- Elevation of Sweat Chloride Levels in a Patient with CFTR-Related Metabolic Syndrome Receiving Dexmethylphenidate and Guanfacine
- Changes in share wave elastography after Lumacaftor/Ivacaftor treatment in children with cystic fibrosis
- The first report on CFTR mutations of meconium ileus in cystic fibrosis population in Saudi Arabia: A single center review
- The first report on the association of celiac disease and cystic fibrosis in a tertiary care center in Saudi Arabia
- Muc5b Contributes to Mucus Abnormality in Rat Models of Cystic Fibrosis
- Ultrasound-guided placement of Long Peripheral Cannula in children with Cystic Fibrosis
- Opposite regulation of F508del-CFTR biogenesis by four poly-lysine ubiquitin chains In vitro
- An Update on CFTR Modulators as New Therapies for Cystic Fibrosis
- Host and pathogen response to bacteriophage engineered against Mycobacterium abscessus lung infection
- Prevalence of Post-Glucose Challenge Hypoglycemia in Adult Patients With Cystic Fibrosis and Relevance to the Risk of Cystic Fibrosis-Related Diabetes
- Development and Evaluation of a Pharmacist-Driven Vitamin D Protocol for a Cystic Fibrosis Clinic
- CFTR Modulator Therapy in an Individual With Cystic Fibrosis Caused by a N1303K CFTR Variant and Infected With Mycobacterium abscessus
- CHST11-mediated microenvironment events contribute to pulmonary fibrosis and cancer progression
- Constipation in Children and Adolescents: Evaluation and Treatment
- Model-based Bayesian inference of the ventilation distribution in patients with Cystic Fibrosis from multiple breath washout, with comparison to ventilation MRI
- In vitro activity of ceragenins against Burkholderia cepacia complex
- Identification of Key Factors for Anoxic Survival of B. cenocepacia H111
- Giving Drugs a Second Chance: Antibacterial and Antibiofilm Effects of Ciclopirox and Ribavirin against Cystic Fibrosis Pseudomonas aeruginosa Strains
- Novel CFTR Activator Cact-3 Ameliorates Ocular Surface Dysfunctions in Scopolamine-Induced Dry Eye Mice
- Surface Hydration Protects Cystic Fibrosis Airways from Infection by Restoring Junctional Networks
- Positively Charged Gold Quantum Dots: An Nanozymatic “Off-On” Sensor for Thiocyanate Detection
- A Compositional Analysis of Physical Activity, Sedentary Time, and Sleep and Associated Health Outcomes in Children and Adults with Cystic Fibrosis
- Renal Function in Patients with Cystic Fibrosis: A Single-Center Study
- Magnesium Status and Calcium/Magnesium Ratios in a Series of Cystic Fibrosis Patients
- CFTR dysfunction and targeted therapies: A vision from non-cystic fibrosis bronchiectasis and COPD
- Loss of Endothelial CFTR Drives Barrier Failure and Edema Formation in Lung Infection and Can Be Targeted by CFTR Potentiation
- VX770 Modulated Bicarbonate Conductance in CFTR-expressing FRT Cell
- Potent and selective inhibition of anion exchange activity of SLC26A9 by A9-301
- Kinetic Characterization of a Crystallized Putative Hydroxybutyrate Dehydrogenase from the Opportunistic Pathogen Burkholderia cenocepacia
- Pharmacological effect of pinostrobin on CFTR-mediated Cl- secretion and renal cyst development in in vitro model of polycystic kidney disease
- Loss of Club Cell Creb Mitigates IL-1B-mediated Muco-obstructive Phenotypes in Male Murine Airways
- Alternative Chloride Channels are Increased in the CFTR-/- Rat Airway
- Regulation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by the Nuclear Bile Acid Receptor, Farnesoid X Receptor
- Colistin Action Against Planktonic Pseudomonas aeruginosa
- Evaluation of Small Molecule Inhibitors of Pseudomonas Virulence factor LasB as Non-Traditional Immunotherapeutics
- In vitro model of pulmonary candidiasis for testing novel therapeutics
- Select Amino Acids Increase Anion Secretion in Human Bronchial Epithelial Cells (HBEC) with F508del and Nonsense Mutations: Alternative Approach to Restoring Airway Anion Secretion in Cystic Fibrosis
- Effect of Ciprofloxacin on Planktonic Pseudomonas aeruginosa
- The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Plays an Important Role in Fetal Human Colon Cell Migration and Proliferation
- Corneal opacification, an atypical presentation of cystic fibrosis: a case report and review of the literature
- In vitro Activity of Antivirulence Drugs Targeting the las or pqs Quorum Sensing Against Cystic Fibrosis Pseudomonas aeruginosa Isolates
- Adaptive Immune Response to Mycobacterium abscessus Complex (MABSC) in Cystic Fibrosis and the Implications of Cross-Reactivity
- Limitations of standard cost-effectiveness methods for health technology assessment of treatments for rare, chronic diseases: a case study of treatment for cystic fibrosis
- Taxonomic position, antibiotic resistance and virulence factor production by Stenotrophomonas isolates from patients with cystic fibrosis and other chronic respiratory infections
- Pseudomonas aeruginosa Alters Peptidoglycan Composition under Nutrient Conditions Resembling Cystic Fibrosis Lung Infections
- Atomic-scale interactions between quorum sensing autoinducer molecules and the mucoid P. aeruginosa exopolysaccharide matrix
- Telemedicine of patients with cystic fibrosis during the COVID-19 pandemic
- Optimization of a Liposomal DNase I Formulation with an Extended Circulating Half-Life
- Prevalence and subtyping of biofilms present in bronchoalveolar lavage from children with protracted bacterial bronchitis or non-cystic fibrosis bronchiectasis: a cross-sectional study
- Urinary bicarbonate and metabolic alkalosis during exacerbations in cystic fibrosis
- Potential Cross-Transmission of Mycobacterium abscessus among Non-Cystic Fibrosis Patients at a Tertiary Hospital in Japan
- Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles
- Tissue-localized immune responses in people with cystic fibrosis and respiratory nontuberculous mycobacteria infection
- Reducing exacerbations in children and adults with primary ciliary dyskinesia using erdosteine and/or azithromycin therapy (REPEAT trial): study protocol for a multicentre, double-blind, double-dummy, 2×2 partial factorial, randomised controlled trial
- Nephronophthisis: a pediatric case report
- Non-Cystic Fibrosis Bronchiectasis Increases the Risk of Lung Cancer Independent of Smoking Status
- Functional Evaluation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in the Endocervix
- Rapid Viscoelastic Characterization of Airway Mucus using a Benchtop Rheometer
- Primary Human Nasal Epithelial Cells: Biobanking in the Context of Precision Medicine
- Discovering Common Pathophysiological Processes between COVID-19 and Cystic Fibrosis by Differential Gene Expression Pattern Analysis
- Short-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor Combination on Glucose Tolerance in Young People With Cystic Fibrosis-An Observational Pilot Study
- Cancer in Cystic Fibrosis: Do Not Neglect Gynecologic Cancers
- Prostaglandin E2 : A Potential Link Between Cystic Fibrosis and Cancer
- [Translated article] Aquagenic Keratoderma: Treatment Update
- Caution advised in the use of CFTR modulator treatment for individuals harboring specific CFTR variants
- Anesthetic impacts on pulmonary function: Implications for cystic fibrosis
- Pathological remodeling of distal lung matrix in end-stage cystic fibrosis patients
- The effect of TGF-β1 polymorphisms on pulmonary disease progression in patients with cystic fibrosis
- The impact of FDA and EMA regulatory decision-making process on the access to CFTR modulators for the treatment of cystic fibrosis
- Amino Acids Improve Aerosolization and Chemical Stability of Potential Inhalable Amorphous Spray-dried Ceftazidime for Pseudomonas aeruginosa Lung Infection
- Systematic review on fecal calprotectin in cystic fibrosis
- Clinical outcomes of a large cohort of individuals with the F508del/5T;TG12 CFTR genotype
- Association between early respiratory viral infections and structural lung disease in infants with cystic fibrosis
- Alterations in innate immune responses of patients with chronic rhinosinusitis related to cystic fibrosis
- A dual center and dual vendor comparison study of automated perfusion-weighted phase-resolved functional lung magnetic resonance imaging with dynamic contrast-enhanced magnetic resonance imaging in patients with cystic fibrosis
- Susceptibility of Burkholderia cepacia Complex to Ceftazidime/Avibactam and Standard Drugs of Treatment for Cystic Fibrosis Patients
- Increased intracellular Cl- concentration mediates neutrophil extracellular traps formation in atherosclerotic cardiovascular diseases
- Blood mRNA biomarkers distinguish variable systemic and sputum inflammation at treatment initiation of inhaled antibiotics in cystic fibrosis: A prospective non-randomized trial
- Impacts of Nontuberculous Mycobacteria Isolates in Non-cystic Fibrosis Bronchiectasis: A 16-Year Cohort Study in Taiwan
- Sputum neutrophil elastase and its relation to pediatric bronchiectasis severity: A cross-sectional study
- Chronic cough in cystic fibrosis: the effect of modulator therapy on objective 24-h cough monitoring
- Distribution of Achromobacter Species in 12 French Cystic Fibrosis Centers in 2020 by a Retrospective MALDI-TOF MS Spectrum Analysis
- Cystic Fibrosis and Diagnostic Imaging
- Angiographic and histopathological study on bronchial-to-pulmonary vascular anastomoses on explants from patients with cystic fibrosis after bronchial artery embolisation
- Extreme electrolyte depletion associated with septic shock as a form of presentation of cystic fibrosis
- Exercise in Child Health
- Early Pseudomonas aeruginosa predicts poorer pulmonary function in preschool children with cystic fibrosis
- Cystic fibrosis and alpha-1 antitrypsin deficiency: case report and review of literature
- Invited editorial: Q and A on hereditary lung cancer
- CFTR protein quantification as a cystic fibrosis diagnostic biomarker in dried blood spots using multiple reaction monitoring tandem mass spectrometry
- The psychological implications and health risks of cystic fibrosis pre- and post- CFTR modulator therapy
- COVID-19 infection and nocardiosis causing the death of an adolescent with cystic fibrosis
- Exploring YAP1-centered networks linking dysfunctional CFTR to epithelial-mesenchymal transition
- Results of Lung Transplantations Among Cystic Fibrosis Patients: A Single-Center Study
- Cystic fibrosis year in review 2021
- Impaired regulation of PMCA activity by defective CFTR expression promotes epithelial cell damage in alcoholic pancreatitis and hepatitis
- Role of Cellular Metabolism in the Formation of Neutrophil Extracellular Traps in Airway Diseases
- Synergy Between Pseudomonas aeruginosa Filtrates And Voriconazole Against Aspergillus fumigatus Biofilm Is Less for Mucoid Isolates From Persons With Cystic Fibrosis
- Cardiac sarcoidosis in an adult person with cystic fibrosis: a case report
- Diffuse pulmonary calcification in allergic bronchopulmonary aspergillosis
- COVID-19 infection in an infant with cystic fibrosis: A case report and possible therapeutic effect of hypertonic saline
- Cystic Fibrosis-Related Liver Disease is an Independent Risk Factor For Mortality And Increased Health Care Resource Utilization In Hospitalized Pediatric Patients With Cystic Fibrosis
- Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis: Authors’ Reply
- Staphylococcus aureus detection from CF respiratory samples is improved using alternative media
- Difficult gall bladder in adolescents with cystic fibrosis and symptomatic cholelithiasis: What is the best treatment choice?
- The Role of HLA-DRB1 Alleles in Pulmonary Cystic Fibrosis
- Breath biomarkers associated withnontuberculosis mycobacteria disease status in persons with cystic fibrosis: a pilot study
- Antibacterial contact-dependent proteins secreted by Gram-negative cystic fibrosis respiratory pathogens
- CFTR mRNAs with nonsense codons are degraded by the SMG6-mediated endonucleolytic decay pathway
- Role of inhaled antibiotics in children and adolescents with cystic fibrosis: Experience from the tertiary care center
- Cystic Fibrosis Airway Mucus Hyperconcentration Produces a Vicious Cycle of Mucin, Pathogen, and Inflammatory Interactions that Promote Disease Persistence
- Continuous glucose monitoring and dysglycaemia in young children with cystic fibrosis: A case series
- Effectiveness of positive expiratory pressure on patients over 16 years of age with cystic fibrosis: systematic review and meta-analysis
- Esophagogastric variceal bleeding as debut of Caroli’s syndrome
- From a medical model to a social model of autonomy for people with cystic fibrosis. A sociological study of the views of professional and associative stakeholders
- Pharmacist-administered audiology screening for pediatric cystic fibrosis patients exposed to high-dose aminoglycosides: a pilot study
- Sustained inhibition of ENaC in CF: Potential RNA-based therapies for mutation-agnostic treatment
- Novel adamantyl clubbed iminothiazolidinones as promising elastase inhibitors: design, synthesis, molecular docking, ADMET and DFT studies
- A Pharmacokinetic Analysis of Tobramycin in Patients Less than Five Years of Age with Cystic Fibrosis: Assessment of Target Attainment with Extended-Interval Dosing through Simulation
- Sputum Metabolites Associated with Nontuberculous Mycobacterial Infection in Cystic Fibrosis
- Use of inhaled antibiotics among Danish patients with cystic fibrosis
- Can continuous glucose monitoring predict cystic fibrosis-related diabetes and worse clinical outcome?
- Diagnosis of cystic fibrosis-related diabetes: too early or too late?
- Cumulative Incidence and Risk Factors for Severe COVID-19 in French People with Cystic Fibrosis
- Paths to Motherhood for Women with Cystic Fibrosis
- Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients
- Molecular Dynamics and Theratyping in Airway and Gut Organoids Reveal R352Q-CFTR Conductance Defect
- Molecular Insight into Gene Response of Diorcinol- and Rubrolide-Treated Biofilms of the Emerging Pathogen Stenotrophomonas maltophilia
- Genetic and Transcriptomic Characteristics of RhlR-Dependent Quorum Sensing in Cystic Fibrosis Isolates of Pseudomonas aeruginosa
- Reporting two novel mutations in two Iranian families with cystic fibrosis, molecular and bioinformatic analysis
- Romosozumab used to treat a patient with cystic fibrosis-related osteoporosis
- Diabetic Ketoacidosis as a Unique Presentation of Cystic Fibrosis
- Contribution of Afferent Renal Nerves to Cystogenesis and Arterial Pressure Regulation in a Preclinical Model of Autosomal Recessive Polycystic Kidney Disease
- Complete Genome Sequence of Stenotrophomonas maltophilia Podophage Pepon
- Downregulation of epithelial sodium channel (ENaC) activity in cystic fibrosis cells by epigenetic targeting
- Antipseudomonal treatment decisions during CF exacerbation management
- Seamless Gene Correction in the Human Cystic Fibrosis Transmembrane Conductance Regulator Locus by Vector Replacement and Vector Insertion Events
- Diagnostics of the Epstein-Barr Virus Before and After Lung Transplantation in a Patient With Developing Posttransplant Lymphoproliferative Disease: A Case Report
- Effect of Bronchoscopic Interventions on Long-Term Lung Function Among Lung Transplant Recipients due to Cystic Fibrosis: A Single-Center Study
- Immunogenicity of the COVID-19 BNT162b2 vaccine in adolescents and young adults with cystic fibrosis
- Ways of coping and survival in Cystic Fibrosis: a 20-year longitudinal study
- Established and novel human translational models to advance cystic fibrosis research, drug discovery, and optimize CFTR-targeting therapeutics
- Overview of CF lung pathophysiology
- The Octopus Sign-A New HRCT Sign in Pulmonary Langerhans Cell Histiocytosis
- Investigation of Direct and Retro Chromone-2-Carboxamides Based Analogs of Pseudomonas aeruginosa Quorum Sensing Signal as New Anti-Biofilm Agents
- Cilia Stimulatory and Antibacterial Activities of T2R Bitter Taste Receptor Agonist Diphenhydramine: Insights into Repurposing Bitter Drugs for Nasal Infections
- Oral Glucose Tolerance Test in Patients with Cystic Fibrosis Compared to the Overweight and Obese: A Different Approach in Understanding the Results
- Theratyping of the Rare CFTR Variants E193K and R334W in Rectal Organoid-Derived Epithelial Monolayers
- Secretory Immunoglobulin A Immunity in Chronic Obstructive Respiratory Diseases
- CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells
- Highway to Cell: Selection of the Best Cell-Penetrating Peptide to Internalize the CFTR-Stabilizing iCAL36 Peptide
- Pseudomonas aeruginosa PAO1 Is Attracted to Bovine Bile in a Novel, Cystic Fibrosis-Derived Bronchial Epithelial Cell Model
- Clinical Impact of Aspergillus fumigatus in Children with Cystic Fibrosis
- The Human Mycobiome in Chronic Respiratory Diseases: Current Situation and Future Perspectives
- Pharmacological Effects of Panduratin A on Renal Cyst Development in In Vitro and In Vivo Models of Polycystic Kidney Disease
- Influence of Bronchoscopic Interventions on Graft Function of Double Lung Transplant Recipients due to Cystic Fibrosis
- Combined machine learning and pharmacophore based virtual screening approaches to screen for antibiofilm inhibitors targeting LasR of Pseudomonas aeruginosa
- Alterations of mucosa-attached microbiome and epithelial cell numbers in the cystic fibrosis small intestine with implications for intestinal disease
- An observational study of Pseudomonas aeruginosa in adult long-term ventilation
- Novel CFTR modulator combinations maximise rescue of G85E and N1303K in rectal organoids
- Onset of systemic arterial hypertension after initiation of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis: A case series
- DNA Sequencing Analysis of Cystic Fibrosis Transmembrane Regulator Gene Identifies Cystic Fibrosis-Associated Variants in the Severe Asthma Research Program
- At-home Compounding Preparation of Slow Desensitization of Elexacaftor/Tezacaftor/Ivacaftor for Delayed Hypersensitivity Rash
- Co-Operative Biofilm Interactions between Aspergillus fumigatus and Pseudomonas aeruginosa through Secreted Galactosaminogalactan Exopolysaccharide
- The Presence of Exophiala dermatitidis in the Respiratory Tract of Cystic Fibrosis Patients Accelerates Lung Function Decline: A Retrospective Review of Lung Function
- Molecular Epidemiological Characteristics of Mycobacterium abscessus Complex Derived from Non-Cystic Fibrosis Patients in Japan and Taiwan
- Interbacterial Antagonism Mediated by a Released Polysaccharide
- Losartan ameliorates TGF-β1-induced CFTR dysfunction and improves correction by cystic fibrosis modulator therapies
- Histomorphological Changes in a Rat Model of Polycystic Ovary Syndrome and the Contribution of Stevia Leaf Extract in Modulating the Ovarian Fibrosis, VEGF, and TGF-β Immunoexpressions: Comparison with Metformin
- The role of Hyaluronan synthesis and degradation in the critical respiratory illness COVID-19
- Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated with one Month of Lumacaftor/ivacaftor
- Current prices versus minimum costs of production for CFTR modulators
- Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D
- Non-visualisation of fetal gallbladder in a Chinese cohort
- Investigation of interspecies crosstalk between probiotic Bacillus subtilis BR4 and Pseudomonas aeruginosa using metabolomics analysis
- Pediatric Paranasal Sinuses- Development, Growth, Pathology & Functional Endoscopic Sinus Surgery
- Living with cystic fibrosis during the COVID-19 pandemic: a social connectedness perspective
- A new era has dawned for persons with cystic fibrosis; however many knowledge gaps exist in our efforts to improve care
- Mucus and mucus flake composition and abundance reflect inflammatory and infection status in cystic fibrosis
- The Effect of Dysglycaemia on Changes in Pulmonary and Aerobic Function in Cystic Fibrosis
- Bone accrual and structural changes over one year in youth with cystic fibrosis
- Clinical practice versus guidelines for the screening of cystic fibrosis-related diabetes: A French survey from the 47 centers
- Using Structure-guided Fragment-Based Drug Discovery to Target Pseudomonas aeruginosa Infections in Cystic Fibrosis
- A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein
- Application of gap time analysis with flexible hazards to pulmonary exacerbations in the EPIC observational study
- ‘We’re kind of like genetic nomads’: Parents’ experiences of biographical disruption and uncertainty following in/conclusive results from newborn cystic fibrosis screening
- Occurrence of Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutations in Patients with Allergic Bronchopulmonary Aspergillosis Complicating Asthma
- Spatial covariance analysis reveals the residue-by-residue thermodynamic contribution of variation to the CFTR fold
- Constipation and cystic fibrosis. Slow movement
- Evaluation of clinically relevant changes in the lung clearance index in children with cystic fibrosis and healthy controls
- Evaluating FEV1 Decline in Diagnosis and Management of Pulmonary Exacerbations in Children with Cystic Fibrosis
- Mental health care needs in cystic fibrosis: A scoping review
- A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus
- Study to Evaluate Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) Long-term Safety and Efficacy in Subjects Without F508del
- Coming Full Circle: Reflections and Inspirations from a Cystic Fibrosis Patient Scientist Panel
- Role of Efflux in Antibiotic Resistance of Achromobacter xylosoxidans and Achromobacter insuavis Isolates From Patients With Cystic Fibrosis
- Repeatability of lung clearance index in infants with cystic fibrosis and recurrent wheeze
- Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice
- Re: Impact of spiroware re-analysis method on multiple-breath washout outcomes in children with cystic fibrosis; M.A. Oestreich, F. Wyler, P. Latzin et al
- CFTR modulator use and risk of nontuberculous mycobacteria positivity in cystic fibrosis, 2011-2018
- Targeting the EGFR-ERK axis using the compatible solute ectoine to stabilize CFTR mutant F508del
- Characterization of skeletal muscle wasting pathways in diaphragm and limb muscles of cystic fibrosis mice
- Correlates of Pancreatic Enzyme Replacement Therapy Intake in Adults with Cystic Fibrosis: Results of a Cross-Sectional Study
- Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis
- CFTR Modulator Therapies: Potential Impact on Airway Infections in Cystic Fibrosis
- The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis
- Comparison of Bioelectrical Impedance Analysis with DXA in Adolescents with Cystic Fibrosis before and after a Resistance Training Intervention
- Cystic Fibrosis-Related Diabetes in Poland
- Bronchiectasis – Exercise as Therapy (BREATH): rationale and study protocol for a multi-center randomized controlled trial
- Biochemical, Biophysical, and Immunological Characterization of Respiratory Secretions in Severe SARS-CoV-2 (COVID-19) Infections
- Feasibility and efficacy of combined pancreatic islet-lung transplantation in cystic fibrosis related diabetes -PIM study: a multicenter phase 1-2 trial
- Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis: Correspondence
- Mucus Clearance Strategies in Mechanically Ventilated Patients
- Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis
- Transcatheter coil embolization of a complex pulmonary artery pseudoaneurysm with thyrocervical trunk-pulmonary arterial fistulization in a patient with cystic fibrosis and massive hemoptysis
- High cystic fibrosis transmembrane conductance regulator expression in childhood B-cell acute lymphoblastic leukemia acts as a potential therapeutic target
- “The Stakes Are Higher”- Patient and Caregiver Perspectives on Cystic Fibrosis Research and Personalized Medicine
- Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry
- Genetic evidence supports the development of SLC26A9 targeting therapies for the treatment of lung disease
- Investigating the Implications of CFTR Exon Skipping Using a Cftr Exon 9 Deleted Mouse Model
- Safety of chronic hypertonic bicarbonate inhalation in a cigarette smoke-induced airway irritation guinea pig model
- Oxylipin profile in saliva from patients with cystic fibrosis reveals a balance between pro-resolving and pro-inflammatory molecules
- Ciliary and immune dysfunctions and their genetic background in patients with non-cystic fibrosis bronchiectasis in Central Iran
- Distinct proteostasis states drive pharmacologic chaperone susceptibility for Cystic Fibrosis Transmembrane Conductance Regulator misfolding mutants
- Medical interventions for chronic rhinosinusitis in cystic fibrosis
- Lung parenchymal calcifications in a child with cystic fibrosis
- Curvilinearity provides additional information to lung clearance index only in a minority of children with early cystic fibrosis lung disease
- A pilot study of disease related education and psychotherapeutic support for unresolved grief in parents of children with CF
- Brazilian Guidelines for Nutrition in Cystic Fibrosis
- Developmental Functioning Outcomes in Infants with Cystic Fibrosis: a 24- to 36-Month Follow-Up Study
- Cefiderocol Pharmacokinetics in Adult Patients With Cystic Fibrosis
- Surprise Foreign Body Aspiration with Pistachios in a Patient with Cystic Fibrosis with Persistent Atelectasis on Chest Radiography
- Glby, Encoded by MAB_3167c , Is Required for In Vivo Growth of Mycobacteroides abscessus and Exhibits Mild β-Lactamase Activity
- Persistent Rhinovirus Infection in a Child With Leukemia
- Proteostasis Regulators in Cystic Fibrosis: Current Development and Future Perspectives
- Microbial Community Composition in Explanted Cystic Fibrosis and Control Donor Lungs
- Targeting the Pseudomonas aeruginosa Virulence Factor Phospholipase C With Engineered Liposomes
- Reinfection or relapse? A case study of whole genome sequencing guided genomic characterization of Mycobacterium abscessus chronic infection in a cystic fibrosis patient
- Association between elevated peripheral blood eosinophil count and respiratory outcomes in adults with cystic fibrosis
- Objective Nebuliser Adherence Data as “Proof” of Adherence in the Management of Cystic Fibrosis: A Qualitative Interview Study
- SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair Modulation
- Moving up: Healthcare transition experiences of adolescents and young adults with cystic fibrosis
- Club cell secretory protein and lung function in children with cystic fibrosis
- Pathophysiology of Lung Disease and Wound Repair in Cystic Fibrosis
- Expression of ACE2 – A Key SARS-CoV-2 Entry Factor – Is Not Increased in the Nasal Mucosa of People with Cystic Fibrosis
- TMEM16A (ANO1) as a therapeutic target in cystic fibrosis
- High frequency of complex CFTR alleles associated with c.1521_1523delCTT (F508del) in Russian cystic fibrosis patients
- Lung clearance index to characterize clinical phenotypes of children and adolescents with cystic fibrosis
- The 49th parallel: Does geographic position affect longevity of patients with cystic fibrosis?
- Mucus concentration-dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis
- Efficacy and Safety of Triple Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Patients With Cystic Fibrosis: A Meta-Analysis of Randomized Controlled Trials
- Network Analysis to Identify Multi-Omic Correlations in the Lower Airways of Children With Cystic Fibrosis
- Efficacy of N-acetylcysteine on idiopathic or postinfective non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis protocol
- The dilemma of initiating ELX/TEZ/IVA in a CF patient recovering from acute-on-chronic liver failure
- Prepuberal insulin secretory indices are long term predictors of short adult stature in cystic fibrosis
- Eosinophilic Esophagitis in Cystic Fibrosis: A Case Series with Long-Term Follow-up
- The Ubiquitin Ligase RNF34 Participates in the Peripheral Quality Control of CFTR (RNF34 Role in CFTR PeriQC)
- A Shaving Proteomic Approach to Unveil Surface Proteins Modulation of Multi-Drug Resistant Pseudomonas aeruginosa Strains Isolated From Cystic Fibrosis Patients
- A PI3Kγ mimetic peptide triggers CFTR gating, bronchodilation, and reduced inflammation in obstructive airway diseases
- Using A Work System Framework to Investigate Pharmacists’ Roles in Cystic Fibrosis Management
- Azithromycin inhibits mucin secretion, mucous metaplasia, airway inflammation and airways hyperresponsiveness in mice exposed to house dust mite extract
- MRI lung lobe segmentation in pediatric cystic fibrosis patients using a recurrent neural network trained with publicly accessible CT datasets
- Things come in threes: A new complex allele and a novel deletion within the CFTR gene complicate an accurate diagnosis of cystic fibrosis
- Azole-Induced Myositis after Combined Lung-Liver Transplantation
- Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis
- Histological Manifestations of Diabetic Kidney Disease and its Relationship with Insulin Resistance
- Cross-talk between enhancers, structural elements and activating transcription factors maintains the 3D architecture and expression of the CFTR gene
- Significance of plasma fibrinogen and malondialdehyde in the post-inflammatory period in patients with cystic fibrosis
- Complete Genome Sequence of Burkholderia cenocepacia Phage Paku
- COVID-19 in a pregnant cystic fibrosis carrier with myasthenia gravis: A case report
- Factors in childhood associated with lung function decline to adolescence in cystic fibrosis
- Global access to affordable CFTR modulator drugs: Time for action!
- Krüppel-Like Factor 5 regulates CFTR expression through repression by maintaining chromatin architecture coupled with direct enhancer activation
- Characterization of glucose metabolism in youth with vs. without cystic fibrosis liver disease: A pilot study
- The Changing Landscape of Nutrition in Cystic Fibrosis: The Emergence of Overweight and Obesity
- Journey on VX-809-Based Hybrid Derivatives towards Drug-like F508del-CFTR Correctors: From Molecular Modeling to Chemical Synthesis and Biological Assays
- Defining and Treating Cystic Fibrosis Liver Disease: Some Things Old and Some Things New
- Pseudomonas aeruginosa polysaccharide Psl supports airway microbial community development
- Exposure to the Pseudomonas aeruginosa secretome alters the proteome and secondary metabolite production of Aspergillus fumigatus
- An 18-Year Dataset on the Clinical Incidence and MICs to Antibiotics of Achromobacter spp. (Labeled Biochemically or by MAL-DI-TOF MS as A. xylosoxidans ), Largely in Patient Groups Other than Those with CF
- Elastase-Activated Antimicrobial Peptide for a Safer Pulmonary Treatment of Cystic Fibrosis Infections
- Lung-Directed Bacteriotherapy in Cystic Fibrosis: Could It Be an Option?
- Structural Comparative Modeling of Multi-Domain F508del CFTR
- The Relation between Vitamin D Level and Lung Clearance Index in Cystic Fibrosis-A Pilot Study
- The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs
- Feasibility and Efficacy of Telehealth-Based Resistance Exercise Training in Adolescents with Cystic Fibrosis and Glucose Intolerance
- Biofilm on Toothbrushes of Children with Cystic Fibrosis: A Potential Source of Lung Re-Infection after Antibiotic Treatment?
- Metrics of Antifungal Effects of Ciprofloxacin on Aspergillus fumigatus Planktonic Growth and Biofilm Metabolism; Effects of Iron and Siderophores
- Targeted Gene Insertion for Functional CFTR Restoration in Airway Epithelium
- One year monitoring of SARS-CoV-2 prevalence in a German cohort of patients with cystic fibrosis
- Inherited pancreatic exocrine insufficiency and pancreatitis: When children transition to adult care
- Digestive outcomes in Cystic fibrosis
- Improving Therapeutic Adherence With a Co-constructed Program Involving Both Patients and Health Care Professionals
- Description of the Short-term Effects of KAFTRIO® by Continuous Monitoring With the PHEAL-CR-K Application in Real Life in Patients With Cystic Fibrosis Eligible for KAFTRIO® Treatment
- Caregiver burden in cystic fibrosis: a systematic literature review
- Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs
- A Potent Inhibitor of the Cystic Fibrosis Transmembrane Conductance Regulator Blocks Disease and Morbidity Due to Toxigenic Vibrio cholerae
- Does ibuprofen affect the expression of alginate genes in pathogenic Pseudomonas aeruginosa strains?
- State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the “iMAging managEment of cySTic fibROsis” (MAESTRO) consortium
- Alginate oligosaccharides enhance diffusion and activity of colistin in a mucin-rich environment
- Respiratory physiotherapy in patients with Cystic Fibrosis and upper limb deep vein thrombosis
- Measurement of exhaled nitric oxide fraction in lung diseases
- The Use of Tobramycin for Pseudomonas aeruginosa : A Review
- Cystic fibrosis transmembrane conductance regulator prevents ischemia/reperfusion induced intestinal apoptosis via inhibiting PI3K/AKT/NF-kappaB pathway
- Cystic fibrosis patients on cystic fibrosis transmembrane conductance regulator modulators have a reduced incidence of cirrhosis
- Pseudomonas aeruginosa modulates neutrophil granule exocytosis in an in vitro model of airway infection
- Resolution of lobe collapse in a child with cystic fibrosis with M. abscessus using serial intrabronchial rhDNase
- CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor
- CFTR-mediated monocyte/macrophage dysfunction revealed by cystic fibrosis proband-parent comparisons
- Systematic estimation of cystic fibrosis prevalence in Chinese and genetic spectrum comparison to Caucasians
- Strong and consistent associations of precedent chronic rhinosinusitis with risk of non-cystic fibrosis bronchiectasis
- The Impact of COVID-19 in Cystic Fibrosis
- Cystic Fibrosis in 2021: “The Times They Are A-Changin”
- Association Between Evolution of Mucus Plugging, Parenchymal Alterations and Air Trapping on Computed Tomography and Risk of Exacerbations in Adults With Cystic Fibrosis
- Variation in CFTR-dependent ‘β-sweating’ among healthy adults
- Virulence Mechanisms of Mycobacterium abscessus : Current Knowledge and Implications for Vaccine Design
- Novel cystic fibrosis transmembrane conductance regulator variant in a cystic fibrosis patient
- Is exercise and electrostimulation effective in improving muscle strength and cardiorespiratory fitness in children with cystic fibrosis and mild-to-moderate pulmonary impairment?: Randomized controlled trial
- Decreased efficacy of antimicrobial agents in a polymicrobial environment
- Mutational Spectrum of the CFTR Gene in the Kazakhstan Population
- HALT-ing Nontuberculous Mycobacteria in CF Centers. Is There Something in The Water?
- The NSAID glafenine rescues class 2 CFTR mutants via cyclooxygenase 2 inhibition of the arachidonic acid pathway
- Pinostrobin inhibits renal CFTR-mediated Cl- secretion and retards cyst growth in cell-derived cyst and polycystic kidney disease rats
- Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis
- Health care costs in a randomized trial of antimicrobial duration among cystic fibrosis patients with pulmonary exacerbations
- A prospective randomised controlled mixed-methods pilot study of home monitoring in adults with cystic fibrosis
- Use of Dynamic Chest Radiography to Assess Treatment of Pulmonary Exacerbations in Cystic Fibrosis
- Impact of lockdown during the COVID-19 pandemic on health status in patients with cystic fibrosis: a mono-centre observational study
- Clinical Outcomes of Antipseudomonal versus Other Antibiotics Among Children with Cystic Fibrosis without Pseudomonas aeruginosa
- Targeting the E1 ubiquitin-activating enzyme (UBA1) improves elexacaftor/tezacaftor/ivacaftor efficacy towards F508del and rare misfolded CFTR mutants
- Informatic analysis of the pulmonary microecology in non-cystic fibrosis bronchiectasis at three different stages
- Predictive value of impulse oscillometry and multiple breath washout parameters in pediatric patients with cystic fibrosis pulmonary exacerbation
- PBPK-led guidance for cystic fibrosis patients taking elexacaftor-tezacaftor-ivacaftor with nirmatrelvir-ritonavir for the treatment of COVID-19
- Charting a New Path: A Single-cell Atlas of Porcine CF Airways at Birth
- Novel volumetric capnography indices measure ventilation inhomogeneity in cystic fibrosis
- Elexacaftor/Tezacaftor/Ivacaftor as a Bridge to Lung Retransplant in a Recipient With Cystic Fibrosis
- Three-hole oesophagectomy following bilateral lung transplant for cystic fibrosis
- The effect of inhaled hypertonic saline on lung structure in children aged 3-6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled trial
- Effect of chest physiotherapy on cystic fibrosis sputum nanostructure: an experimental and theoretical approach
- Inhaled Dry Powder Mannitol Treatment in Pediatric Patients with Cystic Fibrosis: Evaluation of Clinical Data in a Real-World Setting
- Efficacy of Intensive Cholecalciferol Monitoring and Supplementation on Serum vitD Levels in Pediatric Patients With CF
- N-Acetyl-Cysteine Increases Activity of Peanut-Shaped Gold Nanoparticles Against Biofilms Formed by Clinical Strains of Pseudomonas aeruginosa Isolated from Sputum of Cystic Fibrosis Patients
- Letter in Reply: Can We Justify Cystic Fibrosis Mutational Analysis among Omani Neonates?
- Can We Justify Cystic Fibrosis Mutational Analysis among Omani Neonates?
- Commentary: Filling in the cracks: How to improve survival for patients with cystic fibrosis
- Cough in adolescent with cystic fibrosis, from nightmare to COVID-19 stigma: A qualitative thematic analysis
- What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?
- Iron Deficiency in Cystic Fibrosis: A Cross-Sectional Single-Centre Study in a Referral Adult Centre
- Intestinal Absorption of Lipids Using a Pancreatic Enzyme-Free Nutritional Supplement in Patients with Cystic Fibrosis: A Randomized, Double-Blind, Crossover Pilot Trial
- Left behind: The potential impact of CFTR modulators on racial and ethnic disparities in cystic fibrosis
- Longitudinal lung clearance index and association with structural lung damage in children with cystic fibrosis
- Comparison of the Lung Clearance Index in Preschool Children with Primary Ciliary Dyskinesia and Cystic Fibrosis
- Comparison of continuous glucose monitoring in cystic fibrosis patients with or without pancreatic exocrine insufficiency
- CFTR suppresses neointimal formation through attenuating proliferation and migration of aortic smooth muscle cells
- Effects of Exercise on Nutritional Status in People with Cystic Fibrosis: A Systematic Review
- Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management
- Anticipating New Treatments for Cystic Fibrosis: A Global Survey of Researchers
- Methods of Sputum and Mucus Assessment for Muco-Obstructive Lung Diseases in 2022: Time to “Unplug” from Our Daily Routine!
- Proximity Profiling of the CFTR Interaction Landscape in Response to Orkambi
- CFTR, Cell Junctions and the Cytoskeleton
- Effectiveness of Video Games as Physical Treatment in Patients with Cystic Fibrosis: Systematic Review
- Evaluation of Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) in Cystic Fibrosis Subjects Without an F508del Mutation
- Reducing prescribing errors: making electronic prescribing work for cystic fibrosis inpatients
- The Prognosis of ART Is Not Altered in Cystic Fibrosis Women: A Case-Report Study
- Polycationic Glycopolymer Demonstrates Activity Against Persisters and Biofilms of Non-tuberculosis Mycobacteria Cystic Fibrosis Clinical Isolates in vitro
- Pseudomonas aeruginosa Affects Airway Epithelial Response and Barrier Function During Rhinovirus Infection
- Establishing Antimicrobial Susceptibility Testing Methods and Clinical Breakpoints for Inhaled Antibiotic Therapy
- Interleukin-1 beta is a potential mediator of airway nitric oxide deficiency in cystic fibrosis
- Impact of azithromycin on serum inflammatory markers in children with cystic fibrosis and new Pseudomonas
- Pilot Study of a CF-specific CBT Intervention for Adolescents
- Development and preliminary validation of the challenges of living with cystic fibrosis (CLCF) questionnaire: a 46-item measure of treatment burden for parent/carers of children with CF
- Benefits of reviewing pancreatic function in children with cystic fibrosis
- Safety of mRNA-based vaccines against SARS-CoV-2 in people with cystic fibrosis aged 12 years and over
- The clinical impact of the Covid-19 pandemic first wave on patients with cystic fibrosis in New York
- Receiving results of uncertain clinical relevance from population genetic screening: systematic review & meta-synthesis of qualitative research
- Association of Body Mass Index With Clinical Outcomes in Patients With Cystic Fibrosis: A Systematic Review and Meta-analysis
- Body Mass Index and Clinical Outcomes in Persons Living With Cystic Fibrosis-Is Bigger Always Better?
- CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages
- Tobramycin safety and efficacy review article
- Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro
- DMBT1 is upregulated in cystic fibrosis, affects ciliary motility, and is reduced by acetylcysteine
- “Be afraid – be very afraid”: Passive air drying of nebulizer parts in cystic fibrosis (CF) – Occult microbiological risks of contamination with Pseudomonas aeruginosa from calyptrate flies (Musca domestica &Calliphora vomitoria)
- Ethmoidal mucocele causing proptosis in a pediatric patient with cystic fibrosis: A case report
- The role of pharmacy services beyond cystic fibrosis: a case for support in childhood interstitial lung disease
- The changing landscape of pediatric lung transplantation
- Platelet CFTR inhibition enhances arterial thrombosis via increasing intracellular Cl- concentration and activation of SGK1 signaling pathway
- Achromobacter xylosoxidans Purulent Bronchitis in a Previously Healthy Child: An Unexpected Consequence of COVID-19 Infection
- Cystic Fibrosis Lung Transplant Recipients 10 years of age or Younger: Predisposing Factors for End-stage Disease
- Prevalence of antibiotic resistance of Pseudomonas aeruginosa in cystic fibrosis infection: A systematic review and meta-analysis
- 3.22 Nutrition in Cystic Fibrosis
- Elexacaftor, tezacaftor and ivacaftor: a case of severe rash and approach to desensitisation
- A Single-Cell Atlas of Large and Small Airways at Birth in a Porcine Model of Cystic Fibrosis
- The Burkholderia cenocepacia iron starvation σ factor, OrbS, possesses an on-board iron sensor
- Addition time plays a major role in the inhibitory effect of chitosan on the production of Pseudomonas aeruginosa virulence factors
- Patienthèque of Finisterian (South of Brittany) Children With Cystic Fibrosis in the Time of Precision Medicine
- Impact of Spiroware re-analysis method on multiple-breath washout outcomes in children with cystic fibrosis
- Risk Assessment for Patients with Chronic Respiratory Conditions in the Context of the SARS-CoV-2 Pandemic Statement of the German Respiratory Society with the Support of the German Association of Chest Physicians
- The impact of COVID-19 upon the delivery of exercise services within cystic fibrosis clinics in the United Kingdom
- Effectiveness of Different Eradication Treatment Protocols for New-Onset Pseudomonas aeruginosa in Children with Cystic Fibrosis
- AZD5634, an inhaled ENaC inhibitor, in healthy subjects and patients with cystic fibrosis
- Epigenetic regulation of inflammation by microRNAs in post-infectious bronchiolitis obliterans
- Functional interplay between CFTR and pendrin: physiological and pathophysiological relevance
- Impaired intestinal stem cell activity in ETEC infection: enterotoxins, cyclic nucleotides, and Wnt signaling
- Testicular cancer in men with cystic fibrosis
- Congenital Bilateral Absence of the Vas Deferens
- Burkholderia cepacia Complex Lumbar Spondylodiscitis: A Rare Nosocomial Infection
- Cystic fibrosis telemedicine in the era of COVID-19
- Staphylococcus aureus in Non-Cystic Fibrosis Bronchiectasis: Prevalence and Genomic Basis of High Inoculum Beta-Lactam Resistance
- Achromobacter spp. in a Cohort of Non-Selected Pre- and Post-Lung Transplant Recipients
- N-acetylcysteine (NAC) and Its Role in Clinical Practice Management of Cystic Fibrosis (CF): A Review
- Utilization of the Healthy Eating Index in Cystic Fibrosis
- Nutrition in Cystic Fibrosis-Some Notes on the Fat Recommendations
- Neutrophil Dysfunction in the Pathogenesis of Cystic Fibrosis
- Cystic fibrosis transmembrane conductance regulator function in patients with chronic pancreatitis
- Protein with negative surface charge distribution, Bnr1, shows characteristics of a DNA-mimic protein and may be involved in the adaptation of Burkholderia cenocepacia
- In Vivo Activity of Metal Complexes Containing 1,10-Phenanthroline and 3,6,9-Trioxaundecanedioate Ligands against Pseudomonas aeruginosa Infection in Galleria mellonella Larvae
- Inhibitor of Hyaluronic Acid Synthesis 4-Methylumbelliferone Suppresses the Secretory Processes That Ensure the Invasion of Neutrophils into Tissues and Induce Inflammation
- Respiratory and Peripheral Muscle Weakness and Body Composition Abnormalities in Non-Cystic Fibrosis Bronchiectasis Patients: Gender Differences
- Safety and Efficacy of Devices Delivering Inhaled Antibiotics among Adults with Non-Cystic Fibrosis Bronchiectasis: A Systematic Review and a Network Meta-Analysis
- Lung Ultrasound in Children with Cystic Fibrosis in Comparison with Chest Computed Tomography: A Feasibility Study
- Pulmonary Exacerbations in Pediatric Patients: Retrospective Study in a Portuguese Cystic Fibrosis Center
- SLC26A9 as a Potential Modifier and Therapeutic Target in Cystic Fibrosis Lung Disease
- Use of Intravenous Pulse Steroids to Treat Allergic Bronchopulmonary Aspergillosis in a Non-Compliant Asthmatic Adolescent
- Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study
- Factors Predisposing the Response to Lumacaftor/Ivacaftor in People with Cystic Fibrosis
- Novel Bacteriophages Show Activity against Selected Australian Clinical Strains of Pseudomonas aeruginosa
- The Arginine Catabolism-Derived Amino Acid l-ornithine Is a Chemoattractant for Pseudomonas aeruginosa
- Induction of Broad β-lactam Resistance in Achromobacter ruhlandii by Exposure to Ticarcillin Is Primarily Linked to Substitutions in Murein Peptide Ligase Mpl
- In vitro assessment of triple combination therapy for the most common disease-associated mutation in cystic fibrosis
- National Guidelines for the Performance of the Sweat Test in Diagnosis of Cystic Fibrosis on behalf of the Croatian Society of Medical Biochemistry and Laboratory Medicine and the Cystic Fibrosis Centre – Paediatrics and adults, University Hospital Centre Zagreb
- Prevalence and Impact of Rheumatologic Pain in Cystic Fibrosis Adult Patients
- Detection of DZIP1L mutations by whole-exome sequencing in consanguineous families with polycystic kidney disease
- A highly selective, cell-permeable furin inhibitor BOS-318 rescues key features of cystic fibrosis airway disease
- Natural killer cells kill extracellular Pseudomonas aeruginosa using contact-dependent release of granzymes B and H
- Serum anti-PAD4 autoantibodies are present in cystic fibrosis children and increase with age and lung disease severity
- Improvements in anthropometric measures and gastrointestinal tolerance in patients with cystic fibrosis by using a digestive enzyme cartridge with overnight enteral nutrition
- Bicarbonate transport of airway surface epithelia in luminally perfused mice bronchioles
- Neutrophil extracellular traps in chronic lung disease: implications for pathogenesis and therapy
- Age at menarche in girls with cystic fibrosis and asthma
- Collaborative research protocol to define patient-reported experience measures of the cystic fibrosis care pathway in France: the ExPaParM study
- Body functions and structure, activity, and participation limitations of adult cystic fibrosis patients under the international classification of functioning, disability, and health framework
- A Pandemic Lesson for Global Lung Diseases: Exacerbations are Preventable
- Cystic Fibrosis-Related Diabetes Mellitus and Pregnancy: A Retrospective Study
- Differential correlation network analysis of rectal transcriptomes reveals cystic fibrosis-related disturbance
- Gait and functional balance in non-CF bronchiectasis
- A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant
- Expression of cystic fibrosis lung disease modifier genes in human airway models
- An Open-label, Phase 1/2 Trial of Gene Therapy 4D-710 in Adults With Cystic Fibrosis
- Study of Satisfaction of Patients and Care Providers for an Alternate Follow-up With In-hospital Consults and Tele-consult for Patients With Cystic Fibrosis
- Two systematic cochrane reviews of the prevention and treatment of distal intestinal obstruction syndrome in cystic fibrosis
- How the sweat gland reveals levels of CFTR activity
- Repurposing the Veterinary Antibiotic Apramycin for Antibacterial and Antibiofilm Activity Against Pseudomonas aeruginosa From Cystic Fibrosis Patients
- Complete Genome Sequencing and Comparative Analysis of the Clinically-Derived Apiotrichum mycotoxinivorans Strain GMU1709
- Cystic fibrosis in the 21st century: what every radiologist should know
- Cystic fibrosis: candidate selection and impact of the cystic fibrosis transmembrane conductance regulator therapy
- Development of a novel rapamycin loaded nano- into micro-formulation for treatment of lung inflammation
- Parenthood impacts short-term health outcomes in people with cystic fibrosis
- Chest x-rays are less sensitive than multiple breath washout examinations when it comes to detecting early cystic fibrosis lung disease
- Alkalosis-induced hypoventilation in cystic fibrosis: The importance of efficient renal adaptation
- Commensal Bacteria in the Cystic Fibrosis Airway Microbiome Reduce P. aeruginosa Induced Inflammation
- Outcomes and survival following lung transplantation in non-cystic fibrosis bronchiectasis
- Controlled inhalation improves central and peripheral deposition in cystic fibrosis patients with moderate lung disease
- The Effect Of Telerehabilitation On Quality Of Life, Anxiety And Depression In Children With Cystic Fibrosis And Caregivers: A Single-Blind Randomized Trial
- Neutrophil-derived extracellular vesicles promote feed-forward inflammasome signaling in cystic fibrosis airways
- High affinity iron uptake by pyoverdine in Pseudomonas aeruginosa involves multiple regulators besides Fur, PvdS, and FpvI
- Everything in excess is opposed to nature, even vitamin D: a case report
- Mutation profiling of the c.1521_1523delCTT (p.Phe508del, F508del) CFTR allele using haplotype-resolved long-read next generation sequencing
- Men’s sexual and reproductive health in cystic fibrosis in the era of highly effective modulator therapies-A qualitative study
- CFTR modulator therapy alters plasma sphingolipid profiles in people with cystic fibrosis
- Clinical outcomes in cystic fibrosis at 6 years of age with tricare insurance coverage
- CONCERNS FOR PEOPLE WITH CYSTIC FIBROSIS (PWCF) WHEN TRAVELLING PRE COVID-19
- A Survey: Understanding the Health and Perspectives of People with CF Not Benefiting from CFTR Modulators
- Managing Pulmonary Infection in Adults With Cystic Fibrosis: Adult Cystic Fibrosis Series
- Feasibility of Home-based Exercise Program for Adults With Cystic Fibrosis
- Inflammation, Fibrosis and Cancer: Mechanisms, Therapeutic Options and Challenges
- Intestinal Inflammation and Alterations in the Gut Microbiota in Cystic Fibrosis: A Review of the Current Evidence, Pathophysiology and Future Directions
- Assays of CFTR Function In Vitro, Ex Vivo and In Vivo
- Airway Delivery of Hydrogel-Encapsulated Niclosamide for the Treatment of Inflammatory Airway Disease
- Unprecedented Epimerization of an Azithromycin Analogue: Synthesis, Structure and Biological Activity of 2′-Dehydroxy-5″-Epi-Azithromycin
- How can the transition of adolescents from a children’s to an adult CF center be improved? Analysis of adolescents’ and parents’ needs during the post-transfer period
- Guanylin and uroguanylin: a promising nexus in intestinal electrolyte and fluid homeostasis
- CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system
- Precision Medicine Based on CFTR Genotype for People with Cystic Fibrosis
- REAL-world clinical effectiveness of ivacaftor therapy in the first 24 months in two infants with cystic fibrosis and different gating mutations-A case report
- Biocompatible antimicrobial colistin loaded calcium phosphate nanoparticles for the counteraction of biofilm formation in cystic fibrosis related infections
- Investigation of respiratory rate in patients with cystic fibrosis using a minimal-impact biomotion system
- Cystic Fibrosis and Sleep Circadian Rhythms
- A Qualitative Exploration into Experiences and Attitudes Regarding Psychosocial Challenges, Self-compassion, and Mindfulness in a Population of Adults with Cystic Fibrosis
- Chasing a Breath of Fresh Air in Cystic Fibrosis (CF): Therapeutic Potential of Selective HDAC6 Inhibitors to Tackle Multiple Pathways in CF Pathophysiology
- Changes in Care during the COVID-19 Pandemic for People with Cystic Fibrosis
- The rapid reduction of infection-related visits and antibiotic use among people with cystic fibrosis after starting Elexacaftor-Tezacaftor-Ivacaftor
- Editorial: Functional Characterization and Pharmaceutical Targets in Common and Rare CFTR Dysfunctions
- Dynamic Upper and Lower Airway Microbiotas in Paediatric Bronchiectasis Exacerbations: A Pilot Study
- The P2X7 Receptor in Cystic Fibrosis Monocytes: Linking CFTR Deficiency to Inflammation
- Towards a new definition of non-cystic fibrosis bronchiectasis
- Continuing Medical Education Questions: December 2021
- Integrative pan cancer analysis reveals the importance of CFTR in lung adenocarcinoma prognosis
- Implementing the use of objective medication adherence data in routine clinical practice via the digital CFHealthHub platform: situation analysis and strategy development using the theoretical domains framework
- Is Positive Expiratory Pressure Physiotherapy an Effective Method for Airway Clearance in People With Cystic Fibrosis? A Cochrane Review Summary With Commentary
- Hypervitaminosis A with fulminant secondary intracranial hypertension following personalized medicine-based Elexacaftor/Tezacaftor/Ivacaftor initiation in a preadolescent with cystic fibrosis
- Pancreatic Insufficiency in a Child With p.Gly542* and c.2657+5G>A Heterozygote CFTR : A Case Report
- Relationship Between the Development of Impaired Glucose Tolerance, the Phenotype of CFLD, and the Risk of Liver Fibrosis
- Worldwide rates of diagnosis and effective treatment for cystic fibrosis
- A Hyperglycemic Microenvironment Inhibits Tendon-to-Bone Healing through the let-7b-5p/CFTR Pathway
- Seasonal variation of lung function in cystic fibrosis: longitudinal modeling to compare a Midwest US cohort to international populations
- Influence of Comorbidities and Airway Clearance on Mortality and Outcomes of Patients With Severe Bronchiectasis Exacerbations in Taiwan
- Engaging Stakeholders in the Development of a Reproductive Goals Decision AID for Women with Cystic Fibrosis
- Leveraging TOPMed imputation server and constructing a cohort-specific imputation reference panel to enhance genotype imputation among cystic fibrosis patients
- Study to Assess the Safety and PK of Oral and IV Xenleta in Adults With Cystic Fibrosis
- Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis
- Synthesis and bioactivity of readily hydrolysable novel cationic lipids for potential lung delivery application of mRNAs
- Human papillomavirus (HPV) and cervical dysplasia in adult female cystic fibrosis (CF) lung transplant recipients
- Preliminary method for profiling volatile organic compounds in breath that correlate with pulmonary function and other clinical traits of subjects diagnosed with cystic fibrosis: a pilot study
- Genetic analysis and intracytoplasmic sperm injection outcomes of Chinese patients with congenital bilateral absence of vas deferens
- Gastroparesis in Cystic Fibrosis
- Static mucus impairs bacterial clearance and allows chronic infection with Pseudomonas aeruginosa in the cystic fibrosis rat
- Identification and selection of healthy spermatozoa in heterozygous carriers of the Phe508del-variant of the CFTR-gene in assisted reproduction
- Nutrition management in adults with cystic fibrosis
- When is cystic fibrosis not cystic fibrosis? The importance of appropriately classifying patients
- Genomic diversity and antimicrobial resistance of Prevotella species isolated from chronic lung disease airways
- Porphyromonas pasteri and Prevotella nanceiensis in the sputum microbiota are associated with increased decline in lung function in individuals with cystic fibrosis
- Hybrid Lipid/Polymer Nanoparticles to Tackle the Cystic Fibrosis Mucus Barrier in siRNA Delivery to the Lungs: Does PEGylation Make the Difference?
- Forskolin induced swelling (FIS) assay in intestinal organoids to guide eligibility for compassionate use treatment in a CF patient with a rare genotype
- The Predictive Role of Psychological Status and Disease Severity Indexes on Quality of Life Among Patients with Non-CF Bronchiectasis
- Genetic and Clinical Demographics of Adult Cystic Fibrosis Patients in a Middle Eastern Population
- Maternal and Fetal Problems in Patients with Non-Cystic Fibrosis Bronchiectasis During Pregnancy
- The Frequency of Obstructive Sleep Apnea in Patients with Non-cystic Fibrosis Bronchiectasis
- Association Between Cystic Fibrosis Severity Markers and CFTR Genotypes in Turkish Children
- Food Insecurity and Mental Health During the COVID-19 Pandemic in Cystic Fibrosis Households
- New concepts in the pathogenesis of cystic fibrosis-related diabetes
- Measuring the effect of elexacaftor/tezacaftor/ivacaftor combination therapy on the respiratory pump in people with CF using dynamic chest radiography
- Functionally Additive Fixed Positive and Negative Charges in the CFTR Channel Pore Control Anion Binding and Conductance
- The Success of the Cystic Fibrosis Registry of Turkey for Improvement of Patient Care
- Pseudomonas aeruginosa adaptation in cystic fibrosis patients increases C5a levels and promotes neutrophil recruitment
- Early Growth Response 1 Suppresses Macrophage Phagocytosis by Inhibiting NRF2 Activation Through Upregulation of Autophagy During Pseudomonas aeruginosa Infection
- What Is Most Suitable for Children With Cystic Fibrosis-The Relationship Between Spirometry, Oscillometry, and Multiple Breath Nitrogen Washout
- Assessing the Utility of an Outpatient Exercise Program for Children With Cystic Fibrosis: A Quality Improvement Project
- Role of Salivary Biomarkers in Cystic Fibrosis: A Systematic Review
- Impaired Regulatory Volume Decrease and Characterization of Underlying Volume-Activated Currents in Cystic Fibrosis Human Cholangiocyte Cell Line
- The First 4 Years – Outcome of Children Identified by Newborn Screening for CF in Germany
- Generation of human induced pluripotent stem cells from cystic fibrosis patient carrying nonsense mutation (p.S308X) in CFTR gene
- PROBIOTIC USE IN ADULTS WITH CYSTIC FIBROSIS IS COMMON AND INFLUENCED BY GASTROINTESTINAL HEALTH NEEDS: A CROSS SECTIONAL SURVEY STUDY
- Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis
- Cystic fibrosis foundation otolaryngology care multidisciplinary consensus recommendations
- Evaluating the alginate oligosaccharide (OligoG) as a therapy for Burkholderia cepacia complex cystic fibrosis lung infection
- Bone mineral density screening by DXA for people with cystic fibrosis: A registry analysis of patient and program factors influencing rates of screening
- Radiomics derived morphological features predict pulmonary function response under lumacaftor-ivacaftor in patients with cystic fibrosis
- Forskolin-induced Organoid Swelling is Associated with Long-term CF Disease Progression
- Fecal Calprotectin and Phenotype Severity in Patients with Cystic Fibrosis: A Systematic Review and Meta-Analysis
- The Challenges of Eating Well for People Living with Cystic Fibrosis: an Interview Study Exploring the Use of Mindful Eating Approaches and Behaviours to Support Optimal Nutritional Status
- Pseudomonas aeruginosa cytochrome P450 CYP168A1 is a fatty acid hydroxylase that metabolizes arachidonic acid to the vasodilator 19-HETE
- Investigating Nontuberculous Mycobacteria Transmission at the Colorado Adult Cystic Fibrosis Program
- Clinical Phenotypes of Cystic Fibrosis Carriers
- Measuring the Impact of an Empiric Antibiotic Algorithm for Pulmonary Exacerbation in Children and Young Adults with Cystic Fibrosis
- Ultrasound-Derived Diaphragm Contractile Reserve as a Marker of Clinical Status in Patients With Cystic Fibrosis
- Roscovitine Worsens Mycobacterium abscessus Infection by Reducing DUOX2-Mediated Neutrophil Response
- Type 2 inflammation in cystic fibrosis: New insights
- Discovery of Novel Inhibitors of Uridine Diphosphate-N-Acetylenolpyruvylglucosamine Reductase (MurB) from Pseudomonas aeruginosa, an Opportunistic Infectious Agent Causing Death in Cystic Fibrosis Patients
- Combined Host- and Pathogen-Directed Therapy for the Control of Mycobacterium abscessus Infection
- Introducing a MAP for adherence care in the paediatric cystic fibrosis clinic: a multiple methods implementation study
- The lung microbiota in children with cystic fibrosis captured by induced sputum sampling
- Mycobacterium abscessus drug discovery using machine learning
- International Approaches to Management of CFTR-Related Metabolic Syndrome/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis
- Poly (acetyl, arginyl) glucosamine disrupts Pseudomonas aeruginosa biofilms and enhances bacterial clearance in a rat lung infection model
- Computational investigations on the potential role of hygrophorones as quorum sensing inhibitors against LasR protein of Pseudomonas aeruginosa
- Determinants of Daily Physical Activity Level in Patients With Cystic Fibrosis – Pilot Study Protocol
- Inhibiting Mycobacterium abscessus Cell Wall Synthesis: Using a Novel Diazabicyclooctane β-Lactamase Inhibitor To Augment β-Lactam Action
- Cystic Fibrosis: A Disease in Transformation, Yet More Work to be Done!
- Cytotoxic activity of Staphylococcus aureus isolates from a cohort of Mexican children with cystic fibrosis
- Mucoid Coating Provides a Growth Advantage to Pseudomonas aeruginosa at Oil-Water Interfaces
- Molecular Mechanisms of Staphylococcus and Pseudomonas Interactions in Cystic Fibrosis
- Sustained Reduction in Time to Data Entry in the Cystic Fibrosis Foundation Registry
- Molecular dynamics and functional characterization of I37R-CFTR lasso mutation provide insights into channel gating activity
- A flexible summary statistics-based colocalization method with application to the mucin cystic fibrosis lung disease modifier locus
- Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains
- Lived experiences of individuals with cystic fibrosis on CFTR-modulators
- Cystic fibrosis: a call for papers for ECFS 2022
- Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme
- Aquagenic wrinkling of the palms in cystic fibrosis patients treated with ivacaftor
- The Effect of Dynasore Upon the Negative Interaction Between ENaC and CFTR Channels in Xenopus laevis Oocytes
- Five cases of missed cystic fibrosis heterozygous mutations identified after a positive newborn screen on a sibling
- Treatment of cystic fibrosis related bone disease
- Weight loss and cystic disease progression in autosomal dominant polycystic kidney disease
- Yield of chest computed tomography angiogram in cystic fibrosis patients with suspected pulmonary embolism
- Lipid-driven CFTR clustering is impaired in CF and restored by corrector drugs
- Fat-Soluble Vitamins in Standard vs. Liposomal Form Enriched with Vitamin K2 in Cystic Fibrosis: A Randomized Multi-Center Trial
- Correction: Dagenais et al. Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review. J. Clin. Med. 2021, 10, 23
- First Case of COVID-19 Treated with Monoclonal Anti-Spike Antibodies in a Patient with Cystic Fibrosis in Romania
- Therapeutic CFTR Correction Normalizes Systemic and Lung-Specific S1P Level Alterations Associated with Heart Failure
- Activation of Notch3 in Renal Tubular Cells Leads to Progressive Cystic Kidney Disease
- The Use of Targeted Monoclonal Antibodies in the Treatment of ABPA-A Case Series
- Aerosol-Mediated Non-Viral Lung Gene Therapy: The Potential of Aminoglycoside-Based Cationic Liposomes
- The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis
- National multi-centre study found a low prevalence of severely impaired lung function in children and adolescents
- Case Report: Japanese Siblings of Cystic Fibrosis With a Novel Large Heterozygous Deletion in the CFTR Gene
- Review article: epidemiology, pathogenesis and management of liver disease in adults with cystic fibrosis
- Cellular and molecular architecture of submucosal glands in wild-type and cystic fibrosis pigs
- Formation of Calprotectin-Derived Peptides in the Airways of Children with Cystic Fibrosis
- Body Mass Index Recovery Following Lung Transplant for Cystic Fibrosis
- The physical therapy consultation: A qualitative study of the experience of parents of infants with cystic fibrosis in Australia
- Malignancies in patients with cystic fibrosis: a case series
- Simulated intravenous versus inhaled tobramycin with and without intravenous ceftazidime evaluated against hypermutable Pseudomonas aeruginosa via a dynamic biofilm model and mechanism-based modeling
- ADHERENCE TO INHALED THERAPIES OVER 4 YEARS IN PEOPLE WITH CYSTIC FIBROSIS
- Nasal epithelial cells as a gold-standard predictive model for personalized medicine in cystic fibrosis
- Development of Inhibitors of SAICAR Synthetase (PurC) from Mycobacterium abscessus Using a Fragment-Based Approach
- Metabolic reprogramming in a slowly developing orthologous model of polycystic kidney disease
- Challenging the paradigm: moving from umbrella labels to treatable traits in airway disease
- Cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)
- Progress in precision medicine in cystic fibrosis: a focus on CFTR modulator therapy
- Preferred health outcome states following treatment for pulmonary exacerbations of cystic fibrosis
- The role of inspiratory capacity and tidal flow in diagnosing exercise ventilatory limitation in Cystic Fibrosis
- The genetics of autosomal recessive polycystic kidney disease (ARPKD)
- Geographic distribution and phenotype of European people with cystic fibrosis carrying A1006E mutation
- Elevation of alkaline phosphatase and long-term drug therapy for cystic fibrosis
- Abdominal Surgical Procedures in Adult Patients with Cystic Fibrosis: What are the Risks?
- Bone Marrow Transplantation Rescues Monocyte Recruitment Defect and Improves Cystic Fibrosis in Mice
- Role and mechanism(s) of incretin-dependent therapies for treating diabetes mellitus
- An LC-MS/MS method for simultaneous analysis of the cystic fibrosis therapeutic drugs colistin, ivacaftor and ciprofloxacin
- Swyer-James syndrome: A cause of adult-onset dyspnea in a patient with adult polycystic kidney disease
- Contemporary N(2) and SF(6) multiple breath washout in infants and toddlers with cystic fibrosis
- Role of AxyABM overexpression in acquired resistance in Achromobacter xylosoxidans
- Successful non-surgical treatment of pseudomonas choroidal abscess in cystic fibrosis with previous double lung transplantation
- The future of dry powder inhaled therapy: Promising or Discouraging for systemic disorders?
- Advanced detection and sensing strategies of Pseudomonas aeruginosa and quorum sensing biomarkers: A review
- Bacterial low-abundant taxa are key determinants of a healthy airway metagenome in the early years of human life
- Cystic fibrosis related liver disease and endocrine considerations
- Multilocus Sequence Typing Reveals Extensive Genetic Diversity of the Emerging Fungal Pathogen Scedosporium aurantiacum
- Sequence diversity of the Pseudomonas aeruginosa population in loci that undergo microevolution in cystic fibrosis airways
- Stage-Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function
- Laparoscopic morphological aspects and tentative explanation of the aetiopathogenesis of isolated endometriosis of the sciatic nerve: a review based on 267 patients
- Trikafta Rescues CFTR and Lowers Monocyte P2X7R-Induced Inflammasome Activation in Cystic Fibrosis
- The effect of Control IQ hybrid closed loop technology on glycemic control in adolescents and adults with cystic fibrosis related diabetes
- Receptor-mediated activation of CFTR via prostaglandin signaling pathways in the airway
- The Changing Face of Cystic Fibrosis: An Update for Anesthesiologists
- Diagnostic Uncertainty, Microbes, and the Isolation of People with Cystic Fibrosis
- Vitamins A, D, E status as related to supplementation and lung disease markers in young children with cystic fibrosis
- Exon-skipping antisense oligonucleotides for cystic fibrosis therapy
- Open reading frame correction using splice-switching antisense oligonucleotides for the treatment of cystic fibrosis
- Environment in the lung of cystic fibrosis patients stimulates the expression of biofilm phenotype in Mycobacterium abscessus
- What Is Cystic Fibrosis?
- Annual decline in lung function in adults with sickle cell disease is similar to that observed in adults with cystic fibrosis
- Report of two events of nosocomial outbreak and pseudo-outbreak due to contamination with Achromobacter spp
- Trabecular bone score in people with cystic fibrosis
- Rare Trafficking CFTR Mutations Involve Distinct Cellular Retention Machineries and Require Different Rescuing Strategies
- Bicarbonate Transport in Cystic Fibrosis and Pancreatitis
- Rescue of Mutant CFTR Trafficking Defect by the Investigational Compound MCG1516A
- Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation
- Correlation between clinical-functional parameters and number of lobes involved in non-cystic fibrosis bronchiectasis
- Treatment of Polarized Cystic Fibrosis Airway Cells With HGF Prevents VX-661-Rescued F508del-CFTR Destabilization Caused by Prolonged Co-exposure to VX-770
- POINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? Yes
- COUNTERPOINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? No
- Revisiting a diagnosis of cystic fibrosis – Uncertainties and considerations
- Exploring provider attitudes and perspectives related to men’s health in cystic fibrosis
- Focusing on powder processing in Dry Powder Inhalation product development, manufacturing and performance
- Understanding motivation for Australian adolescents and young adults with cystic fibrosis: Modifiable factors to support self-management
- Mechanism of CFTR correction by type I folding correctors
- CFTR variants are associated with chronic bronchitis in smokers
- Scrutiny of Metal Ion Binding Sites in Different Alginate Lyases through In Silico Analysis
- CFTR deficiency aggravates Ang II induced vasoconstriction and hypertension by regulating Ca2+ influx and RhoA/Rock pathway in VSMCs
- What is the role of Achromobacter species in patients with cystic fibrosis?
- Cardiac involvement in cystic fibrosis evaluated using cardiopulmonary magnetic resonance
- Neutrophil respiratory burst activity is not exaggerated in cystic fibrosis
- Systems Approaches to Unravel Molecular Function: High-content siRNA Screen Identifies TMEM16A Traffic Regulators as Potential Drug Targets for Cystic Fibrosis
- The Pseudomonas aeruginosa homeostasis enzyme AlgL clears the periplasmic space of accumulated alginate during polymer biosynthesis
- Evaluation of the safety of cefepime prolonged infusions in pediatric patients with cystic fibrosis
- The Effects of Sub-inhibitory Antibiotic Concentrations on Pseudomonas aeruginosa : Reduced Susceptibility Due to Mutations
- CF and male health: Sexual and reproductive health, hypogonadism, and fertility
- Carbonic anhydrase and soluble adenylate cyclase regulation of cystic fibrosis cellular phenotypes
- An update on methods for assessing bone quality and health in Cystic fibrosis
- Gestational and pregestational diabetes in pregnant women with cystic fibrosis
- Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe
- Utilizing in silico and in vitro methods to identify possible binding sites of a novel ligand against Pseudomonas aeruginosa phospholipase toxin ExoU
- Cystic Fibrosis Transmembrane Conductance Regulator: Roles in Chronic Obstructive Pulmonary Disease
- Prenatal imaging features and perinatal outcomes of foetal volvulus-A literature review
- Culture and Imaging of Human Nasal Epithelial Organoids
- The nutritional environment is sufficient to select coexisting biofilm and quorum-sensing mutants of Pseudomonas aeruginosa
- Depressive symptoms in cystic fibrosis patients and their caretakers are best predicted by their respective sense of belonging
- An Overview on the Upper and Lower Airway Microbiome in Cystic Fibrosis Patients
- The role of genetic modifiers, inflammation and CFTR in the pathogenesis of Cystic fibrosis related diabetes
- Use of estrogen supplementation is associated with higher quality of life scores in women with cystic fibrosis
- Non-invasive tools for detection of liver disease in children and adolescents with cystic fibrosis
- Case report of compound CFTR variants in Korean siblings with cystic fibrosis: importance of differentiating cystic fibrosis from inflammatory bowel disease
- The sodium/glucose cotransporters as potential therapeutic targets for CF lung diseases revealed by human lung organoid swelling assay
- What’s in a name: The importance of lung transplant at Cystic Fibrosis Foundation Accredited Care Centers for patients with Cystic Fibrosis
- Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation
- Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis
- Lung nodules due to Candida parapsilosis in a person with cystic fibrosis
- Case Report: Embolization of the Left Atrial Coronary Artery in Cystic Fibrosis for Control of Massive Hemoptysis due to Coronary to Bronchial Artery Communication
- Esc peptides as novel potentiators of defective cystic fibrosis transmembrane conductance regulator: an unprecedented property of antimicrobial peptides
- Trends in Cystic Fibrosis survival over 40 years in South Africa: an observational cohort study
- Opportunity for pharmacogenomic testing in patients with cystic fibrosis
- RISK FACTORS FOR OBSTRUCTIVE SLEEP APNEA IN CYSTIC FIBROSIS
- Variable Susceptibility to Gallium Compounds of Major Cystic Fibrosis Pathogens
- Genetic counseling access for parents of newborns who screen positive for cystic fibrosis: consensus guidelines
- Pediatric Pulmonology 2020 Year in Review: Rare and Diffuse Lung Disease
- Six minute walk test in Italian children with cystic fibrosis aged 6 and 11
- Remotely Supervised Exercise for Adults With Cystic Fibrosis
- Cystic Fibrosis in Children: A Pediatric Anesthesiologist’s Perspective
- Deposition of Inhaled Levofloxacin in Cystic Fibrosis Lungs Assessed by Functional Respiratory Imaging
- Probing Allosteric Hsp70 Inhibitors by Molecular Modelling Studies to Expedite the Development of Novel Combined F508del CFTR Modulators
- Vitamin D Status in Pediatric and Young Adult Cystic Fibrosis Patients. Are the New Recommendations Effective?
- Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood
- Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial
- Safety and pharmacokinetics of Roscovitine (Seliciclib) in cystic fibrosis patients chronically infected with Pseudomonas aeruginosa, a randomized, placebo-controlled study
- Structural lung disease in preschool children with cystic fibrosis: An 18 month natural history study
- Comprehensive Analysis of Chemical Structures That Have Been Tested as CFTR Activating Substances in a Publicly Available Database CandActCFTR
- Long-term macrolide therapy in asthma
- Exercise intolerance in cystic fibrosis-the role of CFTR modulator therapies
- Portuguese adolescents with cystic fibrosis and their parents: An intervention proposal for nursing clinical practice
- Quality improvement and rapid PDSA cycles to maintain routine surveillance of pulmonary pathogens during the COVID-19 pandemic in a pediatric cystic fibrosis clinic
- Gamma-glutamyl transpeptidase-to-platelet ratio as a biomarker of liver disease and hepatic fibrosis severity in paediatric Cystic Fibrosis
- Clinical complications in children with false-negative results in cystic fibrosis newborn screening
- Bactericidal effects and stability of LL-37 and CAMA in the presence of human lung epithelial cells
- Specialized pro-resolving mediators in respiratory diseases
- A disorder clinically resembling cystic fibrosis caused by biallelic variants in the AGR2 gene
- Genome-Wide RNAi Screening Identifies Novel Pathways/Genes Involved in Oxidative Stress and Repurposable Drugs to Preserve Cystic Fibrosis Airway Epithelial Cell Integrity
- Standards of Nutritional Care for Patients with Cystic Fibrosis: A Methodological Primer and AGREE II Analysis of Guidelines
- New Auranofin Analogs with Antibacterial Properties against Burkholderia Clinical Isolates
- Potential Therapeutic Targets for Combination Antibody Therapy against Pseudomonas aeruginosa Infections
- A Polyclonal Antibody Raised against the Burkholderia cenocepacia OmpA-like Protein BCAL2645 Impairs the Bacterium Adhesion and Invasion of Human Epithelial Cells In Vitro
- Revisiting the Role of Leukocytes in Cystic Fibrosis
- High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids
- Immunoglobulin A Mucosal Immunity and Altered Respiratory Epithelium in Cystic Fibrosis
- Causes of Exocrine Pancreatic Insufficiency Other Than Chronic Pancreatitis
- Genetic Modifying Factors of Cystic Fibrosis Phenotype: A Challenge for Modern Medicine
- The Need for Precision Therapies as Determined by Genetic Signature for Cystic Fibrosis
- Prediction of Real-World Long-Term Outcomes of People with CF Homozygous for the F508del Mutation Treated with CFTR Modulators
- In Vitro Activity of 22 Antibiotics against Achromobacter Isolates from People with Cystic Fibrosis. Are There New Therapeutic Options?
- Evaluation of Three Culture Media for Isolation of Burkholderia cepacia Complex from Respiratory Samples of Patients with Cystic Fibrosis
- Revisiting CFTR Interactions: Old Partners and New Players
- Advanced but not mild liver disease is a predictor of decreased survival in children with cystic fibrosis, with far greater impact in females: A 27-year real-life cohort study
- Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward
- Outcomes following lung re-transplantation in patients with cystic fibrosis
- SARS-CoV-2 antibodies among people with cystic fibrosis prior to the vaccination campaign: A seroprevalence study in two specialized centres in Northern Italy
- Comparison of the airway microbiota in children with chronic suppurative lung disease
- Flagellin From Pseudomonas aeruginosa Modulates SARS-CoV-2 Infectivity in Cystic Fibrosis Airway Epithelial Cells by Increasing TMPRSS2 Expression
- Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial
- Comparison of biosimilar Tigerase and Pulmozyme in long-term symptomatic therapy of patients with cystic fibrosis and severe pulmonary impairment (subgroup analysis of a Phase III randomized open-label clinical trial (NCT04468100))
- MutTMPredictor: Robust and accurate cascade XGBoost classifier for prediction of mutations in transmembrane proteins
- Prospects of Inhaled Phage Therapy for Combatting Pulmonary Infections
- A Volatile and Dynamic Longitudinal Microbiome Is Associated With Less Reduction in Lung Function in Adolescents With Cystic Fibrosis
- Family planning, pregnancy and birth in women with lung conditions: a worldwide survey
- Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles
- Coming Up for Air: The Role of Anaerobes in Cystic Fibrosis
- Ceftaroline Versus Vancomycin for the Treatment of Acute Pulmonary Exacerbations of Cystic Fibrosis in Adults
- Health-Related Quality of Life Assessment: An Inexpensive Tool for Cystic Fibrosis Care
- Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis
- Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis
- Phosphodiesterase 8A Regulates CFTR Activity in Airway Epithelial Cells
- Cystic Fibrosis Mucus Model to Design More Efficient Drug Therapies
- Cystic fibrosis: current concepts
- The Impact of COVID-19 in Cystic Fibrosis
- The Management of Cystic Fibrosis Chronic Rhinosinusitis: An Evidenced-Based Review with Recommendations
- Comparison of Transient Elastography, ShearWave Elastography, Magnetic Resonance Elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with Cystic Fibrosis
- Long-term macrolide treatment for non-cystic fibrosis bronchiectasis in children: a meta-analysis
- Mutations in CFTR genes are associated with oligoasthenospermia in infertile men undergoing IVF
- Impact of the COVID-19 pandemic: How our response is shaping the future of cystic fibrosis care
- Financial impacts of the COVID-19 pandemic on cystic fibrosis care: lessons for the future
- Financial impacts of the COVID-19 pandemic on cystic fibrosis care: Lessons for the future (Commentary) A.L. Stephenson
- Barriers and facilitators to implementing telehealth services during the COVID-19 pandemic: A qualitative analysis of interviews with cystic fibrosis care team members
- Design and methods for understanding the state of cystic fibrosis care amid the COVID-19 pandemic
- Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis
- Impact of COVID-19 on mental health: Effects on screening, care delivery, and people with cystic fibrosis (Commentary)
- Patient and family experience of telehealth care delivery as part of the CF chronic care model early in the COVID-19 pandemic
- Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors
- Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network
- Evaluating barriers to and promoters of telehealth during the COVID-19 pandemic at U.S. cystic fibrosis programs
- The effect of the cystic fibrosis care center on outcomes after lung transplantation for cystic fibrosis
- Healthcare-associated links in transmission of nontuberculous mycobacteria among people with cystic fibrosis (HALT NTM) study: Rationale and study design
- Cystic fibrosis related diabetes (CFRD) prognosis
- Cystic fibrosis-related diabetes: The patient perspective
- A growing trend: CFTR modulators for cystic fibrosis lung transplant recipients
- Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers
- Low body mass index as a barrier to lung transplant in cystic fibrosis
- Magnetic resonance imaging of the gastrointestinal tract shows reduced small bowel motility and altered chyme in cystic fibrosis compared to controls
- Developing Ciprofloxacin Dry Powder for Inhalation: A Story of Challenges and Rational Design in the Treatment of Cystic Fibrosis Lung Infection
- Clinical Outcomes of Cystic Fibrosis Patients with Pseudomonas aeruginosa Bloodstream Infections
- Management of Multidrug Resistant Infections in Lung Transplant Recipients with Cystic Fibrosis
- Eating disorders and body image in cystic fibrosis
- The role of modulators in cystic fibrosis related diabetes
- Cystic fibrosis-related diabetes: Prevalence, screening, and diagnosis
- Impact of Achromobacter spp. Isolation on Clinical Outcomes in Children with Cystic Fibrosis
- Evaluation of a novel CFTR potentiator in copd ferrets with acquired cftr dysfunction
- The effect of danusertib, an Aurora kinase inhibitor, onto the cytotoxicity, cell cycle and apoptosis in pancreatic ductal adenocarcinoma cells
- Perception, experience and relationship with food and eating in adults with cystic fibrosis
- Gene mutations in congenital bilateral absence of the vas deferens: An update
- Cross-talk of inflammatory mediators and airway epithelium reveals the cystic fibrosis transmembrane conductance regulator as a major target
- Acute ST-elevation myocardial infarction in two young women with cystic fibrosis and cystic fibrosis-related diabetes
- Autogenic drainage for airway clearance in cystic fibrosis
- Major Decrease in Lung Transplantation for Patients with Cystic Fibrosis in France
- The Controversy of Drug Hypersensitivity in Patients with Cystic Fibrosis and Review of the Literature
- Olfactory dysfunction in people with cystic fibrosis with at least one copy of F508del
- Circadian Rhythmicity in Cerebral Microvascular Tone Influences Subarachnoid Hemorrhage-Induced Injury
- The journal of cystic fibrosis’ 20th anniversary
- Home monitoring for cystic fibrosis: The future is now
- The Relationship Between CFTR Gene Mutations and Exercise Capacity
- Cystic fibrosis associated liver disease in children
- Food protein-induced enterocolitis syndrome in a patient with pseudo-Bartter syndrome associated with cystic fibrosis. A case report
- Combining Ivacaftor and Intensive Antibiotics Achieves Limited Clearance of Cystic Fibrosis Infections
- Increased susceptibility of cystic fibrosis airway epithelial cells to ferroptosis
- Forewarned is forearmed: The cardiovascular time bomb in Cystic Fibrosis
- Meteorological Factors Influence the Presence of Fungi in the Air; A 14-Month Surveillance Study at an Adult Cystic Fibrosis Center
- Patient and Provider Experience With Cystic Fibrosis Telemedicine Clinic
- Cystic Fibrosis: Systems Biology Analysis from Homozygous p.Phe508del Variant Patients’ Samples Reveals Perturbations in Tissue-Specific Pathways
- Genetic determinants of virulence and antibiotic resistance are common for Pseudomonas aeruginosa ST235 isolates from cystic fibrosis patients from various geographical regions
- In vitro differentiation of ciliated cells in ALI-cultured human airway epithelium – The framework for functional studies on airway differentiation in ciliopathies
- Intestinal function and transit associate with gut microbiota dysbiosis in cystic fibrosis
- Hepatic inflammation and liver fibrogenesis: a potential target for the treatment of cystic echinococcosis-associated hepatic injury
- Real-World Outcomes in Cystic Fibrosis Telemedicine Clinical Care in a Time of a Global Pandemic
- Evaluation of Long-term Safety and Efficacy of ELX/TEZ/IVA in Cystic Fibrosis (CF) Participants 2 Years and Older
- Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis
- CFTR mutational screening by next-generation sequencing reveals novel variants and a high carrier rate in a Middle Eastern population
- Clinical validation of digital biomarkers for pediatric patients with asthma and cystic fibrosis – Potential for clinical trials and clinical care
- Synergy in Cystic Fibrosis Therapies: Targeting SLC26A9
- Defining the Importance of Age-Related Changes in Drug Clearance to Optimizing Aminoglycoside Dosing Regimens for Adult Patients with Cystic Fibrosis
- Environmental predictors of pulmonary nontuberculous mycobacteria (NTM) sputum positivity among persons with cystic fibrosis in the state of Florida
- Role of the flagellar hook in the structural development and antibiotic tolerance of Pseudomonas aeruginosa biofilms
- Nebulisation therapy in patients with cystic fibrosis – consensus of the Polish Cystic Fibrosis Society
- Magnetic resonance imaging of cystic fibrosis: Multi-organ imaging in the age of CFTR modulator therapies
- A new path for CF clinical trials through the use of historical controls
- The safety and sustainability of bottle-pep therapy in pediatric patients with cystic fibrosis
- Transcriptome analysis of Pseudomonas aeruginosa biofilm infection in an ex vivo pig model of the cystic fibrosis lung
- Goblet Cell Hyperplasia is not Epithelial-Autonomous in the Cftr Knockout Intestine
- Translating in vitro CFTR rescue into small molecule correctors for cystic fibrosis using the Library of Integrated Network-based Cellular Signatures drug discovery platform
- Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes
- Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes. Reply
- Comparison of endoscopic sinus surgery timing in lung transplant patients with cystic fibrosis
- Frequency of CFTR variants in southern Brazil and indication for modulators therapy in patients with cystic fibrosis
- Clinical course and risk factors for severe COVID-19 among Italian patients with cystic fibrosis: a study within the Italian Cystic Fibrosis Society
- Not a wild goose chase: long-lasting MRSA negative status following eradication therapy for chronic MRSA infection in patients with cystic fibrosis
- Infant BMI or Weight-for-Length and Risk of Undernutrition in Childhood Among Children with Cystic Fibrosis
- The Effect of Telerehabilitation on Functional Capacity, Oxidative Stress and Respiratory Parameters in Cystic Fibrosis
- Molecular pathology of the R117H cystic fibrosis mutation is explained by loss of a hydrogen bond
- Endobronchial Therapy With Gentamicin and Dexamethasone After Airway Clearance by Bronchoscopy in Exacerbation of Non-Cystic Fibrosis Bronchiectasis: A Real-World Observational Study
- Editorial: Novel Anti-Inflammatory Approaches for Cystic Fibrosis Lung Disease: Identification of Molecular Targets and Design of Innovative Therapies
- Acidic Microenvironment Determines Antibiotic Susceptibility and Biofilm Formation of Pseudomonas aeruginosa
- miR-224-5p and miR-545-5p Levels Relate to Exacerbations and Lung Function in a Pilot Study of X-Linked MicroRNA Expression in Cystic Fibrosis Monocytes
- Impaired glucose tolerance and indeterminate glycemia in cystic fibrosis
- Obesity in cystic fibrosis
- Long-Term Outcomes in Real Life of Lumacaftor-Ivacaftor Treatment in Adolescents With Cystic Fibrosis
- Incidence of breast cancer in people with cystic fibrosis: A cause for concern?
- Development and evaluation of a virtual patient-centered outcomes research training program for the cystic fibrosis community
- Sweat metabolomics before and after intravenous antibiotics for pulmonary exacerbation in people with cystic fibrosis
- Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis
- Synchronous association of hepatocellular carcinoma and cystic echinococcosis with unusual pathology: report on diagnostic and pathological variability
- Diagnosis and management of non-cystic fibrosis bronchiectasis
- Burden of cystic fibrosis in children <12 years of age prior to the introduction of CFTR modulator therapies
- Primary nasal epithelial cells from patients with cystic fibrosis hold promise for guiding precision medicine and expanding treatment
- S-nitrosothiols signaling in cystic fibrosis airways
- A Comparative Analysis of Pricing and Reimbursement of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Europe
- Might Routine Vitamin A Monitoring in Cystic Fibrosis Patients Reduce Virus-Mediated Lung Pathology?
- Editorial: Fungal Respiratory Infections in Cystic Fibrosis
- Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients
- Non-liver mRNA Delivery
- The Genetic Analysis of Cystic Fibrosis Patients With Seven Novel Mutations in the CFTR Gene in the Central Anatolian Region of Turkey
- Non-Pharmaceutical Techniques for Obstructive Airway Clearance Focusing on the Role of Oscillating Positive Expiratory Pressure (OPEP): A Narrative Review
- 25-Hydroxy vitamin D and body composition are associated with pulmonary function in non-cystic fibrosis bronchiectasis: A cross-sectional study
- Association of site of treatment with clinical outcomes following intravenous antimicrobial treatment of a pulmonary exacerbation
- Disconcerting and Counter-Intuitive Findings from a Trial of Exercise in Cystic Fibrosis- Can Exercise Make our Patients Worse?
- Inquilinus limosus isolated from a patient with chronic cystic fibrosis. First report in Mexico and evidence that co-infection with Pseudomonas aeruginosa promotes the accelerated and increased formation of extracellular neutrophil traps
- Nonspecific binding of common anti-CFTR antibodies in ciliated cells of human airway epithelium
- Effects of Training Intensity on Physical Fitness and Body Composition in Cystic Fibrosis
- Clinical characteristics and outcome of SARS -CoV-2 infection in patients with cystic fibrosis managed at home
- Ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis
- Delayed HIV diagnosis in a cystic fibrosis patient: Not just another exacerbation
- Gynecologic health care for females with cystic fibrosis
- Continuous Glucose Monitoring and HbA1c in Cystic Fibrosis: Clinical Correlations and Implications for CFRD Diagnosis
- The use of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis post-liver transplant: a case series
- CFTR limits F-actin formation and promotes morphological alignment with flow in human lung microvascular endothelial cells
- Mycobacterium abscessus, a model of resistance to multiple antibiotic classes
- A step back in time: The basics of CF care still matter!
- Perceptions of barriers to and facilitators of physical activity in adults with cystic fibrosis
- Molecular epidemiology and phylogenomic analysis of Mycobacterium abscessus clinical isolates in an Asian population
- Emotional Impact of COVID-19 Pandemic on Adults with Cystic Fibrosis
- Validity and reliability of the one-minute sit-to-stand test for the measurement of cardio-respiratory responses in children with cystic fibrosis
- Cystic fibrosis: a diagnosis in an adolescent
- Continuous glucose monitoring systems for monitoring cystic fibrosis-related diabetes
- Validation of a Custom Next-Generation Sequencing Assay for Cystic Fibrosis Newborn Screening
- Is mHealth a useful therapy for improving physical or emotional health in adolescents with cystic fibrosis? A systematic review
- Urticaria multiforme-like eruption due to a novel agent elexacaftor/tezacaftor/ivacaftor in a pediatric patient with cystic fibrosis
- Transplantation of human iPS cell-derived airway cells on vitrigel membrane into rat nasal cavity
- Collection, Expansion, and Differentiation of Primary Human Nasal Epithelial Cell Models for Quantification of Cilia Beat Frequency
- Cystic Fibrosis Newborn Screening: Five-year Experience from a Tertiary Care Center
- Assessment of body composition in cystic fibrosis: agreement between skinfold measurement and densitometry
- The bitter end: T2R bitter receptor agonists elevate nuclear calcium and induce apoptosis in non-ciliated airway epithelial cells
- Evaluation of Pulmonary Structure and Function in Patients with Cystic Fibrosis from Electrical Impedance Tomography Data
- Nano- and Macroscale Imaging of Cholesterol Linoleate and Human Beta Defensin 2-Induced Changes in Pseudomonas aeruginosa Biofilms
- Sub-Inhibitory Antibiotic Exposure and Virulence in Pseudomonas aeruginosa
- Integrity and Stability of PTC Bearing CFTR mRNA and Relevance to Future Modulator Therapies in Cystic Fibrosis
- The Effects of Aspergillus fumigatus Colonization on Lung Function in Patients with Cystic Fibrosis
- Lung and Gut Microbiota Changes Associated with Pseudomonas aeruginosa Infection in Mouse Models of Cystic Fibrosis
- Putative RNA Ligase RtcB Affects the Switch between T6SS and T3SS in Pseudomonas aeruginosa
- Methods Used to Evaluate the Immediate Effects of Airway Clearance Techniques in Adults with Cystic Fibrosis: A Systematic Review and Meta-Analysis
- miR-125b Promotes Colorectal Cancer Migration and Invasion by Dual-Targeting CFTR and CGN
- CFTR Protein: Not Just a Chloride Channel?
- TMEM16A/ANO1: Current Strategies and Novel Drug Approaches for Cystic Fibrosis
- The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
- Apparent Yield Stress of Sputum as a Relevant Biomarker in Cystic Fibrosis
- Impact of Airway Inflammation on the Efficacy of CFTR Modulators
- Employment Status and Work Ability in Adults with Cystic Fibrosis
- The Distribution and Role of the CFTR Protein in the Intracellular Compartments
- Identification of cbiO Gene Critical for Biofilm Formation by MRSA CFSa36 Strain Isolated from Pediatric Patient with Cystic Fibrosis
- Defective Glyoxalase 1 Contributes to Pathogenic Inflammation in Cystic Fibrosis
- Anti-Virulence Properties of Coridothymus capitatus Essential Oil against Pseudomonas aeruginosa Clinical Isolates from Cystic Fibrosis Patients
- Geospatial Analysis of Food Deserts and Their Impact on Health Outcomes in Children with Cystic Fibrosis
- Vitamin D Status and Risk of Cystic Fibrosis-Related Diabetes: A Retrospective Single Center Cohort Study
- Handgrip Strength: Associations with Clinical Variables, Body Composition, and Bone Mineral Density in Adults with Cystic Fibrosis
- Genetic Evaluation of Pancreatitis
- Genome evolution drives transcriptomic and phenotypic adaptation in Pseudomonas aeruginosa during 20 years of infection
- Predictors of mortality in children with cystic fibrosis in India
- Lung Transplant READY Pilot Study
- A restructuring of microbiome niche space is associated with Elexacaftor-Tezacaftor-Ivacaftor therapy in the cystic fibrosis lung
- Differentiation of Cystic Fibrosis-Related Pathogens by Volatile Organic Compound Analysis with Secondary Electrospray Ionization Mass Spectrometry
- Consideration of height-based tobramycin dosing regimens for the treatment of adult cystic fibrosis pulmonary exacerbations
- Optimising the care and quality of life of people with cystic fibrosis: the influence of cystic fibrosis transmembrane conductance regulator modulators
- Discovery of inhibition of Burkholderia cenocepacia, Pseudomonas aeruginosa and Stenotrophomonas maltophilia by the Brown Rot Basidiomycete Fungus, Postia placenta
- Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio)
- A Clinician’s guide to vitamin D supplementation for patients with cystic fibrosis
- The adverse association between stimulant use for attention deficit hyperactivity disorder (ADHD) and semen parameters
- Physical Activity and Sedentary Behavior in Adults With Cystic Fibrosis: Association With Aerobic Capacity, Lung Function, Sleep, Well-Being, and Quality of Life
- Parent-Collected Oropharyngeal Swabs in Cystic Fibrosis
- Outcomes of Cystic Fibrosis Screening-Positive Infants With Inconclusive Diagnosis at School Age
- Transitioning to telehealth during the coronavirus disease 2019 pandemic: Perspectives from partners of women with cystic fibrosis and healthcare providers
- Effect of Salbutamol on Lung Ventilation in Children with Cystic Fibrosis: Comprehensive Assessment Using Spirometry, Multiple-Breath Washout, and Functional Lung Magnetic Resonance Imaging
- Intravenous or oral antibiotic treatment in adults and children with cystic fibrosis and Pseudomonas aeruginosa infection: the TORPEDO-CF RCT
- Hereditary Pancreatitis Related to SPINK-1 Mutation. Is There an Increased Risk of Developing Pancreatic Cancer?
- Organic acids and their salts potentiate the activity of selected antibiotics against Pseudomonas aeruginosa biofilms grown in a synthetic cystic fibrosis sputum medium
- Body composition in individuals with cystic fibrosis
- Fertility preservation in women with cystic fibrosis pre-lung transplantation: A mixed methods study
- Endoscopic Sinus Surgery for Cystic Fibrosis: Variables Influencing Sinonasal and Pulmonary Outcomes
- Facilitators and barriers to empowerment in children and young people with cystic fibrosis: a meta-synthesis of the qualitative literature
- Urinary sodium/creatinine ratio is a predictor for fractional sodium excretion and related to age in patients with cystic fibrosis
- Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis
- Approach and anesthetic management for kidney transplantation in a patient with bilateral lung transplantation: case report
- Macrophages from gut-corrected CF mice express human CFTR and lack a pro-inflammatory phenotype
- Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip: Cystic fibrosis airway chip
- Measurements of spontaneous CFTR-mediated ion transport without acute channel activation in airway epithelial cultures after modulator exposure
- Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis
- Surgical treatment of non-cystic fibrosis bronchiectasis in Central Europe
- Right hepatectomy with a cholangiojejunostomy and hepaticojejunostomy for unilobar Caroli’s syndrome
- Small-molecule drugs for cystic fibrosis: Where are we now?
- A Phase 2a randomized, single-center, double-blind, placebo-controlled study to evaluate the safety and preliminary efficacy of oral iOWH032 against cholera diarrhea in a controlled human infection model
- Aspergillus-related lung disease in people with cystic fibrosis: can imaging help us to diagnose disease?
- Abdominal Symptoms Assessed with the CFAbd-Score are Associated with Intestinal Inflammation in Patients with Cystic Fibrosis
- An empirical comparison of segmented and stochastic linear mixed effects models to estimate rapid disease progression in longitudinal biomarker studies
- Erratum to: Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance
- Membrane Transporters of the Major Facilitator Superfamily Are Essential for Long-Term Maintenance of Phenotypic Tolerance to Multiple Antibiotics in E. coli
- Management of Cystic Fibrosis during COVID-19: Patient Reported Outcomes based remote follow-up among CF patients in Denmark – A feasibility study
- Clinical Effectiveness of Elexacaftor/Tezacftor/Ivacaftor in People with Cystic Fibrosis
- Translation, cross-cultural adaptation and psychometric evaluation of the Brazilian version of the Cystic Fibrosis Knowledge Scale (CFKS)
- CyFi-MAP: an interactive pathway-based resource for cystic fibrosis
- Complete CFTR gene sequencing in 5,058 individuals with cystic fibrosis informs variant-specific treatment
- Nontypeable Haemophilus influenzae infection impedes Pseudomonas aeruginosa colonization and persistence in mouse respiratory tract
- Conjugation of Silver Nanoparticles with De Novo Engineered Cationic Antimicrobial Peptides: An Exploratory Proposal
- Unique Features of Mycobacterium abscessus Biofilms Formed in Synthetic Cystic Fibrosis Medium
- Circulating Fibrocyte Level in Children with Cystic Fibrosis
- Quality of home spirometry performance amongst adults with cystic fibrosis
- Emerging non-pulmonary complications for adults with cystic fibrosis
- Genetic Variation in ABCC4 and CFTR and Acute Pancreatitis during Treatment of Pediatric Acute Lymphoblastic Leukemia
- Lung Clearance Index in Children with Cystic Fibrosis during Pulmonary Exacerbation
- Comprehensive Analysis of Combinatorial Pharmacological Treatments to Correct Nonsense Mutations in the CFTR Gene
- Multi-Omics Study of Keystone Species in a Cystic Fibrosis Microbiome
- Human Recombinant DNase I (Pulmozyme® ) Inhibits Lung Metastases in Murine Metastatic B16 Melanoma Model That Correlates with Restoration of the DNase Activity and the Decrease SINE/LINE and c-Myc Fragments in Blood Cell-Free DNA
- Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge
- Cystic Fibrosis Transmembrane Conductance Regulator Folding Mutations Reveal Differences in Corrector Efficacy Linked to Increases in Immature Cystic Fibrosis Transmembrane Conductance Regulator Expression
- Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China
- Telemedicine in cystic fibrosis
- Final results of the southwest German pilot study on cystic fibrosis newborn screening – Evaluation of an IRT/PAP protocol with IRT-dependent safety net: Results of the Southwest German CFNBS pilot study
- SPLUNC1 comes of age? Predicting acute exacerbations in cystic fibrosis
- Epigenetic reprogramming of airway macrophages promotes polarization and inflammation in muco-obstructive lung disease
- The challenges of managing thoracic pain in cystic fibrosis (CF)
- Expanding clinical phage microbiology: simulating phage inhalation for respiratory tract infections
- Predicting mortality in non-cystic fibrosis bronchiectasis patients using distance-saturation product
- Correlating genotype with phenotype using CFTR-mediated whole-cell Cl- currents in human nasal epithelial cells
- Unexpected associations between respiratory viruses and bacteria with Pulmonary Function Testing in children suffering from Cystic Fibrosis (MUCOVIB study)
- When CFSPID becomes CF
- CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the beta-adrenergic sweat rate assay
- No drug-drug interaction between tezacaftor-ivacaftor and clofazimine: A case report
- Methodological tools to study species of the genus Burkholderia
- CFTR mutations causing congenital unilateral absence of the vas deferens (CUAVD) and congenital absence of the uterus (CAU) in a consanguineous family
- The effectiveness of exercise interventions to increase physical activity in Cystic Fibrosis: A systematic review
- Using negative control outcomes and difference-in-differences to estimate treatment effects in an entirely treated cohort: the effect of ivacaftor in cystic fibrosis
- Exploring Associations Between Self-Compassion, Self-Criticism, Mental Health, and Quality of Life in Adults with Cystic Fibrosis: Informing Future Interventions
- An Overview of Noninvasive Ventilation in Cystic Fibrosis
- Spotlighting “Neutrophil Elastase Triggers the Release of Macrophage Extracellular Traps”: A New Catch in CF?
- The determinants of survival among adults with cystic fibrosis-a cohort study
- Spontaneous and iatrogenic hypoglycemia in cystic fibrosis
- Altered Pseudomonas Strategies to Inhibit Surface Aspergillus Colonies
- spa Typing and Molecular Characterization of Antimicrobial Resistance in Staphylococcus aureus Strains from Patients with Cystic Fibrosis
- Mental status changes during elexacaftor/tezacaftor / ivacaftor therapy
- Fenretinide inhibits vitamin A formation from β-carotene and regulates carotenoid levels in mice
- The Epidemiology and Pathogenesis and Treatment of Pseudomonas aeruginosa Infections: An Update
- Lung ultrasound for the diagnosis of cystic fibrosis pulmonary exacerbation
- A Study Evaluating the Safety of Elexacaftor/Tezacaftor/Ivacaftor in Participants With Cystic Fibrosis (CF)
- The Immunomodulatory Effects of Macrolide Antibiotics in Respiratory Disease
- Frequency of allele variations in the CFTR gene in a Mexican population
- Treating nontuberculous mycobacteria in children with cystic fibrosis: a multicentre retrospective study
- Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis
- Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis
- Overweight and Cystic Fibrosis: An Unexpected Challenge
- Therapeutic education and cystic fibrosis
- Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter Randomized Controlled Trial (ACTIVATE-CF)
- CFTR modulators increase risk of acute pancreatitis in pancreatic insufficient patients with cystic fibrosis
- Contrast Enhanced Magnetic Resonance Imaging Does Not Detect a Progression in Lung Morphological Score in Preschool Children with Cystic Fibrosis
- Reply to: Contrast Enhanced Magnetic Resonance Imaging Does Not Detect a Progression in Lung Morphological Score in Preschool Children with Cystic Fibrosis
- Obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment: a systematic review and meta-analysis of prevalence
- Validation of a Spanish version of the Leicester Cough Questionnaire in cystic fibrosis
- Toxic Epidermal Necrolysis Induced by Cystic Fibrosis Transmembrane Conductance Regulator modulators
- Review of current and future therapeutics in ABPA
- Decreased CFTR/PPARγ and increased transglutaminase 2 in nasal polyps
- Arm muscle area for the longitudinal assessment of nutritional status in paediatric patients with cystic fibrosis – A single centre experience
- Parent-Collected Oropharyngeal Swabs in Cystic Fibrosis
- Visualization of Ectopic Serine Protease Activity by Förster Resonance Energy Transfer-Based Reporters
- Renal transplantation in a patient with cystic fibrosis: a changing disease landscape
- Current evidence on the effect of highly effective CFTR modulation on interleukin-8 in cystic fibrosis
- MICROBIOLOGICAL FEATURES OF STAPHYLOCOCCUS AUREUS ISOLATED FROM RESPIRATORY TRACT OF CHILDREN WITH CYSTIC FIBROSIS
- Metabolomic profiling of Burkholderia cenocepacia in synthetic cystic fibrosis sputum medium reveals nutrient environment-specific production of virulence factors
- Need of gastrointestinal surgery in cystic fibrosis? Do not forget the chest!
- Apixaban Causing Hepatic Cystic Bleeding: A Rare but a Life-Threatening Complication
- Biofilm Formation in Methicillin-Resistant Staphylococcus aureus Isolated in Cystic Fibrosis Patients Is Strain-Dependent and Differentially Influenced by Antibiotics
- Mapping Pulmonary and Systemic Inflammation in Preschool Aged Children With Cystic Fibrosis
- COVID-19: Pathophysiology and Implications for Cystic Fibrosis, Diabetes and Cystic Fibrosis-Related Diabetes
- Phase II Study of a Nebulised Nitric Oxide Generating Solution in Patients With Cystic Fibrosis
- Analysis of multiple gene co-expression networks to discover interactions favoring CFTR biogenesis and ΔF508-CFTR rescue
- Gene therapy for cystic fibrosis: new tools for precision medicine
- Rugose small colony variant and its hyper-biofilm in Pseudomonas aeruginosa: Adaption, evolution, and biotechnological potential
- Pretransplant Quality of Life and Post-Transplant Survival in Adolescents with Cystic Fibrosis
- Age-Related Progression of Microvascular Dysfunction in Cystic Fibrosis: New Detection Ways and Clinical Outcomes
- Pharmacological chaperones improve intra-domain stability and inter-domain assembly via distinct binding sites to rescue misfolded CFTR
- Pseudomonas aeruginosa: an antibiotic resilient pathogen with environmental origin
- Non-nuberculous mycobacteria infection treated with intermittently inhaled high-dose nitric oxide
- Validation of Therapeutic Efficacy Targeting the Splicing Variants in Cystic Fibrosis and CFTR Pathologies
- A Developmental Role of the Cystic Fibrosis Transmembrane Conductance Regulator in Cystic Fibrosis Lung Disease Pathogenesis
- One more piece in the pancreatic chloride puzzle
- Olfaction before and after initiation of elexacaftor-tezacaftor-ivacaftor in a cystic fibrosis cohort
- Testing the effects of combining azithromycin with inhaled tobramycin for P. aeruginosa in cystic fibrosis: a randomised, controlled clinical trial
- Respiratory syncytial virus persistent infection causes acquired CFTR dysfunction in human bronchial epithelial cells
- Looking Toward the Future: Approaching Care of the Aging CF Patient
- Ivacaftor restores delayed mucociliary transport caused by Pseudomonas aeruginosa-induced acquired cystic fibrosis transmembrane conductance regulator dysfunction in rabbit nasal epithelia
- A Study of Inhaled KB407 for the Treatment of Cystic Fibrosis
- 50 Years Ago in TheJournalofPediatrics: Carbenicillin and Cystic Fibrosis
- Impact of CFTR large deletions and insertions on the clinical and laboratory severity of cystic fibrosis: a serial case report
- Achieving respiratory excellence in pre-school children with cystic fibrosis
- Association of bacterial community types, functional microbial processes and lung disease in cystic fibrosis airways
- Bacterial Subversion of Autophagy in Cystic Fibrosis
- Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease with the Phe508del/minimal function genotype
- Genetic mutation analysis of 22 patients with congenital absence of vas deferens: A single-center study†
- Effects of Tham Nasal Alkalinization on Airway Microbial Communities: A Pilot Study in Non-CF and CF Adults
- Clinical characteristics and outcomes of non-cystic fibrosis patients with Burkholderia cepacia complex bacteremia at a medical center in Taiwan
- Understanding the Pancreatic Islet Microenvironment in Cystic Fibrosis and the Extrinsic Pathways Leading to Cystic Fibrosis Related Diabetes
- A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients
- Cystic fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies
- A model for building a national, patient-driven database to track contraceptive use in women with rare diseases
- NBD2 Is Required for the Rescue of Mutant F508del CFTR by a Thiazole-Based Molecule: A Class II Corrector for the Multi-Drug Therapy of Cystic Fibrosis
- Dysfunction in the Cystic Fibrosis Transmembrane Regulator in Chronic Obstructive Pulmonary Disease as a Potential Target for Personalised Medicine
- Pseudomonas aeruginosa mexR and mexEF Antibiotic Efflux Pump Variants Exhibit Increased Virulence
- Effect of N-Acetylcysteine in Combination with Antibiotics on the Biofilms of Three Cystic Fibrosis Pathogens of Emerging Importance
- Consistent Assignment of Risk and Benign Allele at rs2303153 in the CF Modifier Gene SCNN1B in Three Independent F508del-CFTR Homozygous Patient Populations
- Forced Oscillation Technique for Monitoring the Respiratory Status of Children with Cystic Fibrosis: A Systematic Review
- Effect of Low Amperage Electric Current on Staphylococcus Aureus-Strategy for Combating Bacterial Biofilms Formation on Dental Implants in Cystic Fibrosis Patients, In Vitro Study
- Therapeutic Approaches for Patients with Cystic Fibrosis Not Eligible for Current CFTR Modulators
- Adaptation and genomic erosion in fragmented Pseudomonas aeruginosa populations in the sinuses of people with cystic fibrosis
- Sleep in children and young adults with cystic fibrosis
- Adherence to CF treatment can be improved with the right approach!
- Clinical characteristics of people with cystic fibrosis and frequent fungal infection
- Comparison of inhaled mannitol/dornase alfa combination and daily dornase alfa alone in children with cystic fibrosis
- Antimicrobial effects of Melaleuca alternifolia (tea tree) essential oil against biofilm-forming multi-drug resistant cystic fibrosis-associated Pseudomonas aeruginosa as a single agent and in combination with commonly nebulised antibiotics
- A Study to Assess the Safety, Tolerability, and Pharmacokinetics of Brensocatib Tablets in Adults With Cystic Fibrosis
- Increasing life expectancy in cystic fibrosis: Advances and challenges
- Airway clearance and exercise for people with cystic fibrosis: balancing longevity with life
- Population Genomics and Inference of Mycobacterium avium Complex Clusters in Cystic Fibrosis Care Centers, United States
- Alkyl-Quinolones derivatives as potential biomarkers for Pseudomonas aeruginosa infection chronicity in Cystic Fibrosis
- Bone involvement in young adults with cystic fibrosis – a Portuguese cohort
- Escherichia coli cryptic prophages sense nutrients to influence persister cell resuscitation
- Isotope dilution LC-MS/MS quantification of the cystic fibrosis transmembrane conductance regulator (CFTR) modulators ivacaftor, lumacaftor, tezacaftor, elexacaftor, and their major metabolites in human serum
- Separating the contributions of SLC26A9 and CFTR to anion secretion in primary human bronchial epithelia (HBE)
- Functional Restoration of CFTR Nonsense Mutations in Intestinal Organoids
- Genome features and antibiotic resistance of Pseudomonas aeruginosa strains isolated in patients with cystic fibrosis in the Russian Federation
- The prevalence of CFTR mutations in patients with chronic rhinosinusitis: A systematic review and meta-analysis
- Cystic Fibrosis Increases Long-Term Revision Rates of Endoscopic Sinus Surgery in Patients With Comorbid Chronic Rhinosinusitis
- Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis
- Myriad Faces of Active Tuberculosis: Intrapulmonary Bronchial Artery Pseudoaneurysm
- Evaluation of different phenotypic methods to detect methicillin resistance in Staphylococcus aureus isolates recovered from cystic fibrosis patients
- VX-809 mitigates disease in a mouse model of autosomal dominant polycystic kidney disease bearing the R3277C human mutation
- Zebrafish Embryo Infection Model to Investigate Pseudomonas aeruginosa Interaction With Innate Immunity and Validate New Therapeutics
- Following the Trace of HVS II Mitochondrial Region Within the Nine Iranian Ethnic Groups Based on Genetic Population Analysis
- Loss of ciliated cells and altered airway epithelial integrity in cystic fibrosis
- Gastrointestinal manifestations of cystic fibrosis in children
- Corrigendum to: “Effect of Sitagliptin on Islet Function in Pancreatic Insufficient Cystic Fibrosis With Abnormal Glucose Tolerance”
- The Role of Nurses in the Management of Respiratory Disorders in Children
- The tyrosine-kinase inhibitor Nintedanib ameliorates autosomal-dominant polycystic kidney disease
- Prevalence, variability, and predictors of sinus surgery in pediatric patients with cystic fibrosis
- Impact of the SARS-CoV-2 pandemic on cystic fibrosis centres and care: survey results from US centres
- Assessment of the microbial load of airway clearance devices used by a cohort of children with cystic fibrosis
- Health Disparity in CF: Perspectives from a Lived Experience
- Bacteria in cystic fibrosis: United they stand – Recent advances in microbiology research through the eyes of ENS students
- The molecular evolution of function in the CFTR chloride channel
- The horizontal transfer of Pseudomonas aeruginosa PA14 ICE PAPI-1 is controlled by a transcriptional triad between TprA, NdpA2 and MvaT
- A small molecule CFTR potentiator restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR
- A Phase 3 Study of VX-121 Combination Therapy in Participants With Cystic Fibrosis (CF) Who Are Homozygous for F508del, Heterozygous for F508del and a Gating (F/G) or Residual Function (F/RF) Mutation, or Have At Least 1 Other Triple Combination Responsive (TCR) CFTR Mutation and No F508del Mutation
- Parasite Survival and Disease Persistence in Cystic Fibrosis, Schistosomiasis and Pathogenic Bacterial Diseases: A Role for Universal Stress Proteins?
- SARS-CoV-2 driving rapid change in adult cystic fibrosis services: the role of the clinical nurse specialist
- Immunometabolic factors in adolescent chronic disease are associated with Th1 skewing of invariant Natural Killer T cells
- Renal plasticity revealed through reversal of polycystic kidney disease in mice
- Prevalence and factors associated with urinary incontinence in females with cystic fibrosis: an Italian single-centre cross-sectional analysis
- Increased Plasma YKL-40 Level and Chitotriosidase Activity in Cystic Fibrosis Patients
- P. aeruginosa Induced Lipid Peroxidation Causes Ferroptotic Cell Death in Airways
- Efficacy of epetraborole against Mycobacterium abscessus is increased with norvaline
- Peripherally Inserted Central Catheters: 100% success providing 13 day antibiotic therapy in cystic fibrosis patients aged 2 to 18 years
- A systematic Cochrane Review of the timing of pancreatic enzyme replacement therapy (PERT) in cystic fibrosis
- The feasibility of home monitoring of young people with cystic fibrosis: Results from CLIMB-CF
- Isolation of Total RNA from Pseudomonas aeruginosa within Biofilms for Measuring Gene Expression
- Preconception risk assessment for thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease
- Sheep models of F508del and G542X cystic fibrosis mutations show cellular responses to human therapeutics
- Epidemiology of Mycobacterium abscessus in England: an observational study
- Risk factors for Pseudomonas aeruginosa airway infection and lung function decline in children with cystic fibrosis
- Outcomes of inpatient cholecystectomy among adults with cystic fibrosis in the United States
- Cysteamine Inhibits Glycine Utilisation and Disrupts Virulence in Pseudomonas aeruginosa
- Regional brain tissue changes in patients with cystic fibrosis
- Growth medium and nitric oxide alter Mycobacterium abscessus morphotype and virulence
- Impact of timing of PERT on gastrointestinal symptoms in Danish children and adolescents with CF
- Spray-freeze-dried inhalable composite microparticles containing nanoparticles of combinational drugs for potential treatment of lung infections caused by Pseudomonas aeruginosa
- Combined use of rheology and portable low-field NMR in cystic fibrosis patients
- COVID-19 outcomes in people with cystic fibrosis
- Granulocytic Myeloid-Derived Suppressor Cells in Cystic Fibrosis
- Prospectively predicting Pseudomonas aeruginosa infection/s using routine data from the UK cystic fibrosis register
- Evaluation of anthropometry as an alternative to DXA as predictor of low bone mineral density in children and adolescents with cystic fibrosis
- Sweat testing in the modern era: A national survey of sweat testing practice in the Republic of Ireland
- The Role of Telehealth Services in Children with Cystic Fibrosis During Coronavirus Disease 2019 Outbreak
- The favorable prognosis of cystic biliary atresia may be related to early surgery and mild liver pathological changes
- Screening strategy for gastrointestinal and hepatopancreatobiliary cancers in cystic fibrosis
- Severe Spontaneous Pneumomediastinum in a Girl with Cystic Fibrosis
- Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment
- Culture with apically applied healthy or disease sputum alters the airway surface liquid proteome and ion transport across human bronchial epithelial cells
- Divergence of bacterial communities in the lower airways of CF patients in early childhood
- Study to Evaluate Symptoms of Exocrine Pancreatic Insufficiency in Adult Participants With Cystic Fibrosis or Chronic Pancreatitis Treated With Creon
- Characteristics of electrolyte imbalance and pseudo-bartter syndrome in hospitalized cystic fibrosis children and adolescents
- Cystic Fibrosis Foundation Nebulizer and Compressor Accessibility Survey
- cAMP triggers Na(+) absorption by distal airway surface epithelium in cystic fibrosis swine
- beta-Catenin-NFkB-CFTR interactions in cholangiocytes regulate inflammation and fibrosis during ductular reaction
- CFTR Rescue in Intestinal Organoids with GLPG/ABBV-2737, ABBV/GLPG-2222 and ABBV/GLPG-2451 Triple Therapy
- A natural language processing pipeline to synthesize patient-generated notes toward improving remote care and chronic disease management: a cystic fibrosis case study
- Improvement of anti-biofilm activities via co-delivery of curcumin and gentamicin in lipid-polymer hybrid nanoparticle
- Tigecycline-associated acute pancreatitis in a child with pulmonary cystic fibrosis: A case report and literature review
- BACH1, the master regulator of oxidative stress, has a dual effect on CFTR expression
- Retracing changes in cystic fibrosis understanding and management over the past twenty years
- Demographic characteristics, clinical and laboratory features, and the distribution of pathogenic variants in the CFTR gene in the Cypriot cystic fibrosis (CF) population demonstrate the utility of a national CF patient registry
- Comparison of the readability of lay summaries and scientific abstracts published in CF Research News and the Journal of Cystic Fibrosis: Recommendations for writing lay summaries
- Effects of environmental air pollutants on CFTR expression and function in human airway epithelial cells
- Olfactory dysfunction in cystic fibrosis: Impact of CFTR modulator therapy
- Improved survival albeit with persistent disparities in prognosis for people with cystic fibrosis in European countries
- Neutrophil Elastase Triggers the Release of Macrophage Extracellular Traps: Relevance to CF
- The Effect of COVID-19 on Anxiety Levels of Children with CF and Healthy Peers
- Pregnancy rates and outcomes amongst women with cystic fibrosis in the UK: comparisons with the general population before and after the introduction of disease modifying treatment, 2003-17
- A Rapid and Sensitive Detection Method for Pseudomonas aeruginosa Using Visualized Recombinase Polymerase Amplification and Lateral Flow Strip Technology
- 2-Alkyl-4-quinolone quorum sensing molecules are biomarkers for culture-independent Pseudomonas aeruginosa burden in adults with cystic fibrosis
- A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis
- Ultrasound findings of pubertal development in girls with cystic fibrosis and their association with clinical outcomes and Tanner staging
- Cystic Fibrosis Transmembrane Conductance Regulator Modulators During Pregnancy: A Case Series
- Administration of pilocarpine by microneedle patch as a novel method for cystic fibrosis sweat testing
- Duration of intravenous antibiotic treatment for acute exacerbations of cystic fibrosis: A systematic review: Duration of treatment for acute exacerbations of cystic fibrosis
- Rothia mucilaginosa is an anti-inflammatory bacterium in the respiratory tract of patients with chronic lung disease
- Mislocalization of CFTR expression in acute pancreatitis and the beneficial effect of VX-661 + VX-770 treatment on disease severity
- Interplay of OpdP Porin and Chromosomal Carbapenemases in the Determination of Carbapenem Resistance/Susceptibility in Pseudomonas aeruginosa
- Assessing Psychosocial Distress in Cystic Fibrosis: Validation of the ‘Distress in Cystic Fibrosis Scale’
- Elexacaftor/tezacaftor/ivacaftor for CFTR variants giving rise to diagnostic uncertainty: Personalised medicine or over-medicalisation?
- Clearance of 25-hydroxyvitamin D in Cystic Fibrosis
- A rare case of pancytopenia in a child with cystic fibrosis: Can copper cure it all?
- Risk factors leading to pulmonary exacerbation in patients with cystic fibrosis: A systematic review
- ‘You’re on mute!’ Does paediatric CF home spirometry require physiologist supervision?
- Palliative Care in Pediatric Pulmonology
- Spns2 Transporter Contributes to the Accumulation of S1P in Cystic Fibrosis Human Bronchial Epithelial Cells
- Anti-Inflammatory Effects of RTD-1 in a Murine Model of Chronic Pseudomonas aeruginosa Lung Infection: Inhibition of NF-κB, Inflammasome Gene Expression, and Pro-IL-1β Biosynthesis
- Increased Inflammatory Markers Detected in Nasal Lavage Correlate with Paranasal Sinus Abnormalities at MRI in Adolescent Patients with Cystic Fibrosis
- Increased Prevalence of Celiac Disease in Patients with Cystic Fibrosis: A Systematic Review and Meta-Analysis
- Planktonic and Biofilm-Associated Pseudomonas aeruginosa and Staphylococcus epidermidis Elicit Differential Human Peripheral Blood Cell Responses
- Current and Emerging Therapies to Combat Cystic Fibrosis Lung Infections
- Preclinical Development of a Bacteriophage Cocktail for Treating Multidrug Resistant Pseudomonas aeruginosa Infections
- State of the Art on Approved Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators and Triple-Combination Therapy
- Staphylococcus aureus and Cystic Fibrosis-A Close Relationship. What Can We Learn from Sequencing Studies?
- The Impact of Highly Effective CFTR Modulators on Growth and Nutrition Status
- Being Underweight Increases the Risk of Non-Cystic Fibrosis Bronchiectasis in the Young Population: A Nationwide Population-Based Study
- Breastfeeding in Cystic Fibrosis: A Systematic Review on Prevalence and Potential Benefits
- The Burkholderia cenocepacia Type VI Secretion System Effector TecA Is a Virulence Factor in Mouse Models of Lung Infection
- Model-based iterative reconstruction in CT of paranasal sinuses in cystic fibrosis
- The impact of modulator therapy in cystic fibrosis on patterns of hospitalization related to age. Results from an analysis of US inpatient episodes 2006-2016
- Criteria and definitions for the radiological and clinical diagnosis of bronchiectasis in adults for use in clinical trials: international consensus recommendations
- Draft Genome Sequence of the Environmental Fungus Scedosporium dehoogii
- Colocolic intussusception mimicking distal intestinal obstruction syndrome in a young adult with cystic fibrosis
- Characterising the allergic profile of children with cystic fibrosis
- Optimizing Sexual and Reproductive Health Across the Lifespan in People with Cystic Fibrosis
- Digital healthcare in cystic fibrosis. Learning from the pandemic to innovate future care
- Using a learning health system to understand the mismatch between medicines supply and actual medicines use among adults with cystic fibrosis
- Type-4 Phosphodiesterase (PDE4) Blockade Reduces NETosis in Cystic Fibrosis
- Mechanisms of Post-Pancreatitis Diabetes Mellitus and Cystic Fibrosis-Related Diabetes: A Review of Preclinical Studies
- Is it Worth Adding Systemic Antibiotics to Inhalational Tobramycin Therapy to Treat Pseudomonas Infections in Cystic Fibrosis?
- Genomic analysis of Burkholderia cenocepacia isolated from a liver abscess in a patient with cystic fibrosis
- An in vitro model for the cultivation of polymicrobial biofilms under continuous-flow conditions
- A review of cystic fibrosis: Basic and clinical aspects
- Description of wylie-stanley agar for the recovery of mycobacterium abscessus
- Self-management intervention to reduce pulmonary exacerbations by supporting treatment adherence in adults with cystic fibrosis: a randomised controlled trial
- Exploring the challenges of accessing medication for patients with cystic fibrosis
- Projecting the impact of triple CFTR modulator therapy on intravenous antibiotic requirements in cystic fibrosis using patient registry data combined with treatment effects from randomised trials
- COVID-19 pandemic restrictions continuously impact on physical activity in adults with cystic fibrosis
- Combined agonists act synergistically to increase mucociliary clearance in a cystic fibrosis airway model
- Anti-IgE therapy for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
- The AWESCORE, a patient-reported outcome measure: development, feasibility, reliability, validity and responsiveness for adults with cystic fibrosis
- Outcomes of early repeat sweat testing in infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome (CRMS)/CF screen-positive, inconclusive diagnosis (CFSPID)
- Clinical Considerations for Routine Auditory and Vestibular Monitoring in Patients With Cystic Fibrosis
- Should the physiotherapy outcomes airway clearance, physical activity and fitness be recorded on the Australian Cystic Fibrosis Data Registry? A consensus approach
- A Case Report of Cystic Fibrosis Plus Tuberous Sclerosis: A Cautionary Tale Regarding Lung Transplantation
- Lung transplantation disparities based on diagnosis for patients bridging to transplant on extracorporeal membrane oxygenation
- Comparison of microbial composition of cough swabs and sputum for pathogen detection in patients with cystic fibrosis
- Inactivation of Nitrite-Dependent Nitric Oxide Biosynthesis Is Responsible for Overlapped Antibiotic Resistance between Naturally and Artificially Evolved Pseudomonas aeruginosa
- Genetic diversification of persistent Mycobacterium abscessus within cystic fibrosis patients
- Dissemination of Mycobacterium abscessus via global transmission networks
- Model Systems to Study the Chronic, Polymicrobial Infections in Cystic Fibrosis: Current Approaches and Exploring Future Directions
- Two Steps Forward: Improving the Management of Cystic Fibrosis Pulmonary Exacerbations
- Chronic Joint Pain in a Young Adult With Cystic Fibrosis
- Magnesium implantation or supplementation ameliorates bone disorder in CFTR-mutant mice through an ATF4-dependent Wnt/β-catenin signaling
- Outcomes of artery embolisation for cystic fibrosis patients with haemoptysis: a 20-year experience at a major Australian tertiary centre
- The cystic fibrosis lung microenvironment alters antibiotic activity: causes and effects
- Colistin neurotoxicity mimicking Guillain-Barre syndrome in a patient with cystic fibrosis: case report and review
- Inflammatory epithelial cytokines after in vitro respiratory syncytial viral infection are associated with reduced lung function
- Elevated Alkaline Phosphatase in a 10-Year-Old With Cystic Fibrosis
- Role of inflammation and oxidative stress in tissue damage associated with cystic fibrosis: CAPE as a future therapeutic strategy
- Mycobacteriophage-antibiotic therapy promotes enhanced clearance of drug-resistant Mycobacterium abscessus
- The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis
- Indoor air pollution exposure is associated with greater morbidity in cystic fibrosis
- Filamentous fungi in the airway of patients with cystic fibrosis: Just spectators?
- Cancer in Cystic Fibrosis: A Narrative Review of Prevalence, Risk Factors, Screening and Treatment Challenges
- Bronchoscopic airway clearance therapy for acute exacerbations of bronchiectasis
- Full The Relationship between Continuous Glucose Monitoring and OGTT in Youth and Young Adults with Cystic Fibrosis
- CFTR modulator use in post lung transplant recipients
- Empire-CF study: A phase 2 clinical trial of leukotriene A4 hydrolase inhibitor acebilustat in adult subjects with cystic fibrosis
- Development of Tinnitus and Hyperacusis in a Mouse Model of Tobramycin Cochleotoxicity
- Cystic Fibrosis – Insulin Deficiency, Early Action
- Infants with cystic fibrosis have altered fecal functional capacities with potential clinical and metabolic consequences
- Evaluation of antimicrobial susceptibility testing methods for Burkholderia cenocepacia and Burkholderia multivorans isolates from cystic fibrosis patients
- CFTR gene mutation spectrum among 735 Iranian patients with cystic fibrosis: A comprehensive systematic review
- Coughing and diaphragmatic breathing exercise: What is the repercussion on respiratory mechanics of children and adolescents with cystic fibrosis?
- Ascending Cholangitis Caused by Methicillin-Resistant Staphylococcus aureus Species in a Patient With Cystic Fibrosis
- Barriers implementing infection prevention and control experienced by healthcare workers, people with CF and parents
- Telehealth after the pandemic: Will the inverse care law apply?
- Outcomes of SARS-CoV-2 infection in patients with cystic fibrosis: A multicenter retrospective research network study
- Social support and social isolation in adults with cystic fibrosis: An integrative review
- Correction to “Discovery of GLPG2451, a Novel Once Daily Potentiator for the Treatment of Cystic Fibrosis”
- Gender differences in non-cystic fibrosis bronchiectasis severity and bacterial load: the potential role of hormones
- Functional correction of CFTR mutations in human airway epithelial cells using adenine base editors
- Assessment of hepatic involvement by two-dimensional shear wave elastography in paediatric patients with cystic fibrosis
- Novel detection of specific bacterial quorum sensing molecules in saliva: Potential non-invasive biomarkers for pulmonary Pseudomonas aeruginosa in cystic fibrosis
- Traditional Chinese Medicine is an Alternative Therapeutic Option for Treatment of Pseudomonas aeruginosa Infections
- ABCC7/CFTR Expression Is Associated with the Clinical Course of Ulcerative Colitis Patients
- Cardiovascular System Involvement in Cystic Fibrosis
- An Innovative Protocol for Metaproteomic Analyses of Microbial Pathogens in Cystic Fibrosis Sputum
- Antimicrobial activity of ceftazidime/avibactam, ceftolozane/tazobactam and comparator agents against Pseudomonas aeruginosa from cystic fibrosis patients
- Mechanistic analysis and significance of sphingomyelinase-mediated decreases in transepithelial CFTR currents in nHBEs
- Association of Quality of Life Measures and Otolaryngologic Care in Cystic Fibrosis Patients
- Diagnosing constipation in patients with cystic fibrosis applying ESPGHAN criteria
- Restoration of exocrine pancreatic function in child with lumacaftor/ivacaftor therapy in cystic fibrosis
- Serological biomarkers for the diagnosis of Mycobacterium abscessus infections in cystic fibrosis patients
- Hypoplasia of Extrahepatic Biliary Tree and Intrahepatic Cholangiolopathy in Cystic Fibrosis Imperfectly Mimic Biliary Atresia in 4 Infants With Cystic Fibrosis and Kasai Portoenterostomy
- Ethical dilemma: ELX/TEZ/IVA or Lung Transplantation in Cystic Fibrosis and End Stage Lung Disease?
- Quality of life and exercise tolerance tools in children/adolescents with cystic fibrosis: Systematic review
- TeleHealth or TeleWealth? Equity challenges for the future of cystic fibrosis care – commentary
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- Efficacy and safety of inhaled ENaC inhibitor BI 1265162 in patients with cystic fibrosis: BALANCE-CF 1 – a randomised, Phase II study
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- Multidimensional Clinical Surveillance of Pseudomonas aeruginosa Reveals Complex Relationships between Isolate Source, Morphology, and Antimicrobial Resistance
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- Satisfaction and effectiveness of opioid pain management among adults with cystic fibrosis: A mixed methods study
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- NUTRITIONAL STATUS BETWEEN 5 – 10 YEARS IS ASSOCIATED WITH CYSTIC FIBROSIS-RELATED DIABETES IN ADOLESCENCE
- Sensitive markers to detect progression of lung disease in children with cystic fibrosis
- Comparison of the predictive value of area under the curve versus maximum serum concentration of intravenous tobramycin in cystic fibrosis patients treated for an acute pulmonary exacerbation
- Peptide inhibition of neutrophil-mediated injury after in vivo challenge with supernatant of Pseudomonas aeruginosa and immune-complexes
- Aerobic fitness is associated with extracellular DNA levels in the sputum of patients with cystic fibrosis
- Mild Cystic Fibrosis Lung Disease Is Associated with Bacterial Community Stability
- Achromobacter spp. genetic adaptation in cystic fibrosis
- Perceptions of Social Media Use to Augment Health Care: A Survey of Adolescents and Young Adults with Cystic Fibrosis
- A Clinician Survey of Use Assessment, Documentation, and Education about Cannabis in Persons with Cystic Fibrosis
- Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation
- New Perspectives in Underlying Molecular Defects Based Cystic Fibrosis Therapeutics
- The Bactericidal Tandem Drug, AB569: How to Eradicate Antibiotic-Resistant Biofilm Pseudomonas aeruginosa in Multiple Disease Settings Including Cystic Fibrosis, Burns/Wounds and Urinary Tract Infections
- Ethnic Differences in the Frequency of CFTR Gene Mutations in Populations of the European and North Caucasian Part of the Russian Federation
- Sputum and Plasma Neutrophil Elastase in Stable Adult Patients With Cystic Fibrosis in Relation to Chronic Pseudomonas Aeruginosa Colonization
- A retrospective ‘real-world’ cohort study of azole therapeutic drug monitoring and evolution of antifungal resistance in cystic fibrosis
- Antibiotic Susceptibility and Biofilm Formation of Bacterial Isolates Derived from Pediatric Patients with Cystic Fibrosis from Tehran, Iran
- MRSA Strains with Distinct Accessory Genes Predominate at Different Ages in Cystic Fibrosis
- Nontuberculous mycobacterial infection and environmental molybdenum in persons with cystic fibrosis: a case-control study in Colorado
- Identification of infiltrating immune cell subsets and heterogeneous macrophages in the lesion microenvironment of hepatic cystic echinococcosis patients with different cyst viability
- Resolution of severe hepatosteatosis in a cystic fibrosis patient with multifactorial choline deficiency: A case report
- Optimization of antibiotics for cystic fibrosis pulmonary exacerbations due to highly resistant nonlactose fermenting Gram negative bacilli: Meropenem-vaborbactam and cefiderocol
- Telemedicine and cystic fibrosis: Do we still need face-to-face clinics?
- A new era for people with cystic fibrosis
- Role of Protein Kinase A-Mediated Phosphorylation in CFTR Channel Activity Regulation
- DETERMINATION OF AMOXICILLIN AND COTRIMOXAZOLE CONCENTRATIONS IN SPUTUM OF PATIENTS WITH CYSTIC FIBROSIS
- Cystic Fibrosis Sputum Impairs the Ability of Neutrophils to Kill Staphylococcus aureus
- Bicarbonate Evokes Reciprocal Changes in Intracellular Cyclic di-GMP and Cyclic AMP Levels in Pseudomonas aeruginosa
- New Therapies to Correct the Cystic Fibrosis Basic Defect
- Cystic Fibrosis Human Organs-on-a-Chip
- Targeted Drug Delivery Technologies Potentiate the Overall Therapeutic Efficacy of an Indole Derivative in a Mouse Cystic Fibrosis Setting
- Systems Biology and Bile Acid Signalling in Microbiome-Host Interactions in the Cystic Fibrosis Lung
- Severe COVID-19 in Hospitalized Carriers of Single CFTR Pathogenic Variants
- The Fitter the Better? Cardiopulmonary Exercise Testing Can Predict Pulmonary Exacerbations in Cystic Fibrosis
- Metaproteomics to Decipher CF Host-Microbiota Interactions: Overview, Challenges and Future Perspectives
- Prevotella melaninogenica, a Sentinel Species of Antibiotic Resistance in Cystic Fibrosis Respiratory Niche?
- The Effects of the COVID-19 Pandemic on Pediatric Cystic Fibrosis Patients
- Serratus Anterior Plane Block as a Primary Anesthetic Technique for Video-Assisted Thoracic Surgery in a Child
- Automatic referrals within a cystic fibrosis multidisciplinary clinic improve patient evaluation and management
- Effect of malnutrition in infants with cystic fibrosis in India: An underestimated danger
- Assessment of a novel genetic counselling intervention to inform assisted reproductive technology treatments and other family-building options in adults with cystic fibrosis
- Elucidating clinical phenotypic variability associated with the polyT tract and TG repeats in CFTR
- Real World Clinical Outcomes With Novel Modulator Therapy Combinations in People With CF (RECOVER)
- Entry of cystic fibrosis transmembrane conductance potentiator ivacaftor into the developing brain and lung
- Isolation of and risk factors for airway infection with Pseudomonas aeruginosa in patients with non-cystic fibrosis bronchiectasis
- Impact of a Pharmacy Technician and Pharmacist on Time to Inhaled Tobramycin Therapy in a Pediatric Cystic Fibrosis Clinic
- Development of an Advance Care Planning Protocol in a Cystic Fibrosis Outpatient Clinic
- A narrative review of in utero gene therapy: advances, challenges, and future considerations
- CFTR-mediated anion secretion in parathyroid hormone-treated Caco-2 cells is associated with PKA and PI3K phosphorylation but not intracellular pH changes or Na+ /K+ -ATPase abundance
- Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment
- The bacterial toxin ExoU requires a host trafficking chaperone for transportation and to induce necrosis
- Measuring Knowledge of Fertility Preservation in Women With Cystic Fibrosis: Instrument Development and Psychometric Analysis
- Real-world adherence among adults with cystic fibrosis is low – a retrospective analysis of the CFHealthHub digital learning health system
- Designing multi-epitope vaccine candidates against functional amyloids in Pseudomonas aeruginosa through immunoinformatic and structural bioinformatics approach
- Perceptions of Diabetes Technology Use in Cystic Fibrosis Related Diabetes Management
- Genomic and Phenotypic Evolution of Achromobacter xylosoxidans during Chronic Airway Infections of Patients with Cystic Fibrosis
- The association between body composition, leptin levels and glucose dysregulation in youth with cystic fibrosis
- Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis
- Cystic fibrosis: From gene discovery to precision medicine
- CFTR and cystic fibrosis, a 50-year long history
- Pycnogenol-Centellicum supplementation improves lung fibrosis and Post-Covid-19 lung healing
- An Organ System-Based Synopsis of Pseudomonas aeruginosa Virulence
- Functional Contribution and Targeted Migration of Group-2 Innate Lymphoid Cells in Inflammatory Lung Diseases: Being at the Right Place at the Right Time
- Decreased Wait Time and Increased Satisfaction With Bedside Pancreatic Enzyme Dosing for the Inpatient Adolescent With Cystic Fibrosis: A Quality Improvement Project Comparing Enzyme Self-Administration to Nurse Administration
- Lingering Identity as Chronically Ill and the Unanticipated Effects of Life-Changing Precision Medicine in Cystic Fibrosis: A Case Report
- Impact of COVID-19 Lockdown on Pulmonary and Nutritional Status in Children and Young Adults With Cystic Fibrosis, in Greece
- Partnership Enhancement Program: Piloting a Communication Training Program for Cystic Fibrosis Care Teams
- Association of vitamin A status with lung function in children and adolescents with cystic fibrosis
- Rewriting CFTR to cure cystic fibrosis
- Breast development in a 7 year old girl with CF treated with ivacaftor: An indication for personalized dosing?
- Association between insurance variability and early lung function in children with cystic fibrosis
- Otitis media prone children with cystic fibrosis: A new normal
- Formal vs. informal transition in adolescents with cystic fibrosis: A retrospective comparison of outcomes
- Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration
- Early Lung Disease Exhibits Bacterial-Dependent and -Independent Abnormalities in Cystic Fibrosis Pigs
- Primary ciliogenesis is a crucial step for multiciliated cell determinism in the respiratory epithelium
- Systemic pseudohypoaldosteronism-1 with episodic dyslipidemia in a Sudanese child
- Inflammatory cytokines TNFalpha and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators
- Notch-Hes1 signaling activation in Caroli disease and polycystic liver disease
- Factors Influencing Lung Function in Patients with Cystic Fibrosis in Western Romania
- Airway Clearance Therapy in Cystic Fibrosis Patients Insights from a Clinician Providing Cystic Fibrosis Care
- Alteration of Membrane Cholesterol Content Plays a Key Role in Regulation of Cystic Fibrosis Transmembrane Conductance Regulator Channel Activity
- Ketone Supplementation in Cystic Fibrosis
- Combined Liver-Lung-Kidney Transplant in a Patient with Cystic Fibrosis
- Short-term effect of elexacaftor-tezacaftor-ivacaftor on lung function and transplant planning in cystic fibrosis patients with advanced lung disease
- Association of Diverse Staphylococcus aureus Populations with Pseudomonas aeruginosa Coinfection and Inflammation in Cystic Fibrosis Airway Infection
- Both Pseudomonas aeruginosa and Candida albicans Accumulate Greater Biomass in Dual-Species Biofilms under Flow
- CFTR Modulators to the Rescue of Individuals with Cystic Fibrosis and Advanced Lung Disease
- Genome-wide association study of signature genetic alterations among pseudomonas aeruginosa cystic fibrosis isolates
- Single versus combination intravenous anti-pseudomonal antibiotic therapy for people with cystic fibrosis
- Lung Inflammation and Simulated Airway Resistance in Infants with Cystic Fibrosis
- Pharmacokinetics and pharmacodynamics of antibiotics in cystic fibrosis: a narrative review
- Untargeted Metabolomics Reveals Species-Specific Metabolite Production and Shared Nutrient Consumption by Pseudomonas aeruginosa and Staphylococcus aureus
- Filamentous Bacteriophages and the Competitive Interaction between Pseudomonas aeruginosa Strains under Antibiotic Treatment: a Modeling Study
- Coping with Cystic Fibrosis: An analysis from the sibling’s point of view
- Paranasal mucoceles in children with cystic fibrosis: Management of a not so rare clinical condition
- Multidisciplinary Management of Non-cystic-fibrosis Bronchiectasis – State of the Art
- TWEAK Signaling Pathway Blockade Slows Cyst Growth and Disease Progression in Autosomal Dominant Polycystic Kidney Disease
- Burkholderia cepacia infection in a non-cystic fibrosis patient: an arcane presentation
- Review of Gastrointestinal Motility in Cystic Fibrosis
- Pulmonary Complications in Cystic Fibrosis: past, present and future
- PepBiotics, novel cathelicidin-inspired antimicrobials to fight pulmonary bacterial infections
- Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection
- Cystic fibrosis transmembrane conductance regulator-related male infertility: Relevance of genetic testing & counselling in Indian population
- Critical roles of SMYD2 lysine methyltransferase in mediating renal fibroblast activation and kidney fibrosis
- Hyperinflation is Associated with Increased Respiratory Rate and is a More Sensitive Measure of Cystic Fibrosis Lung Disease During Infancy Compared to Forced Expiratory Measures
- Pancreas in Hereditary Syndromes: Cross-sectional Imaging Spectrum
- Novel CFTR Modulators for Treating Cystic Fibrosis
- Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator
- Physiotherapy service provision in a specialist adult cystic fibrosis service: A pre-post design study with the inclusion of an allied health assistant
- Cardiovascular complications in cystic fibrosis: A review of the literature
- Non-invasive ventilation is associated with long-term improvements in lung function and gas exchange in cystic fibrosis adults with hypercapnic respiratory failure
- Neonatal cystic fibrosis screening: Analysis and differences in immunoreactive trypsin levels in newborns with a positive screen
- The impact of the high emergency lung transplantation program in cystic fibrosis in France: insight from a comparison with Canada
- Serum hepatocyte growth factor as a predictor of disease severity and future exacerbations in patients with non-cystic fibrosis bronchiectasis
- Potential role for the use of gliptins in cystic fibrosis-related diabetes
- Aggressive vasculitis after lung transplantation for cystic fibrosis
- Healthcare Resource Utilization and Costs Among Children With Cystic Fibrosis in the United States
- COVID-19: Impact, experiences and support needs of children and young adults with Cystic Fibrosis and parents
- System-Level Variation in Multiple Sclerosis Care Outcomes: Initial Findings from the Multiple Sclerosis Continuous Quality Improvement Research Collaborative
- Cystic fibrosis-related diabetes (CFRD) and cognitive function in adults with cystic fibrosis
- Pyoverdine Inhibitors and Gallium Nitrate Synergistically Affect Pseudomonas aeruginosa
- Combined indeterminate and impaired glucose tolerance is a novel group at high risk of Cystic Fibrosis-related Diabetes
- Combined indeterminate and impaired glucose tolerance is a novel group at high risk of Cystic Fibrosis-related Diabetes
- Safety, Tolerability and Efficacy of S-1226 in Cystic Fibrosis and Non CF Bronchiectasis
- USE OF COMPUTED TOMOGRAPHY (CT) TO DETERMINE THE SENSITIVITY OF CLINICAL SIGNS AS A DIAGNOSTIC TOOL FOR RESPIRATORY DISEASE IN BORNEAN ORANGUTANS (PONGO PYGMAEUS )
- Ivacaftor-elexacaftor-tezacaftor and tacrolimus combination in cystic fibrosis
- Clinical findings of patients with cystic fibrosis according to newborn screening results
- Italian Cystic Fibrosis Registry (ICFR). Report 2017-2018
- Colonic Transit Time and Fecal Impaction in Children and Adolescents with Cystic Fibrosis-Associated Constipation
- Primary care providers’ role in newborn screening result notification for cystic fibrosis
- Newborn screening for cystic fibrosis: Role of primary care providers in caring for infants with positive screening results
- Diffuse cystic lung disease in sickle cell anaemia: a series of 22 cases and a case-control study
- A First in Human Study to Evaluate the Safety, Tolerability and Pharmacokinetics of Single and Multiple Ascending Doses of Inhaled ETD001 in Healthy Subjects
- Aquagenic Keratoma: Treatment Update
- A Report of Candida blankii Fungemia and Possible Endocarditis in an Immunocompetent Individual and the Review of Literature
- Predictive factors for premature birth and respiratory exacerbation in pregnancies of women with cystic fibrosis
- Disruption of biofilms and killing of Burkholderia cenocepacia from cystic fibrosis lung using an antioxidant-antibiotic combination therapy
- Microbial interaction: Prevotella spp. reduce P. aeruginosa induced inflammation in cystic fibrosis bronchial epithelial cells
- Pragmatic Implementation Trial of a CF Primary Palliative Care Intervention
- Cystic Fibrosis and Urinary Incontinence
- Bright Light Therapy for Depression Symptoms in Adults With Cystic Fibrosis (CF)
- Long-term bone mineral density changes and fractures in lung transplant recipients with cystic fibrosis
- Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung
- Citizen-science based study of the oral microbiome in Cystic fibrosis and matched controls reveals major differences in diversity and abundance of bacterial and fungal species
- Cystic fibrosis foundation consensus statements for the care of cystic fibrosis lung transplant recipients
- Comparison of Cas9 and Cas12a CRISPR editing methods to correct the W1282X-CFTR mutation
- Tezacaftor-ivacaftor use in routine care of adults with cystic fibrosis: a medicine use evaluation
- Phenotypic characteristics of incident and chronic MRSA isolates in cystic fibrosis
- Airway Administration of Flagellin Regulates the Inflammatory Response to Pseudomonas aeruginosa
- Association Between Number of Intravenous Antipseudomonal Antibiotics and Clinical Outcomes of Pediatric Cystic Fibrosis Pulmonary Exacerbations
- Real-World Long-Term Ivacaftor for Cystic Fibrosis in France: Clinical Effectiveness and Healthcare Resource Utilization
- Effectiveness of enteral nutrition by percutaneous endoscopic gastrostomy in malnourished patients with cystic fibrosis: Does the gender gap play a role?
- Inhaled mannitol (Bronchitol) for cystic fibrosis
- Airway Microbiology in Children with Cystic Fibrosis: A Prospective Cohort Study from Northern India
- Clinical impact of levofloxacin inhalation solution in cystic fibrosis patients in a real-world setting
- Treatment-resistant hypertension in a post-transplant patient with cystic fibrosis: a rare case of phaeochromocytoma
- Biofilm inhibitory effect of alginate lyases on mucoid P. aeruginosa from a cystic fibrosis patient
- An Animated Functional Data Analysis Interface to Cluster Rapid Lung Function Decline and Enhance Center-Level Care in Cystic Fibrosis
- A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?
- Cystic fibrosis
- Sex differences in treatment patterns in cystic fibrosis pulmonary exacerbations
- HOMA indices as screening tests for cystic fibrosis-related diabetes
- Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease
- Survival Benefits Following Liver Transplantation – A Matched-Pair Analysis in Pediatric Patients with Cystic Fibrosis
- Pulmonary Biofilm-Based Chronic Infections and Inhaled Treatment Strategies
- Respiratory symptoms do not reflect functional impairment in early CF lung disease
- Tensin 1 (TNS1) is a modifier gene for low body mass index (BMI) in homozygous [F508del]CFTR patients
- Correction to: Cystic Fibrosis Revisited – A Review Study
- Association of the CFTR gene with asthma and airway mucus hypersecretion
- Single-Dose Lentiviral Mediated Gene Therapy Recovers CFTR Function in Cystic Fibrosis Knockout Rats
- A chromobacter xylosoxidans airway infection is associated with lung disease severity in children with cystic fibrosis
- Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study
- Beyond the Guidelines: Treatment of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis
- Diabetes outbreak during COVID19 lock-down in a prediabetic patient with cystic fibrosis long treated with glargine
- Ivacaftor and Airway Inflammation in Preschool Children with Cystic Fibrosis
- Does Cefiderocol Have a Potential Role in Cystic Fibrosis Pulmonary Exacerbation Management?
- Best Practices: Imaging Strategies for Reduced-Dose Chest CT in the Management of Cystic Fibrosis-Related Lung Disease
- Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era
- Airway Epithelial Nucleotide Release Contributes to Mucociliary Clearance
- Proteases, Mucus, and Mucosal Immunity in Chronic Lung Disease
- Personalized Medicine Based on Nasal Epithelial Cells: Comparative Studies with Rectal Biopsies and Intestinal Organoids
- Potential of Intestinal Current Measurement for Personalized Treatment of Patients with Cystic Fibrosis
- Computed Tomographic Changes in Patients with Cystic Fibrosis Treated by Combination Therapy with Lumacaftor and Ivacaftor
- The Equitable Implementation of Cystic Fibrosis Personalized Medicines in Canada
- Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment
- Increased Incidence and Associated Risk Factors of Aspergillosis in Patients with Bronchiectasis
- Fungal Infection and Inflammation in Cystic Fibrosis
- Plasmonic Sensing Studies of a Gas-Phase Cystic Fibrosis Marker in Moisture Laden Air
- Comparison of Physical Activity and Sedentary Behaviour between Schoolchildren with Cystic Fibrosis and Healthy Controls: A Gender Analysis
- Quantification of Phenotypic Variability of Lung Disease in Children with Cystic Fibrosis
- Health Characteristics of Patients with Cystic Fibrosis whose Genotype Includes a Variant of the Nucleotide Sequence c.3140-16T>A and Functional Analysis of this Variant
- Cystic Fibrosis, New Frontier: Exploring the Functional Connectivity of the Brain Default Mode Network. Comment on Elce et al. Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis. Diagnostics 2020, 10, 489
- Reply to Gambazza et al. Cystic Fibrosis, New Frontier: Exploring the Functional Connectivity of the Brain Default Mode Network. Comment on “Elce et al. Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis. Diagnostics 2020, 10, 489”
- Change in Nutrient and Dietary Intake in European Children with Cystic Fibrosis after a 6-Month Intervention with a Self-Management mHealth Tool
- CFTR Modulators: Does One Dose Fit All?
- Pulmonary Mycobacterium abscessus Infection in an 11-Year-Old Child, Successfully Treated with Inhaled/Parenteral Amikacin: A Case Report and Review of Literature
- Papilledema and hypervitaminosis A after elexacaftor/tezacaftor/ivacaftor for cystic fibrosis
- Enhancing Cystic Fibrosis Immune Regulation
- Enhanced Expression of Human Epididymis Protein 4 (HE4) Reflecting Pro-Inflammatory Status Is Regulated by CFTR in Cystic Fibrosis Bronchial Epithelial Cells
- Rating of Perceived Exertion in Three-Minute Step Test in Children with Cystic Fibrosis
- A rare case of idiopathic intrauterine intestinal volvulus complicated with intestinal perforation: a case report from Syria
- Renal Ciliopathies: Sorting Out Therapeutic Approaches for Nephronophthisis
- Biomarkers in Cystic Fibrosis Lung Disease – A Review
- Overweight and obesity in adults with cystic fibrosis: An Italian multicenter cohort study
- The immune response to airway mycosis
- Automated antimicrobial susceptibility testing of slow-growing Pseudomonas aeruginosa strains in the presence of tetrazolium salt WST-1
- Epigenome editing of the CFTR-locus for treatment of cystic fibrosis
- Enabling Antibiotic Allergy Evaluations and Reintroduction of First-Line Antibiotics for Cystic Fibrosis Patients
- Compensatory evolution of Pseudomonas aeruginosa’s slow growth phenotype suggests mechanisms of adaptation in cystic fibrosis
- Ivacaftor Inhibits Glioblastoma Stem Cell Maintenance and Tumor Progression
- Would it have happened without Christmas? Thoracic emphysema and allergic bronchopulmonary aspergillosis in a juvenile cystic fibrosis patient with the G551D mutation receiving ivacaftor
- Testing of aerosolized ciprofloxacin nanocarriers on cystic fibrosis airway cells infected with P. aeruginosa biofilms
- Allergic Broncho-Pulmonary Aspergillosis
- Erratum: Explaining the Sex Effect on Survival in Cystic Fibrosis: A Joint Modeling Study of UK Registry Data
- Orthopedic Manifestations of Cystic Fibrosis
- Actigraphic and patient and family reported sleep outcomes in children and youth with cystic fibrosis: A systematic review
- Use of the NINJA (Nephrotoxic Injury Negated by Just-in-Time Action) Program to Identify Nephrotoxicity in Pediatric Patients with Cystic Fibrosis
- Association of Cystic Fibrosis Transmembrane Conductance Regulator with Epithelial Sodium Channel Subunits Carrying Liddle’s Syndrome Mutations
- Tackling the Misconception That Cystic Fibrosis Is a “White People’s Disease”
- VIP reduction in the pancreas of F508del homozygous CF mice and early signs of Cystic Fibrosis Related Diabetes (CFRD)
- Risk factors for cystic fibrosis arthropathy: Data from the German cystic fibrosis registry
- The Current Practice of Noninvasive Ventilation in Patients With Cystic Fibrosis
- Correlation of computed tomography findings and lung function in children and adolescents with cystic fibrosis
- DRESS syndrome in a patient with cystic fibrosis: a pediatric case report
- Gain-of-Function Polymorphisms in Human Inflammasomes: Implications for Cystic Fibrosis
- Cystic fibrosis year in review 2020: Section 2 pulmonary disease, infections, and inflammation
- Phosphorus stress induces the synthesis of novel glycolipids in Pseudomonas aeruginosa that confer protection against a last-resort antibiotic
- Monovalent: Divalent Anion Selectivity in the CFTR Channel Pore
- Chronic bacterial pulmonary infections in advanced cystic fibrosis differently affect the level of sputum neutrophil elastase, IL-8 and IL-6
- Pancreas transplantation for Cystic Fibrosis: A Frequently Missed Opportunity
- The long-term safety of chronic azithromycin use in adult patients with cystic fibrosis, evaluating biomarkers for renal function, hepatic function and electrical properties of the heart
- Pseudomonas aeruginosa infection, but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis
- Pharmacokinetics of oral antimycobacterials and dosing guidance for Mycobacterium avium complex treatment in cystic fibrosis
- Cerebral paradoxical embolisation in a patient with cystic fibrosis with patent foramen ovale: a comparative review of literature
- Survival models to support shared decision-making about advance care planning for people with advanced stage cystic fibrosis
- The role of suboptimal concentrations of nebulized tobramycin in driving antimicrobial resistance in Pseudomonas aeruginosa isolates in cystic fibrosis – an in vitro study
- The Impact of Resistant Bacterial Pathogens including Pseudomonas aeruginosa and Burkholderia on Lung Transplant Outcomes
- Ivacaftor Reduces Inflammatory Mediators in Upper Airway Lining Fluid From Cystic Fibrosis Patients With a G551D Mutation: Serial Non-Invasive Home-Based Collection of Upper Airway Lining Fluid
- Novel reaction to new cystic fibrosis medication Trikafta
- Honeycomb-like structures in sarcoidosis pathologically showing granulomas in walls of clustered bronchioles
- Bone Cells Differentiation: How CFTR Mutations May Rule the Game of Stem Cells Commitment?
- The Human Microbiome, an Emerging Key-Player in the Sex Gap in Respiratory Diseases
- Cystic fibrosis in the kidney: new lessons from impaired renal HCO3- excretion
- Perinatal outcomes in women with cystic fibrosis: data from the Italian Cystic Fibrosis Registry
- Discovery of Icenticaftor (QBW251), a Cystic Fibrosis Transmembrane Conductance Regulator Potentiator with Clinical Efficacy in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease
- Two positively charged amino acid side-chains in the inner vestibule of the CFTR channel pore play analogous roles in controlling anion binding and anion conductance
- Laryngeal Injury Due to Amikacin Inhalation for Refractory Mycobacterium avium Complex Infection
- Randomized, Controlled Trial Of Sitagliptin And Islet Function In Cystic Fibrosis With Abnormal Glucose Tolerance
- Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors
- Importance of beta-lactam pharmacokinetics and pharmacodynamics on the recovery of microbial diversity in the airway of persons with cystic fibrosis
- Diabetes and Prediabetes in Children With Cystic Fibrosis: A Systematic Review of the Literature and Recommendations of the Italian Society for Pediatric Endocrinology and Diabetes (ISPED)
- Sweat Testing and Recent Advances
- The Correlation of Health-Related Quality of Life with Cystic Fibrosis Severity Markers in Chest CT Scan and 6-Minute Walk Test: A Cross-Sectional Study
- Systematic review: cystic fibrosis in the SARS-CoV-2/COVID-19 pandemic
- Targeted exhaled breath analysis for detection of Pseudomonas aeruginosa in cystic fibrosis patients
- Lack of CFTR alters the ferret pancreatic ductal epithelial secretome and cellular proteome: Implications for exocrine/endocrine signaling
- Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020
- Aquagenic wrinkling in children under two years of age: Could this be a potential clinical referral tool for cystic fibrosis among non-screened populations?
- Management of an unusual cause of life-threatening haemoptysis: a multidisciplinary approach
- Complete Genome Sequence of Burkholderia gladioli Myophage Mana
- Gastrointestinal Factors Associated With Hospitalization in Infants With Cystic Fibrosis: Results from the BONUS Study
- Topic of the Month: Transient Elastography in Pediatric Liver Disease
- National Trends of Hospitalizations in Cystic Fibrosis Highlight a Need for Pediatric to Adult Transition Clinics
- Cystic fibrosis: A changing landscape
- Cystic fibrosis: A changing landscape
- Evaluating the Impact of Education on Pharmacist Tobramycin Dose Recommendations for Cystic Fibrosis and a Review of Perceptions on Pharmacist-Led Charting
- VEGF receptor 2 (KDR) protects airways from mucus metaplasia through a Sox9-dependent pathway
- CFTR Function and Clinical Response to Modulators Parallel Nasal Epithelial Organoid Swelling
- Value Assessment in Cystic Fibrosis: ICER’s Rejection of the Axioms of Fundamental Measurement
- Aquagenic Wrinkling of the Palm: A Rare Diagnostic Clue of Cystic Fibrosis and the Response to CFTR-Modulating Therapy
- Atypical haemolytic uraemic syndrome in a child with cystic fibrosis
- Pharmacokinetic interactions between ivacaftor and cytochrome P450 3A4 inhibitors in people with cystic fibrosis and healthy controls
- Sleep disturbances in children with cystic fibrosis, primary ciliary dyskinesia and typically developing children during COVID-19 pandemic
- The associations between pediatric weight status and cystic fibrosis-related diabetes status and health-related quality of life among children and young adults with cystic fibrosis: A systematic review
- Cystic fibrosis being a polyendocrine disease (Review)
- Neonatal screening in Europe revisited: An ISNS-perspective on the current state and developments since 2010
- Newborn screening for cystic fibrosis in France
- Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Advanced Cystic Fibrosis Lung Disease
- Safety and Feasibility of High-intensity Interval Training Program in CF Patients
- Aquagenic wrinkling of the palms is a rare dermatological disease associated with cystic fibrosis
- Vibrio parahaemolyticus induces inflammation-associated fluid accumulation via activation of the cystic fibrosis transmembrane conductance regulator
- Defining the role of CFTR channel blocker and ClC-2 activator in DNBS induced gastrointestinal inflammation
- Fungal infection in cystic fibrosis
- Towards harmonization of solutions used for cystic fibrosis diagnosis by nasal potential difference measurements: a formulation approach with CHESS software
- An integrated liquid crystal sensing device assisted by the surfactant-embedded smart hydrogel
- Sleep assessment in cystic fibrosis patients using a minimal-impact biomotion system
- Elevated sweat chloride test: is it always cystic fibrosis?
- On the Corner of Models and Cure: Gene Editing in Cystic Fibrosis
- Nocturnal non invasive ventilation in normocapnic cystic fibrosis patients: a pilot study
- The lived experience of Jordanian parents of children with cystic fibrosis: Qualitative study
- Aminoglycoside dosing and monitoring for Pseudomonas aeruginosa during acute pulmonary exacerbations in cystic fibrosis
- SARS-CoV-2 infection in cystic fibrosis: A multicentre prospective study with a control group, Italy, February-July 2020
- The Evaluation of Determinants and Impacts of Co-Production in Healthcare: A Research Protocol for OPAT in Cystic Fibrosis
- Bactericidal/Permeability-Increasing Protein Preeminently Mediates Clearance of Pseudomonas aeruginosa In Vivo via CD18-Dependent Phagocytosis
- Clinical Pharmacokinetic and Pharmacodynamic Considerations in the Drug Treatment of Non-Tuberculous Mycobacteria in Cystic Fibrosis
- A short-term evaluation of a prototype disposable Oscillating Positive Expiratory Pressure (OPEP) device in a cohort of children with cystic fibrosis
- The measurement properties of tests and tools used in cystic fibrosis studies: a systematic review
- Lung function in children with cystic fibrosis in the USA and UK: a comparative longitudinal analysis of national registry data
- Catamenial haemoptysis in females with cystic fibrosis: a case series with review of management strategies
- Tobramycin Liquid Crystal Nanoparticles Eradicate Cystic Fibrosis-Related Pseudomonas aeruginosa Biofilms
- New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via Sodium/Hydrogen Exchanger inhibition
- Bacille Calmette-Guerin (BCG) Vaccine for Immune Protection Against Infections
- SLEEP IN CHILDREN WITH CYSTIC FIBROSIS: MORE UNDER THE COVERS
- LPS decreases CFTR open probability and mucociliary transport through generation of reactive oxygen species
- General Health, Attitudes, and Activity in Self-directed Exercise in Patients With Cystic Fibrosis (GAINS-CF)
- Cystic fibrosis improves COVID-19 survival and provides clues for treatment of SARS-CoV-2
- Gene Therapy: A Possible Alternative to CFTR Modulators?
- Azithromycin Exhibits Activity Against Pseudomonas aeruginosa in Chronic Rat Lung Infection Model
- Adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: analysis of a national specialty pharmacy database
- Long-term safety of lumacaftor-ivacaftor in children aged 2-5 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a multicentre, phase 3, open-label, extension study
- Combination CFTR modulator therapy in children and adults with cystic fibrosis
- Személyre szabott, pozitív nyomású légzésterápia cystás fibrosisban
- In Vitro Antimicrobial Susceptibility Testing of Biofilm-Growing Bacteria: Current and Emerging Methods
- The molecular mechanism of CFTR- and secretin-dependent renal bicarbonate excretion
- Rapid cystic fibrosis lung-function decline and in-vitro CFTR modulation
- Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study
- Chronic Pseudomonas aeruginosa Lung Infection Is IL-1R Independent, but Relies on MyD88 Signaling
- SPLUNC1: a novel marker of cystic fibrosis exacerbations
- Transcriptional analysis of cystic fibrosis airways at single-cell resolution reveals altered epithelial cell states and composition
- Clinical Determinants of the Six-Minute Walk Test (6MWT) in Stable Non-Cystic Fibrosis Bronchiectasis Patients
- Association Between Non-Cystic Fibrosis Bronchiectasis and Quality of Life: A Single-Center Cross-Sectional Study
- Molecular genetics of renal ciliopathies
- Cystic fibrosis mutation classes in pediatric otitis media – Fickle or faulty?
- Liver-first strategy for a combined lung and liver transplant in patients with cystic fibrosis
- CFTR regulates embryonic T lymphopoiesis via Wnt signaling in zebrafish
- Many patients with cystic fibrosis have a better quality of life now
- Cystic fibrosis and coeliac disease: a case report of an unusual association
- The clinical effects of combining postural exercises with chest physiotherapy in cystic fibrosis: A single-blind, randomized-controlled trial
- Transcriptional firing represses bactericidal activity in cystic fibrosis airway neutrophils
- Evaluation of Two Chest Tomosynthesis Cystic Fibrosis Scoring Systems Using High-Resolution Computed Tomography Brody Scoring as Reference
- Patient personalized translational tools in Cystic fibrosis to transform data from bench to bed-side and back
- Cystic fibrosis in Brazil: achievements in survival
- Effect of Alpha-1 Antitrypsin on CFTR Levels in Primary Human Airway Epithelial Cells Grown at the Air-Liquid-Interface
- Prenatal Ultrasound Suspicion of Cystic Fibrosis in a Multiethnic Population: Is Extensive CFTR Genotyping Needed?
- The effects of conditioning and lentiviral vector pseudotype on short- and long-term airway reporter gene expression in mice
- Inhaled antibiotics for treatment of adults with non-cystic fibrosis bronchiectasis: A systematic review and meta-analysis
- Heterogenous Susceptibility to R-Pyocins in Populations of Pseudomonas aeruginosa Sourced from Cystic Fibrosis Lungs
- Development and Preclinical Evaluation of New Inhaled Lipoglycopeptides for the Treatment of Persistent Pulmonary MRSA Infections
- Systematic depression and anxiety screening for patients and caregivers: implementation and process improvement in a cystic fibrosis clinic
- Bayesian forecasting for intravenous tobramycin dosing in adults with cystic fibrosis using one versus two serum concentrations in a dosing interval
- The cAMP-phosphodiesterase 4 (PDE4) controls β-adrenoceptor- and CFTR-dependent saliva secretion in mice
- Decreasing the Cut-off Score Value of MALDI-ToF MS Increase the Identities of Burkholderia cepacia Complex Species
- COVID-19 vaccine prioritisation for people with cystic fibrosis
- Cystic Fibrosis Lung Disease in the Aging Population
- Case Report: White Colored Stool: An Early Sign of Cystic Fibrosis in Infants
- Prevalence and serotyping of S. pneumoniae in a large vaccine-naive cohort of adults with cystic fibrosis
- Management of chronic pulmonary disease in the time of coronavirus disease 2019
- The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations
- Lung transplantation for non-cystic fibrosis bronchiectasis in Turkey: Initial institutional experience
- Diagnostic tools and CFTR functional assays in cystic fibrosis: utility and availability in Switzerland
- Integrative chemogenomic analysis identifies small molecules that partially rescue deltaF508-CFTR for cystic fibrosis
- Characterizing medication management and the role of pharmacists in caring for people living with cystic fibrosis: A work system approach
- Virtual medication tours with a pharmacist as part of a cystic fibrosis telehealth visit
- Diversity of cystic fibrosis chronic rhinosinusitis microbiota correlates with different pathogen dominance
- A histone-like nucleoid structuring protein regulates several virulence traits in Burkholderia multivorans
- Peptide 1018 inhibits swarming and influences Anr-regulated gene expression downstream of the stringent stress response in Pseudomonas aeruginosa
- A medication adherence-enhancing simulation intervention in pediatric cystic fibrosis
- Burkholderia cepacia complex bacteremia outbreaks among non-cystic fibrosis patients in the pediatric unit of a university hospital
- ACINETOBACTER BAUMANNII Infection in a Child with Cystic Fibrosis
- Revealing the Metabolic Alterations during Biofilm Development of Burkholderia cenocepacia Based on Genome-Scale Metabolic Modeling
- DNA Methylation Patterns Correlate with the Expression of SCNN1A , SCNN1B , and SCNN1G (Epithelial Sodium Channel, ENaC) Genes
- Real-World Outcomes of Ivacaftor Treatment in People with Cystic Fibrosis: A Systematic Review
- Evaluation of Aerosol Therapy during the Escalation of Care in a Model of Adult Cystic Fibrosis
- Achromobacter xylosoxidans and Stenotrophomonas maltophilia: Emerging Pathogens Well-Armed for Life in the Cystic Fibrosis Patients’ Lung
- DeltaF508 CFTR Hetero- and Homozygous Paediatric Patients with Cystic Fibrosis Do Not Differ with Regard to Nutritional Status
- The Impact of an Efflux Pump Inhibitor on the Activity of Free and Liposomal Antibiotics against Pseudomonas aeruginosa
- Identification of Potential Leukocyte Biomarkers Related to Drug Recovery of CFTR: Clinical Applications in Cystic Fibrosis
- Biofilm Formation on Dental Implant Biomaterials by Staphylococcus aureus Strains Isolated from Patients with Cystic Fibrosis
- Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTATM
- Isorhamnetin Ameliorates Dry Eye Disease via CFTR Activation in Mice
- Unknown Renal Impairment: A Rare Case of Inhaled Tobramycin Induced Acute Kidney Injury in a Cystic Fibrosis Patient
- Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review
- New Sequence Type ST3449 in Multidrug-Resistant Pseudomonas aeruginosa Isolates from a Cystic Fibrosis Patient
- Transduction of Pig Small Airway Epithelial Cells and Distal Lung Progenitor Cells by AAV4
- Nasal Epithelial Cell-Based Models for Individualized Study in Cystic Fibrosis
- Virus Infection of Aspergillus fumigatus Compromises the Fungus in Intermicrobial Competition
- Small Hsps as Therapeutic Targets of Cystic Fibrosis Transmembrane Conductance Regulator Protein
- Cystic Fibrosis Lung Disease Modifiers and Their Relevance in the New Era of Precision Medicine
- Physical Activity Regulates TNFalpha and IL-6 Expression to Counteract Inflammation in Cystic Fibrosis Patients
- In-Host Emergence of Linezolid Resistance in a Complex Pattern of Toxic Shock Syndrome Toxin-1-Positive Methicillin-Resistant Staphylococcus aureus Colonization in Siblings with Cystic Fibrosis
- Individual and Group Response of Treatment with Ivacaftor on Airway and Gut Microbiota in People with CF and a S1251N Mutation
- Pseudomonas aeruginosa Virulence Factors Support Voriconazole Effects on Aspergillus fumigatus
- Home physiotherapists assisting follow-up treatment in cystic fibrosis: a multicenter observational study
- Allergic Broncho-Pulmonary Aspergillosis (ABPA) in cystic fibrosis: Mechanisms, diagnosis and therapeutic options
- Glycated Hemoglobin as a First-line Screening Test for Cystic Fibrosis-Related Diabetes and Impaired Glucose Tolerance in Children With Cystic Fibrosis: A Validation Study
- Stepwise pathogenic evolution of Mycobacterium abscessus
- Mycobacterium abscessus in cystic fibrosis
- Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension
- Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis
- Huff and puff of exercise for airway clearance in cystic fibrosis: how clear is the evidence?
- Calprotectin-mediated zinc chelation inhibits Pseudomonas aeruginosa protease activity in cystic fibrosis sputum
- Characteristics and Outcomes of Children With Cystic Fibrosis Hospitalized With Cirrhosis in the United States
- Loss of PKD1 and PKD2 share common effects on intracellular Ca2+ signaling
- Aquagenic Wrinkling of the Palms after Brief Immersion to Water test as a screening tool for Cystic Fibrosis diagnosis
- Mycobacterium abscessus biofilms produce an extracellular matrix and have a distinct mycolic acid profile
- Long-Term Impact of Ivacaftor on Healthcare Resource Utilization Among People with Cystic Fibrosis in the United States
- Effects of patient room layout on viral accruement on healthcare professionals’ hands
- CFTR MODULATOR DRUG DESENSITIZATION: PRESERVING THE HOPE OF LONG TERM IMPROVEMENT
- Risk factors for Pseudomonas aeruginosa colonization in non-cystic fibrosis bronchiectasis and clinical implications
- Ultrasound Echogenicity of Papillary Thyroid Cancer Is Affected by Tumor Growth Patterns and Tumor Fibrosis
- Rapid macrolide and amikacin resistance testing for Mycobacterium abscessus in people with cystic fibrosis
- The impact of plasma 25-hydroxyvitamin D on pulmonary function and exercise physiology in cystic fibrosis: a multicentre retrospective study
- Could automated analysis of chest X-rays detect early bronchiectasis in children?
- Systematic Review of the Prevalence of Kidney Stones in Cystic Fibrosis
- Hyperinflammation and airway surface liquid dehydration in cystic fibrosis: purinergic system as therapeutic target
- Drug therapies for reducing gastric acidity in people with cystic fibrosis
- The anti-sigma factor MucA is required for viability in Pseudomonas aeruginosa
- Psychological interventions during COVID pandemic: Telehealth for individuals with cystic fibrosis and caregivers
- Sweat sticker for cystic fibrosis
- Respiratory pathogens in patients with acute exacerbation of non-cystic fibrosis bronchiectasis from a developing country
- The Future of Highly Effective Modulator Therapy in Cystic Fibrosis
- Tools for the Real-Time Assessment of a Pseudomonas aeruginosa Infection Model
- Phenotypic and Genomic Variability of Serial Peri-Lung Transplantation Pseudomonas aeruginosa Isolates From Cystic Fibrosis Patients
- Full characterization of plasmids from Achromobacter ruhlandii isolates recovered from a single patient with cystic fibrosis (CF)
- Lumacaftor/ivacaftor changes the lung microbiome and metabolome in cystic fibrosis patients
- Cystic fibrosis airway microbiota associated with outcomes of nontuberculous mycobacterial infection
- Guiding the rational design of patient-centred drug trials in Cystic Fibrosis: A Delphi study
- Integrating Electronic Patient Reported Biometric Measures (ePReBMs) From Wearable Devices in Respiratory Diseases
- Lives changed: A new era for people with cystic fibrosis
- Crowdsourcing airway annotations in chest computed tomography images
- Assessing Practices, Beliefs, and Attitudes about Palliative Care among People with Cystic Fibrosis, Their Caregivers, and Clinicians: Results of a Content Analysis
- Lumacaftor and Ivacaftor
- Tezacaftor and Ivacaftor
- Ivacaftor
- Elexacaftor, Tezacaftor and Ivacaftor
- Use of hemoglobin A1c to identify dysglycemia in cystic fibrosis
- A multi-centre, randomized, controlled trial on coaching and telemonitoring in patients with cystic fibrosis: conneCT CF
- SARS-CoV-2 and Burkholderia cenocepacia infection in a patient with Cystic Fibrosis: An unfavourable conjunction?
- A survey of the prevalence, management and outcome of infants with an inconclusive diagnosis following newborn bloodspot screening for cystic fibrosis (CRMS/CFSPID) in six Italian centres
- Varied clinical significance of ATP-binding cassette C sub-family members for lung adenocarcinoma
- Extensive Mycobacterium abscessus Pneumonia in an Immunocompetent Infant with No Underlying Lung Pathology
- Facile antibacterial materials with turbine-like structure for P. aeruginosa infected scald wound healing
- The effects of nano-curcumin as a nutritional strategy on clinical and inflammatory factors in children with cystic fibrosis: the study protocol for a randomized controlled trial
- Improving and standardizing protocols for cystic fibrosis routine microbiology
- Study to Evaluate Adverse Events and Change in Disease Activity With Oral Capsules of Galicaftor/Navocaftor/ABBV-119 Combination Therapy in Adult Participants With Cystic Fibrosis
- Survival and lung transplant outcomes for individuals with advanced cystic fibrosis lung disease in the United States and Canada: an analysis of national registries
- Antimicrobial Activity of Dalbavancin and Comparators against Staphylococcus aureus Causing Pneumonia in Patients with and without Cystic Fibrosis
- A systematic cochrane review of corrector therapies (with or without potentiators) for people with cystic fibrosis with class II gene variants (most commonly F508DEL)
- Optimising equity of access: how should we allocate slots to the most competitive trials in Cystic Fibrosis (CF)?
- Inhibition of Pseudomonas aeruginosa Alginate Synthesis by Ebselen Oxide and Its Analogues
- Antibodies Against Pseudomonas aeruginosa Alkaline Protease Directly Enhance Disruption of Neutrophil Extracellular Traps Mediated by This Enzyme
- Subinhibitory Concentrations of Biogenic Silver Nanoparticles Affect Motility and Biofilm Formation in Pseudomonas aeruginosa
- Early Interleukin-22 and Neutrophil Proteins Are Correlated to Future Lung Damage in Children With Cystic Fibrosis
- Case Report: Maintenance of Desensitization to Nebulized Colomycin Over 10 Years
- Dose Escalation Study of ALX-009 in Healthy Men and Cystic Fibrosis (CF) and Non-CF Bronchiectasis Patients
- Temporal trends in healthcare resource use and associated costs of patients with cystic fibrosis
- Maintaining normal lung function in children with cystic fibrosis is possible with aggressive treatment regardless of Pseudomonas aeruginosa infections
- Smoothing the transition of adolescents with CF from pediatric to adult care: Pre-transfer needs
- Can clinical benefits of modulators effectively ‘modulate’ adherence in people with CF?
- Trichomonas vaginalis infection impairs anion secretion in vaginal epithelium
- Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies
- Effects of a Short-Term Resistance-Training Program on Heart Rate Variability in Children With Cystic Fibrosis-A Randomized Controlled Trial
- USE OF PROTON PUMP INHIBITORS IS ASSOCIATED WITH LOWER HEMOGLOBIN LEVELS IN PEOPLE WITH CYSTIC FIBROSIS
- Clinical characteristics of children with cystic fibrosis infected with unusual bacteria
- Caregiver burden and related factors in mothers of children and adolescents with cystic fibrosis
- Comparative meta-analysis of cystic fibrosis cell models suggests partial endothelial-to-mesenchymal transition
- Rectal organoid morphology analysis (ROMA) as a promising diagnostic tool in cystic fibrosis
- Population Genomics of Mycobacterium abscessus from United States Cystic Fibrosis Care Centers
- A Novel Cystic Fibrosis Gene Mutation C.4242+1G>C in an Omani Patient: A Case Report
- Acute hyperglycaemia in cystic fibrosis pulmonary exacerbations
- The c.3140-26A>G Variant of the CFTR Gene in Homozygous State Causes Mild Cystic Fibrosis – Overview of Longitudinal Clinical Data of the Patient Managed in our CF Center and Review of the Literature
- Children with Cystic Fibrosis Hospitalised with COVID-19: Multicentre Experience
- Cystic fibrosis in disguise – the wolf in sheep’s clothing, a case report
- Development and validation of the Cystic Fibrosis Decisional Balance for Physical Activity scale (CF-DB-PA)
- Developing a Decision Aid to Facilitate Informed Decision Making About Invasive Mechanical Ventilation and Lung Transplantation Among Adults With Cystic Fibrosis: Usability Testing
- miR-125b/NRF2/HO-1 axis is involved in protection against oxidative stress of cystic fibrosis: A pilot study
- Genetics of CFTR and male infertility
- How do we counsel men with obstructive azoospermia due to CF mutations?-a review of treatment options and outcomes
- Longitudinal analysis of sinus microbiota post endoscopic surgery in patients with cystic fibrosis and chronic rhinosinusitis: a pilot study
- Inflammasome Genetic Variants, Macrophage Function, and Clinical Outcomes in Cystic Fibrosis
- Tobramycin and Colistin display anti-inflammatory properties in CuFi-1 cystic fibrosis cell line
- The Natural Alkaloid Berberine Can Reduce the Number of Pseudomonas aeruginosa Tolerant Cells
- Remote support by multidisciplinary teams: a crucial means to cope with the psychological impact of SARS-COV-2 pandemic on patients with cystic fibrosis and inflammatory bowel disease in Lombardia
- Detection of Bacterial Pathogens Using Home Oropharyngeal Swab Collection in Children with Cystic Fibrosis
- CFTR chloride channel activity modulates the mitochondrial morphology in cultured epithelial cells
- A multimodal approach to detect and monitor early lung disease in cystic fibrosis
- Interferon regulatory factor 8 regulates expression of acid ceramidase and infection susceptibility in cystic fibrosis
- Non-contrast pulmonary perfusion MRI in patients with cystic fibrosis
- A Qualitative Exploration of Sleep Habits and Intervention Needs Among Youth with Cystic Fibrosis
- Pseudomonas aeruginosa, the type of mutation, lung function, and nutritional status in adults with cystic fibrosis
- Predictors of sinonasal disease onset, progression, and severity in pediatric cystic fibrosis patients
- Impact of newborn screening for cystic fibrosis on clinical outcomes of pediatric patients: 10 years’ experience in Lodz Voivodship
- NLR family pyrin domain containing 3 (NLRP3) and caspase 1 (CASP1) modulation by intracellular Cl- concentration
- Association of Medication Regimen Complexity With Clinical Endpoints in Pediatric Patients With Cystic Fibrosis
- Allergic Reactions to Serine Protease-Like Proteins of Staphylococcus aureus
- Can Targeting Iron Help in Combating Chronic Pseudomonas Infection? A Systematic Review
- Ophthalmologic manifestations of adult patients with cystic fibrosis
- Pregnancy in women with Cystic Fibrosis in the 21(st) century
- Peripheral lung effect of elexacaftor/tezacaftor/ivacaftor in adult cystic fibrosis
- Vitamin D intoxication in patients with cystic fibrosis: report of a single-center cohort
- Fatal Pandoraea nosoerga infection after combined liver-lung transplantation for cystic fibrosis: a recontamination by the pre-transplantation strain
- Bronchodilator Responsiveness in Cystic Fibrosis Children Treated for Pulmonary Exacerbations
- Use of a Portable Spirometer in Pediatric Patients With Cystic Fibrosis at the Nancy CHRU: Feasibility Study
- Percussion Palm Cup: Safety and Usability in Infants and Children With Cystic Fibrosis
- Fibrotic expression profile analysis reveals repurposed drugs with potential anti-fibrotic mode of action
- Quinomycin A reduces cyst progression in polycystic kidney disease
- Audit of sweat chloride testing reveals analytical errors
- Pseudo-Bartter Syndrome and Intermediate Sweat Chloride Levels-It Could Still be Cystic Fibrosis!
- Ivacaftor partially corrects airway inflammation in a humanized G551D rat
- Mini-gut feelings: perspectives of people with cystic fibrosis on the ethics and governance of organoid biobanking
- Discovery of CFTR Modulators for the Treatment of Cystic Fibrosis
- Co-infection with Pseudomonas aeruginosa impacts virulence of Staphylococcus aureus and intensifies the severity of infection
- Burkholderia cenocepacia BCAM2418-induced antibody inhibits bacterial adhesion, confers protection to infection and enables identification of host glycans as adhesin targets
- ROLE OF SWEAT ION RATIOS IN DIAGNOSING CYSTIC FIBROSIS
- Thoracic movement screening in adults with cystic fibrosis: reliability of the Manchester musculoskeletal screening tool
- A complicated association between two different genetic rare disorders: Cystic Fibrosis and Spinal Muscular Atrophy
- Trends of antimicrobial resistance and combination susceptibility testing of cystic fibrosis multidrug-resistant Pseudomonas aeruginosa: A ten-year update
- Severe obstructive biliopathy mimicking biliary atresia in an infant with cystic fibrosis
- Development and evaluation of an internet-based cognitive behavioral therapy intervention for anxiety and depression in adults with cystic fibrosis (eHealth CF-CBT): An international collaboration
- Changes in the Cystic Fibrosis Airway Microbiome in Response to CFTR Modulator Therapy
- Nasal high-flow therapy as an adjunct to exercise in patients with cystic fibrosis: A pilot feasibility trial
- Optical Measurements of Sweat for in Vivo Quantification of CFTR Function in Individual Sweat Glands
- A mutational approach to dissect the functional role of the putative CFTR “PTM-CODE”
- Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study
- Microbiota: A Missing Link in The Pathogenesis of Chronic Lung Inflammatory Diseases
- Case Report of a Successful Pregnancy in a Cystic Fibrosis Patient with The c.1521_1523delCTT/c.3718-2477C>t Genotypes
- CF Fungal Disease in the Age of CFTR Modulators
- Simultaneous pancreas and kidney transplant after bilateral lung transplant for a recipient with Cystic Fibrosis
- Pharmacokinetics of Meropenem in People with Cystic Fibrosis-A Proof of Concept Clinical Trial
- Is the ENaC Dysregulation in CF an Effect of Protein-Lipid Interaction in the Membranes?
- Allergic Diseases Caused by Aspergillus Species in Patients with Cystic Fibrosis
- Actin-Resistant DNase1L2 as a Potential Therapeutics for CF Lung Disease
- IRE1alpha Is a Therapeutic Target for Cystic Fibrosis Airway Inflammation
- Functional and Transcriptional Adaptations of Blood Monocytes Recruited to the Cystic Fibrosis Airway Microenvironment In Vitro
- Oxidative Stress and Endoplasmic Reticulum Stress in Rare Respiratory Diseases
- Pseudomonas aeruginosa : An Audacious Pathogen with an Adaptable Arsenal of Virulence Factors
- Antimicrobial Resistance in Common Respiratory Pathogens of Chronic Bronchiectasis Patients: A Literature Review
- Copper-Associated Oxidative Stress Contributes to Cellular Inflammatory Responses in Cystic Fibrosis
- A Novel Infection Protocol in Zebrafish Embryo to Assess Pseudomonas aeruginosa Virulence and Validate Efficacy of a Quorum Sensing Inhibitor In Vivo
- The Application of Bicarbonate Recovers the Chemical-Physical Properties of Airway Surface Liquid in Cystic Fibrosis Epithelia Models
- Lumacaftor and Matrine: Possible Therapeutic Combination to Counteract the Inflammatory Process in Cystic Fibrosis
- Airway Surface Liquid pH Regulation in Airway Epithelium Current Understandings and Gaps in Knowledge
- Phenotypic Presentations of Cystic Fibrosis in Children of African Descent
- Screening Methods for Diagnosing Cystic Fibrosis-Related Diabetes: A Network Meta-Analysis of Diagnostic Accuracy Studies
- CFTR Cooperative Cis -Regulatory Elements in Intestinal Cells
- Two Siblings Homozygous for F508del-CFTR Have Varied Disease Phenotypes and Protein Biomarkers
- Neonatal Screening in Europe Revisited: An ISNS Perspective on the Current State and Developments Since 2010
- Bacterial Re-Colonization Occurs Early after Lung Transplantation in Cystic Fibrosis Patients
- CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis
- DNA Methylation at ATP11A cg11702988 Is a Biomarker of Lung Disease Severity in Cystic Fibrosis: A Longitudinal Study
- Cystic Fibrosis-Related Diabetes (CFRD): Overview of Associated Genetic Factors
- Cystic Fibrosis: Recent Insights into Inhaled Antibiotic Treatment and Future Perspectives
- Modulation of Ion Transport to Restore Airway Hydration in Cystic Fibrosis
- Variability in Bacteriophage and Antibiotic Sensitivity in Serial Pseudomonas aeruginosa Isolates from Cystic Fibrosis Airway Cultures over 12 Months
- Factors influencing physical activity in adults with cystic fibrosis
- Real-world use of ivacaftor in Canada: A retrospective analysis using the Canadian Cystic Fibrosis Registry
- Treatment of Cystic Fibrosis: From Gene- to Cell-Based Therapies
- Could Azithromycin Be Part of Pseudomonas aeruginosa Acute Pneumonia Treatment?
- Sclerosing mucoepidermoid carcinoma of the salivary glands: report of three cases with special concern to the counterpart accompanied by eosinophilia
- Insomnia in adults with cystic fibrosis: strong association with anxiety/depression and impaired quality of life
- Simultaneous Double Intussusception in a Patient with Cystic Fibrosis
- Targeted replacement of full-length CFTR in human airway stem cells by CRISPR/Cas9 for pan-mutation correction in the endogenous locus
- Effect of transfer from a paediatric to adult cystic fibrosis centre on clinical status and hospital attendance
- Managing Fungal Infections in Cystic Fibrosis Patients: Challenges in Clinical Practice
- The impact of COVID-19 shielding on the wellbeing, mental health and treatment adherence of adults with cystic fibrosis
- Pulmonary rehabilitation for cystic fibrosis: A narrative review of current literature
- Remote Monitoring of High-Risk Patients With Chronic Cardiopulmonary Diseases
- MyVoice:CF Decision Aid for Women With Cystic Fibrosis
- Racially equitable diagnosis of cystic fibrosis using next-generation DNA sequencing: a case report
- Early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study
- Soft, skin-interfaced sweat stickers for cystic fibrosis diagnosis and management
- V-type ATPase Mediates Airway Surface Liquid Acidification in Pig Small Airway Epithelial Cells
- Characterization of a novel mCH3 conjugated anti-PcrV scFv molecule
- A Rare Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Mutation Associated With Typical Cystic Fibrosis in an Arab Child
- Azithromycin and the microbiota of cystic fibrosis sputum
- Cystic fibrosis transmembrane conductance regulator modulators for cystic fibrosis: a new dawn?
- A Pseudomonas aeruginosa Antimicrobial Affects the Biogeography but Not Fitness of Staphylococcus aureus during Coculture
- Ventilation Heterogeneity in Asthma and COPD: The Value of the Poorly Communicating Fraction as the Ratio of Total Lung Capacity to Alveolar Volume
- Minimising the risk of cross infection between siblings with cystic fibrosis (CF) within the home: Successful domestic steam disinfection of CF bacterial and foodborne pathogens on common household cutlery and crockery utensils
- Continuous glucose monitoring indices predict poor FEV(1) recovery following cystic fibrosis pulmonary exacerbations
- The CFTR P67L variant reveals a key role for N-terminal lasso helices in channel folding, maturation, and pharmacologic rescue
- Increased mortality in patients with non cystic fibrosis bronchiectasis with respiratory comorbidities
- Immunoglobulin G: A useful outcome marker in the follow-up of cystic fibrosis patients?
- Update on Calcium Signaling in Cystic Fibrosis Lung Disease
- Murine Model of Sinusitis Infection for Screening Antimicrobial and Immunomodulatory Therapies
- Chronic infection by nontypeable Haemophilus influenzae fuels airway inflammation
- Quantification of Cystic Fibrosis Lung Disease with Radiomics-based CT Scores
- CFTR modulator therapy improves cystic fibrosis-related diabetes. But how?
- Impact of airway Exophiala spp. on children with cystic fibrosis
- Emergence and impact of oprD mutations in Pseudomonas aeruginosa strains in cystic fibrosis
- Elexacaftor co-potentiates the activity of F508del and gating mutants of CFTR
- Patient and family perceptions of telehealth as part of the cystic fibrosis care model during COVID-19
- Clostridioides difficile colonisation and infection in a cohort of Australian adults with cystic fibrosis
- I won’t let cystic fibrosis stop me from living my best life
- Co-translational folding of the first transmembrane domain of ABC-transporter CFTR is supported by assembly with the first cytosolic domain
- High-fiber diets attenuate emphysema development via modulation of gut microbiota and metabolism
- An Unusual Presentation of Chest Pain in a Patient With Cystic Fibrosis: A Case Report
- Staphylococcus aureus Potentiates the Hemolytic Activity of Burkholderia cepacia Complex (Bcc) Bacteria
- An observational study of anaerobic bacteria in cystic fibrosis lung using culture dependant and independent approaches
- New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation
- Increased CFTR expression and function from an optimized lentiviral vector for cystic fibrosis gene therapy
- Fighting diagnostic confirmation bias: Cystic fibrosis, allergic bronchopulmonary aspergillus, or both?
- Prediction and Validation of a Druggable Site on Virulence Factor of Drug Resistant Burkholderia cenocepacia
- Post COVID-19 large pneumatocele: clinical and pathological perspectives
- Does cystic fibrosis make susceptible to celiac disease?
- Trainability of Health-Related and Motor Performance Fitness in Adults with Cystic Fibrosis within a 12-Month Partially Supervised Exercise Program
- Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during pregnancy and lactation
- Improved detection of air trapping on expiratory computed tomography using deep learning
- Luminal Gastrointestinal Manifestations of Cystic Fibrosis
- Clinical use of shear-wave elastography for detecting liver fibrosis in children and adolescents with cystic fibrosis
- A bacterial stimulation assay for bronchoalveolar lavage immune cells from young children with cystic fibrosis
- Tobramycin and vestibulotoxicity: retrospective analysis of four cases
- Characterizing mucociliary clearance in young children with cystic fibrosis
- Isolated Liver Rejection After Lung and Combined Kidney-Liver Transplantation: A Case Report
- A formalized transition program for cystic fibrosis: a ten-year retrospective analysis of 97 patients in Lyon
- A rare case of a cystic renal mass with heterotopic ossification and a mini literature review
- Untreated dental disease and lung transplant waitlist evaluation time for individuals with cystic fibrosis
- Resolvin D1 and D2 reduce SARS-CoV-2-induced inflammatory responses in cystic fibrosis macrophages
- How Bacterial Adaptation to Cystic Fibrosis Environment Shapes Interactions Between Pseudomonas aeruginosa and Staphylococcus aureus
- Changes in handgrip strength in children with cystic fibrosis compared to children without cystic fibrosis
- Cyproheptadine as an appetite stimulant in children with cystic fibrosis
- Commentary on early management of meconium ileus in infants with cystic fibrosis: A prospective population cohort study
- Rates of adverse and serious adverse events in children with cystic fibrosis
- Acceptability, Feasibility and Preliminary Efficacy of CFWP to Treat Clinically Elevated Fatigue in Adults With CF
- Computed tomography in cystic fibrosis lung disease: a focus on radiation exposure
- Anti-quorum sensing and antibiofilm potential of 1,8-cineole derived from Musa paradisiaca against Pseudomonas aeruginosa strain PAO1
- Cytokine signaling pathway in cystic fibrosis: expression of SOCS and STATs genes in different clinical phenotypes of the disease
- Evaluation of bone metabolism in children with cystic fibrosis
- Assisting PNA transport through cystic fibrosis human airway epithelia with biodegradable hybrid lipid-polymer nanoparticles
- A Phase 3 Open-Label Study of ELX/TEZ/IVA in Children 6 Through 11 Years of Age With CF and at Least One F508del Allele
- Is Impulse Oscillometry System a Useful Method for the Evaluation and Follow-Up of Patients with Cystic Fibrosis?
- Impact of 1h oral glucose tolerance test on the clinical status of adult cystic fibrosis patients over a 4-year period
- Dornase alfa for cystic fibrosis
- Cystic fibrosis and noninvasive liver fibrosis assessment methods in children
- Telerehabilitation Using Fitness Application in Patients with Severe Cystic Fibrosis Awaiting Lung Transplant: A Pilot Study
- A retrospective evaluation of vitamin K for hemoptysis in adult cystic fibrosis patients
- Physiotherapy via telehealth for acute respiratory exacerbations in paediatric cystic fibrosis
- One versus Many: Polymicrobial Communities and the Cystic Fibrosis Airway
- SPI-1005 for Prevention and Treatment of Tobramycin Induced Ototoxicity
- The clinical features that contribute to poor bone health in young Australians living with cystic fibrosis: A recommendation for BMD screening
- Managing Cystic Fibrosis related diabetes via telehealth during COVID-19 pandemic
- Assessing the Potency of β-Lactamase Inhibitors with Diverse Inactivation Mechanisms against the PenA1 Carbapenemase from Burkholderia multivorans
- Computationally designed pyocyanin demethylase acts synergistically with tobramycin to kill recalcitrant Pseudomonas aeruginosa biofilms
- Molecular mechanism mediating enteric bacterial translocation after severe burn: the role of cystic fibrosis transmembrane conductance regulator
- Stability Prediction for Mutations in the Cytosolic Domains of Cystic Fibrosis Transmembrane Conductance Regulator
- Transmission, adaptation and geographical spread of the Pseudomonas aeruginosa Liverpool epidemic strain
- Genomics of an endemic cystic fibrosis Burkholderia multivorans strain reveals low within-patient evolution but high between-patient diversity
- Toward the Establishment of New Clinical Endpoints for Cystic Fibrosis: The Role of Lung Clearance Index and Cardiopulmonary Exercise Testing
- Chest physiotherapy enhances detection of Pseudomonas aeruginosa in nonexpectorating children with cystic fibrosis
- Geographic distribution of cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in Saudi Arabia
- Role of Toilet Bronchoscopy in RICU
- Care of people with Cystic Fibrosis: what is the role of specialists in Endocrinology and Nutrition?
- Efficacy and safety of inhaled dry-powder mannitol in adults with cystic fibrosis: An international, randomized controlled study
- Ex vivo model predicted in vivo efficacy of CFTR modulator therapy in a child with rare genotype
- An innovative role for luteolin as a natural quorum sensing inhibitor in Pseudomonas aeruginosa
- Expression and Role of CFTR in Human Esophageal Squamous Cell Carcinoma
- Pseudomonas aeruginosa PA80 is a cystic fibrosis isolate deficient in RhlRI quorum sensing
- Burkholderia cenocepacia transcriptome during the early contacts with giant plasma membrane vesicles derived from live bronchial epithelial cells
- VJ Segment Usage of TCR-Beta Repertoire in Monozygotic Cystic Fibrosis Twins
- Ceftolozane/tazobactam for Pseudomonas aeruginosa pulmonary exacerbations in cystic fibrosis adult patients: a case series
- Tele-Coaching Intervention to Improve Treatment Adherence in Cystic Fibrosis
- Impact of Bronchial Drainage Technique by the Medical Device Simeox® on Respiratory Function and Symptoms in Adult Patients With Cystic Fibrosis
- Induction of Ciliary Orientation by Matrix Patterning and Characterization of Mucociliary Transport
- The genetic background significantly impacts the severity of kidney cystic disease in the Pkd1(RC/RC) mouse model of autosomal dominant polycystic kidney disease
- How abnormal is the normal? Clinical characteristics of CF patients with normal FEV1
- Towards better understanding of the heparin role in NETosis: feasibility of using native mass spectrometry to monitor interactions of neutrophil elastase with heparin oligomers
- Immune Responses to Pseudomonas aeruginosa Biofilm Infections
- Current therapies for gastro-oesophageal reflux in the setting of chronic lung disease: state of the art review
- Association of Intensity of Anti-Pseudomonal Antibiotic Therapy With Risk of Treatment-Emergent Organisms in Cystic Fibrosis Children with Newly Acquired Pseudomonas Aeruginosa
- Pneumocystis jirovecii among patients with cystic fibrosis and their household members
- Peak glucose during an oral glucose tolerance test is associated with future diabetes risk in adults with cystic fibrosis
- LasR-deficient Pseudomonas aeruginosa variants increase airway epithelial mICAM-1 expression and enhance neutrophilic lung inflammation
- Pharmacological approaches to cystic fibrosis
- A non-olfactory shark adenosine receptor activates CFTR with unique pharmacology and structural features
- Strain-Resolved Dynamics of the Lung Microbiome in Patients with Cystic Fibrosis
- Inadequate Functioning of Nebulizer System Compressors Used by Individuals With Cystic Fibrosis
- Timing of dornase alfa inhalation for cystic fibrosis
- Effect of Endoscopic Sinus Surgery on Clinical Outcomes in DeltaF508 Cystic Fibrosis Patients
- The effect of oral and intravenous antimicrobials on pulmonary exacerbation recovery in cystic fibrosis
- Derivation of iPSC line (RCMGi002-A) from dermal fibroblasts of a cystic fibrosis female patient with homozygous F508del mutation
- The correlation between 6-min walk test and respiratory parameters in children with cystic fibrosis
- Proteomics insights into the Burkholderia cenocepacia phosphorus stress response
- Long-term benefits of nusinersen in a child affected by cystic fibrosis and spinal muscular atrophy type 1
- Quantification of Pulmonary Pathology in Cystic Fibrosis-Comparison Between Digital Chest Tomosynthesis and Computed Tomography
- Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry Cohort study
- Genetic Association With Pseudomonas aeruginosa Acquisition in Cystic Fibrosis: Influence of Surfactant Protein D and Mannose-Binding Lectin
- Comparison of typical and atypical computed tomography patterns regarding reversibility and fibrosis in pulmonary sarcoidosis
- Azole Resistance in Aspergillus fumigatus: A Five-Year Follow Up Experience in a Tertiary Hospital With a Special Focus on Cystic Fibrosis
- The Frequency and Related Factors of Non-Tuberculosis Mycobacteria Infections among Patients with Cystic Fibrosis
- Pancreatitis severity in mice with impaired CFTR function but pancreatic sufficiency is mediated via ductal and inflammatory cells-Not acinar cells
- Cystic fibrosis prognosis in Europa : chronicle of an announced dilemma
- The Impact of Environmental Factors on Monogenic Mendelian Diseases
- Assessment of treatment burden and complexity in cystic fibrosis: A quality improvement project
- Cystic Fibrosis Newborn Screening in Austria Using PAP and the Numeric Product of PAP and IRT Concentrations as Second-Tier Parameters
- In Vitro Newly Isolated Environmental Phage Activity against Biofilms Preformed by Pseudomonas aeruginosa from Patients with Cystic Fibrosis
- Dysfunctional Inflammation in Cystic Fibrosis Airways: From Mechanisms to Novel Therapeutic Approaches
- Intestinal Dysbiosis in Young Cystic Fibrosis Rabbits
- The Diagnosis of Cystic Fibrosis in Adult Age. Data from the Italian Registry
- Severe Genotype, Pancreatic Insufficiency and Low Dose of Pancreatic Enzymes Associate with Abnormal Serum Sterol Profile in Cystic Fibrosis
- Oral Nutritional Supplements in Adults with Cystic Fibrosis: Effects on Intake, Levels of Fat-Soluble Vitamins, and Bone Remodeling Biomarkers
- Bacterial Interactions with Aspergillus fumigatus in the Immunocompromised Lung
- Approaches to Targeting Bacterial Biofilms in Cystic Fibrosis Airways
- Impact of Altered Gut Microbiota and Its Metabolites in Cystic Fibrosis
- Development of a simple medium for the selective isolation of bacteria belonging to the Streptococcus anginosus group
- Chronic incretin-based therapy in cystic fibrosis-related diabetes: A tale of 3 patients treated with sitagliptin for over 5 years
- SLC26A9 SNP rs7512462 is not associated with lung disease severity or lung function response to ivacaftor in cystic fibrosis patients with G551D-CFTR
- Impairment in inflammasome signaling by the chronic Pseudomonas aeruginosa isolates from cystic fibrosis patients results in an increase in inflammatory response
- Inhaled Sodium Nitrite as an Antimicrobial for Cystic Fibrosis
- Cyclic nucleotide phosphodiesterase inhibitors as therapeutic interventions for cystic fibrosis
- Wideband Acoustic Reflex Growth in Adults With Cystic Fibrosis
- Educational Needs and Preferences for Patient-Centered Outcomes Research in the Cystic Fibrosis Community: Mixed Methods Study
- SARS-CoV-2 infection in a pediatric patient with cystic fibrosis
- Micro-gallbladder: A rare surgical problem
- Mycobacterium abscessus biofilms have viscoelastic properties which may contribute to their recalcitrance in chronic pulmonary infections
- CFTR Deficiency Affects Glucose Homeostasis via Regulating GLUT4 Plasma Membrane Transportation
- Anxiety in Children with Cystic Fibrosis and Their Parents: A Systematic Review
- PAR-2-activated secretion by airway gland serous cells: role for CFTR and inhibition by Pseudomonas aeruginosa
- Impact of hypoxia and AMPK on CFTR-mediated bicarbonate secretion in human cholangiocyte organoids
- Exercise-induced desaturation in subjects with non-cystic fibrosis bronchiectasis: laboratory-based tests versus field-based exercise tests
- Cystic fibrosis-related mortality trends in Brazil for the 1999-2017 period: a multiple-cause-of-death study
- Outcomes Of Non-Cystic Fibrosis Related Bronchiectasis Post Lung Transplantation
- Reflex zone stimulation reduces ventilation inhomogeneity in cystic fibrosis: A randomised controlled cross-over study
- State of the Art in Cystic Fibrosis Pharmacology Optimization of anti-microbials in the treatment of Cystic Fibrosis Pulmonary Exacerbations: III. Executive Summary
- Disparities in cystic fibrosis survival in Mexico: Impact of socioeconomic status
- The Lung Microbiome in Young Children with Cystic Fibrosis: A Prospective Cohort Study
- Evaluation of Fused Pyrrolothiazole Systems as Correctors of Mutant CFTR Protein
- Discrete choice experiment (DCE) to quantify the influence of trial features on the decision to participate in cystic fibrosis (CF) clinical trials
- Inflammation in children with cystic fibrosis: contribution of bacterial production of long-chain fatty acids
- A pilot study of cystic fibrosis exacerbation response phenotypes reveals contrasting serum and sputum iron trends
- Effect of CFTR correctors on the traffic and the function of intracellularly retained ABCB4 variants
- Clinical use of the modified shuttle test in children with cystic fibrosis: Is one test sufficient?
- Limb muscle size and contractile function in adults with cystic fibrosis: A systematic review and meta-analysis
- “Il faut continuer a poser des questions” patient reported outcome measures in cystic fibrosis: An anthropological perspective
- Clinical Effort Against Smoke Exposure in Cystic Fibrosis
- Primary Immun Deficiency in Patients with Non-Cystic Fibrosis Bronchiectasis and Its Relationship with Clinical Parameters
- Genetic modification of cystic fibrosis with deltaF508 mutation of CFTR gene using the CRISPR system in peripheral blood mononuclear cells
- Multidrug resistance protein structure of Trypanosoma evansi isolated from buffaloes in Ngawi District, Indonesia: A bioinformatics analysis
- Identifying children with Cystic Fibrosis in population-scale routinely collected data in Wales: A Retrospective Review
- Thriving alongside CF: Developing a Grounded Theory of Empowerment in Children and Young People with Cystic Fibrosis During Key Life Transitions
- The impact of physical activity and exercise interventions for physical health in people with cystic fibrosis: protocol for a systematic review
- Defining key outcomes to evaluate performance of newborn screening programmes for cystic fibrosis
- Favorable Clinician Acceptability of Telehealth as Part of the Cystic Fibrosis Care Model during the COVID-19 Pandemic
- Corrigendum: Stenotrophomonas maltophilia phenotypic and genotypic features through 4-year cystic fibrosis lung colonization
- Reference Values Matter: Fewer Patients with Malnutrition using American Compared to more Recent German Growth Charts
- Comparative evaluation of expiratory airflow limitation between patients with COPD and BE using IOS
- A rabbit model of cystic fibrosis
- Exhaled volatile organic compounds detect pulmonary exacerbations early in children with cystic fibrosis: results of a 1 year observational pilot study
- Pseudomonas aeruginosa aggregates in cystic fibrosis sputum produce exopolysaccharides that likely impede current therapies
- Cystic Fibrosis Polymorphic Variants in a Russian Population
- Downregulation of epithelial sodium channel (ENaC) activity in human airway epithelia after low temperature incubation
- Preliminary national report on cystic fibrosis epidemiology in Tunisia: the actual state of affairs
- Virulence attenuating combination therapy: a potential multi-target synergy approach to treat Pseudomonas aeruginosa infections in cystic fibrosis patients
- Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis
- Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
- Attitudes Towards Involving Children in Decision-Making Surrounding Lung Transplantation
- Frequent Pet Contact as Risk Factor for Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis
- Burkholderia vietnamiensis Causing a Non-lactational Breast Abscess in a Non-cystic Fibrosis Patient in Tamil Nadu, India
- Neutrophil dysfunction in cystic fibrosis
- Contribution of common CFTR variants (M470V, T854, and Q1463) to cystic fibrosis in Tunisia: haplotype analysis
- How Many Maneuvers Should We Do for Maximal Inspiratory and Expiratory Muscle Pressure Testing in Children: A Retrospective Review in Children with Cystic Fibrosis
- Disparities in insurance coverage among hospitalized adult congenital heart disease patients before and after the Affordable Care Act
- Reproductive Health Counseling and Contraceptive Use in Adolescents with Cystic Fibrosis
- Feasibility study on exhaled-breath analysis by untargeted Selected-Ion Flow-Tube Mass Spectrometry in children with cystic fibrosis, asthma, and healthy controls: Comparison of data pretreatment and classification techniques
- Cystic fibrosis-related diabetes and lung disease: an update
- Aspergillus fumigatus Strain-Specific Conidia Lung Persistence Causes an Allergic Broncho-Pulmonary Aspergillosis-Like Disease Phenotype
- Comparative genomics of ST5 and ST30 methicillin-resistant Staphylococcus aureus sequential isolates recovered from paediatric patients with cystic fibrosis
- An evaluation of healthcare utilization and clinical charges in children and adults with cystic fibrosis
- The Incidence of Glucose Disorders Related to Cystic Fibrosis Could Be Higher with Current Criteria (Re: Carbohydrate Metabolism Changes in Cystic Fibrosis. J Pediatr Endocrinol Metab 2007; 20: 621-663)
- Rapid Improvement After Starting Elexacaftor-tezacaftor-ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease
- Effects of Lumacaftor-Ivacaftor on Lung Clearance Index, Magnetic Resonance Imaging and Airway Microbiome in Phe508del Homozygous Patients with Cystic Fibrosis
- Bewertung der Funktionen des rechten Herzens bei Kindern mit leichter Mukoviszidose
- Virtual Screening Approach to Identifying a Novel and Tractable Series of Pseudomonas aeruginosa Elastase Inhibitors
- Lung Transplantation for Cystic Fibrosis in Turkey: First Report
- Failed eradication therapy of new onset Pseudomonas aeruginosa infections in cystic fibrosis children is associated with bacterial resistance to neutrophil functions
- Generation of two induced pluripotent stem cell lines (RCMGi004-A and -B) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/W1282X mutations in CFTR gene
- The Upd3 cytokine couples inflammation to maturation defects in Drosophila
- Beyond phenotype: The genomic heterogeneity of co-infecting Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients
- Outcomes of cystic fibrosis pulmonary exacerbations treated with antibiotics with activity against anaerobic bacteria
- Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough
- Functional relationship between CFTR and RAC3 expression for maintaining cancer cell stemness in human colorectal cancer
- CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells
- Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort
- Williams-Campbell syndrome: an unusual presentation in an adult patient
- Change in circulating proteins during treatment of pulmonary exacerbation in patients with cystic fibrosis
- Clinical characteristics associated with lung function decline in individuals with adult-diagnosed cystic fibrosis: Contemporary analysis of the Canadian CF Registry
- Interval versus constant-load exercise training in adults with Cystic Fibrosis
- PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy
- Withdrawal of dornase alfa increases ventilation inhomogeneity in children with cystic fibrosis
- Outcomes of cystic fibrosis pulmonary exacerbations treated with antibiotics with activity against anaerobic bacteria.
- CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.
- Next-Generation Sequencing for Molecular Diagnosis of Cystic Fibrosis in a Brazilian Cohort.
- Williams-Campbell syndrome: an unusual presentation in an adult patient.
- Change in circulating proteins during treatment of pulmonary exacerbation in patients with cystic fibrosis.
- Novel Correctors and Potentiators Enhance Functional Rescue of CFTR Nonsense Mutation Translational Readthrough.
- Functional relationship between CFTR and RAC3 expression for maintaining cancer cell stemness in human colorectal cancer.
- Clinical characteristics associated with lung function decline in individuals with adult-diagnosed cystic fibrosis: Contemporary analysis of the Canadian CF Registry.
- Interval versus constant-load exercise training in adults with Cystic Fibrosis.
- Beyond phenotype: The genomic heterogeneity of co-infecting Mycobacterium abscessus smooth and rough colony variants in cystic fibrosis patients.
- Prostaglandin E2 stimulates anion and fluid secretion triggered by lipopolysaccharide in rat vaginal epithelium.
- Hospice care access inequalities: a systematic review and narrative synthesis.
- Immune transcriptomes of highly exposed SARS-CoV-2 asymptomatic seropositive versus seronegative individuals from the Ischgl community.
- The Upd3 cytokine couples inflammation to maturation defects in Drosophila.
- Inhalable nano into micro dry powders for ivacaftor delivery: The role of mannitol and cysteamine as mucus-active agents.
- Genetic markers for treatment-related pancreatitis in a cohort of Hispanic children with acute lymphoblastic leukemia.
- Antibiotic use evaluation in hospitalized pediatric patients with respiratory tract infections: A retrospective chart review study.
- Virtual Screening Approach to Identifying a Novel and Tractable Series of Pseudomonas aeruginosa Elastase Inhibitors.
- Diaphragmatic thickness and excursion by lung ultrasound in pediatric chronic pulmonary diseases.
- Lung Transplantation for Cystic Fibrosis in Turkey: First Report.
- Failed eradication therapy of new onset Pseudomonas aeruginosa infections in cystic fibrosis children is associated with bacterial resistance to neutrophil functions.
- Generation of two induced pluripotent stem cell lines (RCMGi004-A and -B) from human skin fibroblasts of a cystic fibrosis patient with compound heterozygous F508del/W1282X mutations in CFTR gene.
- Editing Myosin VB Gene to Create Porcine Model of Microvillus Inclusion Disease, With Microvillus-Lined Inclusions and Alterations in Sodium Transporters.
- Feasibility study on exhaled-breath analysis by untargeted Selected-Ion Flow-Tube Mass Spectrometry in children with cystic fibrosis, asthma, and healthy controls: Comparison of data pretreatment and classification techniques.
- GRAF2, WDR44, and MICAL1 mediate Rab8/10/11-dependent export of E-cadherin, MMP14, and CFTR ΔF508.
- Burkholderia vietnamiensis Causing a Non-lactational Breast Abscess in a Non-cystic Fibrosis Patient in Tamil Nadu, India.
- Neutrophil dysfunction in cystic fibrosis.
- Contribution of common CFTR variants (M470V, T854, and Q1463) to cystic fibrosis in Tunisia: haplotype analysis.
- How Many Maneuvers Should We Do for Maximal Inspiratory and Expiratory Muscle Pressure Testing in Children: A Retrospective Review in Children with Cystic Fibrosis.
- A novel highly bio-available itraconazole formulation (SUBA®-Itraconazole) for anti-fungal prophylaxis in Lung Transplant recipients.
- Disparities in insurance coverage among hospitalized adult congenital heart disease patients before and after the Affordable Care Act.
- Reproductive Health Counseling and Contraceptive Use in Adolescents with Cystic Fibrosis.
- Longitudinal effects of ivacaftor and medicine possession ratio in people with the Gly551Asp mutation: a 5-year study.
- Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
- Complete Genome Sequence of Burkholderia cenocepacia Phage Mica.
- Phenotyping of Rare CFTR Mutations Reveals Distinct Trafficking and Functional Defects.
- Current Status of Genetic Diagnosis Laboratories and Frequency of Genetic Variants Associated with Cystic Fibrosis through a Newborn-Screening Program in Turkey.
- Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.
- Associations Between Religious/Spiritual Coping and Depression Among Adults with Cystic Fibrosis: A 12-Month Longitudinal Study.
- Friends or enemies? The complicated relationship between Pseudomonas aeruginosa and Staphylococcus aureus.
- Effect of omeprazole on symptoms of gastroesophageal reflux disease in children with cystic fibrosis. A randomized, double-blind, placebo-controlled trial.
- Measurement properties of the one-minute sit-to-stand test in children and adolescents with cystic fibrosis: A multicenter randomized cross-over trial.
- F1099L-CFTR (c.3297C>G) has Impaired Channel Function and Associates with Mild Disease Phenotypes in Two Pediatric Patients.
- First clinical trials of the inhaled epithelial sodium channel inhibitor BI 1265162 in healthy volunteers.
- Lumacaftor/ivacaftor-associated health stabilisation in adults with severe cystic fibrosis.
- Cystic fibrosis transmembrane conductance regulator-associated ligand protects dopaminergic neurons by differentially regulating metabotropic glutamate receptor 5 in the progression of neurotoxin 6-hydroxydopamine-induced Parkinson’s disease model.
- Diversity and importance of tracheobronchial glands in mammalian species.
- Culture-independent multilocus sequence typing of Pseudomonas aeruginosa for cross-infection screening.
- Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: A double-blind, placebo-controlled, randomized, crossover study with open-label extension.
- Human Genetic Variation Influences Enteric Fever Progression.
- Contribution of Drugs Interfering with Protein and Cell Wall Synthesis to the Persistence of Pseudomonas aeruginosa Biofilms: An In Vitro Model.
- Pulmonary Aspergillosis: Spectrum of Disease.
- Erratum to ‘From micro to macro; joining the dots of early CF lung disease’ [Journal of Cystic Fibrosis (2020) 850-851/2102].
- Determinants of COVID-19 preventive behaviours among adults with chronic diseases in the USA: an analysis of the nationally representative COVID-19 impact survey.
- Percutaneous endoscopic gastrostomy in children: A single center experience in Saudi Arabia.
- Differential diagnosis of perinatal Bartter, Bartter and Gitelman syndromes.
- Detection of cytosine methylation in Burkholderia cenocepacia by single-molecule real-time sequencing and whole-genome bisulfite sequencing.
- Leukocyte Telomere Length Is Not Reduced in Children and Adults with Cystic Fibrosis but Associates with Clinical Characteristics-A Cross-Sectional Study.
- Animal models for cystic fibrosis: a systematic search and mapping review of the literature. Part 2: nongenetic models.
- The Potential for Phospholipids in the Treatment of Airway Inflammation: An Unexplored Solution.
- Targeting G542X CFTR nonsense alleles with ELX-02 restores CFTR function in human-derived intestinal organoids.
- Effect of CFTR modulator therapy on cystic fibrosis-related diabetes.
- Pediatric pulmonology 2019 year in review: rare and diffuse lung disease.
- Asthma in Cystic Fibrosis: Definitions and Implications of This Overlap Syndrome.
- History of Newborn Screening for Cystic Fibrosis-The Early Years.
- Performance of a Three-Tier (IRT-DNA-IRT) Cystic Fibrosis Screening Algorithm in British Columbia.
- It All Depends What You Count-The Importance of Definitions in Evaluation of CF Screening Performance.
- Constructing a Bioethical Framework to Evaluate and Optimise Newborn Bloodspot Screening for Cystic Fibrosis.
- Newborn Screening for CF across the Globe-Where Is It Worthwhile?
- Inconclusive Diagnosis after Newborn Screening for Cystic Fibrosis.
- The Role of Extended CFTR Gene Sequencing in Newborn Screening for Cystic Fibrosis.
- Processing Newborn Bloodspot Screening Results for CF.
- Psychological Impact of NBS for CF.
- Pancreatitis-Associated Protein in Neonatal Screening for Cystic Fibrosis: Strengths and Weaknesses.
- Production of CFTR-ΔF508 Rabbits.
- Prospective Evaluation of Aspergillus fumigatus-Specific IgG in Patients With Cystic Fibrosis.
- Comparative Analysis of Clinical Parameters and Sputum Biomarkers in Establishing the Relevance of Filamentous Fungi in Cystic Fibrosis.
- COVID-19 and Pediatric Lung Disease: A South African Tertiary Center Experience.
- Differential effects on the miRNome of the treatment of human airway epithelial Calu-3 cells with peptide-nucleic acids (PNAs) targeting microRNAs miR-101-3p and miR-145-5p: Next generation sequencing datasets.
- Correction of the Gene Defect in Cystic Fibrosis: Is it too late for bone?
- Antibiotic Efficacy Testing in an Ex vivo Model of Pseudomonas aeruginosa and Staphylococcus aureus Biofilms in the Cystic Fibrosis Lung.
- Pulmonary Infection Secondary to Blastobotrys raffinosifermentans in a Cystic Fibrosis Patient: Review of the Literature.
- Telemedicine in Pediatrics: A Systematic Review of Randomized Controlled Trials.
- Extended-culture and culture-independent molecular analysis of the airway microbiota in cystic fibrosis following CFTR modulation with ivacaftor.
- First and second wave of SARS-CoV2 in Italian Cystic Fibrosis patients: Data from Italian Cystic Fibrosis Registry.
- Home-Care Bronchiectasis
- Airway Clearance for Pediatric and Adult Cystic Fibrosis Patients Using a Portable Intra-Pulmonary Percussion Device
- Prognosis-Based Early Intervention Strategies to Resolve Exacerbation and Progressive Lung Function Decline in Cystic Fibrosis.
- Formulation and In Vitro and In Silico Characterization of “Nano-in-Micro” Dry Powder Inhalers Containing Meloxicam.
- Urinary stone disease prevalence and associations in cystic fibrosis.
- Pseudomonas aeruginosa Resistance to Bacteriophages and Its Prevention by Strategic Therapeutic Cocktail Formulation.
- Complete Genome Sequence of Burkholderia gladioli Phage Maja.
- Determinants of lung disease progression measured by lung clearance index in children with cystic fibrosis.
- Eradication success for non-tuberculous mycobacteria in children with cystic fibrosis.
- Balkan Endemic Nephropathy: An Autopsy Case Report.
- The effects of the addition of a new airway clearance device to chest physiotherapy in children with cystic fibrosis pulmonary exacerbations.
- A Prospective Study of the Effects of Sex Hormones on Lung Function and Inflammation in Women with Cystic Fibrosis.
- Multidisciplinary approach to patients with manifestations and pulmonary complications of cystic fibrosis.
- Clinical Implications of Polymicrobial Synergism Effects on Antimicrobial Susceptibility.
- The Effects of Telerehabilitation on Muscle Function, Physical Activity and Sleep in Cystic Fibrosis During Pandemic
- Interspecies Metabolic Complementation in Cystic Fibrosis Pathogens via Purine Exchange.
- Considerations for Phage Therapy Against Mycobacterium abscessus.
- Gender-affirming hormone therapy in cystic fibrosis – A case of new Pseudomonas infection.
- Intestinal TMEM16A control luminal chloride secretion in a NHERF1 dependent manner.
- Improvement of Pulmonary Function in Cystic Fibrosis Patients following Endoscopic Sinus Surgery.
- Telemedicine Use and Health-related Concerns of Patients with Chronic Conditions During COVID-19: A Survey of Members of Online Health Communities.
- The Effects of Nebulizer Hygiene Training on the Practices of Cystic Fibrosis Patients and Caregivers.
- Development and in vitro characterization of a novel pMDI diclofenac formulation as an inhalable anti-inflammatory therapy for cystic fibrosis.
- A Peptide-Nucleic Acid Targeting miR-335-5p Enhances Expression of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Gene with the Possible Involvement of the CFTR Scaffolding Protein NHERF1.
- Reflections on the Importance of Cost of Illness Analysis in Rare Diseases: A Proposal.
- Primary care during the transition to adult care for adolescents involved with pediatric specialty services: a scoping review protocol.
- Challenges in the use of highly effective modulator treatment for cystic fibrosis.
- Mesenchymal Stem Cell exosome delivered Zinc Finger Protein activation of cystic fibrosis transmembrane conductance regulator.
- Histomorphology and Immunohistochemistry of a Congenital Nephromegaly Demonstrate Concurrent Features of Heritable and Acquired Cystic Nephropathies in a Girgentana Goat (Capra falconeri).
- Losartan reduces cigarette smoke-induced airway inflammation and mucus hypersecretion.
- Avatar acceptability: views from the Australian Cystic Fibrosis community on the use of personalised organoid technology to guide treatment decisions.
- Sex disparities in cystic fibrosis: review on the effect of female sex hormones on lung pathophysiology and outcomes.
- Longitudinal non-cystic fibrosis trends of pulmonary Mycobacterium abscessus disease from 2010 to 2017: spread of the “globally successful clone” in Asia.
- Large pH oscillations promote host defense against human airways infection.
- DNAJB12 and Hsp70 Triage Arrested Intermediates of N1303K-CFTR for ER Associated-Autophagy.
- Pilocarpine Microneedles for Sweat Induction (PMN-SI)
- Microbial Biomarkers of EArly Pseudomonas Aeruginosa Colonization in CHildren With Cystic Fibrosis
- Environmental Microbial Contamination during Cystic Fibrosis Group-Based Psychotherapy.
- JCF Year in Review.
- Sclerosing Polycystic Adenoma.
- Cystic Fibrosis.
- Patterns of azithromycin use in obstructive airway diseases: a real-world observational study.