cystic Fibrosis
- Airway clearance physiotherapy and health-related quality of life in cystic fibrosis
- Discovery of N -β-l-Fucosyl Amides as High-Affinity Ligands for the Pseudomonas aeruginosa Lectin LecB
- Antibiotic Resistance in Pseudomonas
- Cell Envelope Stress Response in Pseudomonas aeruginosa
- Pseudomonas aeruginosa in the Cystic Fibrosis Lung
- Mixed Populations and Co-Infection: Pseudomonas aeruginosa and Staphylococcus aureus
- Association between exercise and risk of cardiovascular diseases in patients with non-cystic fibrosis bronchiectasis
- The risk of colorectal cancer in individuals with mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene: An English population-based study
- Effects of prolonged proton pump inhibitor treatment on nutritional status and respiratory infection risk in cystic fibrosis: A matched cohort study
- Variability of clinically measured lung clearance index in children with cystic fibrosis
- Aryl Hydrocarbon Receptor Agonism Antagonizes the Hypoxia-driven Inflammation in Cystic Fibrosis
- Identification of cystic fibrosis transmembrane conductance regulator as a prognostic marker for juvenile myelomonocytic leukemia via the whole-genome bisulfite sequencing of monozygotic twins and data mining
- Synthesis of C6-modified mannose 1-phosphates and evaluation of derived sugar nucleotides against GDP-mannose dehydrogenase
- Specific circulating neutrophils subsets are present in clinically stable adults with cystic fibrosis and are further modulated by pulmonary exacerbations
- Determination of Cystic Fibrosis Mutation Frequency in Preterm and Term Neonates with Respiratory Tract Problems
- Now What? Collective Sensemaking and Sensegiving in the Cystic Fibrosis Community in Sweden During the Initial Phase of the COVID-19 Pandemic
- Pulmonary disorders in pregnancy: Bronchiectasis, cystic fibrosis, sarcoidosis and interstitial diseases
- Guidelines for Cystic Fibrosis Carrier Screening in the Prenatal/Preconception Period
- Inhalable ceftazidime-roflumilast powder targeting infection and inflammation: Influence of incorporating roflumilast into ceftazidime-leucine co-amorphous formulation
- CFTR Modulators and Reduction of Airway Inflammation in Cystic Fibrosis: How Much is Enough?
- An inflammatory Signature of Glucose Impairment in Cystic Fibrosis
- Plasticity towards Rigidity: A Macrophage Conundrum in Pulmonary Fibrosis
- Modulator Combination Improves In Vitro the Microrheological Properties of the Airway Surface Liquid of Cystic Fibrosis Airway Epithelia
- Detection of Disease-Causing CFTR Variants In State Newborn Screening Programs
- Genotype-phenotype correlations of cystic fibrosis in siblings compound heterozygotes for rare variant combinations: Review of literature and case report
- Improved Trimethylangelicin Analogs for Cystic Fibrosis: Design, Synthesis and Preliminary Screening
- Synthesis and Biomedical Applications of Mucin Mimic Materials
- Clinically undetected polyclonal heteroresistance among Pseudomonas aeruginosa isolated from cystic fibrosis respiratory specimens
- Evidence of validity of internal structure of the Functional Assessment of Chronic Illness Therapy-Spiritual Well-Being Scale (FACIT-Sp-12) in Brazilian adolescents with chronic health conditions
- Deletion of sphingosine kinase 2 attenuates cigarette smoke-mediated chronic obstructive pulmonary disease-like symptoms by reducing lung inflammation
- MRI evaluation of right heart functions in children with mild cystic fibrosis
- Idiopathic pulmonary fibrosis: Diagnosis, biomarkers and newer treatment protocols
- ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria
- The efficacy of azithromycin to prevent exacerbation of non-cystic fibrosis bronchiectasis: a meta-analysis of randomized controlled studies
- 129 Xe and Free-Breathing 1 H Ventilation MRI in Patients With Cystic Fibrosis: A Dual-Center Study
- Investigation of the Mechanism and Chemistry Underlying Staphylococcus aureus’ Ability to Inhibit Pseudomonas aeruginosa Growth In Vitro
- Metabolic Mechanism and Physiological Role of Glycerol 3-Phosphate in Pseudomonas aeruginosa PAO1
- Airway surface hyperviscosity and defective mucociliary transport by IL-17/TNF-α are corrected by beta-adrenergic stimulus
- CGM patterns in adults with cystic fibrosis-related diabetes before and after elexacaftor-tezacaftor-ivacaftor therapy
- Restoring airway epithelial homeostasis in Cystic Fibrosis
- Structure basis of CFTR folding, function and pharmacology
- Discovery of Homogentisic Acid as a Precursor in Trimethoprim Metabolism and Natural Product Biosynthesis
- Meconium Ileus, Distal Intestinal Obstruction Syndrome, and Other Gastrointestinal Pathology in the Cystic Fibrosis Patient
- Quantity and quality of airway clearance in children and young people with cystic fibrosis
- Sexual and reproductive health experiences and care of adult women with cystic fibrosis
- High Rates of Extensively Drug-Resistant Pseudomonas aeruginosa in Children with Cystic Fibrosis
- ECFS standards of care on CFTR-related disorders: Diagnostic criteria of CFTR dysfunction
- Activity of newest generation β-lactam/β-lactamase inhibitor combination therapies against multidrug resistant Pseudomonas aeruginosa
- Pleiotropic modifiers of age-related diabetes and neonatal intestinal obstruction in cystic fibrosis
- Thoracic configuration in patients ageing with cystic fibrosis: A retrospective single-blinded cohort study
- Exploring the impact of elexacaftor-tezacaftor-ivacaftor treatment on opinions regarding airway clearance techniques and nebulisers: TEMPO a qualitative study in children with cystic fibrosis, their families and healthcare professionals
- Limited evidence of patient-to-patient transmission of Staphylococcus aureus strains between children with cystic fibrosis, Queensland, Australia
- Changing racial and ethnic differences for lung transplantation in cystic fibrosis
- Pharmacological inhibitors of the cystic fibrosis transmembrane conductance regulator exert off-target effects on epithelial cation channels
- Investigating the genetic profile of familial atypical cystic fibrosis patients (DeltaF508-CFTR) with neonatal biliary atresia
- Retrospective Review of Vancomycin Monitoring via Trough Only vs. Two- Point Estimated Area Under the Curve in Pediatric and Adult Patients with Cystic Fibrosis
- Scars of COVID-19: A bibliometric analysis of post-COVID-19 fibrosis
- Longitudinal Assessment of Educational Risk for K-12 Students with Cystic Fibrosis
- Cough suppression and HRQoL in adult people with cystic fibrosis: an unexplored correlation
- Trends in and Outcomes of Deliveries Complicated by Cystic Fibrosis
- Assessing the Occurrence of Reproductive Health Counseling in Adolescents with Cystic Fibrosis
- Comparison of continuous glucose monitoring to reference standard oral glucose tolerance test for the detection of dysglycemia in cystic Fibrosis: A systematic review
- Parent’s Report on Oral Health-Related Quality of Life of Children with Cystic Fibrosis
- Phage therapy for pulmonary infections: lessons from clinical experiences and key considerations
- Increased expression of glucagon-like peptide-1 and cystic fibrosis transmembrane conductance regulator in the ileum and colon in mouse treated with metformin
- In Silico Identification of Potential Insect Peptides against Biofilm-Producing Staphylococcus aureus
- In vivo correction of cystic fibrosis mediated by PNA nanoparticles
- Disulfiram Is Effective against Drug-Resistant Mycobacterium abscessus in a Zebrafish Embryo Infection Model
- False negatives in the newborn screening for Cystic Fibrosis in Western Andalusia: Results from a 10- year experience
- Young’s syndrome, a rare syndrome that can cause infertility and mimics cystic fibrosis and immotile-cilia syndrome: a case report
- Single-Cell RNA-Sequencing Reveals New Basic and Translational Insights in the Cystic Fibrosis Lung
- Pseudomonas aeruginosa utilises the host-derived polyamine spermidine to facilitate antimicrobial tolerance
- The Role of the Physical Therapist in Cystic Fibrosis Care
- Coexistence of Cystic Fibrosis with other genetic disorders: A case series
- Beta-adrenergic sweat test in children with inconclusive cystic fibrosis diagnosis: do we need new reference ranges?
- Reproductive genetic carrier screening for cystic fibrosis, fragile X syndrome and spinal muscular atrophy: patterns of community and healthcare provider participation in a Victorian screening program
- Evolution of Quorum Sensing in Pseudomonas aeruginosa Can Occur via Loss of Function and Regulon Modulation
- Impact of Patient-Specific Aminoglycoside Monitoring for Treatment of Pediatric Cystic Fibrosis Pulmonary Exacerbations
- Monitoring disease progression in childhood bronchiectasis
- Effects of a therapeutic exercise program in children with non-cystic fibrosis bronchiectasis: A pilot randomized controlled trial
- Serum leptin and neuropeptide Y in patients with cystic fibrosis-A single center study
- Evaluation of phages and liposomes as combination therapy to counteract Pseudomonas aeruginosa infection in wild-type and CFTR -null models
- ULTRASONOGRAPHIC PRESENTATION OF NODULAR CYSTIC FAT NECROSIS AFTER A LOW-VELOCITY TRAUMA: A CASE REPORT
- Genetic factors help explain the variable responses of young children with cystic fibrosis to vitamin D supplements
- The SLC26A9 inhibitor S9-A13 provides no evidence for a role of SLC26A9 in airway chloride secretion but suggests a contribution to regulation of ASL pH and gastric proton secretion
- Role of physical activity and airway clearance therapy in cystic fibrosis: moving forward in a rapidly changing landscape
- Web-based physical activity promotion in young people with CF: a randomised controlled trial
- Enabling Asymmetric Synthesis of ABBV-3748, a Corrector Compound for the Treatment of Cystic Fibrosis
- Evaluation of the Interstitial Histological Lesions in Pulmonary Langerhans Cell Histiocytosis
- Jaw and neck muscle activity during chewing and craniocervical dysfunction index in children, adolescents, and adults with and without cystic fibrosis: a comparative analysis
- Body mass index and additional risk factors for cancer in adults with cystic fibrosis
- Imipenem/Cilastatin/Relebactam Pharmacokinetics, Safety, and Outcomes in Adults and Adolescents With Cystic Fibrosis
- Closed-loop for People Living With Cystic Fibrosis Related Diabetes
- Distal Intestinal Obstruction Syndrome Relieved by Ileoscopy in a Patient With Cystic Fibrosis: A Case Report and Literature Review
- Intercellular communication and social behaviors in mycobacteria
- Mechanism of Xiaoqinglong Decoction and Qingqi Huatan Pills in improving pathological airway mucus based on nuclear factor-κB/microRNA-494 signaling regulation of mucin 5AC and cystic fibrosis transmembrane conductance regulator
- Targeting a Multidrug-Resistant Pathogen: First Generation Antagonists of Burkholderia cenocepacia ‘s BC2L-C Lectin
- Multicenter Surveillance of Cystic Fibrosis in Korean Children
- Take on transplant: human-centered design of a patient education tool to facilitate informed discussions about lung transplant among people with cystic fibrosis
- Personalized tobramycin dosing in children with cystic fibrosis: a comparative clinical evaluation of log-linear and Bayesian methods
- Targeted AntiBiotics for Chronic pulmonary diseases (TARGET ABC): can targeted antibiotic therapy improve the prognosis of Pseudomonas aeruginosa-infected patients with chronic pulmonary obstructive disease, non-cystic fibrosis bronchiectasis, and asthma? A multicenter, randomized, controlled, open-label trial
- Genomic epidemiology of Mycobacterium abscessus in a Canadian cystic fibrosis centre
- Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites
- Antimicrobial susceptibility and genotyping of microorganisms isolated from sputum culture of children with cystic fibrosis in an Iranian referral children’s hospital
- Impact of Antibiotics on the Lung Microbiome and Lung Function in Children With Cystic Fibrosis 1 Year After Hospitalization for an Initial Pulmonary Exacerbation
- Acute Infection with a Tobramycin-Induced Small Colony Variant of Staphylococcus aureus Causes Increased Inflammation in the Cystic Fibrosis Rat Lung
- Generation of two TMEM16A knockout iPSC clones each from a healthy human iPSC line, from a Cystic Fibrosis patient specific line with p.Phe508del mutation and from the gene corrected iPSC line
- Clinical outcomes of adults and children with cystic fibrosis during the COVID-19 pandemic
- Impact of Elexacaftor/Tezacaftor/Ivacaftor Therapy on the Cystic Fibrosis Airway Microbial Metagenome
- Intra- and Interspecies Spread of a Novel Conjugative Multidrug Resistance IncC Plasmid Coharboring bla OXA-181 and armA in a Cystic Fibrosis Patient
- Telemonitoring for Cystic fibrosis patients of Bambino Gesù Children’s Hospital during COVID-19
- Evaluation of nutritional status through bioimpedance analysis in a group of Cystic Fibrosis patients
- Coxsackievirus B infections are common in Cystic Fibrosis and experimental evidence supports protection by vaccination
- Innovative cystic fibrosis drug development: A perspective
- Tranexamic Acid Use for Massive Hemoptysis in a Child: A Case Report
- Allergic Bronchopulmonary Aspergillosis (ABPA) Diagnosis Missed in the Context of Asthma Exacerbation Due to Medication Nonadherence
- Lymph node regression grading of locally advanced rectal cancer treated with neoadjuvant chemoradiotherapy
- A pilot study investigating the effects of a manuka honey sinus rinse compared to a standard sinus rinse on sino-nasal outcome test scores in cystic fibrosis patients
- Editorial overview – 2022 respiratory issue: Cystic fibrosis pathophysiology, models, and novel therapies
- Protocol for establishing a core outcome set for evaluation in studies of pulmonary exacerbations in people with cystic fibrosis
- Cytokine patterns in cystic fibrosis patients with different microbial infections in oropharyngeal samples
- Author’s reply: “Serum bile acids in cystic fibrosis patients-Glycodeoxycholic acid as a potential marker of liver disease”
- Advent of elexacaftor/tezacaftor/ivacaftor for cystic fibrosis treatment: What consequences on Aspergillus-related diseases? Preliminary insights
- One-minute sit-to-stand test is practical to assess and follow the muscle weakness in cystic fibrosis
- Appetite stimulants for people with cystic fibrosis
- Multidrug-Resistant Bacteria in Children and Adolescents with Cystic Fibrosis
- Non-Cystic Fibrosis Bronchiectasis in Pediatric Age: A Case Series in a Metropolitan Area of Northern Italy
- Mucins and CFTR: Their Close Relationship
- Evaluation of the Complex p.[Leu467Phe;Phe508del] CFTR Allele in the Intestinal Organoids Model: Implications for Therapy
- In Vitro Rescue of the Bile Acid Transport Function of ABCB11 Variants by CFTR Potentiators
- The CFTR Amplifier Nesolicaftor Rescues TGF-β1 Inhibition of Modulator-Corrected F508del CFTR Function
- Modulator Therapy in Cystic Fibrosis Patients with cis Variants in F508del Complex Allele: A Short-Term Observational Case Series
- Trikafta-Extending Its Success to Less Common Mutations
- Malassezia Folliculitis following Triple Therapy for Cystic Fibrosis
- α-Tocopherol Pharmacokinetics in Adults with Cystic Fibrosis: Benefits of Supplemental Vitamin C Administration
- Effects of Ceragenins and Antimicrobial Peptides on the A549 Cell Line and an In Vitro Co-Culture Model of A549 Cells and Pseudomonas aeruginosa
- Self-Emulsifying Formulations to Increase the Oral Bioavailability of 4,6,4′-Trimethylangelicin as a Possible Treatment for Cystic Fibrosis
- Clinical associations with carriage of pulmonary Stenotrophomonas maltophilia
- Feasibility of unsedated lung MRI in young children with cystic fibrosis
- The natural history and genetic diversity of Haemophilus influenzae infecting the airways of adults with cystic fibrosis
- Standardizing Treatments for Pulmonary Exacerbations – Aminoglycoside Study
- Integrating specialist palliative care to improve care and reduce suffering: cystic fibrosis (InSPIRe:CF) – study protocol for a multicentre randomised clinical trial
- Evaluation of Growth Hormone Deficiency in Children with Cystic Fibrosis
- Real-world feasibility of short-term, unsupervised home spirometry in CF
- Significance of Molecular Identification of Genomic Variants of Pseudomonas aeruginosa in Children with Cystic Fibrosis in Mexico
- Lysosomal cathepsin D mediates endogenous mucin glycodomain catabolism in mammals
- COPD and the modulation of CFTR by acute exposure to cigarette smoke
- Withaferin A targets the membrane of Pseudomonas aeruginosa and mitigates the inflammation in zebrafish larvae; an in vitro and in vivo approach
- Mucus aberrant properties in CF: Insights from cells and animal models
- Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis
- Non-respiratory health-related quality of life in people with cystic fibrosis receiving elexacaftor/tezacaftor/ivacaftor
- Short-term effects of a novel bronchial drainage device: A pilot cohort study in subjects with cystic fibrosis
- Bone metabolism and incretin hormones following glucose ingestion in young adults with pancreatic insufficient cystic fibrosis
- Biphasic regulation of CFTR expression by ENaC in epithelial cells: The involvement of Ca2+ -modulated cAMP production
- Beyond borders: cystic fibrosis survival between Australia, Canada, France and New Zealand
- Efficacy and Mode of Action of a Direct Inhibitor of Mycobacterium abscessus InhA
- DNA Methyltransferase Regulates Nitric Oxide Homeostasis and Virulence in a Chronically Adapted Pseudomonas aeruginosa Strain
- Is Measuring Physical Literacy in School-Aged Children With Cystic Fibrosis or Congenital Heart Disease Needed?
- Italian Cystic Fibrosis Registry (ICFR). Report 2019-2020
- Association between phenotypic and genotypic characteristics and disease severity in individuals with cystic fibrosis
- Nutritional and metabolic management for cystic fibrosis in a post-cystic fibrosis transmembrane conductance modulator era
- Pseudomonas aeruginosa Strains from Both Clinical and Environmental Origins Readily Adopt a Stable Small-Colony-Variant Phenotype Resulting from Single Mutations in c-di-GMP Pathways
- Progress and challenges in fungal lung disease in cystic fibrosis
- Infection control in cystic fibrosis: evolving perspectives and challenges
- Is Cystic Fibrosis Contributing Significantly to Infant Mortality Rate in India?
- Preclinical evaluation of the ENaC inhibitor AZD5634 and implications on human translation
- Fibrosis quística: patogenia bacteriana y moduladores del CFTR (regulador de conductancia transmembranal de la fibrosis quística)
- Dental Caries and Associated Salivary Biomarkers in Patients with Cystic Fibrosis
- Low Diversity and Instability of the Sinus Microbiota over Time in Adults with Cystic Fibrosis
- Treatment of Pseudomonas aeruginosa infectious biofilms: Challenges and strategies
- Diet quality in cystic fibrosis – associations with patient reported outcome measures and enablers and barriers to eating a healthy diet: A protocol paper for a mixed methods study
- Trajectories of early growth and subsequent lung function in cystic fibrosis: An observational study using UK and Canadian registry data
- CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway
- Drug-induced acne with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis
- The feasibility of collecting the physiotherapy outcomes airway clearance, physical activity and fitness for the Australian Cystic Fibrosis Data Registry
- Mental health screening in Cystic Fibrosis as an intervention: Patient and caregiver feedback on improving these processes
- A Study to Evaluate the Relative Bioavailability of a Fixed-dose Combination Tablet of VX-121/Tezacaftor/Deutivacaftor
- Differential Performance and Lung Deposition of Levofloxacin with Different Nebulisers Used in Cystic Fibrosis
- KDM2A and KDM3B as Potential Targets for the Rescue of F508del-CFTR
- Ultrastructural Characterization of Human Bronchial Epithelial Cells during SARS-CoV-2 Infection: Morphological Comparison of Wild-Type and CFTR-Modified Cells
- The NHE3 Inhibitor Tenapanor Prevents Intestinal Obstructions in CFTR-Deleted Mice
- Exercise Capacity and Physical Activity in Non-Cystic Fibrosis Bronchiectasis after a Pulmonary Rehabilitation Home-Based Programme: A Randomised Controlled Trial
- Competitive fitness of Pseudomonas aeruginosa isolates in human and murine precision-cut lung slices
- Cystic fibrosis – A look at Belgium in 2022
- Mediation of the total effect of cystic fibrosis-related diabetes on mortality: A UK Cystic Fibrosis Registry cohort study
- Progress in Respiratory Gene Therapy
- Longitudinal Evolution of the Pseudomonas-Derived Cephalosporinase (PDC) Structure and Activity in a Cystic Fibrosis Patient Treated with β-Lactams
- A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase
- Xylitol vs Saline Nasal Irrigations in CF-CRS
- Tromethamine improves mucociliary clearance in cystic fibrosis pigs
- New Recommendations of a Height-Based Dosing Regimen of Tobramycin for Cystic Fibrosis in Adults: A Population Pharmacokinetic Analysis
- In vitro synergy between manuka honey and amikacin against Mycobacterium abscessus complex shows potential for nebulisation therapy
- Airway Infection in Cystic Fibrosis: Microbiology and Management
- Adaptation and Evolution of Pathogens in the Cystic Fibrosis Lung
- Antimicrobial Stewardship in Cystic Fibrosis
- The Elusive Role of Airway Infection in Cystic Fibrosis Exacerbation
- Drugs, Drugs, Drugs: Current Treatment Paradigms in Cystic Fibrosis Airway Infections
- The Sense and Nonsense of Antimicrobial Susceptibility Testing in Cystic Fibrosis
- Exploring the Cystic Fibrosis Lung Microbiome: Making the Most of a Sticky Situation
- Opportunistic Pathogens in Cystic Fibrosis: Epidemiology and Pathogenesis of Lung Infection
- Community Perspective on Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis
- Health-related quality of life in a prospective study of ultrasound to detect cystic fibrosis-related liver disease in children
- Using the genome to correct the ion transport defect in cystic fibrosis
- A Study to Assess the Pharmacokinetics and Safety of Co-administered Oral Galicaftor, Navocaftor, and ABBV-576 in Healthy Adults for the Treatment of Cystic Fibrosis
- Generation of two induced pluripotent stem cell lines (RCMGi005-A/B) from human skin fibroblasts of a cystic fibrosis patient with homozygous F508del mutation in CFTR gene
- The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus
- Glycemic indices at night measured by CGM are predictive for a lower pulmonary function in adults but not in children with cystic fibrosis
- Clinimetric analysis of outcome measures for airway clearance in people with cystic fibrosis: a systematic review
- KCa3.1 potentiation stimulates Cl- secretion in F508del and G551D CFTR corrected primary human bronchial epithelial cells
- The circadian system in cystic fibrosis mice is regulated by histone deacetylase 6
- Development of algorithm for diagnosis of Cystic Fibrosis in absence of sweat chloride testing in resource limited setting
- Pulmonary exacerbations, airway pathogens, and long-term course of lung clearance index in children and young adults with Cystic Fibrosis
- The role of mechanosensitive ion channels in the gastrointestinal tract
- Niche, not phylogeny, governs the response to oxygen availability among diverse Pseudomonas aeruginosa strains
- Experiments with LDA and Top2Vec for embedded topic discovery on social media data-A case study of cystic fibrosis
- Clinical outcomes of digital health in adults with cystic fibrosis
- Research advances on the regulatory mechanism of sweat secretion ion channels of eccrine sweat glands
- Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
- Faecal calprotectin and rectal histological inflammatory markers in cystic fibrosis: a single-centre study
- Real-world Efficacy and Safety of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulator Therapy in Adult Patients With Cystic Fibrosis (CF)
- The association between Sodium Urinary Discharge (FENa) and growth parameters in pediatrics with cystic fibrosis
- In response to “who are the 10%? – Non eligibility of cystic fibrosis (CF) patients for highly effective modulator therapies”
- Intracellular replication of Inquilinus limosus in bronchial epithelial cells
- Analysis of cystic fibrosis patient survival confirms STAT3 as a CF modifying gene with changing impact over time
- Combining Gallium Protoporphyrin and Gallium Nitrate Enhances In Vitro and In Vivo Efficacy against Pseudomonas aeruginosa : Role of Inhibition of Bacterial Antioxidant Enzymes and Resultant Increase in Cytotoxic Reactive Oxygen Species
- Prevalence, trends and outcomes of long-term inhaled antibiotic treatment in people with cystic fibrosis without chronic Pseudomonas aeruginosa infection – A European cystic fibrosis patient registry data analysis
- Keratin 8 is a scaffolding and regulatory protein of ERAD complexes
- Detecting Ion Levels, Changes to Monitor Health
- Systemic Corticosteroids in the Management of Pediatric Cystic Fibrosis Pulmonary Exacerbations
- Exercise performance in children and adolescents with cystic fibrosis with and without abnormal glucose tolerance: a single center cross-sectional study
- High expression of CFTR in cumulus cells from mature oocytes is associated with high-quality of oocyte and subsequent embryonic development
- Case report of a Hispanic female with cystic fibrosis and short stature
- Evaluation of an association between RANKL and OPG with bone disease in people with cystic fibrosis
- SOCIAL DETERMINANTS OF HEALTH SCREENING AND INTERVENTION: A CYSTIC FIBROSIS QUALITY IMPROVEMENT PROCESS
- Polyguluronate simulations shed light onto the therapeutic action of OligoG CF-5/20
- Clinical phenotypes and genotypic spectrum of cystic fibrosis with pancreatic insufficiency in children
- Phase 1 Study to Assess the Safety and Pharmacokinetics of Elexacaftor/Tezacaftor/Ivacaftor in Subjects Without Cystic Fibrosis With Moderate Hepatic Impairment
- Long-term azithromycin in children with bronchiectasis unrelated to cystic fibrosis: treatment effects over time
- Self-reported dental attendance, oral hygiene habits, and dietary habits of adults with cystic fibrosis
- Insulinogenic index and early phase insulin secretion predict increased risk of worsening glucose tolerance and of cystic fibrosis-related diabetes
- Availability of CFTR modulators in countries of Eastern Europe: The reality in 2022
- Comment on: “Serum bile acids in cystic fibrosis patients – glycodeoxycholic acid as a potential marker of liver disease”
- Physical activity assessment and vascular function in adults with cystic fibrosis and their non-CF peers
- Immunogenicity of BNT162b2 mRNA-Based Vaccine against SARS-CoV-2 in People with Cystic Fibrosis According to Disease Characteristics and Maintenance Therapies
- Effects of Levofloxacin, Aztreonam, and Colistin on Enzyme Synthesis by P. aeruginosa Isolated from Cystic Fibrosis Patients
- Antibiotherapy in Children with Cystic Fibrosis-An Extensive Review
- Differential CFTR-Interactome Proximity Labeling Procedures Identify Enrichment in Multiple SLC Transporters
- Conformational Variability in Ground-State CFTR Lipoprotein Particle Cryo-EM Ensembles
- Achromobacter spp. Adaptation in Cystic Fibrosis Infection and Candidate Biomarkers of Antimicrobial Resistance
- Combined Treatment of Bronchial Epithelial Calu-3 Cells with Peptide Nucleic Acids Targeting miR-145-5p and miR-101-3p: Synergistic Enhancement of the Expression of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene
- Can Leukotriene Receptor Antagonist Therapy Improve the Control of Patients with Severe Asthma on Biological Therapy and Coexisting Bronchiectasis? A Pilot Study
- Advances in Preclinical In Vitro Models for the Translation of Precision Medicine for Cystic Fibrosis
- Cystic Fibrosis and Oxidative Stress: The Role of CFTR
- Usefulness of Muscle Ultrasonography in the Nutritional Assessment of Adult Patients with Cystic Fibrosis
- Ruthenium(II) Polypyridyl Complexes for Antimicrobial Photodynamic Therapy: Prospects for Application in Cystic Fibrosis Lung Airways
- Therapeutic Drug Monitoring of Ivacaftor, Lumacaftor, Tezacaftor, and Elexacaftor in Cystic Fibrosis: Where Are We Now?
- Implementation of Model-Based Dose Adjustment of Tobramycin in Adult Patients with Cystic Fibrosis
- BEGIN Novel ImagiNG Biomarkers
- Response to inhaled ceftazidime in patients with non-cystic fibrosis bronchiectasis and chronic bronchial infection unrelated to Pseudomonas aeruginosa
- A missense mutation in the proprotein convertase gene furinb causes hepatic cystogenesis during liver development in zebrafish
- Cystic fibrosis transmembrane conductance regulator modulators and the exocrine pancreas: A scoping review
- Whole-genome analysis of Haemophilus influenzae strains isolated from persons with cystic fibrosis
- Lower airway microbiota and decreasing lung function in young Brazilian cystic fibrosis patients with pulmonary Staphylococcus and Pseudomonas infection
- Allergic Bronchopulmonary Aspergillosis in Children Presenting as Lung Masses
- Functional Evaluation and Genetic Landscape of Children and Young Adults Referred for Assessment of Bronchiectasis
- Risk factors for severe COVID-19 in people with cystic fibrosis: A systematic review
- Outcome of lung transplantation in cystic fibrosis patients with severe asymmetric chest cavities
- Commentary: Creative solutions for lung transplantation with chest wall asymmetry and cystic fibrosis
- Neuromodulation for Children With Cystic Fibrosis Experiencing Chronic Abdominal Pain
- T helper 2 Mechanisms involved in Human Rhinovirus Infections and Asthma
- Historical note: Horace Dobell’s report of cystic fibrosis in 1872
- The effect of a training webinar on decreasing inter-observer variability in making a radiologic diagnosis of bronchiectasis
- Supplemental Oxygen Alters the Airway Microbiome in Cystic Fibrosis
- Impact of pharmacy services on time to elexacaftor-tezacaftor-ivacaftor initiation
- The Value of Sputum Polymerase Chain Reaction for Detection of Nontuberculous Mycobacteria in Cystic Fibrosis Patients with Negative Nontuberculous Mycobacteria Sputum Culture
- A successful treatment of a lobar atelectasis in a patient with Cystic Fibrosis
- The Utility of Glucose Area Under the Curve from The Oral Glucose Tolerance Test as a Screening Tool for Cystic Fibrosis -Related Diabetes
- The respiratory microbiota alpha-diversity in chronic lung diseases: first systematic review and meta-analysis
- Update on the management of vitamins and minerals in cystic fibrosis
- Impact of Pancreatitis-Associated Protein on Newborn Screening Outcomes and Detection of CFTR-Related Metabolic Syndrome (CRMS)/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): A Monocentric Prospective Pilot Experience
- PHLPP1 regulates CFTR activity and lumen expansion through AMPK
- Profiling the response to Lumacaftor-Ivacaftor in children with Cystic Fibrosis New insight from a French-Italian real-life cohort
- Pulmonary medication adherence among children and adults with Cystic Fibrosis: Is there an association with disease severity?
- Impact of antibiotic eradication therapy of Pseudomonas aeruginosa on long term lung function in cystic fibrosis
- Carbon nanoparticles adversely affect CFTR expression and toxicologically relevant pathways
- An optimized protocol for assessment of sputum macrorheology in health and muco-obstructive lung disease
- Oligonucleotide-based therapies for cystic fibrosis
- Three Complex alleles associated with N1303K mutation and their molecular consequences
- Nitric oxide inhibits alginate biosynthesis in Pseudomonas aeruginosa and increases its sensitivity to tobramycin by downregulating algU gene expression
- Human pluripotent stem cell-derived macrophages host Mycobacterium abscessus infection
- Long-term follow-up and liver outcomes in children with cystic fibrosis and nodular liver on ultrasound in a multi-center study
- The disease-specific clinical trial network for primary ciliary dyskinesia: PCD-CTN
- Pharmacokinetic/Pharmacodynamic Evaluation of the Dipeptidyl Peptidase 1 Inhibitor Brensocatib for Non-cystic Fibrosis Bronchiectasis
- A Study Assessing KB407 for the Treatment of Cystic Fibrosis
- Prevalence, clinical features, and outcomes of COVID-19 in persons with cystic fibrosis: a systematic review protocol
- Cystic Fibrosis Modulator Therapies
- β-sitosterol ameliorates inflammation and Pseudomonas aeruginosa lung infection in a mouse model
- Prevalence of unmet pain and symptom management needs in adults with cystic fibrosis
- Antibiotics Drive Expansion of Rare Pathogens in a Chronic Infection Microbiome Model
- Bacterial Culture Underestimates Lung Pathogen Detection and Infection Status in Cystic Fibrosis
- CFTR Modulators: From Mechanism to Targeted Therapeutics
- Time to change course and tackle CF related obesity
- Frailty predicts outcomes in cystic fibrosis patients listed for lung transplantation
- Partner perspectives on women’s sexual and reproductive healthcare in cystic fibrosis
- Microrheology of Mucin-Albumin Assembly Using Diffusing Wave Spectroscopy
- Effects of Mucin and DNA Concentrations in Airway Mucus on Pseudomonas aeruginosa Biofilm Recalcitrance
- Identification of differentially expressed genes and their major pathways among the patient with COVID-19, cystic fibrosis, and chronic kidney disease
- Reduced Expression of TMEM16A Impairs Nitric Oxide-Dependent Cl- Transport in Retinal Amacrine Cells
- Cystic fibrosis transmembrane regulator correction attenuates heart failure-induced lung inflammation
- Multidrug-resistant Pseudomonas aeruginosa is predisposed to lasR mutation through up-regulated activity of efflux pumps in non-cystic fibrosis bronchiectasis patients
- Laboratory biomarkers in the diagnosis and follow-up of treatment of allergic bronchopulmonary aspergillosis in cystic fibrosis
- Structural characterization of the novel stress response facilitator (SrfA) from Pseudomonas aeruginosa
- Predicting risk-adjusted incidence rates of methicillin-resistant Staphylococcus Aureus and Pseudomonas Aeruginosa in cystic fibrosis programs in the United States
- Elexacaftor-Tezacaftor-Ivacaftor Overdose in an Adolescent Female with Cystic Fibrosis
- Association between early chronic methicillin-susceptible Staphylococcus aureus colonization and lung function in children with cystic fibrosis
- G378X-I148T CFTR variant: A new complex allele in a cystic fibrosis newborn with pancreatic insufficiency
- Super resolution microscopy analysis reveals increased Orai1 activity in asthma and cystic fibrosis lungs
- Sweat Proteomics in Cystic Fibrosis: Discovering Companion Biomarkers for Precision Medicine and Therapeutic Development
- Pseudomonas Aeruginosa Lung Infection Subverts Lymphocytic Responses through IL-23 and IL-22 Post-Transcriptional Regulation
- Probiotics Administration in Cystic Fibrosis: What Is the Evidence?
- Clinical Features and Treatment Outcomes of Pulmonary Mycobacterium avium-intracellulare Complex With and Without Coinfections
- Personalized medicine with drugs targeting the underlying protein defect in cystic fibrosis: is monitoring of treatment response necessary?
- The Microbiology of Non-aeruginosa Pseudomonas Isolated From Adults With Cystic Fibrosis: Criteria to Help Determine the Clinical Significance of Non-aeruginosa Pseudomonas in CF Lung Pathology
- Silver-manganese nanocomposite modified screen-printed carbon electrode in the fabrication of an electrochemical, disposable biosensor strip for cystic fibrosis
- A Giant Sporadic Intra-abdominal Desmoid Tumor in a Male Patient: A Case Report
- Cystic fibrosis and jejunoileal atresia: A clinical case
- Cystic fibrosis transmembrane conductance regulator (CFTR): a missing link between smoking and chronic airway diseases?
- Evidence for Early CFTR Modulator Treatment for Children with Cystic Fibrosis Keeps Growing
- Antimicrobial peptides for tackling cystic fibrosis related bacterial infections: A review
- Antibiotics and outcomes of CF pulmonary exacerbations in children infected with MRSA and Pseudomonas aeruginosa
- Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
- Physical activity and exercise training in cystic fibrosis
- Molecular Epidemiology of Mycobacterium abscessus Isolates Recovered from German Cystic Fibrosis Patients
- Congenital etiologies of exocrine pancreatic insufficiency
- Disease severity of people with cystic fibrosis carrying residual function mutations: Data from the ECFS Patient Registry
- Chronic rhinosinusitis in people with Cystic Fibrosis: Expanding evidence and future directions
- CFTR gene variants, air pollution, and childhood asthma in a California Medicaid population
- Cystic fibrosis exacerbations treated with medical therapy with and without concurrent sinus surgery
- Achromobacter spp. prevalence and adaptation in cystic fibrosis lung infection
- Eating Disorders in Adolescents and Young Adults with Cystic Fibrosis
- FEASIBILITY AND ACCEPTABILITY OF A CF-SPECIFIC COGNITIVE-BEHAVIORAL PREVENTIVE INTERVENTION FOR ADULTS INTEGRATED INTO TEAM-BASED CARE
- Spectrum of Cystic Fibrosis Conductance Regulator Gene Mutations Reported in Pakistani Descent Cystic Fibrosis Patients
- Current knowledge in the use of bacteriophages to combat infections caused by Pseudomonas aeruginosa in cystic fibrosis
- Considering the impact of patient ethnicity on cystic fibrosis related bone disease
- Novel Macrocyclic 1,3,4-Oxadiazoles as CFTR Modulators for Treating Cystic Fibrosis
- A systematic review of lung clearance index in non-cystic fibrosis, non-primary ciliary dyskinesia bronchiectasis
- A step forward for an intermediate cystic fibrosis population
- Improving the management of chronic Pseudomonas aeruginosa infection in lower respiratory tract
- Stress and mental health in adolescents and young adults with cystic fibrosis one year after the COVID pandemic: Findings from an Italian sample
- Plasma and cellular ivacaftor concentrations in patients with CF
- Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index
- Modelling Successful Self-Management in Adults With Cystic Fibrosis: Vicarious Self-Efficacy From Videos of ‘People Like Me’
- Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia
- Heterogeneous spectrum of CFTR gene mutations in Chinese patients with CAVD and the dilemma of genetic blocking strategy
- Surgical considerations in cystic fibrosis: what every general surgeon needs to know
- A novel free-breathing abdominal RAVE T2 /T1 hybrid MRI sequence in patients with cystic fibrosis: Preliminary results
- Global prevalence of non-tuberculous mycobacteria in adults with non-cystic fibrosis bronchiectasis 2006-2021: a systematic review and meta-analysis
- Impact of highly effective CFTR modulator therapy on digital clubbing in patients with cystic fibrosis
- Developing an ecological approach to physical activity promotion in adults with Cystic fibrosis
- Inhaled antibiotics for pulmonary exacerbations in cystic fibrosis
- Real-world Associations of US Cystic Fibrosis Newborn Screening Programs With Nutritional and Pulmonary Outcomes
- Pilot Trial of ExACT (Exercise as Airway Clearance Therapy) for People With Cystic Fibrosis
- Educational achievements of children aged 10-11 years with cystic fibrosis. A data linkage study in Wales
- Effects of the Sex Steroid Hormone Estradiol on Biofilm Growth of Cystic Fibrosis Pseudomonas aeruginosa Isolates
- A retrospective correlative profiling of lung functions, microbiological, radiological, periodontal, hematological parameters in noncystic fibrosis bronchiectasis patients of North India
- Corrigendum to “Using a learning health system to understand the mismatch between medicines supply and actual medicines use among adults with cystic fibrosis” [J Cyst Fibros (2022), 21/2, 323-331]
- The omicron wave modifies the COVID-19 paradigm in children with cystic fibrosis
- Continuous glucose monitoring versus self-monitoring of blood glucose in the management of cystic fibrosis related diabetes: A systematic review and meta-analysis
- A multimodal iPSC platform for cystic fibrosis drug testing
- Non-cell-autonomous activation of Hedgehog signaling contributes to disease progression in a mouse model of renal cystic ciliopathy
- Therapeutic applications of curcumin nanomedicine formulations in cystic fibrosis
- Editorial: Mechanisms of Novel Drugs and Gene Modifiers in the Treatment of Cystic Fibrosis
- Insulin-Like Growth Factor Binding Protein (IGFBP-6) as a Novel Regulator of Inflammatory Response in Cystic Fibrosis Airway Cells
- Oxidative stress and impaired insulin secretion in cystic fibrosis pig pancreas
- Trends in Growth and Maturation in Children with Cystic Fibrosis Throughout Nine Decades
- Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study
- Gut Bifidobacteria enrichment following oral Lactobacillus-supplementation is associated with clinical improvements in children with cystic fibrosis
- Small molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough
- Depression, anxiety, substance misuse and self-harm in children and young people with rare chronic liver disease
- Is there a role of viral infection in cystic fibrosis exacerbation in children?
- A female with excessive wrinkling of the hands
- Monoallelic pathogenic ALG5 variants cause atypical polycystic kidney disease and interstitial fibrosis
- Biologic drugs in treating allergic bronchopulmonary aspergillosis in patients with cystic fibrosis: a systematic review
- Cystic fibrosis associated with Wernicke’s encephalopathy in an older adult
- Clinical and gene mutation features of cystic fibrosis: an analysis of 8 cases
- Variation in lung function and clinical aspects in adults with cystic fibrosis
- Characterization of the cluster MabR prophages of Mycobacterium abscessus and Mycobacterium chelonae
- The Use of Thiocyanate Formulations to Create Manganese Porphyrin Antioxidants That Supplement Innate Immunity
- IL-17 Cytokines and Chronic Lung Diseases
- Going the Extra Mile: Why Clinical Research in Cystic Fibrosis Must Include Children
- Nontuberculous Mycobacterial Lung Disease in the Patients with Cystic Fibrosis-A Challenging Diagnostic Problem
- Health-Related Quality of Life in Adults with Cystic Fibrosis: Familial, Occupational, Social, and Mental Health Predictors
- A Cross-Sectional Study of the Marital Attitudes of Pregnant Women at Risk for Cystic Fibrosis and Psychological Impact of Prenatal Screening
- A Splice Switch in SIGIRR Causes a Defect of IL-37-Dependent Anti-Inflammatory Activity in Cystic Fibrosis Airway Epithelial Cells
- Emerging Concepts in Defective Macrophage Phagocytosis in Cystic Fibrosis
- Macrophage Lysosomal Alkalinization Drives Invasive Aspergillosis in a Mouse Cystic Fibrosis Model of Airway Transplantation
- Identification of Pathogenic Variant Burden and Selection of Optimal Diagnostic Method Is a Way to Improve Carrier Screening for Autosomal Recessive Diseases
- Serum Fibrinogen as a Biomarker for Disease Severity and Exacerbation in Patients with Non-Cystic Fibrosis Bronchiectasis
- Auxological and Endocrinological Features in Children and Adolescents with Cystic Fibrosis
- Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis
- Loss of Serum Glucocorticoid-Inducible Kinase 1 SGK1 Worsens Malabsorption and Diarrhea in Microvillus Inclusion Disease (MVID)
- The Use of Artificial Sputum Media to Enhance Investigation and Subsequent Treatment of Cystic Fibrosis Bacterial Infections
- Activity of Drug Combinations against Mycobacterium abscessus Grown in Aerobic and Hypoxic Conditions
- Mycobacterium abscessus : It’s Complex
- Newborn Screening for Cystic Fibrosis: A Qualitative Study of Successes and Challenges from Universal Screening in the United States
- Pseudomonas aeruginosa : Recent Advances in Vaccine Development
- Absence of EPAC1 Signaling to Stabilize CFTR in Intestinal Organoids
- Novel Treatment Approach for Aspergilloses by Targeting Germination
- Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease
- Nebulizer Cleaning Practices and Adherence to Nebulized Therapies in People With Cystic Fibrosis When Traveling
- Abdominal ultrasound findings in adult patients with cystic fibrosis
- Pediatric Medical Traumatic Stress in Inflammatory Bowel Disease, Pancreatitis and Cystic Fibrosis
- Aquagenic keratoderma
- Telehealth and CFTR modulators: Accelerating innovative models of cystic fibrosis care
- Reproducibility of pulmonary magnetic resonance angiography in adults with muco-obstructive pulmonary disease
- Clinician attitudes and practices on pregnancy planning and care in cystic fibrosis
- Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study
- An invisible threat? Aspergillus positive cultures and co-infecting bacteria in airway samples
- Disparities in first evaluation of infants with cystic fibrosis since implementation of newborn screening
- Unveiling Co-Infection in Cystic Fibrosis Airways: Transcriptomic Analysis of Pseudomonas aeruginosa and Staphylococcus aureus Dual-Species Biofilms
- The Role of Pulmonary Function Test in Perioperative Management of Patients with Cystic Fibrosis
- Resistance Training Recommendations for Children and Adolescents With Cystic Fibrosis-Related Diabetes
- The Role of PaFicT in Pseudomonas aeruginosa Persister Cell Formation
- Non-invasive diagnostics of pathogenic bacteria using a breath sampler in children with cystic fibrosis
- Treatment preference amongst people with cystic fibrosis: the importance of reducing treatment burden
- Different management approaches and outcome for infants with an inconclusive diagnosis following newborn screening for cystic fibrosis (CRMS/CFSPID) and Pseudomonas aeruginosa isolation
- Staphylococcus aureus and Pseudomonas aeruginosa Isolates from the Same Cystic Fibrosis Respiratory Sample Coexist in Coculture
- Perspectives on Genetic Medicine for Cystic Fibrosis
- PlayPhysio: Making Physio Fun
- Survival difference between high-risk and low-risk CFTR genotypes after lung transplant
- Surface Motility Favors Codependent Interaction between Pseudomonas aeruginosa and Burkholderia cenocepacia
- Specificity of Immunoglobulin Response to Nontuberculous Mycobacteria Infection in People with Cystic Fibrosis
- Exploring the role of antibiotics and steroids in managing respiratory diseases
- Evaluating Differences in the Disease Experiences of Minority Adults With Cystic Fibrosis
- Genetic spectrum of Chinese children with cystic fibrosis: comprehensive data analysis from the main referral centre in China
- Resistance evolution can disrupt antibiotic exposure protection through competitive exclusion of the protective species
- Secondary Metabolites Produced during Aspergillus fumigatus and Pseudomonas aeruginosa Biofilm Formation
- The In Vitro Replication Cycle of Achromobacter xylosoxidans and Identification of Virulence Genes Associated with Cytotoxicity in Macrophages
- A New Era for Cystic Fibrosis and CFTR Modulator Trials in Infants
- Extreme electrolyte depletion associated with septic shock as a form of presentation of cystic fibrosis
- Content validation for the Chilean population of the quality of life assessment instrument in children, adolescents and adults with Cystic Fibrosis: CFQ-R CYSTIC FIBROSIS QUESTIONNAIRE-REVISED version in Spanish, Chile
- CFTR genotype analysis of Asians in international registries highlights disparities in the diagnosis and treatment of Asian patients with cystic fibrosis
- Reducing human DNA bias in cystic fibrosis airway specimens for microbiome analysis
- Azithromycin and cystic fibrosis
- The potential of volatile organic compound analysis for pathogen detection and disease monitoring in patients with cystic fibrosis
- The changing epidemiology of cystic fibrosis: the implications for adult care
- Elexacaftor-Tezacaftor-Ivacaftor improves exercise capacity in adolescents with cystic fibrosis
- Drugs in Clinical Development to Treat Autosomal Dominant Polycystic Kidney Disease
- Potential opportunities and challenges for infection prevention and control for cystic fibrosis in the modern era
- WNK Inhibition Increases Surface Liquid pH and Host Defense in Cystic Fibrosis Airway Epithelia
- If At First You Don’t Succeed, Trikafta Again
- Effectiveness and Safety of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Children With Cystic Fibrosis: A Meta-Analysis
- Rechallenge of Elexacaftor/Tezacaftor/Ivacaftor After Skin Rash in Two Pediatric Patients
- ACCESS 2: AI for pediatriC diabetiC Eye examS Study 2
- Generation of induced pluripotent stem cell line (RCMGi008-A) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/CFTRdele2.3 mutations in CFTR gene
- Development and outcomes of a patient driven cystic fibrosis quality of care improvement project
- Chronic rhinosinusitis is associated with non-cystic fibrosis bronchiectasis – a new clinical implication?
- A case of Elexacaftor-Tezacaftor-Ivacaftor induced rash resolving without interruption of treatment
- Longitudinal assessment of renal function after lung transplantation for cystic fibrosis: transition from post-operative acute kidney injury to acute kidney disease and chronic kidney failure
- Pharmacokinetics of Intermittent Dosed Intravenous Vancomycin in Adult Persons with Cystic Fibrosis
- Membrane lipid renovation in Pseudomonas aeruginosa – implications for phage therapy?
- The Exocrine Pancreas in Cystic Fibrosis in the Era of CFTR Modulation: A Mini Review
- Psychological status of mothers of children with cystic fibrosis and primary ciliary dyskinesia
- Epithelial damage in the cystic fibrosis lung: the role of host and microbial factors
- Antibiotic allergy in children with cystic fibrosis: a retrospective case-control study
- Limitations of the dichotomized 6-minute walk distance when computing lung allocation score for cystic fibrosis: a 16-year retrospective cohort study
- ScRNA-seq expression of IFI27 and APOC2 identifies four alveolar macrophage superclusters in healthy BALF
- Erratum: “Volatile organic compound breath signatures of children with cystic fibrosis by real-time SESI-HRMS”. Ronja Weber, Naemi Haas, Astghik Baghdasaryan, Tobias Bruderer, Demet Inci, Srdjan Micic, Nathan Perkins, Renate Spinas, Renato Zenobi and Alexander Moeller for the Paediatric Exhalomics Group at the University Children’s Hospital Zurich. ERJ Open Res 2020; 6: 00171-2019
- Pregnancy experience in the setting of cystic fibrosis: A systematic review and thematic synthesis
- Effects of Hypoxia on Respiratory Diseases: Perspective View of Epithelial Ion Transport
- A Phase 1b/2 Trial of the Safety and Microbiological Activity of Bacteriophage Therapy in Cystic Fibrosis Subjects Colonized With Pseudomonas Aeruginosa
- Improving nutrition in cystic fibrosis: A systematic literature review
- Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3B, Randomized, Placebo-Controlled Study
- The Cystic Fibrosis Action Plan: a low health literacy, pictographic self-management tool with clinical automation
- Time Free From Hospitalization in Children and Adolescents With Cystic Fibrosis: Findings From FEV1 , Lung Clearance Index and Peak Work Rate
- Pharmacological Responses of the G542X-CFTR to CFTR Modulators
- Management of Cepacia Syndrome in an Immunocompetent Non-Cystic Fibrosis Adult Patient
- The pharmacology of the TMEM16A channel: therapeutic opportunities
- The role of viral infections in pulmonary exacerbations of patients with non-cystic fibrosis bronchiectasis: A systematic review
- Feasibility of the A-STEP for the assessment of exercise capacity in people with cystic fibrosis
- Development of excipients free inhalable co-spray-dried tobramycin and diclofenac formulations for cystic fibrosis using two and three fluid nozzles
- Daily versus three-times-weekly azithromycin in Chinese patients with non-cystic fibrosis bronchiectasis: protocol for a prospective, open-label and randomised controlled trial
- Multi-omic comparisons between CFBE41o- cells stably expressing wild-type CFTR and F508del-mutant CFTR
- Cystic fibrosis related diabetes is not associated with maximal aerobic exercise capacity in cystic fibrosis: a cross-sectional analysis of an international multicenter trial
- Effects of a Long-Term Monitored Exercise Program on Aerobic Fitness in a Small Group of Children with Cystic Fibrosis
- Experiences and Perspectives of Individuals with Cystic Fibrosis and Their Families Related to Food Insecurity
- Main Metabolites of Pseudomonas aeruginosa : A Study of Electrochemical Properties
- Effects of a Long-Term Wearable Activity Tracker-Based Exercise Intervention on Cardiac Morphology and Function of Patients with Cystic Fibrosis
- Mucosal Immunity in Cystic Fibrosis
- Effects of GLP-1 and GIP on Islet Function in Glucose Intolerant, Pancreatic Insufficient Cystic Fibrosis
- Gold nanocluster adjuvant enables the eradication of persister cells by antibiotics and abolishes the emergence of resistance
- 2D Shear Wave Elastography, a promising screening tool for Cystic Fibrosis liver disease, shows a correlation between vitamin D and liver stiffness
- Correlation among clinical, functional and morphological indexes of the respiratory system in non-cystic fibrosis bronchiectasis patients
- The changing landscape of the cystic fibrosis lung environment: From the perspective of Pseudomonas aeruginosa
- Bilateral Pseudomonas aeruginosa keratitis as presenting feature in an infant of cystic fibrosis
- Adverse Childhood Experiences (ACEs) in Adults with Cystic Fibrosis
- Persistent Aspergillus fumigatus infection in cystic fibrosis: impact on lung function and role of treatment of asymptomatic colonization-a registry-based case-control study
- Ivacaftor, not ivacaftor/lumacaftor, associated with lower pulmonary inflammation in preschool cystic fibrosis
- Microsporidial myositis, keratitis and hypercalcaemia in a cystic fibrosis lung transplant recipient
- Generation of Airway Epithelial Cell Air-Liquid Interface Cultures from Human Pluripotent Stem Cells
- Real Life With Tezacaftor and Ivacaftor in Adult Patients With Cystic Fibrosis: Spanish Multicenter Study
- Characterization of the Achromobacter xylosoxidans Type VI Secretion System and Its Implication in Cystic Fibrosis
- Heart rate variability, exercise capacity and levels of daily physical activity in children and adolescents with mild-to-moderate cystic fibrosis
- CF Patients’ Airway Epithelium and Sex Contribute to Biosynthesis Defects of Pro-Resolving Lipids
- Allicin Facilitates Airway Surface Liquid Hydration by Activation of CFTR
- Peer i-Coaching for Activated Self-Management Optimization in Adolescents and Young Adults With Chronic Conditions
- A Study Evaluating the Long-term Safety and Efficacy of VX-121 Combination Therapy
- Future therapies for cystic fibrosis
- Pediatric Pulmonary Year in Review 2021: Sleep Medicine
- An Update on Ductal Plate Malformations and Fibropolycystic Diseases of the Liver
- Reclassifying inconclusive diagnosis for Cystic Fibrosis with new generation sweat test
- The U-shaped association of post-lung transplant mortality with pretransplant eGFR underscores possible limitations of creatinine-based estimation equations for risk stratification
- MicroRNA global profiling in cystic fibrosis cell lines reveals dysregulated pathways related with inflammation, cancer, growth, glucose and lipid metabolism, and fertility: an exploratory study
- The Role of Hypoxia in Improving the Therapeutic Potential of Mesenchymal Stromal Cells. A Comparative Study From Healthy Lung and Congenital Pulmonary Airway Malformations in Infants
- Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: Early report
- Prevalence and characteristics of cystic fibrosis liver disease: a study highlighting the lack of histological diagnosis
- Targeting fibrosis, mechanisms and cilinical trials
- Exposure to bile and gastric juice can impact the aerodigestive microbiome in people with cystic fibrosis
- Behavioral Health Screening in Military Cystic Fibrosis Centers: A Survey
- A Phase 3, Open-Label Study of Lumacaftor/Ivacaftor in Children 1 to Less Than 2 Years of Age With Cystic Fibrosis Homozygous for F508del-CFTR
- Pharmacokinetics and Safety Assessment of VX-121/Tezacaftor/Deutivacaftor in Participants With Moderate Hepatic Impairment
- Modulation, microbiota and inflammation in the adult CF gut: A prospective study
- A Middle East systematic review and meta-analysis of prevalence and antibiotic susceptibility pattern in MRSA Staphylococcus aureus isolated from patients with cystic fibrosis
- Stem Cells, Cell Therapies, and Bioengineering in Lung Biology and Disease 2021
- In Vitro Comparison of Aztreonam/Amoxicillin-Clavulanate Versus Aztreonam/Ceftazidime-Avibactam on Ceftazidime-Avibactam Resistant Stenotrophomonas maltophilia
- Expression of active B-Raf proto-oncogene in kidney collecting ducts induces cyst formation in normal mice and accelerates cyst growth in mice with polycystic kidney disease
- ELX/TEZ/IVA use in cystic fibrosis liver disease: Is the perspective of improved lung function worth the risk?
- Pancreatic, hepatobiliary, and gastrointestinal manifestations of children with cystic fibrosis: A 10-year experience from a tertiary care center in southern India
- Collaboration Between Two CF Centers; one in USA and one in Turkey before and during CoV2 Pandemic
- Antimicrobial Peptides against Multidrug-Resistant Pseudomonas aeruginosa Biofilm from Cystic Fibrosis Patients
- Crosstalk between lysine methyltransferase Smyd2 and TGF-β-Smad3 signaling promotes renal fibrosis in autosomal dominant polycystic kidney disease
- An Update on CFTR Drug Discovery: Opportunities and Challenges
- Hemoptysis and the Risk for Lung Transplant or Death without Transplant in Individuals with Cystic Fibrosis in the United States
- T-cell-mediated hypersensitivity to lumacaftor and ivacaftor in cystic fibrosis
- Correlation of Gut Microbiota, Vitamin D Status, and Pulmonary Function Tests in Children With Cystic Fibrosis
- Editorial: Genome Editing to Treat Cystic Fibrosis and Other Pulmonary Diseases
- Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis
- Systemic oxidative stress in children with cystic fibrosis with bacterial infection including Pseudomonas Aeruginosa
- Comparative analysis of respiratory symptom scores to detect acute respiratory events in children with cystic fibrosis
- Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study
- Evaluation of Aminopyrrolidine Amide to improve chloride transport in CFTR-defective cells
- High prevalence of Merkel cell polyomavirus is associated with dysregulation in transcript levels of TLR9 and type I IFNs in a large cohort of CF patients from the Italian (Lazio) reference center for cystic fibrosis
- Eruptive melanocytic naevi following initiation of elexacaftor/ivacaftor/tezacaftor for cystic fibrosis
- Pseudomonas aeruginosa: pathogenesis, virulence factors, antibiotic resistance, interaction with host, technology advances and emerging therapeutics
- Functional role of the ER stress transducer IRE1α in CF airway epithelial inflammation
- Being affiliated to a cystic fibrosis centre is important for parents’ everyday life
- Short-acting inhaled bronchodilators for cystic fibrosis
- Eating Disorders and Disordered Eating Behaviors in Cystic Fibrosis: A Neglected Issue
- One Size Does Not Fit All: The Past, Present and Future of Cystic Fibrosis Causal Therapies
- CFTR Rescue by Lumacaftor (VX-809) Induces an Extensive Reorganization of Mitochondria in the Cystic Fibrosis Bronchial Epithelium
- CFTR and Gastrointestinal Cancers: An Update
- Sleep, Sedentary Time and Physical Activity Levels in Children with Cystic Fibrosis
- The effect of cystic fibrosis transmembrane conductance regulator modulators on impaired glucose tolerance and cystic fibrosis related diabetes
- Altered intravenous drug disposition in people living with cystic fibrosis: a meta-analysis integrating top-down and bottom-up data
- PF-06409577 inhibits renal cyst progression by concurrently inhibiting the mTOR pathway and CFTR channel activity
- TMED3 Complex Mediates ER Stress-Associated Secretion of CFTR, Pendrin, and SARS-CoV-2 Spike
- Mannose-Binding Lectin (MBL) and Gap Junction Protein Alpha 4 (GJA4) Gene Heterogeneity in Relation to Severity of Clinical Disease in Cystic Fibrosis
- COVID-19 Lockdown Impacts Among Patients with Cystic Fibrosis: An Italian Regional Reference Centre Experience
- Macrocycle-stabilization of its interaction with 14-3-3 increases plasma membrane localization and activity of CFTR
- Analyzing the use and impact of elexacaftor/tezacaftor/ivacaftor on total cost of care and other health care resource utilization in a commercially insured population
- The thematic role of extracellular loop of VraG in activation of the membrane sensor GraS in a cystic fibrosis MRSA strain differs in nuance from the CA-MRSA strain JE2
- Activity of N -Acetylcysteine Alone and in Combination with Colistin against Pseudomonas aeruginosa Biofilms and Transcriptomic Response to N -Acetylcysteine Exposure
- Detection of Metallo Beta-Lactamase (MBL) Producing Pseudomonas aeruginosa in a Tertiary Care Hospital, Ghanpur, Medchal, India
- Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway disease
- Strain-specific predation of Bdellovibrio bacteriovorus on Pseudomonas aeruginosa with a higher range for cystic fibrosis than for bacteremia isolates
- Exercise versus airway clearance techniques for people with cystic fibrosis
- Emerging medicines to improve the basic defect in cystic fibrosis
- Taxonomic position, antibiotic resistance and virulence factors of clinical Achromobacter isolates
- Beneficial short-term effect of autogenic drainage on peripheral resistance in childhood cystic fibrosis disease
- Synergistic Activity of Repurposed Peptide Drug Glatiramer Acetate with Tobramycin against Cystic Fibrosis Pseudomonas aeruginosa
- A novel role for CFTR interaction with LH and FGF in azoospermia and epididymal maldevelopment caused by cryptorchidism
- Revealing the human mucinome
- Du modèle médical vers un modèle social de l’autonomie des personnes atteintes de mucoviscidose. Une étude sociologique du point de vue des acteurs professionnels et associatifs
- Temporal Modulation of Drug Desensitization Procedures
- Editorial: Co-Infection and Consequences in Cystic Fibrosis
- Satisfaction and Concerns with Telemedicine Endocrine Care of Patients with Cystic Fibrosis
- Development of an Interdisciplinary Telehealth Care Model in a Pediatric Cystic Fibrosis Center
- Elexacaftor-Tezacaftor-Ivacaftor Treatment Reduces Abdominal Symptoms in Cystic Fibrosis-Early results Obtained With the CF-Specific CFAbd-Score
- The Impact of Air Pollution on the Course of Cystic Fibrosis: A Review
- COMMUNICATING RESEARCH FINDINGS TO PATIENTS:: Comparison of readability of Patient Lay Summaries written by eight clinical disciplines of the Cystic Fibrosis (CF) Multidisciplinary Team (MDT)
- Elexacaftor-Tezacaftor-Ivacaftor improve Gastro-Oesophageal reflux and Sinonasal symptoms in advanced cystic fibrosis
- Clearing the air: Uniquely engaging furin as an approach to cystic fibrosis therapy
- Acute Pulmonary Exacerbation Phenotypes in Patients with Cystic Fibrosis
- CF-Seq, an accessible web application for rapid re-analysis of cystic fibrosis pathogen RNA sequencing studies
- The Beneficial Role of Probiotic Lactobacillus in Respiratory Diseases
- Ivacaftor Alters Macrophage and Lymphocyte Infiltration in the Lungs Following Lipopolysaccharide Exposure
- Metabolomic Biomarkers to Predict and Diagnose Cystic Fibrosis Pulmonary Exacerbations: A Systematic Review
- Defining and identifying early-onset lung disease in cystic fibrosis with cumulative clinical characteristics
- CFTR RNA- and DNA-based therapies
- Prospectively evaluating maternal and fetal outcomes in the era of CFTR modulators: the MAYFLOWERS observational clinical trial study design
- Anesthesia and Mucociliary Clearance: Implications for Cystic Fibrosis
- Evaluation of VX-121/Tezacaftor/Deutivacaftor in Cystic Fibrosis (CF) Participants 1 Through 11 Years of Age
- Molecular mechanisms of Cystic Fibrosis – how mutations lead to misfunction and guide therapy
- Food-web and metabolic interactions of the lung inhabitants Streptococcus pneumoniae and Pseudomonas aeruginosa
- Impact of illness on mothers of children with cystic fibrosis in Tunisia: A qualitative interview study: Impact of cystic fibrosis on Tunisian mothers
- Socioeconomic determinants of respiratory health in patients with cystic fibrosis: implications for treatment strategies
- Basic Pancreatic Lesions: Radiologic-pathologic Correlation
- Prolonged treatment with inhaled ampicillin in children with non-cystic fibrosis bronchiectasis
- Pilot Study to Evaluate a Voice Journaling Application in Adolescents With Cystic Fibrosis
- Geographical distribution of cystic fibrosis carriers as population genetic determinant of COVID-19 spread and fatality in 37 countries
- The Role of Bronchoscopy in the Management of Children With Cystic Fibrosis
- Cystic fibrosis: Quality of life and radiological monitoring
- Long-term Follow-up in Adult Patients with Cystic Fibrosis and Deep Intronic Splicing Variants
- Can we Train the Immune System of Patients With Cystic Fibrosis?
- Effects of Controlled Voluntary Increase in the Ventilatory Demand on Respiratory System Resistance in Healthy and Non-Cystic Fibrosis Bronchiectasis Subjects: A Cross-Sectional Study
- Mycobacterium abscessus : insights from a bioinformatic perspective
- Analysis of the genotypic profile and its relationship with the clinical manifestations in people with cystic fibrosis: study from a rare disease registry
- Coefficient of Fat Absorption to Measure the Efficacy of Pancreatic Enzyme Replacement Therapy in People With Cystic Fibrosis: Gold Standard or Coal Standard?
- Calprotectin: the link between acute lung injury and gastrointestinal injury in Covid-19: Ban or boon
- Mucus-targeting therapies of defective mucus clearance for cystic fibrosis: A short review
- Severity of the S1251N allele in cystic fibrosis is affected by the presence of the F508C variant in cis
- Poor Tolerability of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Lung Transplant Recipients
- Discovery and SAR of 4-Aminopyrrolidine-2-carboxylic Acid Correctors of CFTR for the Treatment of Cystic Fibrosis
- Biologically synthesized silver nanoparticles as potent antibacterial effective against multi drug-resistant Pseudomonas aeruginosa
- CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France
- Caroli’s disease incidentally discovered in a 16-years-old female: a case report
- Risk Factors of Incident Lung Cancer in Patients with Non-Cystic Fibrosis Bronchiectasis: A Korean Population-Based Study
- Nanomechanics combined with HDX reveals allosteric drug binding sites of CFTR NBD1
- rAAV-MEDIATED EDITING OF THE G551D CFTR MUTATION IN FERRET AIRWAY BASAL CELLS
- Five-year performance analysis of a cystic fibrosis newborn screening program in northeastern Brazil
- Lubiprostone is non-selective activator of cAMP-gated ion channels and Clc-2 has a minor role in its prosecretory effect in intestinal epithelial cells
- New Insights into Clinical and Mechanistic Heterogeneity of the Acute Respiratory Distress Syndrome: Summary of the Aspen Lung Conference 2021
- Acute kidney injury in cystic fibrosis patients treated with intravenous colistimethate sodium or tobramycin
- The effect of probiotic administration on metabolomics and glucose metabolism in CF patients
- The Short Term Influence of Chest Physiotherapy on Lung Function Parameters in Children With Cystic Fibrosis and Primary Ciliary Dyskinesia
- “Acquiring the Skills Needed to Communicate What Is in Our Teams’ Heart: Love, Compassion and Partnership”: Qualitative Analysis of Intact Multidisciplinary Teams’ Experience of Relationship-Centered Communication Training
- Use of mucoactive agents in cystic fibrosis: A consensus survey of Italian specialists
- The inhibition of CFTR in the descended testis of SD rats with unilateral cryptorchidism induced by di-(2-ethylhexyl) phthalate (DEHP)
- Routine spirometry in cystic fibrosis patients: impact on pulmonary exacerbation diagnosis and FEV1 decline
- Diabetic foot infection caused by bacteria of the Burkholderia cepacia complex: report of an unusual case and a scoping literature review
- Early Disseminated Mycobacterium Abscessus Complex Infection in an Infant with Coexisting Cystic Fibrosis and Progressive Familial Intrahepatic Cholestasis: Case report and literature review
- Structural Insights into the Inhibition Site in the Phosphorylcholine Phosphatase Enzyme of Pseudomonas aeruginosa
- Antibiotic hypersensitivity in cystic fibrosis – low frequency of anaphylaxis over 16,000 courses
- Rifaximin for Treatment of Bloating in Children and Adults With Cystic Fibrosis
- Treatment With LAU-7b Complements CFTR Modulator Therapy by Improving Lung Physiology and Normalizing Lipid Imbalance Associated With CF Lung Disease
- Disease Prevention and Treatment Using β-Carotene: the Ultimate Provitamin A
- Video Game-Based Exercise in Children and Adolescents with Non-Cystic Fibrosis Bronchiectasis: A Randomized Comparative Study of Aerobic and Breathing Exercises
- Pharmacogenomics: a tool to improve medication safety and efficacy in patients with cystic fibrosis
- The first lung retransplantation with “ECMO bridge” in the Czech Republic – case report
- Change in exercise capacity measured by Cardio-pulmonary Exercise Testing (CPET) in Danish people with cystic fibrosis after initiation of treatment with Lumacaftor/Ivacaftor and Tezacaftor/Ivacaftor
- No patient left behind! Therapeutic options for cystic fibrosis patients living with lung transplantation
- Changes in airway bacterial communities occur soon after initiation of antibiotic treatment of pulmonary exacerbations in cystic fibrosis
- Up-to-date incidence and initial characteristics of cystic fibrosis in Tunisia
- CFTR heterozygosity in severe asthma with recurrent airway infections: a retrospective review
- Depression and anxiety prevalence in people with cystic fibrosis and their caregivers: a systematic review and meta-analysis
- The effect of azithromycin on structural lung disease in infants with cystic fibrosis (COMBAT CF): a phase 3, randomised, double-blind, placebo-controlled clinical trial
- Ivacaftor in Omani children with cystic fibrosis caused by p.Ser549Arg CFTR mutation
- COMBATing airway inflammation in infants with cystic fibrosis
- The Progress of the Prevention and Treatment of Vitamin D to Tuberculosis
- First report on the prevalence of bacteria in cystic fibrosis patients (CF) in a tertiary care center in Saudi Arabia
- Liver disease in cystic fibrosis patients in a tertiary care center in Saudi Arabia
- Isolation, cultivation, and application of primary respiratory epithelial cells obtained by nasal brushing, polyp samples, or lung explants
- Efficient suppression of endogenous CFTR nonsense mutations using anticodon-engineered transfer RNAs
- Loss-of-function CFTR p.G970D missense mutation might cause congenital bilateral absence of the vas deferens and be associated with impaired spermatogenesis
- The negative impact of chronic rhinosinusitis on the health-related quality of life among adult patients with cystic fibrosis
- Genomics of Diversification of Pseudomonas aeruginosa in Cystic Fibrosis Lung-like Conditions
- The Genetic relatedness of Burkholderia contaminans clinical isolates from cystic fibrosis and non-cystic fibrosis patients in Argentina
- Regulation of Biofilm Exopolysaccharide Biosynthesis and Degradation in Pseudomonas aeruginosa
- The sweat chloride test has lived up to the changes in CF care
- The MUC5B Promoter Polymorphism is Not Associated With Non-ILD Chronic Respiratory Diseases or Post-transplant Outcome
- Translational Research in Cystic Fibrosis: From Bench to Beside
- Metabolomics of airways disease in cystic fibrosis
- The effect of antibiotic changes during treatment of cystic fibrosis pulmonary exacerbations
- Splicing mutations in the CFTR gene as therapeutic targets
- Development and internal validation of a prognostic model of 2-year death or lung transplant for cystic fibrosis
- Improvement in sinonasal quality of life indicators for pediatric patients with cystic fibrosis treated with elexacaftor-tezacaftor- ivacaftor
- Effectiveness of omalizumab as treatment of allergic pulmonary aspergillosis in patients with cystic fibrosis and asthma
- The Relation of CFTR-Genotype and Associated Comorbidities to Development of Pulmonary Atelectasis in Cystic Fibrosis Patients
- A Public Dialogue to Inform the Use of Wider Genomic Testing When Used as Part of Newborn Screening to Identify Cystic Fibrosis
- The prevalence of common CFTR gene mutations and polymorphisms in infertile Iranian men with very severe oligozoospermia
- Accounting for population structure in genetic studies of cystic fibrosis
- High-risk twin pregnancy: case report of an adolescent patient with cystic fibrosis and systemic lupus erythematosus
- The Human ATP-Binding Cassette (ABC) Transporter Superfamily
- Increased sputum lactate during oral glucose tolerance test in cystic fibrosis
- Inhibition of the sodium-dependent HCO3 – transporter SLC4A4, produces a cystic fibrosis-like airway disease phenotype
- Overcoming Health Disparities in Access to CFTR Modulator Therapies: One Child’s Journey with Cystic Fibrosis
- Competition between Pseudomonas aeruginosa and Staphylococcus aureus is dependent on intercellular signaling and regulated by the NtrBC two-component system
- Improved in-vivo airway gene transfer via magnetic-guidance, with protocol development informed by synchrotron imaging
- PILOT STUDY OF INFLAMMATORY BIOMARKERS IN MATCHED INDUCED SPUTUM AND BRONCHOALVEOLAR LAVAGE OF 2-YEAR-OLDS WITH CYSTIC FIBROSIS
- Towards Innovative Antibacterial-Correctors for Cystic Fibrosis Targeting the Lung Microbiome with a Multifunctional Effect
- Unconventional T Cell Immunity in the Lungs of Young Children with Cystic Fibrosis
- Characterization of the Extracellular Volatile Metabolome of Pseudomonas Aeruginosa Applying an in vitro Biofilm Model under Cystic Fibrosis-Like Conditions
- T405, a New Penem, Exhibits In Vivo Efficacy against M. abscessus and Synergy with β-Lactams Imipenem and Cefditoren
- Changes in Microbiome Dominance Are Associated With Declining Lung Function and Fluctuating Inflammation in People With Cystic Fibrosis
- Prevalence and Determinants of Wheezing and Bronchodilatation in Children With Cystic Fibrosis: A Retrospective Cohort Study
- Establishment of a CFTR-based detection method for the second messenger cAMP in the cytoplasm
- Who are the 10%? – Non eligibility of cystic fibrosis (CF) patients for highly effective modulator therapies
- Gene-specific nonsense-mediated mRNA decay targeting for cystic fibrosis therapy
- Links between Disease Severity, Bacterial Infections and Oxidative Stress in Cystic Fibrosis
- Gene Polymorphism of Biotransformation Enzymes and Ciprofloxacin Pharmacokinetics in Pediatric Patients with Cystic Fibrosis
- Colonic Fluid and Electrolyte Transport 2022: An Update
- Complex Presentation of Hao-Fountain Syndrome Solved by Exome Sequencing Highlighting Co-Occurring Genomic Variants
- Evaluating the Effect of Kaftrio on Perspectives of Health and Wellbeing in Individuals with Cystic Fibrosis
- Oral Hygiene and Periodontal Treatment Needs in Adult Patients with Cystic Fibrosis (CF)
- Increased Safety Behavior and COVID-19-Related Fear in Adults with Cystic Fibrosis during the Pandemic
- Oral Care Recommendation for Cystic Fibrosis Patients-Recommendation for Dentists
- Association between Smoking Status and Incident Non-Cystic Fibrosis Bronchiectasis in Young Adults: A Nationwide Population-Based Study
- Human Amniotic Mesenchymal Stem Cells and Fibroblasts Accelerate Wound Repair of Cystic Fibrosis Epithelium
- Essential Oils Biofilm Modulation Activity and Machine Learning Analysis on Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients
- Evaluation of a Novel Chromogenic Medium for the Detection of Pseudomonas aeruginosa in Respiratory Samples from Patients with Cystic Fibrosis
- The Role of Zinc in the Pathogenesis of Lung Disease
- Covalent Inhibition of the Human 20S Proteasome with Homobelactosin C Inquired by QM/MM Studies
- Elexacaftor-Tezacaftor-Ivacaftor as a Final Frontier in the Treatment of Cystic Fibrosis: Definition of the Clinical and Microbiological Implications in a Case-Control Study
- Liposome-Encapsulated Tobramycin and IDR-1018 Peptide Mediated Biofilm Disruption and Enhanced Antimicrobial Activity against Pseudomonas aeruginosa
- Current clinical opinion on CFTR dysfunction and patient risk of pancreatitis: diagnostic and therapeutic considerations
- Viruses to the rescue-Use of bacteriophage to treat resistant pulmonary infections
- Children with cystic fibrosis have elevated levels of fecal chitinase-3-like-1
- A Commensal Streptococcus Dysregulates the Pseudomonas aeruginosa Nitrosative Stress Response
- Antitoxin CrlA of CrlTA Toxin-Antitoxin System in a Clinical Isolate Pseudomonas aeruginosa Inhibits Lytic Phage Infection
- New drugs in cystic fibrosis: what has changed in the last decade?
- Genomic characterization of lytic bacteriophages targeting genetically diverse Pseudomonas aeruginosa clinical isolates
- High Adherence, Microbiological Control and Reduced Exacerbations in Patients With Non-Cystic Fibrosis Bronchiectasis Treated With Nebulised Colistin. A Prospective Observational Study
- Polymicrobial infections can select against Pseudomonas aeruginosa mutators because of quorum-sensing trade-offs
- Symptom Based Performance of Airway Clearance After Starting Highly Effective Modulators for Cystic Fibrosis (SPACE-CF)
- ERS International Congress 2021: highlights from the Paediatric Assembly
- VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non-G551D-CFTR gating mutations
- Characteristics of Stenotrophomonas maltophilia isolates from cystic fibrosis patients in Russia
- The Use of Machine Learning Techniques to Predict Diabetes in Patients with Cystic Fibrosis
- Author Reply to: Empowering Without Misinforming Adolescents and Young Adults with Cystic Fibrosis. Comment on “Perceptions of Social Media Use to Augment Health Care Among Adolescents and Young Adults With Cystic Fibrosis: Survey Study”
- Empowering Without Misinforming Adolescents and Young Adults with Cystic Fibrosis. Comment on “Perceptions of Social Media Use to Augment Health Care Among Adolescents and Young Adults With Cystic Fibrosis: Survey Study”
- Evaluation of nutritional status and related factors in children with cystic fibrosis
- Current state of CFTR modulators for treatment of Cystic Fibrosis
- Coexistence of pan-hypogammaglobulinaemia and primary ciliary dyskinesia
- Reversible bronchiectasis caused by influenza virus mimicking Williams-Campbell syndrome
- Glycosylation increases the anti-QS as well as anti-biofilm and anti-adhesion ability of the cyclo (L-Trp-L-Ser) against Pseudomonas aeruginosa
- Analysis of CFTR gene expression as an immunological and prognostic biomarker in pan-cancers
- Pulmonary hemorrhage in a neonate born to a woman with cystic fibrosis treated with targeted cystic fibrosis transmembrane conductance regulator modulator elexacaftor-tezacaftor-ivacaftor during pregnancy
- Fever in a Patient with a Central Venous Catheter Colonized by Pandoraea pnomenusa
- Isolation of Exophiala dermatitidis is not associated with worse clinical outcomes during acute pulmonary exacerbations in cystic fibrosis
- Mental health screening of patients with cystic fibrosis through telehealth during COVID-19: Evaluation of feasibility and process adoption
- Identification of IQGAP1 as a SLC26A4 (Pendrin)-Binding Protein in the Kidney
- Effect of Triple Combination CFTR Modulator Therapy on Sleep in Adult Patients with Cystic Fibrosis
- First in Poland Simultaneous Liver-Lung Transplantation With Liver-First Approach for Recipient Due to Cystic Fibrosis: A Case Report
- Parenthood challenged by cystic fibrosis. Experience of affected parents
- Complex and unexpected outcomes of antibiotic therapy against a polymicrobial infection
- Preliminary results of abdominal simultaneous multi-slice accelerated diffusion-weighted imaging with motion-correction in patients with cystic fibrosis and impaired compliance
- Objective and patient-based measures of chronic rhinosinusitis in people with cystic fibrosis treated with highly effective modulator therapy
- Does the effect of comprehensive respiratory physiotherapy home-program differ in children with cystic fibrosis and non-cystic fibrosis bronchiectasis?
- Effect of airway clearance therapies on mucociliary clearance in adults with cystic fibrosis: A randomized controlled trial
- Dysregulation of the Scribble/YAP/β-catenin axis sustains the fibroinflammatory response in a PKHD1-/- mouse model of congenital hepatic fibrosis
- Fibrotic Cystic Lung Disease Post Hematopoietic Stem Cell Transplant: Who is the Culprit?
- Editorial: Cystic Fibrosis in Children
- Cystic fibrosis learning network telehealth innovation lab during the COVID-19 pandemic: a success QI story for interdisciplinary care and agenda setting
- Inhibition mechanism of the chloride channel TMEM16A by the pore blocker 1PBC
- JMM Profile: Achromobacter xylosoxidans : the cloak-and-dagger opportunist
- Diagnosis and predicted outcomes of patients with cystic fibrosis related liver disease considered for lung transplantation
- Genomics of diversification of Pseudomonas aeruginosa in cystic fibrosis lung-like conditions
- Evaluation of Frequency and Characteristics of Drug Allergy in Pediatric Patients with Cystic Fibrosis
- Infection and Inflammation MUC Up the Cystic Fibrosis Airway
- Novel CFTR Modulators for Treating Cystic Fibrosis
- Parenting Children with Cystic Fibrosis: Developmental Acquisition of Expertise
- Association between stool consistency and clinical variables among infants with cystic fibrosis: Findings from the BONUS study
- Research letter: The impact of elexacaftor/tezacaftor/ivacaftor on adherence to nebulized maintenance therapies in people with cystic fibrosis
- Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis
- Dornase Alfa
- IgA Serological Response for the Diagnosis of Mycobacterium abscessus Infections in Patients with Cystic Fibrosis
- Comparison of Airway Clearance Therapies for Cystic Fibrosis: A Cochrane Review Summary With Commentary
- Recruitment of monocytes primed to express heme oxygenase-1 ameliorates pathological lung inflammation in cystic fibrosis
- Validation of nasal tracheal aspiration in children with lung disease
- Asymptomatic Pyuria as a Prognostic Biomarker in Autosomal Dominant Polycystic Kidney Disease
- Vitamin D Receptor Activation Exerts Anti-Secretory Actions In Colonic Epithelial Cells
- How Should the Effects of CFTR Modulator Therapy on Cystic Fibrosis Lung Disease be Monitored?
- Animal models of cystic fibrosis in the era of highly effective modulator therapies
- Rapid chloride and bicarbonate determination by capillary electrophoresis for confirmatory testing of cystic fibrosis infants with volume-limited sweat specimens
- ELEXACAFTOR/TEZACAFTOR/IVACAFTOR IN CHILDREN AGED 6-11 YEARS WITH CYSTIC FIBROSIS, AT LEAST ONE F508DEL ALLELE, AND ADVANCED LUNG DISEASE: A 24-WEEK OBSERVATIONAL STUDY
- Elevation of Sweat Chloride Levels in a Patient with CFTR-Related Metabolic Syndrome Receiving Dexmethylphenidate and Guanfacine
- Changes in share wave elastography after Lumacaftor/Ivacaftor treatment in children with cystic fibrosis
- The first report on CFTR mutations of meconium ileus in cystic fibrosis population in Saudi Arabia: A single center review
- The first report on the association of celiac disease and cystic fibrosis in a tertiary care center in Saudi Arabia
- Muc5b Contributes to Mucus Abnormality in Rat Models of Cystic Fibrosis
- Ultrasound-guided placement of Long Peripheral Cannula in children with Cystic Fibrosis
- Opposite regulation of F508del-CFTR biogenesis by four poly-lysine ubiquitin chains In vitro
- An Update on CFTR Modulators as New Therapies for Cystic Fibrosis
- Host and pathogen response to bacteriophage engineered against Mycobacterium abscessus lung infection
- Prevalence of Post-Glucose Challenge Hypoglycemia in Adult Patients With Cystic Fibrosis and Relevance to the Risk of Cystic Fibrosis-Related Diabetes
- Development and Evaluation of a Pharmacist-Driven Vitamin D Protocol for a Cystic Fibrosis Clinic
- CFTR Modulator Therapy in an Individual With Cystic Fibrosis Caused by a N1303K CFTR Variant and Infected With Mycobacterium abscessus
- CHST11-mediated microenvironment events contribute to pulmonary fibrosis and cancer progression
- Constipation in Children and Adolescents: Evaluation and Treatment
- Model-based Bayesian inference of the ventilation distribution in patients with Cystic Fibrosis from multiple breath washout, with comparison to ventilation MRI
- In vitro activity of ceragenins against Burkholderia cepacia complex
- Identification of Key Factors for Anoxic Survival of B. cenocepacia H111
- Giving Drugs a Second Chance: Antibacterial and Antibiofilm Effects of Ciclopirox and Ribavirin against Cystic Fibrosis Pseudomonas aeruginosa Strains
- Novel CFTR Activator Cact-3 Ameliorates Ocular Surface Dysfunctions in Scopolamine-Induced Dry Eye Mice
- Surface Hydration Protects Cystic Fibrosis Airways from Infection by Restoring Junctional Networks
- Positively Charged Gold Quantum Dots: An Nanozymatic “Off-On” Sensor for Thiocyanate Detection
- A Compositional Analysis of Physical Activity, Sedentary Time, and Sleep and Associated Health Outcomes in Children and Adults with Cystic Fibrosis
- Renal Function in Patients with Cystic Fibrosis: A Single-Center Study
- Magnesium Status and Calcium/Magnesium Ratios in a Series of Cystic Fibrosis Patients
- CFTR dysfunction and targeted therapies: A vision from non-cystic fibrosis bronchiectasis and COPD
- Loss of Endothelial CFTR Drives Barrier Failure and Edema Formation in Lung Infection and Can Be Targeted by CFTR Potentiation
- VX770 Modulated Bicarbonate Conductance in CFTR-expressing FRT Cell
- Potent and selective inhibition of anion exchange activity of SLC26A9 by A9-301
- Kinetic Characterization of a Crystallized Putative Hydroxybutyrate Dehydrogenase from the Opportunistic Pathogen Burkholderia cenocepacia
- Pharmacological effect of pinostrobin on CFTR-mediated Cl- secretion and renal cyst development in in vitro model of polycystic kidney disease
- Loss of Club Cell Creb Mitigates IL-1B-mediated Muco-obstructive Phenotypes in Male Murine Airways
- Alternative Chloride Channels are Increased in the CFTR-/- Rat Airway
- Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by the Nuclear Bile Acid Receptor, Farnesoid X Receptor
- Colistin Action Against Planktonic Pseudomonas aeruginosa
- Evaluation of Small Molecule Inhibitors of Pseudomonas Virulence factor LasB as Non-Traditional Immunotherapeutics
- In vitro model of pulmonary candidiasis for testing novel therapeutics
- Select Amino Acids Increase Anion Secretion in Human Bronchial Epithelial Cells (HBEC) with F508del and Nonsense Mutations: Alternative Approach to Restoring Airway Anion Secretion in Cystic Fibrosis
- Effect of Ciprofloxacin on Planktonic Pseudomonas aeruginosa
- The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Plays an Important Role in Fetal Human Colon Cell Migration and Proliferation
- Corneal opacification, an atypical presentation of cystic fibrosis: a case report and review of the literature
- In vitro Activity of Antivirulence Drugs Targeting the las or pqs Quorum Sensing Against Cystic Fibrosis Pseudomonas aeruginosa Isolates
- Adaptive Immune Response to Mycobacterium abscessus Complex (MABSC) in Cystic Fibrosis and the Implications of Cross-Reactivity
- Limitations of standard cost-effectiveness methods for health technology assessment of treatments for rare, chronic diseases: a case study of treatment for cystic fibrosis
- Taxonomic position, antibiotic resistance and virulence factor production by Stenotrophomonas isolates from patients with cystic fibrosis and other chronic respiratory infections
- Pseudomonas aeruginosa Alters Peptidoglycan Composition under Nutrient Conditions Resembling Cystic Fibrosis Lung Infections
- Atomic-scale interactions between quorum sensing autoinducer molecules and the mucoid P. aeruginosa exopolysaccharide matrix
- Telemedicine of patients with cystic fibrosis during the COVID-19 pandemic
- Optimization of a Liposomal DNase I Formulation with an Extended Circulating Half-Life
- Prevalence and subtyping of biofilms present in bronchoalveolar lavage from children with protracted bacterial bronchitis or non-cystic fibrosis bronchiectasis: a cross-sectional study
- Urinary bicarbonate and metabolic alkalosis during exacerbations in cystic fibrosis
- Potential Cross-Transmission of Mycobacterium abscessus among Non-Cystic Fibrosis Patients at a Tertiary Hospital in Japan
- Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles
- Tissue-localized immune responses in people with cystic fibrosis and respiratory nontuberculous mycobacteria infection
- Reducing exacerbations in children and adults with primary ciliary dyskinesia using erdosteine and/or azithromycin therapy (REPEAT trial): study protocol for a multicentre, double-blind, double-dummy, 2×2 partial factorial, randomised controlled trial
- Nephronophthisis: a pediatric case report
- Non-Cystic Fibrosis Bronchiectasis Increases the Risk of Lung Cancer Independent of Smoking Status
- Functional Evaluation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in the Endocervix
- Rapid Viscoelastic Characterization of Airway Mucus using a Benchtop Rheometer
- Primary Human Nasal Epithelial Cells: Biobanking in the Context of Precision Medicine
- Discovering Common Pathophysiological Processes between COVID-19 and Cystic Fibrosis by Differential Gene Expression Pattern Analysis
- Short-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor Combination on Glucose Tolerance in Young People With Cystic Fibrosis-An Observational Pilot Study
- Cancer in Cystic Fibrosis: Do Not Neglect Gynecologic Cancers
- Prostaglandin E2 : A Potential Link Between Cystic Fibrosis and Cancer
- [Translated article] Aquagenic Keratoderma: Treatment Update
- Caution advised in the use of CFTR modulator treatment for individuals harboring specific CFTR variants
- Anesthetic impacts on pulmonary function: Implications for cystic fibrosis
- Pathological remodeling of distal lung matrix in end-stage cystic fibrosis patients
- The effect of TGF-β1 polymorphisms on pulmonary disease progression in patients with cystic fibrosis
- The impact of FDA and EMA regulatory decision-making process on the access to CFTR modulators for the treatment of cystic fibrosis
- Amino Acids Improve Aerosolization and Chemical Stability of Potential Inhalable Amorphous Spray-dried Ceftazidime for Pseudomonas aeruginosa Lung Infection
- Systematic review on fecal calprotectin in cystic fibrosis
- Clinical outcomes of a large cohort of individuals with the F508del/5T;TG12 CFTR genotype
- Association between early respiratory viral infections and structural lung disease in infants with cystic fibrosis
- Alterations in innate immune responses of patients with chronic rhinosinusitis related to cystic fibrosis
- A dual center and dual vendor comparison study of automated perfusion-weighted phase-resolved functional lung magnetic resonance imaging with dynamic contrast-enhanced magnetic resonance imaging in patients with cystic fibrosis
- Susceptibility of Burkholderia cepacia Complex to Ceftazidime/Avibactam and Standard Drugs of Treatment for Cystic Fibrosis Patients
- Increased intracellular Cl- concentration mediates neutrophil extracellular traps formation in atherosclerotic cardiovascular diseases
- Blood mRNA biomarkers distinguish variable systemic and sputum inflammation at treatment initiation of inhaled antibiotics in cystic fibrosis: A prospective non-randomized trial
- Impacts of Nontuberculous Mycobacteria Isolates in Non-cystic Fibrosis Bronchiectasis: A 16-Year Cohort Study in Taiwan
- Sputum neutrophil elastase and its relation to pediatric bronchiectasis severity: A cross-sectional study
- Chronic cough in cystic fibrosis: the effect of modulator therapy on objective 24-h cough monitoring
- Distribution of Achromobacter Species in 12 French Cystic Fibrosis Centers in 2020 by a Retrospective MALDI-TOF MS Spectrum Analysis
- Cystic Fibrosis and Diagnostic Imaging
- Angiographic and histopathological study on bronchial-to-pulmonary vascular anastomoses on explants from patients with cystic fibrosis after bronchial artery embolisation
- Extreme electrolyte depletion associated with septic shock as a form of presentation of cystic fibrosis
- Exercise in Child Health
- Early Pseudomonas aeruginosa predicts poorer pulmonary function in preschool children with cystic fibrosis
- Cystic fibrosis and alpha-1 antitrypsin deficiency: case report and review of literature
- Invited editorial: Q and A on hereditary lung cancer
- CFTR protein quantification as a cystic fibrosis diagnostic biomarker in dried blood spots using multiple reaction monitoring tandem mass spectrometry
- The psychological implications and health risks of cystic fibrosis pre- and post- CFTR modulator therapy
- COVID-19 infection and nocardiosis causing the death of an adolescent with cystic fibrosis
- Exploring YAP1-centered networks linking dysfunctional CFTR to epithelial-mesenchymal transition
- Results of Lung Transplantations Among Cystic Fibrosis Patients: A Single-Center Study
- Cystic fibrosis year in review 2021
- Impaired regulation of PMCA activity by defective CFTR expression promotes epithelial cell damage in alcoholic pancreatitis and hepatitis
- Role of Cellular Metabolism in the Formation of Neutrophil Extracellular Traps in Airway Diseases
- Synergy Between Pseudomonas aeruginosa Filtrates And Voriconazole Against Aspergillus fumigatus Biofilm Is Less for Mucoid Isolates From Persons With Cystic Fibrosis
- Cardiac sarcoidosis in an adult person with cystic fibrosis: a case report
- Diffuse pulmonary calcification in allergic bronchopulmonary aspergillosis
- COVID-19 infection in an infant with cystic fibrosis: A case report and possible therapeutic effect of hypertonic saline
- Cystic Fibrosis-Related Liver Disease is an Independent Risk Factor For Mortality And Increased Health Care Resource Utilization In Hospitalized Pediatric Patients With Cystic Fibrosis
- Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis: Authors’ Reply
- Staphylococcus aureus detection from CF respiratory samples is improved using alternative media
- Difficult gall bladder in adolescents with cystic fibrosis and symptomatic cholelithiasis: What is the best treatment choice?
- The Role of HLA-DRB1 Alleles in Pulmonary Cystic Fibrosis
- Breath biomarkers associated withnontuberculosis mycobacteria disease status in persons with cystic fibrosis: a pilot study
- Antibacterial contact-dependent proteins secreted by Gram-negative cystic fibrosis respiratory pathogens
- CFTR mRNAs with nonsense codons are degraded by the SMG6-mediated endonucleolytic decay pathway
- Role of inhaled antibiotics in children and adolescents with cystic fibrosis: Experience from the tertiary care center
- Cystic Fibrosis Airway Mucus Hyperconcentration Produces a Vicious Cycle of Mucin, Pathogen, and Inflammatory Interactions that Promote Disease Persistence
- Continuous glucose monitoring and dysglycaemia in young children with cystic fibrosis: A case series
- Effectiveness of positive expiratory pressure on patients over 16 years of age with cystic fibrosis: systematic review and meta-analysis
- Esophagogastric variceal bleeding as debut of Caroli’s syndrome
- From a medical model to a social model of autonomy for people with cystic fibrosis. A sociological study of the views of professional and associative stakeholders
- Pharmacist-administered audiology screening for pediatric cystic fibrosis patients exposed to high-dose aminoglycosides: a pilot study
- Sustained inhibition of ENaC in CF: Potential RNA-based therapies for mutation-agnostic treatment
- Novel adamantyl clubbed iminothiazolidinones as promising elastase inhibitors: design, synthesis, molecular docking, ADMET and DFT studies
- A Pharmacokinetic Analysis of Tobramycin in Patients Less than Five Years of Age with Cystic Fibrosis: Assessment of Target Attainment with Extended-Interval Dosing through Simulation
- Sputum Metabolites Associated with Nontuberculous Mycobacterial Infection in Cystic Fibrosis
- Use of inhaled antibiotics among Danish patients with cystic fibrosis
- Can continuous glucose monitoring predict cystic fibrosis-related diabetes and worse clinical outcome?
- Diagnosis of cystic fibrosis-related diabetes: too early or too late?
- Cumulative Incidence and Risk Factors for Severe COVID-19 in French People with Cystic Fibrosis
- Paths to Motherhood for Women with Cystic Fibrosis
- Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients
- Molecular Dynamics and Theratyping in Airway and Gut Organoids Reveal R352Q-CFTR Conductance Defect
- Molecular Insight into Gene Response of Diorcinol- and Rubrolide-Treated Biofilms of the Emerging Pathogen Stenotrophomonas maltophilia
- Genetic and Transcriptomic Characteristics of RhlR-Dependent Quorum Sensing in Cystic Fibrosis Isolates of Pseudomonas aeruginosa
- Reporting two novel mutations in two Iranian families with cystic fibrosis, molecular and bioinformatic analysis
- Romosozumab used to treat a patient with cystic fibrosis-related osteoporosis
- Diabetic Ketoacidosis as a Unique Presentation of Cystic Fibrosis
- Contribution of Afferent Renal Nerves to Cystogenesis and Arterial Pressure Regulation in a Preclinical Model of Autosomal Recessive Polycystic Kidney Disease
- Complete Genome Sequence of Stenotrophomonas maltophilia Podophage Pepon
- Downregulation of epithelial sodium channel (ENaC) activity in cystic fibrosis cells by epigenetic targeting
- Antipseudomonal treatment decisions during CF exacerbation management
- Seamless Gene Correction in the Human Cystic Fibrosis Transmembrane Conductance Regulator Locus by Vector Replacement and Vector Insertion Events
- Diagnostics of the Epstein-Barr Virus Before and After Lung Transplantation in a Patient With Developing Posttransplant Lymphoproliferative Disease: A Case Report
- Effect of Bronchoscopic Interventions on Long-Term Lung Function Among Lung Transplant Recipients due to Cystic Fibrosis: A Single-Center Study
- Immunogenicity of the COVID-19 BNT162b2 vaccine in adolescents and young adults with cystic fibrosis
- Ways of coping and survival in Cystic Fibrosis: a 20-year longitudinal study
- Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics
- Overview of CF lung pathophysiology
- The Octopus Sign-A New HRCT Sign in Pulmonary Langerhans Cell Histiocytosis
- Investigation of Direct and Retro Chromone-2-Carboxamides Based Analogs of Pseudomonas aeruginosa Quorum Sensing Signal as New Anti-Biofilm Agents
- Cilia Stimulatory and Antibacterial Activities of T2R Bitter Taste Receptor Agonist Diphenhydramine: Insights into Repurposing Bitter Drugs for Nasal Infections
- Oral Glucose Tolerance Test in Patients with Cystic Fibrosis Compared to the Overweight and Obese: A Different Approach in Understanding the Results
- Theratyping of the Rare CFTR Variants E193K and R334W in Rectal Organoid-Derived Epithelial Monolayers
- Secretory Immunoglobulin A Immunity in Chronic Obstructive Respiratory Diseases
- CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells
- Highway to Cell: Selection of the Best Cell-Penetrating Peptide to Internalize the CFTR-Stabilizing iCAL36 Peptide
- Pseudomonas aeruginosa PAO1 Is Attracted to Bovine Bile in a Novel, Cystic Fibrosis-Derived Bronchial Epithelial Cell Model
- Clinical Impact of Aspergillus fumigatus in Children with Cystic Fibrosis
- The Human Mycobiome in Chronic Respiratory Diseases: Current Situation and Future Perspectives
- Pharmacological Effects of Panduratin A on Renal Cyst Development in In Vitro and In Vivo Models of Polycystic Kidney Disease
- Influence of Bronchoscopic Interventions on Graft Function of Double Lung Transplant Recipients due to Cystic Fibrosis
- Combined machine learning and pharmacophore based virtual screening approaches to screen for antibiofilm inhibitors targeting LasR of Pseudomonas aeruginosa
- Alterations of mucosa-attached microbiome and epithelial cell numbers in the cystic fibrosis small intestine with implications for intestinal disease
- An observational study of Pseudomonas aeruginosa in adult long-term ventilation
- Novel CFTR modulator combinations maximise rescue of G85E and N1303K in rectal organoids
- Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A case series
- DNA Sequencing Analysis of Cystic Fibrosis Transmembrane Regulator Gene Identifies Cystic Fibrosis-Associated Variants in the Severe Asthma Research Program
- At-home Compounding Preparation of Slow Desensitization of Elexacaftor/Tezacaftor/Ivacaftor for Delayed Hypersensitivity Rash
- Co-Operative Biofilm Interactions between Aspergillus fumigatus and Pseudomonas aeruginosa through Secreted Galactosaminogalactan Exopolysaccharide
- The Presence of Exophiala dermatitidis in the Respiratory Tract of Cystic Fibrosis Patients Accelerates Lung Function Decline: A Retrospective Review of Lung Function
- Molecular Epidemiological Characteristics of Mycobacterium abscessus Complex Derived from Non-Cystic Fibrosis Patients in Japan and Taiwan
- Interbacterial Antagonism Mediated by a Released Polysaccharide
- Losartan ameliorates TGF-β1-induced CFTR dysfunction and improves correction by cystic fibrosis modulator therapies
- Histomorphological Changes in a Rat Model of Polycystic Ovary Syndrome and the Contribution of Stevia Leaf Extract in Modulating the Ovarian Fibrosis, VEGF, and TGF-β Immunoexpressions: Comparison with Metformin
- The role of Hyaluronan synthesis and degradation in the critical respiratory illness COVID-19
- Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated with one Month of Lumacaftor/ivacaftor
- Current prices versus minimum costs of production for CFTR modulators
- Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D
- Non-visualisation of fetal gallbladder in a Chinese cohort
- Investigation of interspecies crosstalk between probiotic Bacillus subtilis BR4 and Pseudomonas aeruginosa using metabolomics analysis
- Pediatric Paranasal Sinuses- Development, Growth, Pathology & Functional Endoscopic Sinus Surgery
- Living with cystic fibrosis during the COVID-19 pandemic: a social connectedness perspective
- A new era has dawned for persons with cystic fibrosis; however many knowledge gaps exist in our efforts to improve care
- Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosis
- The Effect of Dysglycaemia on Changes in Pulmonary and Aerobic Function in Cystic Fibrosis
- Bone accrual and structural changes over one year in youth with cystic fibrosis
- Clinical practice versus guidelines for the screening of cystic fibrosis-related diabetes: A French survey from the 47 centers
- Using Structure-guided Fragment-Based Drug Discovery to Target Pseudomonas aeruginosa Infections in Cystic Fibrosis
- A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein
- Application of gap time analysis with flexible hazards to pulmonary exacerbations in the EPIC observational study
- ‘We’re kind of like genetic nomads’: Parents’ experiences of biographical disruption and uncertainty following in/conclusive results from newborn cystic fibrosis screening
- Occurrence of Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations in Patients with Allergic Bronchopulmonary Aspergillosis Complicating Asthma
- Spatial covariance analysis reveals the residue-by-residue thermodynamic contribution of variation to the CFTR fold
- Constipation and cystic fibrosis. Slow movement
- Evaluation of clinically relevant changes in the lung clearance index in children with cystic fibrosis and healthy controls
- Evaluating FEV1 Decline in Diagnosis and Management of Pulmonary Exacerbations in Children with Cystic Fibrosis
- Mental health care needs in cystic fibrosis: A scoping review
- A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus
- Study to Evaluate Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) Long-term Safety and Efficacy in Subjects Without F508del
- Coming Full Circle: Reflections and Inspirations from a Cystic Fibrosis Patient Scientist Panel
- Role of Efflux in Antibiotic Resistance of Achromobacter xylosoxidans and Achromobacter insuavis Isolates From Patients With Cystic Fibrosis
- Repeatability of lung clearance index in infants with cystic fibrosis and recurrent wheeze
- Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice
- Re: Impact of spiroware re-analysis method on multiple-breath washout outcomes in children with cystic fibrosis; M.A. Oestreich, F. Wyler, P. Latzin et al
- CFTR modulator use and risk of nontuberculous mycobacteria positivity in cystic fibrosis, 2011-2018
- Targeting the EGFR-ERK axis using the compatible solute ectoine to stabilize CFTR mutant F508del
- Characterization of skeletal muscle wasting pathways in diaphragm and limb muscles of cystic fibrosis mice
- Correlates of Pancreatic Enzyme Replacement Therapy Intake in Adults with Cystic Fibrosis: Results of a Cross-Sectional Study
- Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis
- CFTR Modulator Therapies: Potential Impact on Airway Infections in Cystic Fibrosis
- The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis
- Comparison of Bioelectrical Impedance Analysis with DXA in Adolescents with Cystic Fibrosis before and after a Resistance Training Intervention
- Cystic Fibrosis-Related Diabetes in Poland
- Bronchiectasis – Exercise as Therapy (BREATH): rationale and study protocol for a multi-center randomized controlled trial
- Biochemical, Biophysical, and Immunological Characterization of Respiratory Secretions in Severe SARS-CoV-2 (COVID-19) Infections
- Feasibility and efficacy of combined pancreatic islet-lung transplantation in cystic fibrosis related diabetes -PIM study: a multicenter phase 1-2 trial
- Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis: Correspondence
- Mucus Clearance Strategies in Mechanically Ventilated Patients
- Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis
- Transcatheter coil embolization of a complex pulmonary artery pseudoaneurysm with thyrocervical trunk-pulmonary arterial fistulization in a patient with cystic fibrosis and massive hemoptysis
- High cystic fibrosis transmembrane conductance regulator expression in childhood B-cell acute lymphoblastic leukemia acts as a potential therapeutic target
- “The Stakes Are Higher”- Patient and Caregiver Perspectives on Cystic Fibrosis Research and Personalized Medicine
- Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry
- Genetic evidence supports the development of SLC26A9 targeting therapies for the treatment of lung disease
- Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse Model
- Safety of chronic hypertonic bicarbonate inhalation in a cigarette smoke-induced airway irritation guinea pig model
- Oxylipin profile in saliva from patients with cystic fibrosis reveals a balance between pro-resolving and pro-inflammatory molecules
- Ciliary and immune dysfunctions and their genetic background in patients with non-cystic fibrosis bronchiectasis in Central Iran
- Distinct proteostasis states drive pharmacologic chaperone susceptibility for Cystic Fibrosis Transmembrane Conductance Regulator misfolding mutants
- Medical interventions for chronic rhinosinusitis in cystic fibrosis
- Lung parenchymal calcifications in a child with cystic fibrosis
- Curvilinearity provides additional information to lung clearance index only in a minority of children with early cystic fibrosis lung disease
- A pilot study of disease related education and psychotherapeutic support for unresolved grief in parents of children with CF
- Brazilian Guidelines for Nutrition in Cystic Fibrosis
- Developmental Functioning Outcomes in Infants with Cystic Fibrosis: a 24- to 36-Month Follow-Up Study
- Cefiderocol Pharmacokinetics in Adult Patients With Cystic Fibrosis
- Surprise Foreign Body Aspiration with Pistachios in a Patient with Cystic Fibrosis with Persistent Atelectasis on Chest Radiography
- Glby, Encoded by MAB_3167c , Is Required for In Vivo Growth of Mycobacteroides abscessus and Exhibits Mild β-Lactamase Activity
- Persistent Rhinovirus Infection in a Child With Leukemia
- Proteostasis Regulators in Cystic Fibrosis: Current Development and Future Perspectives
- Microbial Community Composition in Explanted Cystic Fibrosis and Control Donor Lungs
- Targeting the Pseudomonas aeruginosa Virulence Factor Phospholipase C With Engineered Liposomes
- Reinfection or relapse? A case study of whole genome sequencing guided genomic characterization of Mycobacterium abscessus chronic infection in a cystic fibrosis patient
- Association between elevated peripheral blood eosinophil count and respiratory outcomes in adults with cystic fibrosis
- Objective Nebuliser Adherence Data as “Proof” of Adherence in the Management of Cystic Fibrosis: A Qualitative Interview Study
- SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair Modulation
- Moving up: Healthcare transition experiences of adolescents and young adults with cystic fibrosis
- Club cell secretory protein and lung function in children with cystic fibrosis
- Pathophysiology of Lung Disease and Wound Repair in Cystic Fibrosis
- Expression of ACE2 – A Key SARS-CoV-2 Entry Factor – Is Not Increased in the Nasal Mucosa of People with Cystic Fibrosis
- TMEM16A (ANO1) as a therapeutic target in cystic fibrosis
- High frequency of complex CFTR alleles associated with c.1521_1523delCTT (F508del) in Russian cystic fibrosis patients
- Lung clearance index to characterize clinical phenotypes of children and adolescents with cystic fibrosis
- The 49th parallel: Does geographic position affect longevity of patients with cystic fibrosis?
- Mucus concentration-dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis
- Efficacy and Safety of Triple Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Patients With Cystic Fibrosis: A Meta-Analysis of Randomized Controlled Trials
- Network Analysis to Identify Multi-Omic Correlations in the Lower Airways of Children With Cystic Fibrosis
- Efficacy of N-acetylcysteine on idiopathic or postinfective non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis protocol
- The dilemma of initiating ELX/TEZ/IVA in a CF patient recovering from acute-on-chronic liver failure
- Prepuberal insulin secretory indices are long term predictors of short adult stature in cystic fibrosis
- Eosinophilic Esophagitis in Cystic Fibrosis: A Case Series with Long-Term Follow-up
- The Ubiquitin Ligase RNF34 Participates in the Peripheral Quality Control of CFTR (RNF34 Role in CFTR PeriQC)
- A Shaving Proteomic Approach to Unveil Surface Proteins Modulation of Multi-Drug Resistant Pseudomonas aeruginosa Strains Isolated From Cystic Fibrosis Patients
- A PI3Kγ mimetic peptide triggers CFTR gating, bronchodilation, and reduced inflammation in obstructive airway diseases
- Using A Work System Framework to Investigate Pharmacists’ Roles in Cystic Fibrosis Management
- Azithromycin inhibits mucin secretion, mucous metaplasia, airway inflammation and airways hyperresponsiveness in mice exposed to house dust mite extract
- MRI lung lobe segmentation in pediatric cystic fibrosis patients using a recurrent neural network trained with publicly accessible CT datasets
- Things come in threes: A new complex allele and a novel deletion within the CFTR gene complicate an accurate diagnosis of cystic fibrosis
- Azole-Induced Myositis after Combined Lung-Liver Transplantation
- Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis
- Histological Manifestations of Diabetic Kidney Disease and its Relationship with Insulin Resistance
- Cross-talk between enhancers, structural elements and activating transcription factors maintains the 3D architecture and expression of the CFTR gene
- Significance of plasma fibrinogen and malondialdehyde in the post-inflammatory period in patients with cystic fibrosis
- Complete Genome Sequence of Burkholderia cenocepacia Phage Paku
- COVID-19 in a pregnant cystic fibrosis carrier with myasthenia gravis: A case report
- Factors in childhood associated with lung function decline to adolescence in cystic fibrosis
- Global access to affordable CFTR modulator drugs: Time for action!
- Krüppel-Like Factor 5 regulates CFTR expression through repression by maintaining chromatin architecture coupled with direct enhancer activation
- Characterization of glucose metabolism in youth with vs. without cystic fibrosis liver disease: A pilot study
- The Changing Landscape of Nutrition in Cystic Fibrosis: The Emergence of Overweight and Obesity
- Journey on VX-809-Based Hybrid Derivatives towards Drug-like F508del-CFTR Correctors: From Molecular Modeling to Chemical Synthesis and Biological Assays
- Defining and Treating Cystic Fibrosis Liver Disease: Some Things Old and Some Things New
- Pseudomonas aeruginosa polysaccharide Psl supports airway microbial community development
- Exposure to the Pseudomonas aeruginosa secretome alters the proteome and secondary metabolite production of Aspergillus fumigatus
- An 18-Year Dataset on the Clinical Incidence and MICs to Antibiotics of Achromobacter spp. (Labeled Biochemically or by MAL-DI-TOF MS as A. xylosoxidans ), Largely in Patient Groups Other than Those with CF
- Elastase-Activated Antimicrobial Peptide for a Safer Pulmonary Treatment of Cystic Fibrosis Infections
- Lung-Directed Bacteriotherapy in Cystic Fibrosis: Could It Be an Option?
- Structural Comparative Modeling of Multi-Domain F508del CFTR
- The Relation between Vitamin D Level and Lung Clearance Index in Cystic Fibrosis-A Pilot Study
- The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs
- Feasibility and Efficacy of Telehealth-Based Resistance Exercise Training in Adolescents with Cystic Fibrosis and Glucose Intolerance
- Biofilm on Toothbrushes of Children with Cystic Fibrosis: A Potential Source of Lung Re-Infection after Antibiotic Treatment?
- Metrics of Antifungal Effects of Ciprofloxacin on Aspergillus fumigatus Planktonic Growth and Biofilm Metabolism; Effects of Iron and Siderophores
- Targeted Gene Insertion for Functional CFTR Restoration in Airway Epithelium
- One year monitoring of SARS-CoV-2 prevalence in a German cohort of patients with cystic fibrosis
- Inherited pancreatic exocrine insufficiency and pancreatitis: When children transition to adult care
- Digestive outcomes in Cystic fibrosis
- Improving Therapeutic Adherence With a Co-constructed Program Involving Both Patients and Health Care Professionals
- Description of the Short-term Effects of KAFTRIO® by Continuous Monitoring With the PHEAL-CR-K Application in Real Life in Patients With Cystic Fibrosis Eligible for KAFTRIO® Treatment
- Caregiver burden in cystic fibrosis: a systematic literature review
- Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs
- A Potent Inhibitor of the Cystic Fibrosis Transmembrane Conductance Regulator Blocks Disease and Morbidity Due to Toxigenic Vibrio cholerae
- Does ibuprofen affect the expression of alginate genes in pathogenic Pseudomonas aeruginosa strains?
- State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the “iMAging managEment of cySTic fibROsis” (MAESTRO) consortium
- Alginate oligosaccharides enhance diffusion and activity of colistin in a mucin-rich environment
- Respiratory physiotherapy in patients with Cystic Fibrosis and upper limb deep vein thrombosis
- Measurement of exhaled nitric oxide fraction in lung diseases
- The Use of Tobramycin for Pseudomonas aeruginosa : A Review
- Cystic fibrosis transmembrane conductance regulator prevents ischemia/reperfusion induced intestinal apoptosis via inhibiting PI3K/AKT/NF-kappaB pathway
- Cystic fibrosis patients on cystic fibrosis transmembrane conductance regulator modulators have a reduced incidence of cirrhosis
- Pseudomonas aeruginosa modulates neutrophil granule exocytosis in an in vitro model of airway infection
- Resolution of lobe collapse in a child with cystic fibrosis with M. abscessus using serial intrabronchial rhDNase
- CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor
- CFTR-mediated monocyte/macrophage dysfunction revealed by cystic fibrosis proband-parent comparisons
- Systematic estimation of cystic fibrosis prevalence in Chinese and genetic spectrum comparison to Caucasians
- Strong and consistent associations of precedent chronic rhinosinusitis with risk of non-cystic fibrosis bronchiectasis
- The Impact of COVID-19 in Cystic Fibrosis
- Cystic Fibrosis in 2021: “The Times They Are A-Changin”
- Association Between Evolution of Mucus Plugging, Parenchymal Alterations and Air Trapping on Computed Tomography and Risk of Exacerbations in Adults With Cystic Fibrosis
- Variation in CFTR-dependent ‘β-sweating’ among healthy adults
- Virulence Mechanisms of Mycobacterium abscessus : Current Knowledge and Implications for Vaccine Design
- Novel cystic fibrosis transmembrane conductance regulator variant in a cystic fibrosis patient
- Is exercise and electrostimulation effective in improving muscle strength and cardiorespiratory fitness in children with cystic fibrosis and mild-to-moderate pulmonary impairment?: Randomized controlled trial
- Decreased efficacy of antimicrobial agents in a polymicrobial environment
- Mutational Spectrum of the CFTR Gene in the Kazakhstan Population
- HALT-ing Nontuberculous Mycobacteria in CF Centers. Is There Something in The Water?
- The NSAID glafenine rescues class 2 CFTR mutants via cyclooxygenase 2 inhibition of the arachidonic acid pathway
- Pinostrobin inhibits renal CFTR-mediated Cl- secretion and retards cyst growth in cell-derived cyst and polycystic kidney disease rats
- Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis
- Health care costs in a randomized trial of antimicrobial duration among cystic fibrosis patients with pulmonary exacerbations
- A prospective randomised controlled mixed-methods pilot study of home monitoring in adults with cystic fibrosis
- Use of Dynamic Chest Radiography to Assess Treatment of Pulmonary Exacerbations in Cystic Fibrosis
- Impact of lockdown during the COVID-19 pandemic on health status in patients with cystic fibrosis: a mono-centre observational study
- Clinical Outcomes of Antipseudomonal versus Other Antibiotics Among Children with Cystic Fibrosis without Pseudomonas aeruginosa
- Targeting the E1 ubiquitin-activating enzyme (UBA1) improves elexacaftor/tezacaftor/ivacaftor efficacy towards F508del and rare misfolded CFTR mutants
- Informatic analysis of the pulmonary microecology in non-cystic fibrosis bronchiectasis at three different stages
- Predictive value of impulse oscillometry and multiple breath washout parameters in pediatric patients with cystic fibrosis pulmonary exacerbation
- PBPK-led guidance for cystic fibrosis patients taking elexacaftor-tezacaftor-ivacaftor with nirmatrelvir-ritonavir for the treatment of COVID-19
- Charting a New Path: A Single-cell Atlas of Porcine CF Airways at Birth
- Novel volumetric capnography indices measure ventilation inhomogeneity in cystic fibrosis
- Elexacaftor/Tezacaftor/Ivacaftor as a Bridge to Lung Retransplant in a Recipient With Cystic Fibrosis
- Three-hole oesophagectomy following bilateral lung transplant for cystic fibrosis
- The effect of inhaled hypertonic saline on lung structure in children aged 3-6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled trial
- Effect of chest physiotherapy on cystic fibrosis sputum nanostructure: an experimental and theoretical approach
- Inhaled Dry Powder Mannitol Treatment in Pediatric Patients with Cystic Fibrosis: Evaluation of Clinical Data in a Real-World Setting
- Efficacy of Intensive Cholecalciferol Monitoring and Supplementation on Serum vitD Levels in Pediatric Patients With CF
- N-Acetyl-Cysteine Increases Activity of Peanut-Shaped Gold Nanoparticles Against Biofilms Formed by Clinical Strains of Pseudomonas aeruginosa Isolated from Sputum of Cystic Fibrosis Patients
- Letter in Reply: Can We Justify Cystic Fibrosis Mutational Analysis among Omani Neonates?
- Can We Justify Cystic Fibrosis Mutational Analysis among Omani Neonates?
- Commentary: Filling in the cracks: How to improve survival for patients with cystic fibrosis
- Cough in adolescent with cystic fibrosis, from nightmare to COVID-19 stigma: A qualitative thematic analysis
- What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?
- Iron Deficiency in Cystic Fibrosis: A Cross-Sectional Single-Centre Study in a Referral Adult Centre
- Intestinal Absorption of Lipids Using a Pancreatic Enzyme-Free Nutritional Supplement in Patients with Cystic Fibrosis: A Randomized, Double-Blind, Crossover Pilot Trial
- Left behind: The potential impact of CFTR modulators on racial and ethnic disparities in cystic fibrosis
- Longitudinal lung clearance index and association with structural lung damage in children with cystic fibrosis
- Comparison of the Lung Clearance Index in Preschool Children with Primary Ciliary Dyskinesia and Cystic Fibrosis
- Comparison of continuous glucose monitoring in cystic fibrosis patients with or without pancreatic exocrine insufficiency
- CFTR suppresses neointimal formation through attenuating proliferation and migration of aortic smooth muscle cells
- Effects of Exercise on Nutritional Status in People with Cystic Fibrosis: A Systematic Review
- Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management
- Anticipating New Treatments for Cystic Fibrosis: A Global Survey of Researchers
- Methods of Sputum and Mucus Assessment for Muco-Obstructive Lung Diseases in 2022: Time to “Unplug” from Our Daily Routine!
- Proximity Profiling of the CFTR Interaction Landscape in Response to Orkambi
- CFTR, Cell Junctions and the Cytoskeleton
- Effectiveness of Video Games as Physical Treatment in Patients with Cystic Fibrosis: Systematic Review
- Evaluation of Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) in Cystic Fibrosis Subjects Without an F508del Mutation
- Reducing prescribing errors: making electronic prescribing work for cystic fibrosis inpatients
- The Prognosis of ART Is Not Altered in Cystic Fibrosis Women: A Case-Report Study
- Polycationic Glycopolymer Demonstrates Activity Against Persisters and Biofilms of Non-tuberculosis Mycobacteria Cystic Fibrosis Clinical Isolates in vitro
- Pseudomonas aeruginosa Affects Airway Epithelial Response and Barrier Function During Rhinovirus Infection
- Establishing Antimicrobial Susceptibility Testing Methods and Clinical Breakpoints for Inhaled Antibiotic Therapy
- Interleukin-1 beta is a potential mediator of airway nitric oxide deficiency in cystic fibrosis
- Impact of azithromycin on serum inflammatory markers in children with cystic fibrosis and new Pseudomonas
- Pilot Study of a CF-specific CBT Intervention for Adolescents
- Development and preliminary validation of the challenges of living with cystic fibrosis (CLCF) questionnaire: a 46-item measure of treatment burden for parent/carers of children with CF
- Benefits of reviewing pancreatic function in children with cystic fibrosis
- Safety of mRNA-based vaccines against SARS-CoV-2 in people with cystic fibrosis aged 12 years and over
- The clinical impact of the Covid-19 pandemic first wave on patients with cystic fibrosis in New York
- Receiving results of uncertain clinical relevance from population genetic screening: systematic review & meta-synthesis of qualitative research
- Association of Body Mass Index With Clinical Outcomes in Patients With Cystic Fibrosis: A Systematic Review and Meta-analysis
- Body Mass Index and Clinical Outcomes in Persons Living With Cystic Fibrosis-Is Bigger Always Better?
- CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages
- Tobramycin safety and efficacy review article
- Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro
- DMBT1 is upregulated in cystic fibrosis, affects ciliary motility, and is reduced by acetylcysteine
- “Be afraid – be very afraid”: Passive air drying of nebulizer parts in cystic fibrosis (CF) – Occult microbiological risks of contamination with Pseudomonas aeruginosa from calyptrate flies (Musca domestica &Calliphora vomitoria)
- Ethmoidal mucocele causing proptosis in a pediatric patient with cystic fibrosis: A case report
- The role of pharmacy services beyond cystic fibrosis: a case for support in childhood interstitial lung disease
- The changing landscape of pediatric lung transplantation
- Platelet CFTR inhibition enhances arterial thrombosis via increasing intracellular Cl- concentration and activation of SGK1 signaling pathway
- Achromobacter xylosoxidans Purulent Bronchitis in a Previously Healthy Child: An Unexpected Consequence of COVID-19 Infection
- Cystic Fibrosis Lung Transplant Recipients 10 years of age or Younger: Predisposing Factors for End-stage Disease
- Prevalence of antibiotic resistance of Pseudomonas aeruginosa in cystic fibrosis infection: A systematic review and meta-analysis
- 3.22 Nutrition in Cystic Fibrosis
- Elexacaftor, tezacaftor and ivacaftor: a case of severe rash and approach to desensitisation
- A Single-Cell Atlas of Large and Small Airways at Birth in a Porcine Model of Cystic Fibrosis
- The Burkholderia cenocepacia iron starvation σ factor, OrbS, possesses an on-board iron sensor
- Addition time plays a major role in the inhibitory effect of chitosan on the production of Pseudomonas aeruginosa virulence factors
- Patienthèque of Finisterian (South of Brittany) Children With Cystic Fibrosis in the Time of Precision Medicine
- Impact of Spiroware re-analysis method on multiple-breath washout outcomes in children with cystic fibrosis
- Risk Assessment for Patients with Chronic Respiratory Conditions in the Context of the SARS-CoV-2 Pandemic Statement of the German Respiratory Society with the Support of the German Association of Chest Physicians
- The impact of COVID-19 upon the delivery of exercise services within cystic fibrosis clinics in the United Kingdom
- Effectiveness of Different Eradication Treatment Protocols for New-Onset Pseudomonas aeruginosa in Children with Cystic Fibrosis
- AZD5634, an inhaled ENaC inhibitor, in healthy subjects and patients with cystic fibrosis
- Epigenetic regulation of inflammation by microRNAs in post-infectious bronchiolitis obliterans
- Functional interplay between CFTR and pendrin: physiological and pathophysiological relevance
- Impaired intestinal stem cell activity in ETEC infection: enterotoxins, cyclic nucleotides, and Wnt signaling
- Testicular cancer in men with cystic fibrosis
- Congenital Bilateral Absence of the Vas Deferens
- Burkholderia cepacia Complex Lumbar Spondylodiscitis: A Rare Nosocomial Infection
- Cystic fibrosis telemedicine in the era of COVID-19
- Staphylococcus aureus in Non-Cystic Fibrosis Bronchiectasis: Prevalence and Genomic Basis of High Inoculum Beta-Lactam Resistance
- Achromobacter spp. in a Cohort of Non-Selected Pre- and Post-Lung Transplant Recipients
- N-acetylcysteine (NAC) and Its Role in Clinical Practice Management of Cystic Fibrosis (CF): A Review
- Utilization of the Healthy Eating Index in Cystic Fibrosis
- Nutrition in Cystic Fibrosis-Some Notes on the Fat Recommendations
- Neutrophil Dysfunction in the Pathogenesis of Cystic Fibrosis
- Cystic fibrosis transmembrane conductance regulator function in patients with chronic pancreatitis
- Protein with negative surface charge distribution, Bnr1, shows characteristics of a DNA-mimic protein and may be involved in the adaptation of Burkholderia cenocepacia
- In Vivo Activity of Metal Complexes Containing 1,10-Phenanthroline and 3,6,9-Trioxaundecanedioate Ligands against Pseudomonas aeruginosa Infection in Galleria mellonella Larvae
- Inhibitor of Hyaluronic Acid Synthesis 4-Methylumbelliferone Suppresses the Secretory Processes That Ensure the Invasion of Neutrophils into Tissues and Induce Inflammation
- Respiratory and Peripheral Muscle Weakness and Body Composition Abnormalities in Non-Cystic Fibrosis Bronchiectasis Patients: Gender Differences
- Safety and Efficacy of Devices Delivering Inhaled Antibiotics among Adults with Non-Cystic Fibrosis Bronchiectasis: A Systematic Review and a Network Meta-Analysis
- Lung Ultrasound in Children with Cystic Fibrosis in Comparison with Chest Computed Tomography: A Feasibility Study
- Pulmonary Exacerbations in Pediatric Patients: Retrospective Study in a Portuguese Cystic Fibrosis Center
- SLC26A9 as a Potential Modifier and Therapeutic Target in Cystic Fibrosis Lung Disease
- Use of Intravenous Pulse Steroids to Treat Allergic Bronchopulmonary Aspergillosis in a Non-Compliant Asthmatic Adolescent
- Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study
- Factors Predisposing the Response to Lumacaftor/Ivacaftor in People with Cystic Fibrosis
- Novel Bacteriophages Show Activity against Selected Australian Clinical Strains of Pseudomonas aeruginosa
- The Arginine Catabolism-Derived Amino Acid l-ornithine Is a Chemoattractant for Pseudomonas aeruginosa
- Induction of Broad β-lactam Resistance in Achromobacter ruhlandii by Exposure to Ticarcillin Is Primarily Linked to Substitutions in Murein Peptide Ligase Mpl
- In vitro assessment of triple combination therapy for the most common disease-associated mutation in cystic fibrosis
- National Guidelines for the Performance of the Sweat Test in Diagnosis of Cystic Fibrosis on behalf of the Croatian Society of Medical Biochemistry and Laboratory Medicine and the Cystic Fibrosis Centre – Paediatrics and adults, University Hospital Centre Zagreb
- Prevalence and Impact of Rheumatologic Pain in Cystic Fibrosis Adult Patients
- Detection of DZIP1L mutations by whole-exome sequencing in consanguineous families with polycystic kidney disease
- A highly selective, cell-permeable furin inhibitor BOS-318 rescues key features of cystic fibrosis airway disease
- Natural killer cells kill extracellular Pseudomonas aeruginosa using contact-dependent release of granzymes B and H
- Serum anti-PAD4 autoantibodies are present in cystic fibrosis children and increase with age and lung disease severity
- Improvements in anthropometric measures and gastrointestinal tolerance in patients with cystic fibrosis by using a digestive enzyme cartridge with overnight enteral nutrition
- Bicarbonate transport of airway surface epithelia in luminally perfused mice bronchioles
- Neutrophil extracellular traps in chronic lung disease: implications for pathogenesis and therapy
- Age at menarche in girls with cystic fibrosis and asthma
- Collaborative research protocol to define patient-reported experience measures of the cystic fibrosis care pathway in France: the ExPaParM study
- Body functions and structure, activity, and participation limitations of adult cystic fibrosis patients under the international classification of functioning, disability, and health framework
- A Pandemic Lesson for Global Lung Diseases: Exacerbations are Preventable
- Cystic Fibrosis-Related Diabetes Mellitus and Pregnancy: A Retrospective Study
- Differential correlation network analysis of rectal transcriptomes reveals cystic fibrosis-related disturbance
- Gait and functional balance in non-CF bronchiectasis
- A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant
- Expression of cystic fibrosis lung disease modifier genes in human airway models
- An Open-label, Phase 1/2 Trial of Gene Therapy 4D-710 in Adults With Cystic Fibrosis
- Study of Satisfaction of Patients and Care Providers for an Alternate Follow-up With In-hospital Consults and Tele-consult for Patients With Cystic Fibrosis
- Two systematic cochrane reviews of the prevention and treatment of distal intestinal obstruction syndrome in cystic fibrosis
- How the sweat gland reveals levels of CFTR activity
- Repurposing the Veterinary Antibiotic Apramycin for Antibacterial and Antibiofilm Activity Against Pseudomonas aeruginosa From Cystic Fibrosis Patients
- Complete Genome Sequencing and Comparative Analysis of the Clinically-Derived Apiotrichum mycotoxinivorans Strain GMU1709
- Cystic fibrosis in the 21st century: what every radiologist should know
- Cystic fibrosis: candidate selection and impact of the cystic fibrosis transmembrane conductance regulator therapy
- Development of a novel rapamycin loaded nano- into micro-formulation for treatment of lung inflammation
- Parenthood impacts short-term health outcomes in people with cystic fibrosis
- Chest x-rays are less sensitive than multiple breath washout examinations when it comes to detecting early cystic fibrosis lung disease
- Alkalosis-induced hypoventilation in cystic fibrosis: The importance of efficient renal adaptation
- Commensal Bacteria in the Cystic Fibrosis Airway Microbiome Reduce P. aeruginosa Induced Inflammation
- Outcomes and survival following lung transplantation in non-cystic fibrosis bronchiectasis
- Controlled inhalation improves central and peripheral deposition in cystic fibrosis patients with moderate lung disease
- The Effect Of Telerehabilitation On Quality Of Life, Anxiety And Depression In Children With Cystic Fibrosis And Caregivers: A Single-Blind Randomized Trial
- Neutrophil-derived extracellular vesicles promote feed-forward inflammasome signaling in cystic fibrosis airways
- High affinity iron uptake by pyoverdine in Pseudomonas aeruginosa involves multiple regulators besides Fur, PvdS, and FpvI
- Everything in excess is opposed to nature, even vitamin D: a case report
- Mutation profiling of the c.1521_1523delCTT (p.Phe508del, F508del) CFTR allele using haplotype-resolved long-read next generation sequencing
- Men’s sexual and reproductive health in cystic fibrosis in the era of highly effective modulator therapies-A qualitative study
- CFTR modulator therapy alters plasma sphingolipid profiles in people with cystic fibrosis
- Clinical outcomes in cystic fibrosis at 6 years of age with tricare insurance coverage
- CONCERNS FOR PEOPLE WITH CYSTIC FIBROSIS (PWCF) WHEN TRAVELLING PRE COVID-19
- A Survey: Understanding the Health and Perspectives of People with CF Not Benefiting from CFTR Modulators
- Managing Pulmonary Infection in Adults With Cystic Fibrosis: Adult Cystic Fibrosis Series
- Feasibility of Home-based Exercise Program for Adults With Cystic Fibrosis
- Inflammation, Fibrosis and Cancer: Mechanisms, Therapeutic Options and Challenges
- Intestinal Inflammation and Alterations in the Gut Microbiota in Cystic Fibrosis: A Review of the Current Evidence, Pathophysiology and Future Directions
- Assays of CFTR Function In Vitro, Ex Vivo and In Vivo
- Airway Delivery of Hydrogel-Encapsulated Niclosamide for the Treatment of Inflammatory Airway Disease
- Unprecedented Epimerization of an Azithromycin Analogue: Synthesis, Structure and Biological Activity of 2′-Dehydroxy-5″-Epi-Azithromycin
- How can the transition of adolescents from a children’s to an adult CF center be improved? Analysis of adolescents’ and parents’ needs during the post-transfer period
- Guanylin and uroguanylin: a promising nexus in intestinal electrolyte and fluid homeostasis
- CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system
- Precision Medicine Based on CFTR Genotype for People with Cystic Fibrosis
- REAL-world clinical effectiveness of ivacaftor therapy in the first 24 months in two infants with cystic fibrosis and different gating mutations-A case report
- Biocompatible antimicrobial colistin loaded calcium phosphate nanoparticles for the counteraction of biofilm formation in cystic fibrosis related infections
- Investigation of respiratory rate in patients with cystic fibrosis using a minimal-impact biomotion system
- Cystic Fibrosis and Sleep Circadian Rhythms
- A Qualitative Exploration into Experiences and Attitudes Regarding Psychosocial Challenges, Self-compassion, and Mindfulness in a Population of Adults with Cystic Fibrosis
- Chasing a Breath of Fresh Air in Cystic Fibrosis (CF): Therapeutic Potential of Selective HDAC6 Inhibitors to Tackle Multiple Pathways in CF Pathophysiology
- Changes in Care during the COVID-19 Pandemic for People with Cystic Fibrosis
- The rapid reduction of infection-related visits and antibiotic use among people with cystic fibrosis after starting Elexacaftor-Tezacaftor-Ivacaftor
- Editorial: Functional Characterization and Pharmaceutical Targets in Common and Rare CFTR Dysfunctions
- Dynamic Upper and Lower Airway Microbiotas in Paediatric Bronchiectasis Exacerbations: A Pilot Study
- The P2X7 Receptor in Cystic Fibrosis Monocytes: Linking CFTR Deficiency to Inflammation
- Towards a new definition of non-cystic fibrosis bronchiectasis
- Continuing Medical Education Questions: December 2021
- Integrative pan cancer analysis reveals the importance of CFTR in lung adenocarcinoma prognosis
- Implementing the use of objective medication adherence data in routine clinical practice via the digital CFHealthHub platform: situation analysis and strategy development using the theoretical domains framework
- Is Positive Expiratory Pressure Physiotherapy an Effective Method for Airway Clearance in People With Cystic Fibrosis? A Cochrane Review Summary With Commentary
- Hypervitaminosis A with fulminant secondary intracranial hypertension following personalized medicine-based Elexacaftor/Tezacaftor/Ivacaftor initiation in a preadolescent with cystic fibrosis
- Pancreatic Insufficiency in a Child With p.Gly542* and c.2657+5G>A Heterozygote CFTR : A Case Report
- Relationship Between the Development of Impaired Glucose Tolerance, the Phenotype of CFLD, and the Risk of Liver Fibrosis
- Worldwide rates of diagnosis and effective treatment for cystic fibrosis
- A Hyperglycemic Microenvironment Inhibits Tendon-to-Bone Healing through the let-7b-5p/CFTR Pathway
- Seasonal variation of lung function in cystic fibrosis: longitudinal modeling to compare a Midwest US cohort to international populations
- Influence of Comorbidities and Airway Clearance on Mortality and Outcomes of Patients With Severe Bronchiectasis Exacerbations in Taiwan
- Engaging Stakeholders in the Development of a Reproductive Goals Decision AID for Women with Cystic Fibrosis
- Leveraging TOPMed imputation server and constructing a cohort-specific imputation reference panel to enhance genotype imputation among cystic fibrosis patients
- Study to Assess the Safety and PK of Oral and IV Xenleta in Adults With Cystic Fibrosis
- Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis
- Synthesis and bioactivity of readily hydrolysable novel cationic lipids for potential lung delivery application of mRNAs
- Human papillomavirus (HPV) and cervical dysplasia in adult female cystic fibrosis (CF) lung transplant recipients
- Preliminary method for profiling volatile organic compounds in breath that correlate with pulmonary function and other clinical traits of subjects diagnosed with cystic fibrosis: a pilot study
- Genetic analysis and intracytoplasmic sperm injection outcomes of Chinese patients with congenital bilateral absence of vas deferens
- Gastroparesis in Cystic Fibrosis
- Static mucus impairs bacterial clearance and allows chronic infection with Pseudomonas aeruginosa in the cystic fibrosis rat
- Identification and selection of healthy spermatozoa in heterozygous carriers of the Phe508del-variant of the CFTR-gene in assisted reproduction
- Nutrition management in adults with cystic fibrosis
- When is cystic fibrosis not cystic fibrosis? The importance of appropriately classifying patients
- Genomic diversity and antimicrobial resistance of Prevotella species isolated from chronic lung disease airways
- Porphyromonas pasteri and Prevotella nanceiensis in the sputum microbiota are associated with increased decline in lung function in individuals with cystic fibrosis
- Hybrid Lipid/Polymer Nanoparticles to Tackle the Cystic Fibrosis Mucus Barrier in siRNA Delivery to the Lungs: Does PEGylation Make the Difference?
- Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype
- The Predictive Role of Psychological Status and Disease Severity Indexes on Quality of Life Among Patients with Non-CF Bronchiectasis
- Genetic and Clinical Demographics of Adult Cystic Fibrosis Patients in a Middle Eastern Population
- Maternal and Fetal Problems in Patients with Non-Cystic Fibrosis Bronchiectasis During Pregnancy
- The Frequency of Obstructive Sleep Apnea in Patients with Non-cystic Fibrosis Bronchiectasis
- Association Between Cystic Fibrosis Severity Markers and CFTR Genotypes in Turkish Children
- Food Insecurity and Mental Health During the COVID-19 Pandemic in Cystic Fibrosis Households
- New concepts in the pathogenesis of cystic fibrosis-related diabetes
- Measuring the effect of elexacaftor/tezacaftor/ivacaftor combination therapy on the respiratory pump in people with CF using dynamic chest radiography
- Functionally Additive Fixed Positive and Negative Charges in the CFTR Channel Pore Control Anion Binding and Conductance
- The Success of the Cystic Fibrosis Registry of Turkey for Improvement of Patient Care
- Pseudomonas aeruginosa adaptation in cystic fibrosis patients increases C5a levels and promotes neutrophil recruitment
- Early Growth Response 1 Suppresses Macrophage Phagocytosis by Inhibiting NRF2 Activation Through Upregulation of Autophagy During Pseudomonas aeruginosa Infection
- What Is Most Suitable for Children With Cystic Fibrosis-The Relationship Between Spirometry, Oscillometry, and Multiple Breath Nitrogen Washout
- Assessing the Utility of an Outpatient Exercise Program for Children With Cystic Fibrosis: A Quality Improvement Project
- Role of Salivary Biomarkers in Cystic Fibrosis: A Systematic Review
- Impaired Regulatory Volume Decrease and Characterization of Underlying Volume-Activated Currents in Cystic Fibrosis Human Cholangiocyte Cell Line
- The First 4 Years – Outcome of Children Identified by Newborn Screening for CF in Germany
- Generation of human induced pluripotent stem cells from cystic fibrosis patient carrying nonsense mutation (p.S308X) in CFTR gene
- PROBIOTIC USE IN ADULTS WITH CYSTIC FIBROSIS IS COMMON AND INFLUENCED BY GASTROINTESTINAL HEALTH NEEDS: A CROSS SECTIONAL SURVEY STUDY
- Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis
- Cystic fibrosis foundation otolaryngology care multidisciplinary consensus recommendations
- Evaluating the alginate oligosaccharide (OligoG) as a therapy for Burkholderia cepacia complex cystic fibrosis lung infection
- Bone mineral density screening by DXA for people with cystic fibrosis: A registry analysis of patient and program factors influencing rates of screening
- Radiomics derived morphological features predict pulmonary function response under lumacaftor-ivacaftor in patients with cystic fibrosis
- Forskolin-induced Organoid Swelling is Associated with Long-term CF Disease Progression
- Fecal Calprotectin and Phenotype Severity in Patients with Cystic Fibrosis: A Systematic Review and Meta-Analysis
- The Challenges of Eating Well for People Living with Cystic Fibrosis: an Interview Study Exploring the Use of Mindful Eating Approaches and Behaviours to Support Optimal Nutritional Status
- Pseudomonas aeruginosa cytochrome P450 CYP168A1 is a fatty acid hydroxylase that metabolizes arachidonic acid to the vasodilator 19-HETE
- Investigating Nontuberculous Mycobacteria Transmission at the Colorado Adult Cystic Fibrosis Program
- Clinical Phenotypes of Cystic Fibrosis Carriers
- Measuring the Impact of an Empiric Antibiotic Algorithm for Pulmonary Exacerbation in Children and Young Adults with Cystic Fibrosis
- Ultrasound-Derived Diaphragm Contractile Reserve as a Marker of Clinical Status in Patients With Cystic Fibrosis
- Roscovitine Worsens Mycobacterium abscessus Infection by Reducing DUOX2-Mediated Neutrophil Response
- Type 2 inflammation in cystic fibrosis: New insights
- Discovery of Novel Inhibitors of Uridine Diphosphate-N-Acetylenolpyruvylglucosamine Reductase (MurB) from Pseudomonas aeruginosa, an Opportunistic Infectious Agent Causing Death in Cystic Fibrosis Patients
- Combined Host- and Pathogen-Directed Therapy for the Control of Mycobacterium abscessus Infection
- Introducing a MAP for adherence care in the paediatric cystic fibrosis clinic: a multiple methods implementation study
- The lung microbiota in children with cystic fibrosis captured by induced sputum sampling
- Mycobacterium abscessus drug discovery using machine learning
- International Approaches to Management of CFTR-Related Metabolic Syndrome/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis
- Poly (acetyl, arginyl) glucosamine disrupts Pseudomonas aeruginosa biofilms and enhances bacterial clearance in a rat lung infection model
- Computational investigations on the potential role of hygrophorones as quorum sensing inhibitors against LasR protein of Pseudomonas aeruginosa
- Determinants of Daily Physical Activity Level in Patients With Cystic Fibrosis – Pilot Study Protocol
- Inhibiting Mycobacterium abscessus Cell Wall Synthesis: Using a Novel Diazabicyclooctane β-Lactamase Inhibitor To Augment β-Lactam Action
- Cystic Fibrosis: A Disease in Transformation, Yet More Work to be Done!
- Cytotoxic activity of Staphylococcus aureus isolates from a cohort of Mexican children with cystic fibrosis
- Mucoid Coating Provides a Growth Advantage to Pseudomonas aeruginosa at Oil-Water Interfaces
- Molecular Mechanisms of Staphylococcus and Pseudomonas Interactions in Cystic Fibrosis
- Sustained Reduction in Time to Data Entry in the Cystic Fibrosis Foundation Registry
- Molecular dynamics and functional characterization of I37R-CFTR lasso mutation provide insights into channel gating activity
- A flexible summary statistics-based colocalization method with application to the mucin cystic fibrosis lung disease modifier locus
- Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains
- Lived experiences of individuals with cystic fibrosis on CFTR-modulators
- Cystic fibrosis: a call for papers for ECFS 2022
- Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme
- Aquagenic wrinkling of the palms in cystic fibrosis patients treated with ivacaftor
- The Effect of Dynasore Upon the Negative Interaction Between ENaC and CFTR Channels in Xenopus laevis Oocytes
- Five cases of missed cystic fibrosis heterozygous mutations identified after a positive newborn screen on a sibling
- Treatment of cystic fibrosis related bone disease
- Weight loss and cystic disease progression in autosomal dominant polycystic kidney disease
- Yield of chest computed tomography angiogram in cystic fibrosis patients with suspected pulmonary embolism
- Lipid-driven CFTR clustering is impaired in CF and restored by corrector drugs
- Fat-Soluble Vitamins in Standard vs. Liposomal Form Enriched with Vitamin K2 in Cystic Fibrosis: A Randomized Multi-Center Trial
- Correction: Dagenais et al. Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review. J. Clin. Med. 2021, 10, 23
- First Case of COVID-19 Treated with Monoclonal Anti-Spike Antibodies in a Patient with Cystic Fibrosis in Romania
- Therapeutic CFTR Correction Normalizes Systemic and Lung-Specific S1P Level Alterations Associated with Heart Failure
- Activation of Notch3 in Renal Tubular Cells Leads to Progressive Cystic Kidney Disease
- The Use of Targeted Monoclonal Antibodies in the Treatment of ABPA-A Case Series
- Aerosol-Mediated Non-Viral Lung Gene Therapy: The Potential of Aminoglycoside-Based Cationic Liposomes
- The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis
- National multi-centre study found a low prevalence of severely impaired lung function in children and adolescents
- Case Report: Japanese Siblings of Cystic Fibrosis With a Novel Large Heterozygous Deletion in the CFTR Gene
- Review article: epidemiology, pathogenesis and management of liver disease in adults with cystic fibrosis
- Cellular and molecular architecture of submucosal glands in wild-type and cystic fibrosis pigs
- Formation of Calprotectin-Derived Peptides in the Airways of Children with Cystic Fibrosis
- Body Mass Index Recovery Following Lung Transplant for Cystic Fibrosis
- The physical therapy consultation: A qualitative study of the experience of parents of infants with cystic fibrosis in Australia
- Malignancies in patients with cystic fibrosis: a case series
- Simulated intravenous versus inhaled tobramycin with and without intravenous ceftazidime evaluated against hypermutable Pseudomonas aeruginosa via a dynamic biofilm model and mechanism-based modeling
- ADHERENCE TO INHALED THERAPIES OVER 4 YEARS IN PEOPLE WITH CYSTIC FIBROSIS
- Nasal epithelial cells as a gold-standard predictive model for personalized medicine in cystic fibrosis
- Development of Inhibitors of SAICAR Synthetase (PurC) from Mycobacterium abscessus Using a Fragment-Based Approach
- Metabolic reprogramming in a slowly developing orthologous model of polycystic kidney disease
- Challenging the paradigm: moving from umbrella labels to treatable traits in airway disease
- Cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)
- Progress in precision medicine in cystic fibrosis: a focus on CFTR modulator therapy
- Preferred health outcome states following treatment for pulmonary exacerbations of cystic fibrosis
- The role of inspiratory capacity and tidal flow in diagnosing exercise ventilatory limitation in Cystic Fibrosis
- The genetics of autosomal recessive polycystic kidney disease (ARPKD)
- Geographic distribution and phenotype of European people with cystic fibrosis carrying A1006E mutation
- Elevation of alkaline phosphatase and long-term drug therapy for cystic fibrosis
- Abdominal Surgical Procedures in Adult Patients with Cystic Fibrosis: What are the Risks?
- Bone Marrow Transplantation Rescues Monocyte Recruitment Defect and Improves Cystic Fibrosis in Mice
- Role and mechanism(s) of incretin-dependent therapies for treating diabetes mellitus
- An LC-MS/MS method for simultaneous analysis of the cystic fibrosis therapeutic drugs colistin, ivacaftor and ciprofloxacin
- Swyer-James syndrome: A cause of adult-onset dyspnea in a patient with adult polycystic kidney disease
- Contemporary N(2) and SF(6) multiple breath washout in infants and toddlers with cystic fibrosis
- Role of AxyABM overexpression in acquired resistance in Achromobacter xylosoxidans
- Successful non-surgical treatment of pseudomonas choroidal abscess in cystic fibrosis with previous double lung transplantation
- The future of dry powder inhaled therapy: Promising or Discouraging for systemic disorders?
- Advanced detection and sensing strategies of Pseudomonas aeruginosa and quorum sensing biomarkers: A review
- Bacterial low-abundant taxa are key determinants of a healthy airway metagenome in the early years of human life
- Cystic fibrosis related liver disease and endocrine considerations
- Multilocus Sequence Typing Reveals Extensive Genetic Diversity of the Emerging Fungal Pathogen Scedosporium aurantiacum
- Sequence diversity of the Pseudomonas aeruginosa population in loci that undergo microevolution in cystic fibrosis airways
- Stage-Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function
- Laparoscopic morphological aspects and tentative explanation of the aetiopathogenesis of isolated endometriosis of the sciatic nerve: a review based on 267 patients
- Trikafta Rescues CFTR and Lowers Monocyte P2X7R-Induced Inflammasome Activation in Cystic Fibrosis
- The effect of Control IQ hybrid closed loop technology on glycemic control in adolescents and adults with cystic fibrosis related diabetes
- Receptor-mediated activation of CFTR via prostaglandin signaling pathways in the airway
- The Changing Face of Cystic Fibrosis: An Update for Anesthesiologists
- Diagnostic Uncertainty, Microbes, and the Isolation of People with Cystic Fibrosis
- Vitamins A, D, E status as related to supplementation and lung disease markers in young children with cystic fibrosis
- Exon-skipping antisense oligonucleotides for cystic fibrosis therapy
- Open reading frame correction using splice-switching antisense oligonucleotides for the treatment of cystic fibrosis
- Environment in the lung of cystic fibrosis patients stimulates the expression of biofilm phenotype in Mycobacterium abscessus
- What Is Cystic Fibrosis?
- Annual decline in lung function in adults with sickle cell disease is similar to that observed in adults with cystic fibrosis
- Report of two events of nosocomial outbreak and pseudo-outbreak due to contamination with Achromobacter spp
- Trabecular bone score in people with cystic fibrosis
- Rare Trafficking CFTR Mutations Involve Distinct Cellular Retention Machineries and Require Different Rescuing Strategies
- Bicarbonate Transport in Cystic Fibrosis and Pancreatitis
- Rescue of Mutant CFTR Trafficking Defect by the Investigational Compound MCG1516A
- Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation
- Correlation between clinical-functional parameters and number of lobes involved in non-cystic fibrosis bronchiectasis
- Treatment of Polarized Cystic Fibrosis Airway Cells With HGF Prevents VX-661-Rescued F508del-CFTR Destabilization Caused by Prolonged Co-exposure to VX-770
- POINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? Yes
- COUNTERPOINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? No
- Revisiting a diagnosis of cystic fibrosis – Uncertainties and considerations
- Exploring provider attitudes and perspectives related to men’s health in cystic fibrosis
- Focusing on powder processing in Dry Powder Inhalation product development, manufacturing and performance
- Understanding motivation for Australian adolescents and young adults with cystic fibrosis: Modifiable factors to support self-management
- Mechanism of CFTR correction by type I folding correctors
- CFTR variants are associated with chronic bronchitis in smokers
- Scrutiny of Metal Ion Binding Sites in Different Alginate Lyases through In Silico Analysis
- CFTR deficiency aggravates Ang II induced vasoconstriction and hypertension by regulating Ca2+ influx and RhoA/Rock pathway in VSMCs
- What is the role of Achromobacter species in patients with cystic fibrosis?
- Cardiac involvement in cystic fibrosis evaluated using cardiopulmonary magnetic resonance
- Neutrophil respiratory burst activity is not exaggerated in cystic fibrosis
- Systems Approaches to Unravel Molecular Function: High-content siRNA Screen Identifies TMEM16A Traffic Regulators as Potential Drug Targets for Cystic Fibrosis
- The Pseudomonas aeruginosa homeostasis enzyme AlgL clears the periplasmic space of accumulated alginate during polymer biosynthesis
- Evaluation of the safety of cefepime prolonged infusions in pediatric patients with cystic fibrosis
- The Effects of Sub-inhibitory Antibiotic Concentrations on Pseudomonas aeruginosa : Reduced Susceptibility Due to Mutations
- CF and male health: Sexual and reproductive health, hypogonadism, and fertility
- Carbonic anhydrase and soluble adenylate cyclase regulation of cystic fibrosis cellular phenotypes
- An update on methods for assessing bone quality and health in Cystic fibrosis
- Gestational and pregestational diabetes in pregnant women with cystic fibrosis
- Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe
- Utilizing in silico and in vitro methods to identify possible binding sites of a novel ligand against Pseudomonas aeruginosa phospholipase toxin ExoU
- Cystic Fibrosis Transmembrane Conductance Regulator: Roles in Chronic Obstructive Pulmonary Disease
- Prenatal imaging features and perinatal outcomes of foetal volvulus-A literature review
- Culture and Imaging of Human Nasal Epithelial Organoids
- The nutritional environment is sufficient to select coexisting biofilm and quorum-sensing mutants of Pseudomonas aeruginosa
- Depressive symptoms in cystic fibrosis patients and their caretakers are best predicted by their respective sense of belonging
- An Overview on the Upper and Lower Airway Microbiome in Cystic Fibrosis Patients
- The role of genetic modifiers, inflammation and CFTR in the pathogenesis of Cystic fibrosis related diabetes
- Use of estrogen supplementation is associated with higher quality of life scores in women with cystic fibrosis
- Non-invasive tools for detection of liver disease in children and adolescents with cystic fibrosis
- Case report of compound CFTR variants in Korean siblings with cystic fibrosis: importance of differentiating cystic fibrosis from inflammatory bowel disease
- The sodium/glucose cotransporters as potential therapeutic targets for CF lung diseases revealed by human lung organoid swelling assay
- What’s in a name: The importance of lung transplant at Cystic Fibrosis Foundation Accredited Care Centers for patients with Cystic Fibrosis
- Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation
- Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis
- Lung nodules due to Candida parapsilosis in a person with cystic fibrosis
- Case Report: Embolization of the Left Atrial Coronary Artery in Cystic Fibrosis for Control of Massive Hemoptysis due to Coronary to Bronchial Artery Communication
- Esc peptides as novel potentiators of defective cystic fibrosis transmembrane conductance regulator: an unprecedented property of antimicrobial peptides
- Trends in Cystic Fibrosis survival over 40 years in South Africa: an observational cohort study
- Opportunity for pharmacogenomic testing in patients with cystic fibrosis
- RISK FACTORS FOR OBSTRUCTIVE SLEEP APNEA IN CYSTIC FIBROSIS
- Variable Susceptibility to Gallium Compounds of Major Cystic Fibrosis Pathogens
- Genetic counseling access for parents of newborns who screen positive for cystic fibrosis: consensus guidelines
- Pediatric Pulmonology 2020 Year in Review: Rare and Diffuse Lung Disease
- Six minute walk test in Italian children with cystic fibrosis aged 6 and 11
- Remotely Supervised Exercise for Adults With Cystic Fibrosis
- Cystic Fibrosis in Children: A Pediatric Anesthesiologist’s Perspective
- Deposition of Inhaled Levofloxacin in Cystic Fibrosis Lungs Assessed by Functional Respiratory Imaging
- Probing Allosteric Hsp70 Inhibitors by Molecular Modelling Studies to Expedite the Development of Novel Combined F508del CFTR Modulators
- Vitamin D Status in Pediatric and Young Adult Cystic Fibrosis Patients. Are the New Recommendations Effective?
- Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood
- Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial
- Safety and pharmacokinetics of Roscovitine (Seliciclib) in cystic fibrosis patients chronically infected with Pseudomonas aeruginosa, a randomized, placebo-controlled study
- Structural lung disease in preschool children with cystic fibrosis: An 18 month natural history study
- Comprehensive Analysis of Chemical Structures That Have Been Tested as CFTR Activating Substances in a Publicly Available Database CandActCFTR
- Long-term macrolide therapy in asthma
- Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies
- Portuguese adolescents with cystic fibrosis and their parents: An intervention proposal for nursing clinical practice
- Quality improvement and rapid PDSA cycles to maintain routine surveillance of pulmonary pathogens during the COVID-19 pandemic in a pediatric cystic fibrosis clinic
- Gamma-glutamyl transpeptidase-to-platelet ratio as a biomarker of liver disease and hepatic fibrosis severity in paediatric Cystic Fibrosis
- Clinical complications in children with false-negative results in cystic fibrosis newborn screening
- Bactericidal effects and stability of LL-37 and CAMA in the presence of human lung epithelial cells
- Specialized pro-resolving mediators in respiratory diseases
- A disorder clinically resembling cystic fibrosis caused by biallelic variants in the AGR2 gene
- Genome-Wide RNAi Screening Identifies Novel Pathways/Genes Involved in Oxidative Stress and Repurposable Drugs to Preserve Cystic Fibrosis Airway Epithelial Cell Integrity
- Standards of Nutritional Care for Patients with Cystic Fibrosis: A Methodological Primer and AGREE II Analysis of Guidelines
- New Auranofin Analogs with Antibacterial Properties against Burkholderia Clinical Isolates
- Potential Therapeutic Targets for Combination Antibody Therapy against Pseudomonas aeruginosa Infections
- A Polyclonal Antibody Raised against the Burkholderia cenocepacia OmpA-like Protein BCAL2645 Impairs the Bacterium Adhesion and Invasion of Human Epithelial Cells In Vitro
- Revisiting the Role of Leukocytes in Cystic Fibrosis
- High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids
- Immunoglobulin A Mucosal Immunity and Altered Respiratory Epithelium in Cystic Fibrosis
- Causes of Exocrine Pancreatic Insufficiency Other Than Chronic Pancreatitis
- Genetic Modifying Factors of Cystic Fibrosis Phenotype: A Challenge for Modern Medicine
- The Need for Precision Therapies as Determined by Genetic Signature for Cystic Fibrosis
- Prediction of Real-World Long-Term Outcomes of People with CF Homozygous for the F508del Mutation Treated with CFTR Modulators
- In Vitro Activity of 22 Antibiotics against Achromobacter Isolates from People with Cystic Fibrosis. Are There New Therapeutic Options?
- Evaluation of Three Culture Media for Isolation of Burkholderia cepacia Complex from Respiratory Samples of Patients with Cystic Fibrosis
- Revisiting CFTR Interactions: Old Partners and New Players
- Advanced but not mild liver disease is a predictor of decreased survival in children with cystic fibrosis, with far greater impact in females: A 27-year real-life cohort study
- Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward
- Outcomes following lung re-transplantation in patients with cystic fibrosis
- SARS-CoV-2 antibodies among people with cystic fibrosis prior to the vaccination campaign: A seroprevalence study in two specialized centres in Northern Italy
- Comparison of the airway microbiota in children with chronic suppurative lung disease
- Flagellin From Pseudomonas aeruginosa Modulates SARS-CoV-2 Infectivity in Cystic Fibrosis Airway Epithelial Cells by Increasing TMPRSS2 Expression
- Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial
- Comparison of biosimilar Tigerase and Pulmozyme in long-term symptomatic therapy of patients with cystic fibrosis and severe pulmonary impairment (subgroup analysis of a Phase III randomized open-label clinical trial (NCT04468100))
- MutTMPredictor: Robust and accurate cascade XGBoost classifier for prediction of mutations in transmembrane proteins
- Prospects of Inhaled Phage Therapy for Combatting Pulmonary Infections
- A Volatile and Dynamic Longitudinal Microbiome Is Associated With Less Reduction in Lung Function in Adolescents With Cystic Fibrosis
- Family planning, pregnancy and birth in women with lung conditions: a worldwide survey
- Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles
- Coming Up for Air: The Role of Anaerobes in Cystic Fibrosis
- Ceftaroline Versus Vancomycin for the Treatment of Acute Pulmonary Exacerbations of Cystic Fibrosis in Adults
- Health-Related Quality of Life Assessment: An Inexpensive Tool for Cystic Fibrosis Care
- Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis
- Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis
- Phosphodiesterase 8A Regulates CFTR Activity in Airway Epithelial Cells
- Cystic Fibrosis Mucus Model to Design More Efficient Drug Therapies
- Cystic fibrosis: current concepts
- The Impact of COVID-19 in Cystic Fibrosis
- The Management of Cystic Fibrosis Chronic Rhinosinusitis: An Evidenced-Based Review with Recommendations
- Comparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis
- Long-term macrolide treatment for non-cystic fibrosis bronchiectasis in children: a meta-analysis
- Mutations in CFTR genes are associated with oligoasthenospermia in infertile men undergoing IVF
- Impact of the COVID-19 pandemic: How our response is shaping the future of cystic fibrosis care
- Financial impacts of the COVID-19 pandemic on cystic fibrosis care: lessons for the future
- Financial impacts of the COVID-19 pandemic on cystic fibrosis care: Lessons for the future (Commentary) A.L. Stephenson
- Barriers and facilitators to implementing telehealth services during the COVID-19 pandemic: A qualitative analysis of interviews with cystic fibrosis care team members
- Design and methods for understanding the state of cystic fibrosis care amid the COVID-19 pandemic
- Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis
- Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis (Commentary)
- Patient and family experience of telehealth care delivery as part of the CF chronic care model early in the COVID-19 pandemic
- Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors
- Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network
- Evaluating barriers to and promoters of telehealth during the COVID-19 pandemic at U.S. cystic fibrosis programs
- The effect of the cystic fibrosis care center on outcomes after lung transplantation for cystic fibrosis
- Healthcare-associated links in transmission of nontuberculous mycobacteria among people with cystic fibrosis (HALT NTM) study: Rationale and study design
- Cystic fibrosis related diabetes (CFRD) prognosis
- Cystic fibrosis-related diabetes: The patient perspective
- A growing trend: CFTR modulators for cystic fibrosis lung transplant recipients
- Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers
- Low body mass index as a barrier to lung transplant in cystic fibrosis
- Magnetic resonance imaging of the gastrointestinal tract shows reduced small bowel motility and altered chyme in cystic fibrosis compared to controls
- Developing Ciprofloxacin Dry Powder for Inhalation: A Story of Challenges and Rational Design in the Treatment of Cystic Fibrosis Lung Infection
- Clinical Outcomes of Cystic Fibrosis Patients with Pseudomonas aeruginosa Bloodstream Infections
- Management of Multidrug Resistant Infections in Lung Transplant Recipients with Cystic Fibrosis
- Eating disorders and body image in cystic fibrosis
- The role of modulators in cystic fibrosis related diabetes
- Cystic fibrosis-related diabetes: Prevalence, screening, and diagnosis
- Impact of Achromobacter spp. Isolation on Clinical Outcomes in Children with Cystic Fibrosis
- Evaluation of a novel CFTR potentiator in copd ferrets with acquired cftr dysfunction
- The effect of danusertib, an Aurora kinase inhibitor, onto the cytotoxicity, cell cycle and apoptosis in pancreatic ductal adenocarcinoma cells
- Perception, experience and relationship with food and eating in adults with cystic fibrosis
- Gene mutations in congenital bilateral absence of the vas deferens: An update
- Cross-talk of inflammatory mediators and airway epithelium reveals the cystic fibrosis transmembrane conductance regulator as a major target
- Acute ST-elevation myocardial infarction in two young women with cystic fibrosis and cystic fibrosis-related diabetes
- Autogenic drainage for airway clearance in cystic fibrosis
- Major Decrease in Lung Transplantation for Patients with Cystic Fibrosis in France
- The Controversy of Drug Hypersensitivity in Patients with Cystic Fibrosis and Review of the Literature
- Olfactory dysfunction in people with cystic fibrosis with at least one copy of F508del
- Circadian Rhythmicity in Cerebral Microvascular Tone Influences Subarachnoid Hemorrhage-Induced Injury
- The journal of cystic fibrosis’ 20th anniversary
- Home monitoring for cystic fibrosis: The future is now
- The Relationship Between CFTR Gene Mutations and Exercise Capacity
- Cystic fibrosis associated liver disease in children
- Food protein-induced enterocolitis syndrome in a patient with pseudo-Bartter syndrome associated with cystic fibrosis. A case report
- Combining Ivacaftor and Intensive Antibiotics Achieves Limited Clearance of Cystic Fibrosis Infections
- Increased susceptibility of cystic fibrosis airway epithelial cells to ferroptosis
- Forewarned is forearmed: The cardiovascular time bomb in Cystic Fibrosis
- Meteorological Factors Influence the Presence of Fungi in the Air; A 14-Month Surveillance Study at an Adult Cystic Fibrosis Center
- Patient and Provider Experience With Cystic Fibrosis Telemedicine Clinic
- Cystic Fibrosis: Systems Biology Analysis from Homozygous p.Phe508del Variant Patients’ Samples Reveals Perturbations in Tissue-Specific Pathways
- Genetic determinants of virulence and antibiotic resistance are common for Pseudomonas aeruginosa ST235 isolates from cystic fibrosis patients from various geographical regions
- In vitro differentiation of ciliated cells in ALI-cultured human airway epithelium – The framework for functional studies on airway differentiation in ciliopathies
- Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis
- Hepatic inflammation and liver fibrogenesis: a potential target for the treatment of cystic echinococcosis-associated hepatic injury
- Real-World Outcomes in Cystic Fibrosis Telemedicine Clinical Care in a Time of a Global Pandemic
- Evaluation of Long-term Safety and Efficacy of ELX/TEZ/IVA in Cystic Fibrosis (CF) Participants 2 Years and Older
- Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis
- CFTR mutational screening by next-generation sequencing reveals novel variants and a high carrier rate in a Middle Eastern population
- Clinical validation of digital biomarkers for pediatric patients with asthma and cystic fibrosis – Potential for clinical trials and clinical care
- Synergy in Cystic Fibrosis Therapies: Targeting SLC26A9
- Defining the Importance of Age-Related Changes in Drug Clearance to Optimizing Aminoglycoside Dosing Regimens for Adult Patients with Cystic Fibrosis
- Environmental predictors of pulmonary nontuberculous mycobacteria (NTM) sputum positivity among persons with cystic fibrosis in the state of Florida
- Role of the flagellar hook in the structural development and antibiotic tolerance of Pseudomonas aeruginosa biofilms
- Nebulisation therapy in patients with cystic fibrosis – consensus of the Polish Cystic Fibrosis Society
- Magnetic resonance imaging of cystic fibrosis: Multi-organ imaging in the age of CFTR modulator therapies
- A new path for CF clinical trials through the use of historical controls
- The safety and sustainability of bottle-pep therapy in pediatric patients with cystic fibrosis
- Transcriptome analysis of Pseudomonas aeruginosa biofilm infection in an ex vivo pig model of the cystic fibrosis lung
- Goblet Cell Hyperplasia is not Epithelial-Autonomous in the Cftr Knockout Intestine
- Translating in vitro CFTR rescue into small molecule correctors for cystic fibrosis using the Library of Integrated Network-based Cellular Signatures drug discovery platform
- Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes
- Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes. Reply
- Comparison of endoscopic sinus surgery timing in lung transplant patients with cystic fibrosis
- Frequency of CFTR variants in southern Brazil and indication for modulators therapy in patients with cystic fibrosis
- Clinical course and risk factors for severe COVID-19 among Italian patients with cystic fibrosis: a study within the Italian Cystic Fibrosis Society
- Not a wild goose chase: long-lasting MRSA negative status following eradication therapy for chronic MRSA infection in patients with cystic fibrosis
- Infant BMI or Weight-for-Length and Risk of Undernutrition in Childhood Among Children with Cystic Fibrosis
- The Effect of Telerehabilitation on Functional Capacity, Oxidative Stress and Respiratory Parameters in Cystic Fibrosis
- Molecular pathology of the R117H cystic fibrosis mutation is explained by loss of a hydrogen bond
- Endobronchial Therapy With Gentamicin and Dexamethasone After Airway Clearance by Bronchoscopy in Exacerbation of Non-Cystic Fibrosis Bronchiectasis: A Real-World Observational Study
- Editorial: Novel Anti-Inflammatory Approaches for Cystic Fibrosis Lung Disease: Identification of Molecular Targets and Design of Innovative Therapies
- Acidic Microenvironment Determines Antibiotic Susceptibility and Biofilm Formation of Pseudomonas aeruginosa
- miR-224-5p and miR-545-5p Levels Relate to Exacerbations and Lung Function in a Pilot Study of X-Linked MicroRNA Expression in Cystic Fibrosis Monocytes
- Impaired glucose tolerance and indeterminate glycemia in cystic fibrosis
- Obesity in cystic fibrosis
- Long-Term Outcomes in Real Life of Lumacaftor-Ivacaftor Treatment in Adolescents With Cystic Fibrosis
- Incidence of breast cancer in people with cystic fibrosis: A cause for concern?
- Development and evaluation of a virtual patient-centered outcomes research training program for the cystic fibrosis community
- Sweat metabolomics before and after intravenous antibiotics for pulmonary exacerbation in people with cystic fibrosis
- Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis
- Synchronous association of hepatocellular carcinoma and cystic echinococcosis with unusual pathology: report on diagnostic and pathological variability
- Diagnosis and management of non-cystic fibrosis bronchiectasis
- Burden of cystic fibrosis in children <12 years of age prior to the introduction of CFTR modulator therapies
- Primary nasal epithelial cells from patients with cystic fibrosis hold promise for guiding precision medicine and expanding treatment
- S-nitrosothiols signaling in cystic fibrosis airways
- A Comparative Analysis of Pricing and Reimbursement of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Europe
- Might Routine Vitamin A Monitoring in Cystic Fibrosis Patients Reduce Virus-Mediated Lung Pathology?
- Editorial: Fungal Respiratory Infections in Cystic Fibrosis
- Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients
- Non-liver mRNA Delivery
- The Genetic Analysis of Cystic Fibrosis Patients With Seven Novel Mutations in the CFTR Gene in the Central Anatolian Region of Turkey
- Non-Pharmaceutical Techniques for Obstructive Airway Clearance Focusing on the Role of Oscillating Positive Expiratory Pressure (OPEP): A Narrative Review
- 25-Hydroxy vitamin D and body composition are associated with pulmonary function in non-cystic fibrosis bronchiectasis: A cross-sectional study
- Association of site of treatment with clinical outcomes following intravenous antimicrobial treatment of a pulmonary exacerbation
- Disconcerting and Counter-Intuitive Findings from a Trial of Exercise in Cystic Fibrosis- Can Exercise Make our Patients Worse?
- Inquilinus limosus isolated from a patient with chronic cystic fibrosis. First report in Mexico and evidence that co-infection with Pseudomonas aeruginosa promotes the accelerated and increased formation of extracellular neutrophil traps
- Nonspecific binding of common anti-CFTR antibodies in ciliated cells of human airway epithelium
- Effects of Training Intensity on Physical Fitness and Body Composition in Cystic Fibrosis
- Clinical characteristics and outcome of SARS -CoV-2 infection in patients with cystic fibrosis managed at home
- Ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis
- Delayed HIV diagnosis in a cystic fibrosis patient: Not just another exacerbation
- Gynecologic health care for females with cystic fibrosis
- Continuous Glucose Monitoring and HbA1c in Cystic Fibrosis: Clinical Correlations and Implications for CFRD Diagnosis
- The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis post-liver transplant: a case series
- CFTR limits F-actin formation and promotes morphological alignment with flow in human lung microvascular endothelial cells
- Mycobacterium abscessus, a model of resistance to multiple antibiotic classes
- A step back in time: The basics of CF care still matter!
- Perceptions of barriers to and facilitators of physical activity in adults with cystic fibrosis
- Molecular epidemiology and phylogenomic analysis of Mycobacterium abscessus clinical isolates in an Asian population
- Emotional Impact of COVID-19 Pandemic on Adults with Cystic Fibrosis
- Validity and reliability of the one-minute sit-to-stand test for the measurement of cardio-respiratory responses in children with cystic fibrosis
- Cystic fibrosis: a diagnosis in an adolescent
- Continuous glucose monitoring systems for monitoring cystic fibrosis-related diabetes
- Validation of a Custom Next-Generation Sequencing Assay for Cystic Fibrosis Newborn Screening
- Is mHealth a useful therapy for improving physical or emotional health in adolescents with cystic fibrosis? A systematic review
- Urticaria multiforme-like eruption due to a novel agent elexacaftor/tezacaftor/ivacaftor in a pediatric patient with cystic fibrosis
- Transplantation of human iPS cell-derived airway cells on vitrigel membrane into rat nasal cavity
- Collection, Expansion, and Differentiation of Primary Human Nasal Epithelial Cell Models for Quantification of Cilia Beat Frequency
- Cystic Fibrosis Newborn Screening: Five-year Experience from a Tertiary Care Center
- Assessment of body composition in cystic fibrosis: agreement between skinfold measurement and densitometry
- The bitter end: T2R bitter receptor agonists elevate nuclear calcium and induce apoptosis in non-ciliated airway epithelial cells
- Evaluation of Pulmonary Structure and Function in Patients with Cystic Fibrosis from Electrical Impedance Tomography Data
- Nano- and Macroscale Imaging of Cholesterol Linoleate and Human Beta Defensin 2-Induced Changes in Pseudomonas aeruginosa Biofilms
- Sub-Inhibitory Antibiotic Exposure and Virulence in Pseudomonas aeruginosa
- Integrity and Stability of PTC Bearing CFTR mRNA and Relevance to Future Modulator Therapies in Cystic Fibrosis
- The Effects of Aspergillus fumigatus Colonization on Lung Function in Patients with Cystic Fibrosis
- Lung and Gut Microbiota Changes Associated with Pseudomonas aeruginosa Infection in Mouse Models of Cystic Fibrosis
- Putative RNA Ligase RtcB Affects the Switch between T6SS and T3SS in Pseudomonas aeruginosa
- Methods Used to Evaluate the Immediate Effects of Airway Clearance Techniques in Adults with Cystic Fibrosis: A Systematic Review and Meta-Analysis
- miR-125b Promotes Colorectal Cancer Migration and Invasion by Dual-Targeting CFTR and CGN
- CFTR Protein: Not Just a Chloride Channel?
- TMEM16A/ANO1: Current Strategies and Novel Drug Approaches for Cystic Fibrosis
- The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
- Apparent Yield Stress of Sputum as a Relevant Biomarker in Cystic Fibrosis
- Impact of Airway Inflammation on the Efficacy of CFTR Modulators
- Employment Status and Work Ability in Adults with Cystic Fibrosis
- The Distribution and Role of the CFTR Protein in the Intracellular Compartments
- Identification of cbiO Gene Critical for Biofilm Formation by MRSA CFSa36 Strain Isolated from Pediatric Patient with Cystic Fibrosis
- Defective Glyoxalase 1 Contributes to Pathogenic Inflammation in Cystic Fibrosis
- Anti-Virulence Properties of Coridothymus capitatus Essential Oil against Pseudomonas aeruginosa Clinical Isolates from Cystic Fibrosis Patients
- Geospatial Analysis of Food Deserts and Their Impact on Health Outcomes in Children with Cystic Fibrosis
- Vitamin D Status and Risk of Cystic Fibrosis-Related Diabetes: A Retrospective Single Center Cohort Study
- Handgrip Strength: Associations with Clinical Variables, Body Composition, and Bone Mineral Density in Adults with Cystic Fibrosis
- Genetic Evaluation of Pancreatitis
- Genome evolution drives transcriptomic and phenotypic adaptation in Pseudomonas aeruginosa during 20 years of infection
- Predictors of mortality in children with cystic fibrosis in India
- Lung Transplant READY Pilot Study
- A restructuring of microbiome niche space is associated with Elexacaftor-Tezacaftor-Ivacaftor therapy in the cystic fibrosis lung
- Differentiation of Cystic Fibrosis-Related Pathogens by Volatile Organic Compound Analysis with Secondary Electrospray Ionization Mass Spectrometry
- Consideration of height-based tobramycin dosing regimens for the treatment of adult cystic fibrosis pulmonary exacerbations
- Optimising the care and quality of life of people with cystic fibrosis: the influence of cystic fibrosis transmembrane conductance regulator modulators
- Discovery of inhibition of Burkholderia cenocepacia, Pseudomonas aeruginosa and Stenotrophomonas maltophilia by the Brown Rot Basidiomycete Fungus, Postia placenta
- Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
- A Clinician’s guide to vitamin D supplementation for patients with cystic fibrosis
- The adverse association between stimulant use for attention deficit hyperactivity disorder (ADHD) and semen parameters
- Physical Activity and Sedentary Behavior in Adults With Cystic Fibrosis: Association With Aerobic Capacity, Lung Function, Sleep, Well-Being, and Quality of Life
- Parent-Collected Oropharyngeal Swabs in Cystic Fibrosis
- Outcomes of Cystic Fibrosis Screening-Positive Infants With Inconclusive Diagnosis at School Age
- Transitioning to telehealth during the coronavirus disease 2019 pandemic: Perspectives from partners of women with cystic fibrosis and healthcare providers
- Effect of Salbutamol on Lung Ventilation in Children with Cystic Fibrosis: Comprehensive Assessment Using Spirometry, Multiple-Breath Washout, and Functional Lung Magnetic Resonance Imaging
- Intravenous or oral antibiotic treatment in adults and children with cystic fibrosis and Pseudomonas aeruginosa infection: the TORPEDO-CF RCT
- Hereditary Pancreatitis Related to SPINK-1 Mutation. Is There an Increased Risk of Developing Pancreatic Cancer?
- Organic acids and their salts potentiate the activity of selected antibiotics against Pseudomonas aeruginosa biofilms grown in a synthetic cystic fibrosis sputum medium
- Body composition in individuals with cystic fibrosis
- Fertility preservation in women with cystic fibrosis pre-lung transplantation: A mixed methods study
- Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes
- Facilitators and barriers to empowerment in children and young people with cystic fibrosis: a meta-synthesis of the qualitative literature
- Urinary sodium/creatinine ratio is a predictor for fractional sodium excretion and related to age in patients with cystic fibrosis
- Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis
- Approach and anesthetic management for kidney transplantation in a patient with bilateral lung transplantation: case report
- Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype
- Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip: Cystic fibrosis airway chip
- Measurements of spontaneous CFTR-mediated ion transport without acute channel activation in airway epithelial cultures after modulator exposure
- Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis
- Surgical treatment of non-cystic fibrosis bronchiectasis in Central Europe
- Right hepatectomy with a cholangiojejunostomy and hepaticojejunostomy for unilobar Caroli’s syndrome
- Small-molecule drugs for cystic fibrosis: Where are we now?
- A Phase 2a randomized, single-center, double-blind, placebo-controlled study to evaluate the safety and preliminary efficacy of oral iOWH032 against cholera diarrhea in a controlled human infection model
- Aspergillus-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?
- Abdominal Symptoms Assessed with the CFAbd-Score are Associated with Intestinal Inflammation in Patients with Cystic Fibrosis
- An empirical comparison of segmented and stochastic linear mixed effects models to estimate rapid disease progression in longitudinal biomarker studies
- Erratum to: Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance
- Membrane Transporters of the Major Facilitator Superfamily Are Essential for Long-Term Maintenance of Phenotypic Tolerance to Multiple Antibiotics in E. coli
- Management of Cystic Fibrosis during COVID-19: Patient Reported Outcomes based remote follow-up among CF patients in Denmark – A feasibility study
- Clinical Effectiveness of Elexacaftor/Tezacftor/Ivacaftor in People with Cystic Fibrosis
- Translation, cross-cultural adaptation and psychometric evaluation of the Brazilian version of the Cystic Fibrosis Knowledge Scale (CFKS)
- CyFi-MAP: an interactive pathway-based resource for cystic fibrosis
- Complete CFTR gene sequencing in 5,058 individuals with cystic fibrosis informs variant-specific treatment
- Nontypeable Haemophilus influenzae infection impedes Pseudomonas aeruginosa colonization and persistence in mouse respiratory tract
- Conjugation of Silver Nanoparticles with De Novo Engineered Cationic Antimicrobial Peptides: An Exploratory Proposal
- Unique Features of Mycobacterium abscessus Biofilms Formed in Synthetic Cystic Fibrosis Medium
- Circulating Fibrocyte Level in Children with Cystic Fibrosis
- Quality of home spirometry performance amongst adults with cystic fibrosis
- Emerging non-pulmonary complications for adults with cystic fibrosis
- Genetic Variation in ABCC4 and CFTR and Acute Pancreatitis during Treatment of Pediatric Acute Lymphoblastic Leukemia
- Lung Clearance Index in Children with Cystic Fibrosis during Pulmonary Exacerbation
- Comprehensive Analysis of Combinatorial Pharmacological Treatments to Correct Nonsense Mutations in the CFTR Gene
- Multi-Omics Study of Keystone Species in a Cystic Fibrosis Microbiome
- Human Recombinant DNase I (Pulmozyme® ) Inhibits Lung Metastases in Murine Metastatic B16 Melanoma Model That Correlates with Restoration of the DNase Activity and the Decrease SINE/LINE and c-Myc Fragments in Blood Cell-Free DNA
- Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge
- Cystic Fibrosis Transmembrane Conductance Regulator Folding Mutations Reveal Differences in Corrector Efficacy Linked to Increases in Immature Cystic Fibrosis Transmembrane Conductance Regulator Expression
- Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China
- Telemedicine in cystic fibrosis
- Final results of the southwest German pilot study on cystic fibrosis newborn screening – Evaluation of an IRT/PAP protocol with IRT-dependent safety net: Results of the Southwest German CFNBS pilot study
- SPLUNC1 comes of age? Predicting acute exacerbations in cystic fibrosis
- Epigenetic reprogramming of airway macrophages promotes polarization and inflammation in muco-obstructive lung disease
- The challenges of managing thoracic pain in cystic fibrosis (CF)
- Expanding clinical phage microbiology: simulating phage inhalation for respiratory tract infections
- Predicting mortality in non-cystic fibrosis bronchiectasis patients using distance-saturation product
- Correlating genotype with phenotype using CFTR-mediated whole-cell Cl- currents in human nasal epithelial cells
- Unexpected associations between respiratory viruses and bacteria with Pulmonary Function Testing in children suffering from Cystic Fibrosis (MUCOVIB study)
- When CFSPID becomes CF
- CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the beta-adrenergic sweat rate assay
- No drug-drug interaction between tezacaftor-ivacaftor and clofazimine: A case report
- Methodological tools to study species of the genus Burkholderia
- CFTR mutations causing congenital unilateral absence of the vas deferens (CUAVD) and congenital absence of the uterus (CAU) in a consanguineous family
- The effectiveness of exercise interventions to increase physical activity in Cystic Fibrosis: A systematic review
- Using negative control outcomes and difference-in-differences to estimate treatment effects in an entirely treated cohort: the effect of ivacaftor in cystic fibrosis
- Exploring Associations Between Self-Compassion, Self-Criticism, Mental Health, and Quality of Life in Adults with Cystic Fibrosis: Informing Future Interventions
- An Overview of Noninvasive Ventilation in Cystic Fibrosis
- Spotlighting “Neutrophil Elastase Triggers the Release of Macrophage Extracellular Traps”: A New Catch in CF?
- The determinants of survival among adults with cystic fibrosis-a cohort study
- Spontaneous and iatrogenic hypoglycemia in cystic fibrosis
- Altered Pseudomonas Strategies to Inhibit Surface Aspergillus Colonies
- spa Typing and Molecular Characterization of Antimicrobial Resistance in Staphylococcus aureus Strains from Patients with Cystic Fibrosis
- Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy
- Fenretinide inhibits vitamin A formation from β-carotene and regulates carotenoid levels in mice
- The Epidemiology and Pathogenesis and Treatment of Pseudomonas aeruginosa Infections: An Update
- Lung ultrasound for the diagnosis of cystic fibrosis pulmonary exacerbation
- A Study Evaluating the Safety of Elexacaftor/Tezacaftor/Ivacaftor in Participants With Cystic Fibrosis (CF)
- The Immunomodulatory Effects of Macrolide Antibiotics in Respiratory Disease
- Frequency of allele variations in the CFTR gene in a Mexican population