Jane Davies

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Jane Davies is affiliated with the following schools: Imperial College London, University of Dundee

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Why Is Jane Davies Influential?

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According to Wikipedia, Jane C. Davies is a British physician who is Professor of Paediatric Respirology at Imperial College School of Medicine. She is an Honorary Consultant at the Royal Brompton and Harefield NHS Foundation Trust.

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Jane Davies's Published Works

Number of citations in a given year to any of this author's works

Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author

Published Works

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. (2011) (1829)
Disordered Microbial Communities in Asthmatic Airways (2010) (1446)
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. (2015) (1178)
Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation. (2013) (446)
The future of cystic fibrosis care: a global perspective. (2019) (418)
Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial (2015) (353)
Tezacaftor–Ivacaftor in Residual‐Function Heterozygotes with Cystic Fibrosis (2017) (349)
CpG-free plasmids confer reduced inflammation and sustained pulmonary gene expression (2008) (287)
Pseudomonas aeruginosa in cystic fibrosis: pathogenesis and persistence. (2002) (269)
Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study. (2016) (235)
Cystic fibrosis (2007) (226)
Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial. (2013) (216)
The Th17 pathway in cystic fibrosis lung disease. (2011) (207)
Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial. (2017) (206)
Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST). (2014) (193)
Impaired innate interferon induction in severe therapy resistant atopic asthmatic children (2012) (189)
VX‐659–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles (2018) (181)
Lung clearance index: evidence for use in clinical trials in cystic fibrosis. (2014) (157)
Increased airway smooth muscle mass in children with asthma, cystic fibrosis, and non-cystic fibrosis bronchiectasis. (2008) (156)
Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study. (2018) (155)
Airway remodelling in children with cystic fibrosis (2007) (144)
Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del‐CFTR or F508del/G551D‐CFTR (2018) (135)
Increased incidence and severity of the systemic inflammatory response syndrome in patients deficient in mannose-binding lectin (2004) (132)
Bugs, biofilms, and resistance in cystic fibrosis. (2009) (132)
Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation (2013) (131)
Pseudomonas aeruginosa, cyanide accumulation and lung function in CF and non-CF bronchiectasis patients (2008) (114)
Preparation for a first-in-man lentivirus trial in patients with cystic fibrosis (2016) (113)
Voriconazole therapy in children with cystic fibrosis. (2005) (104)
Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report. (2015) (103)
A Cell-Free Biosensor for Detecting Quorum Sensing Molecules in P. aeruginosa-Infected Respiratory Samples. (2017) (103)
CFTR biomarkers: time for promotion to surrogate end-point (2012) (97)
CFTR gene transfer reduces the binding of Pseudomonas aeruginosa to cystic fibrosis respiratory epithelium. (1996) (97)
Pre-clinical evaluation of three non-viral gene transfer agents for cystic fibrosis after aerosol delivery to the ovine lung (2011) (95)
Airway remodelling and its relationship to inflammation in cystic fibrosis (2010) (95)
Pseudomonas aeruginosa infection in cystic fibrosis: pathophysiological mechanisms and therapeutic approaches (2016) (87)
Diagnosis of Cystic Fibrosis in Screened Populations (2017) (87)
Emerging and unusual gram-negative infections in cystic fibrosis. (2007) (85)
Colistin kills bacteria by targeting lipopolysaccharide in the cytoplasmic membrane (2018) (83)
Increased nuclear suppressor of cytokine signaling 1 in asthmatic bronchial epithelium suppresses rhinovirus induction of innate interferons (2015) (83)
Lung clearance index and high-resolution computed tomography scores in primary ciliary dyskinesia. (2013) (82)
Cystic fibrosis (2021) (81)
Impaired pulmonary status in cystic fibrosis adults with two mutated MBL-2 alleles (2004) (77)
Current and future therapies for Pseudomonas aeruginosa infection in patients with cystic fibrosis (2017) (76)
An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5 years (KLIMB). (2019) (75)
Modifier genes in cystic fibrosis (2004) (75)
A Phase 3 Open-Label Study of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 through 11 Years of Age with Cystic Fibrosis and at Least One F508del Allele (2021) (73)
Antipseudomonal Bacteriophage Reduces Infective Burden and Inflammatory Response in Murine Lung (2015) (73)
High rhinovirus burden in lower airways of children with cystic fibrosis. (2013) (72)
A randomised, double-blind, placebo-controlled phase IIB clinical trial of repeated application of gene therapy in patients with cystic fibrosis (2013) (71)
Mannose-binding lectin is present in the infected airway: a possible pulmonary defence mechanism (2008) (69)
Assessment of F/HN-pseudotyped lentivirus as a clinically relevant vector for lung gene therapy. (2012) (69)
Lung clearance index in cystic fibrosis subjects treated for pulmonary exacerbations (2015) (66)
Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis (2011) (66)
Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence. (2001) (64)
Use of cough swabs in a cystic fibrosis clinic (2001) (60)
Differential binding of mannose-binding lectin to respiratory pathogens in cystic fibrosis (2000) (57)
Retrospective review of the effects of rhDNase in children with cystic fibrosis (1997) (56)
Novel Keto-phospholipids Are Generated by Monocytes and Macrophages, Detected in Cystic Fibrosis, and Activate Peroxisome Proliferator-activated Receptor-γ* (2012) (56)
Cystic fibrosis in the 21st century (2005) (55)
Differential global gene expression in cystic fibrosis nasal and bronchial epithelium. (2011) (53)
Biomarkers for cystic fibrosis lung disease: application of SELDI-TOF mass spectrometry to BAL fluid. (2008) (53)
Monitoring respiratory disease severity in cystic fibrosis. (2009) (49)
A Phase I/IIa Safety and Efficacy Study of Nebulized Liposome-mediated Gene Therapy for Cystic Fibrosis Supports a Multidose Trial. (2015) (46)
Quality, size, and composition of pediatric endobronchial biopsies in cystic fibrosis. (2007) (46)
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID). (2020) (46)
Bronchoscopy in Cystic Fibrosis Infants Diagnosed by Newborn Screening (2011) (46)
Bronchoscopy following diagnosis with cystic fibrosis (2006) (46)
Ivacaftor in Infants Aged 4 to <12 Months with Cystic Fibrosis and a Gating Mutation. Results of a Two-Part Phase 3 Clinical Trial (2020) (45)
Lung clearance index in CF: a sensitive marker of lung disease severity (2007) (45)
Reduction in the adherence of Pseudomonas aeruginosa to native cystic fibrosis epithelium with anti-asialoGM1 antibody and neuraminidase inhibition. (1999) (45)
Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications (2016) (43)
Genetic medicines for CF: Hype versus reality (2016) (41)
Basic science for the chest physician: Pseudomonas aeruginosa and the cystic fibrosis airway (2012) (41)
Gene therapy for the treatment of cystic fibrosis (2012) (40)
Recent advances in the management of cystic fibrosis (2014) (40)
Bone marrow stem cells do not repopulate the healthy upper respiratory tract (2002) (37)
Beta-defensin genomic copy number is not a modifier locus for cystic fibrosis (2005) (37)
Toxicology study assessing efficacy and safety of repeated administration of lipid/DNA complexes to mouse lung (2013) (36)
Potential difference measurements in the lower airway of children with and without cystic fibrosis. (2005) (35)
Nasal potential difference measurements in diagnosis of cystic fibrosis: an international survey. (2014) (34)
Integrating the multiple breath washout test into international multicentre trials. (2019) (34)
Inconclusive diagnosis after a positive newborn bloodspot screening result for cystic fibrosis; clarification of the harmonised international definition. (2019) (33)
Gene therapy in cystic fibrosis (2014) (33)
The safety profile of a cationic lipid-mediated cystic fibrosis gene transfer agent following repeated monthly aerosol administration to sheep. (2013) (33)
Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor. (2018) (32)
Variability of sweat chloride concentration in subjects with cystic fibrosis and G551D mutations. (2017) (30)
Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor. (2020) (29)
Exploring the mechanisms of macrolides in cystic fibrosis. (2006) (29)
The reproducibility and responsiveness of the lung clearance index in bronchiectasis (2015) (29)
The role of the collectin system in pulmonary defence. (2001) (27)
Theranostics by testing CFTR modulators in patient-derived materials: The current status and a proposal for subjects with rare CFTR mutations. (2019) (26)
Limitations of the murine nose in the development of nonviral airway gene transfer. (2010) (25)
Outdoor air pollution and cystic fibrosis. (2018) (25)
Cephalosporin nitric oxide-donor prodrug DEA-C3D disperses biofilms formed by clinical cystic fibrosis isolates of Pseudomonas aeruginosa (2019) (24)
Cyanide levels found in infected cystic fibrosis sputum inhibit airway ciliary function (2014) (23)
Cystic fibrosis gene therapy: a mutation-independent treatment (2016) (23)
Cystic fibrosis modifier genes. (2005) (23)
Pseudomonas aeruginosa in cystic fibrosis: pathogenesis and new treatments. (2011) (23)
A randomised, double-blind, placebo-controlled trial of repeated nebulisation of non-viral cystic fibrosis transmembrane conductance regulator ( CFTR) gene therapy in patients with cystic fibrosis (2016) (23)
Comparison of the upper and lower airway microbiota in children with chronic lung diseases (2018) (22)
Cystic fibrosis: therapies targeting specific gene defects. (2012) (21)
Airway gene therapy. (2005) (20)
Detection of antibodies to Pseudomonas aeruginosa in serum and oral fluid from patients with cystic fibrosis. (2007) (20)
Developments in multiple breath washout testing in children with cystic fibrosis (2017) (19)
Building global development strategies for cf therapeutics during a transitional cftr modulator era. (2020) (19)
GLPG1837, a CFTR potentiator, in p.Gly551Asp (G551D)-CF patients: An open-label, single-arm, phase 2a study (SAPHIRA1). (2019) (19)
P95 Assessment of F/HN-pseudotyped Lentivirus as a Clinically Relevant Vector For Lung Gene Therapy (2012) (19)
The Immunomodulatory Drug Glatiramer Acetate is Also an Effective Antimicrobial Agent that Kills Gram-negative Bacteria (2017) (19)
Time required to obtain endobronchial biopsies in children during fiberoptic bronchoscopy (2009) (18)
The future of CFTR modulating therapies for cystic fibrosis (2015) (18)
Metabolic Phenotyping and Strain Characterisation of Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients Using Rapid Evaporative Ionisation Mass Spectrometry (2018) (18)
Rebuttal: You are wrong, Dr. Mallory … (2006) (18)
Longitudinal development of the airway microbiota in infants with cystic fibrosis (2019) (18)
Gene and cell therapy for cystic fibrosis. (2006) (17)
Simultaneous sulfur hexafluoride and nitrogen multiple-breath washout (MBW) to examine inherent differences in MBW outcomes (2019) (17)
Preparation for a first-inman lentivirus trial in patients with cystic fibrosis (2016) (17)
Entering the era of highly effective modulator therapies (2021) (16)
New anti-pseudomonal agents for cystic fibrosis- still needed in the era of small molecule CFTR modulators? (2018) (16)
Gene therapy for cystic fibrosis. (2010) (16)
Speeding up access to new drugs for CF: Considerations for clinical trial design and delivery. (2019) (16)
Nasal abnormalities in cystic fibrosis mice independent of infection and inflammation. (2008) (16)
Telemedicine and cystic fibrosis: Do we still need face-to-face clinics? (2021) (15)
Where are we with transformational therapies for patients with cystic fibrosis? (2017) (15)
Tezacaftor – Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe 508 del Alleles (2020) (14)
How to use: bacterial cultures in diagnosing lower respiratory tract infections in cystic fibrosis (2013) (14)
Ivacaftor in People with Cystic Fibrosis and a 3849+10kb C→T or D1152H Residual Function Mutation (2020) (14)
Children with cystic fibrosis demonstrate no respiratory immunological, infective or physiological, consequences of vitamin D deficiency. (2018) (14)
A molecular comparison of microbial communities in bronchiectasis and cystic fibrosis (2013) (14)
Prospects for gene therapy in lung disease. (2001) (14)
The future in paediatric respirology (2010) (14)
Monitoring early stage lung disease in cystic fibrosis (2020) (14)
Cystic Fibrosis Lung Disease Modifiers and Their Relevance in the New Era of Precision Medicine (2021) (14)
Model Systems to Study the Chronic, Polymicrobial Infections in Cystic Fibrosis: Current Approaches and Exploring Future Directions (2021) (13)
WS6.4 Long-term safety and efficacy of ivacaftor in subjects with cystic fibrosis who have the G551D-CFTR Mutation (2012) (13)
Non! to non-steroidal anti-inflammatory therapy for inflammatory lung disease in cystic fibrosis (at least at the moment). (2007) (13)
Inhaled dry powder alginate oligosaccharide in cystic fibrosis: a randomised, double-blind, placebo-controlled, crossover phase 2b study (2020) (13)
Cystic fibrosis drug trial design in the era of CFTR modulators associated with substantial clinical benefit: stakeholders' consensus view. (2020) (13)
Increased airway smooth muscle mass in children with asthma, cystic fibrosis and bronchiectasis (2008) (13)
Quantification of periciliary fluid height in human airway biopsies is feasible, but not suitable as a biomarker. (2011) (13)
WS6.5 Ivacaftor in subjects 6 to 11 years of age with cystic fibrosis and the G551D-CFTR mutation (2012) (13)
Does mass spectrometric breath analysis detect Pseudomonas aeruginosa in cystic fibrosis? (2016) (12)
Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis (2021) (12)
Disease-modifying drug therapy in cystic fibrosis. (2017) (12)
WS7.6 Effect of ivacaftor on lung function in subjects with CF who have the G551D-CFTR mutation and mild lung disease: a comparison of lung clearance index (LCI) vs. spirometry (2012) (12)
Response of Pseudomonas aeruginosa to the Innate Immune System-Derived Oxidants Hypochlorous Acid and Hypothiocyanous Acid (2020) (12)
Ion transport in lung disease (2004) (12)
Biomarkers for cystic fibrosis: are we progressing? (2007) (12)
Abnormal pro-gly-pro pathway and airway neutrophilia in pediatric cystic fibrosis (2020) (11)
Insights into the variability of nasal potential difference, a biomarker of CFTR activity. (2019) (11)
Multiple breath washouts in children can be shortened without compromising quality (2015) (10)
Gene therapy for cystic fibrosis (2001) (10)
UK cystic fibrosis gene therapy consortium tracking study: Exhaled breath condensate pH increases with treatment of an infective exacerbation (2007) (10)
Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study. (2021) (9)
Early detection of lung disease in preschool children with cystic fibrosis (2005) (9)
Self-reactive CFTR T cells in humans: implications for gene therapy. (2013) (9)
Pulmonary Gene Therapy (2003) (9)
The journey of a thousand miles (2011) (9)
WS01.5 An open-label study of the safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2 to 5 years with cystic fibrosis and a CFTR gating mutation: The KIWI study (2015) (9)
The coronavirus pandemic has forced rapid changes in care protocols for cystic fibrosis (2020) (9)
Severe Small Airways Disease Resistant to Medical Treatment in a Child with Cystic Fibrosis (1996) (9)
WS01.6 Preliminary safety and efficacy of triple combination CFTR modulator regimens in cystic fibrosis (2018) (9)
Whole Gene Sequencing of CFTR Reveals a High Prevalence of the Intronic Variant c.3874-4522A>G in Cystic Fibrosis. (2020) (9)
Impact of cross-sensitivity error correction on representative nitrogen-based multiple breath washout data from clinical trials. (2021) (9)
An immunocytochemical assay to detect human CFTR expression following gene transfer. (2009) (9)
Research applications of bronchoscopy. (2003) (8)
Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial (2020) (8)
The feasibility of home monitoring of young people with cystic fibrosis: Results from CLIMB-CF. (2021) (8)
New therapeutic approaches for cystic fibrosis lung disease. (2002) (8)
Lung Clearance Index As An Endpoint In A Multicenter Randomized Control Trial Of Ivacaftor In Subjects With Cystic Fibrosis Who Have Mild Lung Disease (2012) (7)
Lung clearance index in primary ciliary dyskinesia and bronchiectasis. (2014) (7)
Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study. (2021) (7)
Genotype-specific small-molecule therapy for cystic fibrosis (2013) (7)
Current and Novel Antimicrobial Approaches (2005) (7)
Who and why; sharing our experiences of developing a standard operating procedure (SOP) to allocate screening slots for highly competitive cystic fibrosis trials. (2019) (7)
Cystic fibrosis: to ion transport and beyond (2010) (6)
Targeted exhaled breath analysis for detection of Pseudomonas aeruginosa in cystic fibrosis patients. (2021) (6)
SAFETY AND EXPRESSION OF A SINGLE DOSE OF LIPID-MEDIATED CFTR GENE THERAPY TO THE UPPER AND LOWER AIRWAYS OF PATIENTS WITH CF (2011) (6)
New drug developments in the management of cystic fibrosis lung disease (2016) (6)
Impact of T2R38 Receptor Polymorphisms on Pseudomonas aeruginosa Infection in Cystic Fibrosis (2018) (6)
Utility of lung clearance index in CF: What we know, what we don't know and musings on how to bridge the gap. (2020) (6)
An Update on CFTR Modulators as New Therapies for Cystic Fibrosis (2022) (6)
309 Piloting the use of the cystic fibrosis questionnaire (CFQ) in CF patients changing to dry powder inhaled colistimethate (2007) (6)
Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3b, Randomized, Placebo-controlled Study (2022) (6)
Pseudomonas aeruginosa induces p38MAP kinase-dependent IL-6 and CXCL8 release from bronchial epithelial cells via a Syk kinase pathway (2021) (6)
WS13.4 Efficacy and safety of lumacaftor/ivacaftor (LUM/IVA) in patients (pts) aged 6–11 years (yrs) with cystic fibrosis (CF) homozygous for F508del-CFTR: a randomized placebo (PBO)-controlled phase 3 trial (2017) (5)
Current strategies for the long-term assessment, monitoring, and management of cystic fibrosis patients treated with CFTR modulator therapy. (2017) (5)
58 Pulmonary exacerbations in CF patients with the G551D-CFTR mutation treated with ivacaftor (2013) (5)
Targeted exhaled breath analysis for detection of Pseudomonas aeruginosa in cystic fibrosis patients (2020) (5)
Combination antifungal therapy for Scedosporium species in cystic fibrosis (2020) (5)
Age-specific effect of the cystic fibrosis modifier gene, MBL-2 (2002) (5)
P028 Riociguat for the treatment of adult Phe508del homozygous cystic fibrosis: efficacy data from the Phase II Rio-CF study (2018) (5)
Cystic fibrosis: bridging the treatment gap in early childhood. (2013) (5)
134 Training and qualifying international teams in standardised procedures: steps on the learning curve from the CTN LCI Core Facility (2016) (5)
Measurement of tobramycin and gentamicin in saliva is not suitable for therapeutic drug monitoring of patients with cystic fibrosis. (2005) (5)
Endobronchial biopsy in childhood. (2008) (5)
Cystic fibrosis presenting as acute upper airway obstruction (2005) (5)
Pseudomonas aeruginosa in the Cystic Fibrosis Lung. (2022) (5)
Clinical trial research in focus: ensuring new cystic fibrosis drugs fulfil their potential. (2017) (5)
New tests for cystic fibrosis. (2006) (5)
Pooling of bronchoalveolar lavage in children with cystic fibrosis does not adversely affect the microbiological yield or sensitivity in detecting pulmonary inflammation. (2017) (5)
Clinical pharmacokinetics and dose recommendations for posaconazole gastroresistant tablets in children with cystic fibrosis (2021) (5)
Variability in Bacteriophage and Antibiotic Sensitivity in Serial Pseudomonas aeruginosa Isolates from Cystic Fibrosis Airway Cultures over 12 Months (2021) (5)
Colistin kills bacteria by targeting lipopolysaccharide in the cytoplasmic membrane. (2021) (4)
Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis. (2021) (4)
EVALUATION OF SAFETY AND GENE EXPRESSION WITH A SINGLE DOSE OF PGM169/GL67A ADMINISTERED TO THE NOSE AND LUNG OF INDIVIDUALS WITH CF: THE UK CF GENE THERAPY CONSORTIUM "PILOT STUDY" (2009) (4)
Potentiators and Correctors in Paediatric Cystic Fibrosis Patients: A Narrative Review (2018) (4)
S93 Effect of lumacaftor/ivacaftor on total, bronchiectasis, and air trapping computed tomography (ct) scores in children homozygous for f508del-cftr: exploratory imaging substudy (2017) (4)
S48 Mucoidy and the microbiome: community composition in relation to the presence of culturable, mucoid Pseudomonas aeruginosa (2011) (4)
Gene therapy for respiratory diseases; progress and a changing context. (2020) (4)
Multiple breath washout in bronchiectasis clinical trials: is it feasible? (2020) (4)
WS13.1 Ataluren significantly reduces exacerbations in nonsense mutation cystic fibrosis patients not receiving tobramycin (2016) (4)
Horses for courses: Learning from functional tests of pulmonary health? (2020) (4)
WS13.6 GLPG1837 in subjects with cystic fibrosis (CF) and the G551D mutation: results from a phase II study (SAPHIRA1) (2017) (4)
WS13.1.1 Ataluren in nonsense mutation cystic fibrosis patients not receiving tobramycin: significant lung function benefits in the paediatric age range (2016) (4)
Training dogs to differentiate Pseudomonas aeruginosa from other cystic fibrosis bacterial pathogens: not to be sniffed at? (2019) (4)
"Fortunate are those who take the first steps"? The psychosocial impact of novel drug development. (2019) (3)
Evaluation of a multiple breath nitrogen washout system in children (2020) (3)
EPS1.3 Safety, tolerability and early signs of efficacy with riociguat for the treatment of adult Phe508del homozygous cystic fibrosis patients: study design and rationale for the Rio-CF study (2017) (3)
Design of gene therapy trials in CF patients. (2011) (3)
The clinical use of rhDNAse (1997) (3)
Primum non nocere: does the current research publication system (or the lay press) harm our patients? (2005) (3)
Cystic fibrosis in 2016: considerable progress, but much more to do. (2016) (3)
Effect of ivacaftor on lung clearance index and FEV1 in subjects with CF who have the G551D-CFTR mutation and mild lung disease (2012) (3)
WS16.5 Selective ion-flow mass spectrometry (SIFT-MS) analysis of exhaled breath as a non-invasive determinant of Pseudomonas aeruginosa infection in CF patients (2014) (3)
Guiding the rational design of patient-centred drug trials in Cystic Fibrosis: A Delphi study. (2021) (3)
EPS4.6 Inherent differences in multiple breath washout (MBW) using N2 and SF6 demonstrated by simultaneous analysis with respiratory mass spectrometry (RMS) (2017) (3)
Long-term safety and efficacy of ivacaftor in subjects with CF who have the G551D-CFTR mutation (2012) (3)
WS06-4 Ivacaftor (IVA) treatment in patients 6 to <12 months old with cystic fibrosis with a CFTR gating mutation: results of a 2-part, single-arm, phase 3 study (2019) (3)
Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis. (2022) (3)
Efficacy and safety of inhaled ENaC inhibitor BI 1265162 in patients with cystic fibrosis: BALANCE-CF 1, a randomised, phase II study (2021) (2)
Response to Mallory: You Are Civilized, but Still Wrong, Dr. Mallory (2007) (2)
S75 The T2R38 bitter taste receptor as a modifier of host response to pseudomonas aeruginosa in cystic fibrosis: does T2R38 genotype impact on clinical infection? (2016) (2)
Frequent detection of rhinovirus in bronchoalveolar lavage samples from children with cystic fibrosis (2011) (2)
56 Effect of withdrawal of ivacaftor therapy on CFTR channel activity and lung function in patients with cystic fibrosis (2013) (2)
P209 Standardisation Of Lung Clearance Index In A Multicentre Clinical Trial (2014) (2)
UKCF gene therapy consortium tracking study: Lung clearance index improves with treatment of an infective exacerbation (2007) (2)
Discrete choice experiment (DCE) to quantify the influence of trial features on the decision to participate in cystic fibrosis (CF) clinical trials (2021) (2)
A PHASE 2B DOUBLE-BLIND, PLACEBO-CONTROLLED TRIAL OF NON-VIRAL MEDIATED GENE THERAPY FOR CF (2013) (2)
Abstracts from the 23rd Italian congress of Cystic Fibrosis and the 13th National congress of Cystic Fibrosis Italian Society (2018) (2)
24 Comparison of the tolerability of posaconazole versus voriconazole in children with cystic fibrosis (2018) (2)
P96 Repeat Administration of GL67A/pGM169 is Feasible, Safe, and Produces Endogenous Levels of CFTR Expression After 12 Doses (2012) (2)
S112 Variability in susceptibility to antibiotics and bacteriophages between individual colonies of Pseudomonas aeruginosa from cystic fibrosis sputum samples: implications for future clinical trial design (2015) (2)
Nasal cavity inflammation in patients with primary ciliary dyskinesia (PCD) is associated with bacterial infection (2017) (2)
WS02-1 Investigating outcome measures for physiotherapy trials of airway clearance in adult patients with cystic fibrosis (2019) (2)
Visualising early lung disease in CF: the emergence of MRI (2017) (2)
WS21.9 Lung clearance index as a clinical trial outcome measure: Establishing the CTN LCI core facility (2015) (2)
Interleukin-17 and Cystic Fibrosis Lung Disease (2012) (2)
Large scale clinical trials: lessons from the COVID-19 pandemic (2022) (2)
Optimising equity of access: how should we allocate slots to the most competitive trials in Cystic Fibrosis (CF)? (2021) (2)
Authors' response (2011) (2)
S18 Longitudinal assessment of biomarkers for clinical trials of novel therapeutic agents: the run-in study (2010) (2)
P361 What influences participation in clinical trials by people with cystic fibrosis? A national delphi study (2020) (2)
The cystic fibrosis transmembrane conductance regulator: state of the art (2008) (2)
Chronic infection by controlling inflammation (2019) (2)
Inflammation in children with neuromuscular disorders and sleep disordered breathing. (2020) (2)
The effect of CFTR modulation on the disease progression of cystic fibrosis in the era of precision medicine (2016) (2)
565: Significant reduction in abdominal symptoms assessed with CFAbd score over 4 weeks of treatment with elexacaftor/tezacaftor/ivacaftor—First results from the RECOVER study (2021) (2)
LUNG CLEARANCE INDEX AND STRUCTURAL LUNG DISEASE IN CYSTIC FIBROSIS: A COMPARISON WITH PRIMARY CILIARY DYSKINESIA (2010) (2)
P243 Outcome measures for airway clearance in adults with cystic fibrosis (CF): a randomised controlled crossover trial (2019) (1)
LARGE-SCALE PRODUCTION OF LENTIVIRAL VECTORS FOR CF LUNG GENE THERAPY (2014) (1)
Comparison of functional residual capacity (FRC) from two multiple breath washout (MBW) systems and body plethysmography (2016) (1)
Update on Clinical Outcomes of Highly Effective Modulator Therapy. (2022) (1)
Disease-modifying drug therapy in CF (2017) (1)
Cumulative CFTR expression following repeated aerosol delivery of non-viral pGM169/GL67A formulation to mouse lung (2012) (1)
Cystic fibrosis and survival to 40 years: a study of CFTR function (2010) (1)
Transepithelial nasal potential difference in patients with, and at risk of acute respiratory distress syndrome (2021) (1)
46: A discrete choice experiment (DCE) to quantify the influence of trial features on the decision to participate in CF trials (2021) (1)
Selective Sampling of the Lower Airway in Children with Cystic Fibrosis: What Are We Missing? (2019) (1)
New concepts in antimicrobial resistance in cystic fibrosis respiratory infections. (2022) (1)
INFECTION IN PATIENTS WITH CYSTIC FIBROSIS AND CONGENITAL IMMUNE DEFICIENCIES (1997) (1)
Early-phase clinical trials in a pandemic: learning from the response to COVID-19 (2022) (1)
ePS5.07 CLInical Monitoring and Biomarkers to stratify severity and predict outcomes in children with cystic fibrosis (CLIMB-CF): results from the feasibility study (2019) (1)
Ivacaftor improved lung function in cystic fibrosis with G551d mutation (2012) (1)
Modifier genes in cystic fibrosis (2004) (1)
Comparison of the airway microbiota in children with chronic suppurative lung disease (2021) (1)
Simultaneous SF6and N2gas multiple breath washout (MBW); understanding the difference between test gases (2016) (1)
Gene therapy for cystic fibrosis: successes and challenges (2006) (1)
Preparation for a fi rst-inman lentivirus trial in patients with cystic fi brosis (2016) (1)
P130 Pseudomonas aeruginosa impairs growth of aspergillus from CF airway samples (2021) (1)
THINK TANK REVIEW (2010) (1)
Migration is not the perfect answer: How the cross‐talk error correction for multiple breath nitrogen washout (MBWN2) parameters differs on directly collected vs. legacy data (2022) (1)
S120 Update on the UK CF Gene Therapy Consortium Multidose, Non-Viral, Gene Therapy Trial (2012) (1)
Clinimetric analysis of outcome measures for airway clearance in people with cystic fibrosis: a systematic review (2021) (1)
A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: A pilot study. (2021) (1)
Fair selection of participants in clinical trials: The challenge to push the envelope further. (2019) (1)
WS17.5 Nasal potential difference measurement increases the diagnostic yield in patients with equivocal first-line cystic fibrosis investigations: the experience of a large national CFTR diagnostic service (2018) (1)
The life rafts sailed; Now let's take stock and set the course ahead (Commentary) (2021) (1)
Ataluren in nonsense mutation cystic fibrosis patients not receiving chronic inhaled tobramycin: Evaluation of exacerbations and lung function (2016) (1)
Predicting the Future of Cystic Fibrosis Lung Disease: Gene Expression Holds Some of the Answers. (2018) (1)
M5 The multiple sclerosis drug, glatiramer acetate, acts as a resistance breaker with antibiotics from different classes against cystic fibrosis strains of pseudomonas aeruginosa (2019) (1)
An invisible threat? Aspergillus positive cultures and co-infecting bacteria in airway samples. (2022) (1)
534. Preparation for a First-in-Man Lentivirus Trial in Cystic Fibrosis Patients (2016) (1)
WS20.2 Intra-patient variability of sweat chloride concentrations in patients with cystic fibrosis (CF) (2015) (1)
Cystic Fibrosis Foundation Therapeutics, Inc. Therapeutics Development Network Center for Sweat Analysis Medical Director: F. Accurso, M.D. (720 777 2522) Center for Sweat Analysis Publications (2012) (1)
Clinical Development of an Optimal F/HN Pseudotyped SIV Vector for Cystic Fibrosis Lung Gene Therapy (2014) (1)
WS07.4 Combined killing effects of lytic bacteriophages and antibiotics in biofilm-grown Pseudomonas aeruginosa from CF airway cultures (2017) (1)
93 Determining the impact of the T2R38 bitter taste receptor on P. aeruginosa infection in the cystic fibrosis airway (2017) (1)
Revisiting a diagnosis of cystic fibrosis – uncertainties and considerations (2021) (1)
564: Impact of elexacaftor/tezacaftor/ivacaftor treatment on clinical outcomes in people with CF in a real-world setting—The RECOVER trial (2021) (1)
WS12-4 Long-term safety and efficacy of lumacaftor/ivacaftor therapy in patients aged 6–11 years with cystic fibrosis homozygous for the F508del-CFTR mutation (F/F) (2019) (1)
MOVING LENTIVIRAL-BASED GENE THERAPY INTO A FIRST-IN-MAN CF TRIAL (2015) (1)
P125 The multiple sclerosis drug, glatiramer acetate, acts as a resistance breaker with antibiotics from different classes against cystic fibrosis strains of Pseudomonas aeruginosa (2019) (1)
Progress in Respiratory Gene Therapy. (2022) (1)
Trials and tribulations: The highs and lows of running cystic fibrosis drug studies. (2019) (1)
S74 Increased respiratory syncytial virus burden leads to more rapid cell death in PHE508DEL bronchial epithelial cells (2016) (1)
Molecular Therapies for Cystic Fibrosis (2019) (1)
S17 Ivacaftor treatment in patients 6 to <12 months old with cystic fibrosis with a CFTR gating mutation: results of a 2-part, single-arm, phase 3 study (2019) (1)
Recent advances in the management of cystic fi brosis (2014) (1)
Endobronchial Biopsy in Childhood. Authors' reply (2008) (1)
WS15.1 Further comparison of lung clearance index (LCI) generated with SF6 and N2-based multiple breath washout (MBW) techniques: differences increase with disease severity and are predominantly related to the prolonged final stage of the N2 washout (2016) (1)
Going the Extra Mile: Why Clinical Research in Cystic Fibrosis Must Include Children (2022) (1)
Comprar Paediatric Respiratory Disease. Parenchymal Diseases. Atlas Of Investigation And Management | A. Bush | 9781846920868 | Clinical Pub. (2011) (1)
Highlights of the North American Cystic Fibrosis Conference 2011 (2012) (1)
104 Clinical impact of Staphylococcus aureus co-infection in sputum-producing cystic fibrosis patients chronically infected with Pseudomonas aeruginosa (2017) (1)
251 Evaluation of the Medication Electronic Monitoring Systems n adherence measurement in a real-world setting (2022) (0)
Development of an immunocytochemical assay to detect human CFTR expression following gene therapy (2008) (0)
224 Is paediatric home monitoring in CF feasible? There's an app for that! (2017) (0)
Pre-existing immunity to human parainfluenza virus (hPIV) does not affect rSIV.F/HN-mediated transduction efficiency. (2016) (0)
Comparison of CF and non CF FRC and LCI values measured with Exhalyzer D and InnocorTMdevices (2015) (0)
WS19.02 Impact of corrected Multiple Breath nitrogenWashout (MBW) software on assessment of under/unventilated lung units (UVLU) with the MBWShX (2022) (0)
Results of a Phase IIb non-viral gene therapy trial from the UK CF Gene Therapy Consortium (2014) (0)
VALIDATION OF THE NDD EASYONE PRO LAB MULTIPLE BREATH NITROGEN WASHOUT SYSTEM IN CHILDREN (2018) (0)
WS17-4 The addition of sinus imaging to a quantitative cystic fibrosis lung MRI protocol demonstrates an association between sinus signal characteristics and lung disease severity (2019) (0)
13 Genotype, disease severity, and healthcare resource use by patients with CF in the UK National Health Service (2013) (0)
ePS04.3 Comparison of CF and non-CF LCI results using the Exhalyzer D and Innocor™ devices (2015) (0)
S63 Ivacaftor in 4- to <6-month-old infants with cystic fibrosis and a gating mutation: results of a 2-part, single-arm, Phase 3 study (2021) (0)
Performance of a Lytic Bacteriophage Cocktail against Pseudomonas aeruginosa in Conditions That Simulate the Cystic Fibrosis Lung Environment (2018) (0)
Tracheal structure abnormalities in Cftr − / − knockout mice (2007) (0)
S56 Moving lentiviral-based gene therapy into a first-in-man CF trial (2015) (0)
Gene therapy in cysticfibrosis (2015) (0)
Comparison Of The Microbiomes Of Two Chronic Airway Diseases Using High-Throughput Sequencing (2011) (0)
Clinical Trials of Novel Treatments for Cystic Fibrosis. (2016) (0)
PRODUCTION OF SIV.F/HN: A NEW LENTIVIRUS VECTOR FOR CF GENE THERAPY (2015) (0)
P042 RECOVER - the Real World Clinical Outcomes with Novel Modulator therapy combinations in people with cystic fibrosis (2021) (0)
S94 Development of assays to assess safety and efficacy of lentiviral gene therapy for cystic fibrosis (2017) (0)
Basic science: What will it deliver? Non-gene therapy treatments (2015) (0)
WS01.1 A phase 3, 2-part, single-arm study of ivacaftor treatment in patients <2 years with a CFTR gating mutation: results from the ARRIVAL study in patients 1 to 2 years (2018) (0)
WS17.4 Anti-pseudomonal bacteriophage cocktail reduces inflammatory responses in the murine lung (2013) (0)
Toxicology studies in support of the UK CF Gene Therapy Consortium's Multi-dose Clinical Trial (2011) (0)
WS07.7 Response of Pseudomonas aeruginosa to hypochlorous acid (HOCl) stress and the role of defence mechanisms in protecting against neutrophil killing (2017) (0)
S123 The Role of Nasal Potential Difference Testing in Difficult Cases of Possible Cystic Fibrosis (2012) (0)
ePS6.10 Glatiramer acetate improves the killing ability of tobramycin in Pseudomonas aeruginosa cultured from cystic fibrosis clinical samples (2020) (0)
Metabolomic characterization of Pseudomonas aeruginosa isolates from cystic fibrosis patients using rapid evaporative ionisation mass spectrometry (2016) (0)
P137 Further exploring phenotype and virulence differences between chronic Pseudomonas aeruginosa isolates from patients with cystic fibrosis and non-cystic fibrosis bronchiectasis (Bx) (2020) (0)
M4 Pseudomonas aeruginosa inhibits aspergillus fumigatus in vitro through multiple mechanisms, including pyoverdine production (2019) (0)
Distinct pattern of inflammation in bronchoalveolar lavage and bronchial mucosa of children with cystic fibrosis Online Repository (2011) (0)
Transepithelial ion transport: the relationship with long-term survivors of cystic fibrosis (2008) (0)
Metabolic Phenotyping and Strain Characterisation of Pseudomonas aeruginosa Isolates from Cystic Fibrosis Patients Using Rapid Evaporative Ionisation Mass Spectrometry (2018) (0)
P128 The use of thoracic CT to determine bone mineral density in adults and children with cystic fibrosis (2021) (0)
S5 Moraxella catarrhalis attaches to the ciliary tip causing ciliary dyskinesia (2018) (0)
73 Detection of antibodies to Pseudomonas aeruginosa in oral fluid from patients with Cystic Fibrosis (2006) (0)
Is there a secondary defect in CFTR in the nasal epithelium of patients with primary ciliary dyskinesia (2016) (0)
P220 Pseudomonas aeruginosa induces inflammation in bronchial epithelial cells via the p38 MAP and Syk tyrosine kinase pathways (2019) (0)
Development of F/HN pseudotyped Lentivirus for airway gene transfer (2014) (0)
42* Validating assays on invasive airway samples as end-points for gene therapy trials. (2007) (0)
P409 A quality improvement project to optimise multidisciplinary team communication about unplanned admissions of clinical trial patients (2019) (0)
New insights into disease progression for the CFTR modulator-treated cystic fibrosis patient (2016) (0)
Mechanisms of airway injury in COPD CIGARETTE SMOKE PROMOTES EXAGGERATED INFLAMMATORY RESPONSE IN THE PRESENCE OF Z-AT (2012) (0)
P231 Home respiratory rate monitoring using pulse oximetry pleth and smartphone app in children with cystic fibrosis (2019) (0)
WS07-1 The applicability of published Lung Clearance Index (LCI) normative values to a local healthy reference population (2019) (0)
WS07.2 Emergent biopsychosocial challenges of novel drug development and clinical trial participation: stopping an experimental modulator drug in order to conceive (2018) (0)
Curvilinearity provides additional information to lung clearance index only in a minority of children with early cystic fibrosis lung disease (2022) (0)
WS06.5 A methodology to evaluate trapped gas using an open circuit Multiple Breath Nitrogen Washout (MBWN2) device (2018) (0)
Lung Clearance Index, in Children and Adults with Cystic Fibrosis, Is More Sensitive to Progressive Airways Disease Than Standard Spirometry. (2009) (0)
S06.5 Adding a Slow Vital Capacity (SVC) manoeuvre to the methodology of Multiple Breath Nitrogen Washout (MBWN2) can enhance the sensitivity of the Lung Clearance Index (LCI) (2020) (0)
Exploiting Single-Cell-PCR Technology To Determine the Percentage of Transfected Cells in the Respiratory Epithelium (2009) (0)
WS11.4 The repurposed multiple sclerosis drug, glatiramer acetate, is an antibiotic resistance breaker in Pseudomonas aeruginosa strains from cystic fibrosis patients (2018) (0)
P163 Pseudomonas aeruginosa inhibits Aspergillus fumigatus in vitro through multiple mechanisms, including pyoverdine production (2019) (0)
569: Agreement of spirometry-controlled computed tomography scans with multiple breath washout with short extension (2021) (0)
S69 Development of an in vitro assay to detect chemically-induced changes in ciliary beat frequency (2015) (0)
Selection of Relevant Bacterial Strains for Novel Therapeutic Testing: a Guidance Document for Priority Cystic Fibrosis Lung Pathogens (2022) (0)
Differential binding of mannose-binding lectin to cystic fibrosis respiratory pathogens (2000) (0)
520: The antimicrobial peptide glatiramer acetate disrupts pseudomonal cell membranes through interaction with lipopolysaccharide (2021) (0)
ePS6.01 Targeted analysis of volatile organic compounds for detection of Pseudomonas aeruginosa in cystic fibrosis patients by exhaled breath analysis (2020) (0)
Patient information sheets (2016) (0)
Inflammation in Children with Neuromuscular Disorders and Sleep Disordered Breathing (2020) (0)
Assessment of immune responses to single and repeat dose aeroslisation of the non-viral formualtion PGM169/GL67A in cystic fibrosis patients (2014) (0)
P034 MRI as the new gold standard in the assessment of cystic fibrosis lung disease severity? A bespoke cystic fibrosis-MRI protocol combining quantitative ventilation and structural MRI measures to replace CT (2019) (0)
ePS6.06 Variability in response of chronic Pseudomonas aeruginosa isolates to a lytic bacteriophage mix and standard antibiotics over a year (2019) (0)
ePS5.09 CLInical Monitoring and Biomarkers to stratify severity and predict outcomes in children with cystic fibrosis (CLIMB-CF): usability results from our feasibility study (2020) (0)
M2 Pseudomonas aeruginosa (Pa) biofilm-forming potential and metabolomic phenotypes differ between chronically infected patients with cystic fibrosis (CF) and non-CF bronchiectasis (Bx) (2019) (0)
320. Selection of Lentiviral Vectors for Gene Transfer to Porcine Airways (2009) (0)
What is the Role of Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Primary Sclerosing Cholangitis (2007) (0)
320. Selection of Lentiviral Vectors for Gene Transfer to Porcine Airways (2009) (0)
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. (2022) (0)
Biomarkers for cystic fibrosis lung disease (2016) (0)
Dose preparation sheet (2016) (0)
P048 “Dr Dog” will see you now? First steps in assessing the utility of trained sniffer dogs to detect Pseudomonas aeruginosa (Pa) airway infection in non-expectorating CF patients (2018) (0)
P204 Immune Responses To Single And Repeated Administration Of Pgm169/gl67a: The Uk Cf Gene Therapy Consortium Clinical Trials (2014) (0)
fibrosisfollowing diagnosis with cystic (2009) (0)
SERUM BRAIN NATRIURETIC FACTOR (BNP) DOES NOT PREDICT RIGHT VENTRICULAR DYSFUNCTION IN CF ADULTS AND CHILDREN (2011) (0)
196 The impact of pH on antibiotic susceptibility of clinical Pseudomonas aeruginosa strains (2017) (0)
EPS3.10 Clinical pharmacokinetics and dose recommendations for posaconazole gastro-resistant tablets in children with cystic fibrosis (2021) (0)
Novel antipseudomonal treatment approaches. (2006) (0)
Rebuttal From Dr Thursfield et al. (2022) (0)
Multiple breath washout in bronchiectasis clinical trials (2020) (0)
WS04.4 Abnormal LTA4H-PGP pathway in early life: a novel mechanism underlying persistent neutrophilic inflammation in the CF airway (2016) (0)
225 The influence of operator professional background on the quality of multiple breath washout (MBW) measurements (2017) (0)
WS01.5 Characterisation of the response to the innate immune oxidant hypochlorous acid in Pseudomonas aeruginosa cystic fibrosis isolates (2021) (0)
Home monitoring of respiratory rate from pulse oximetry in children with cystic fibrosis (2018) (0)
EPS3.04 Depolarisation of the Pseudomonas aeruginosa cytoplasmic membrane by Glatiramer acetate potentiates the antibacterial activity of tobramycin (2021) (0)
P035 Differential gene expression by Pseudomonas aeruginosa in response to bacteriophage infection (2018) (0)
Update on the UK CF Gene Therapy Consortium Multidoes, non-viral, gene therapy trial (2012) (0)
UKCF gene therapy consortium tracking study: Sputum and serum calprotectin are useful novel measurements in CF exacerbation (2007) (0)
JCF Year in Review (2021) (0)
Optimising Harvest of Bronchial Brush Biopsy Samples To Maximise Cell and RNA Yield in Gene Therapy Studies (2009) (0)
P187 Measuring the effect of airway clearance in adults with cystic fibrosis – a systematic review (2021) (0)
Pooling of Bronchoalveolar Lavage in Children with Cystic Fibrosis Does Not Affect the Microbiological Yield and May Allow Earlier Detection of Pulmonary Inflammation (2016) (0)
The Immunomodulatory Drug Glatiramer Acetate is Also an Effective Antimicrobial Agent that Kills Gram-negative Bacteria (2017) (0)
90 Using cell-free biosensors to monitor Pseudomonas aeruginosa infection in cystic fibrosis sputum (2017) (0)
WS06.02 Impact of one year of treatment with elexacaftor/tezacaftor/ivacaftor on clinical outcomes in people with cystic fibrosis in a real-world setting – the RECOVER study (2022) (0)
P066 Disease progression and burden in patients with cystic fibrosis homozygous for F508del across Europe in an observational registry (VOICE Study) (2019) (0)
WS14.6 Increased respiratory syncytial virus burden leads to more rapid cell death in Phe508del bronchial epithelial cells (2016) (0)
WS11.6 Interpretation of nasal potential difference (nPD) measurements in difficult cases of possible cystic fibrosis and the role of published equations (2014) (0)
Nitrogen offset in N2 multiple washout method (2020) (0)
P238 Superior yield of positive bacterial cultures from sputum induction vs cough swab in children, and its utility in assessing success of pseudomonas aeruginosa eradication therapy (2019) (0)
A NOVEL EXERCISE TEST DEMONSTRATES REDUCED STROKE VOLUME AUGMENTATION BUT NO EVIDENCE OF PULMONARY HYPERTENSION IN PATIENTS WITH ADVANCED CYSTIC FIBROSIS (2011) (0)
20 – Modern Molecular Therapies for Respiratory Disease (2012) (0)
36 Feasibility of airway surface liquid (ASL) height measurement in human nasal and bronchial biopsies (2007) (0)
P029 Curvilinearity (Curv) in healthy children and those with cystic fibrosis (2019) (0)
A RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED TRIAL OF REPEATED NEBULISATION OF NON-VIRAL CFTR GENE THERAPY IN PATIENTS WITH CYSTIC FIBROSIS (2015) (0)
Preparation for a ﬁ rst-in-man lentivirus trial in patients with cystic ﬁ brosis (2016) (0)
Methods: clinical trial design and outcome measures (2016) (0)
Author response: Colistin kills bacteria by targeting lipopolysaccharide in the cytoplasmic membrane (2021) (0)
Results of mechanistic substudy (2016) (0)
The Uk Cystic Fibrosis Gene Therapy Consortium: Normal Values And Reproducibility Of Forced Expiratory Flow Volume Curves In Sheep (2011) (0)
Chapter 12 Future treatments (2010) (0)
Safety and adverse events (2016) (0)
Lytic Bacteriophage Is a Promising Adjunct to Common Antibiotics across Cystic Fibrosis Clinical Strains and Culture Models of Pseudomonas aeruginosa Infection (2023) (0)
Future therapies for cystic fibrosis (2023) (0)
Authors' reply (2003) (0)
Gene Therapy for Lung Diseases (2006) (0)
Clinical evaluation of gene therapy for cystic fibrosis: safety and expression of a single dose of lipid-mediated CFTR gene therapy to the upper and lower airways of patients (2012) (0)
Synergistic Activity of Repurposed Peptide Drug Glatiramer Acetate with Tobramycin against Cystic Fibrosis Pseudomonas aeruginosa (2022) (0)
P223 Urinary Matrix Metalloproteinase-9 (MMP-9) is not a biomarker of pulmonary exacerbation in cystic fibrosis (2019) (0)
Current & Emerging Pharmaceutical Treatments for Cystic Fibrosis Lung Disease (2013) (0)
What have we learned over the last 5 years (2015) (0)
562: Elexacaftor/tezacaftor/ivacaftor in children aged 6 and older with cystic fibrosis and at least 1 F508del allele: Interim results from a Phase 3 open-label extension study (2021) (0)
Results of clinical efficacy outcomes (2016) (0)
560: Real-world impact of lumacaftor/ivacaftor on pulmonary outcomes in children aged 6 to 11 with CF (2021) (0)
Cystic Fibrosis Questionnaire – Revised quality-of-life questionnaire (2016) (0)
140 Towards the clinical application of anti-pseudomonal bacteriophages: Activity is retained following nebulisation with a range of commercially available nebuliser systems (2015) (0)
Diversity and prevalence of type VI secretion system effectors in clinical Pseudomonas aeruginosa isolates (2023) (0)
Wheezing in infants and pre-school children accounts for a considerable proportion of acute hospital admissions. (2003) (0)
The ratio IL-8:IL-10 is increased in the paediatric CF airway as compared to other neutrophilic lung diseases (2015) (0)
MINI-SYMPOSIUM: Optimising Cystic Fibrosis Outcomes: Screening and Treating in 2013 Cystic Fibrosis: therapies targeting specific gene defects (2012) (0)
Lung Infection in Cystic Fibrosis and Other Chronic Suppurative Lung Diseases (2010) (0)
Comprar Paediatric Respiratory Disease: Airways And Infection: An Atlas Of Investigation And Management | DAVIES, J | 9781904392972 | Clinical Pub. (2010) (0)
WS16.2 The prevalence of gastroesophageal reflux disease in infants with cystic fibrosis diagnosed by newborn screening and the relationship with lung infection (2015) (0)
WS23.4 High Rhinovirus burden in lower airways of children with cystic fibrosis (2012) (0)
300: Screening for depression and anxiety in parents of children with cystic fibrosis over 6 months (2021) (0)
M3 Pseudomonas aeruginosa induces inflammation in bronchial epithelial cells via the p38 MAP and Syk tyrosine kinase pathways (2019) (0)
Simultaneous SF6 and Nitrogen Multiple Breath Washout (MBW) to examine inherent differences in MBW outcomes (2019) (0)
Outcome of clinical trials: Electricity, induced sputum, and breath (2015) (0)
95 Phenotyping cystic fibrosis clinical isolates of Pseudomonas aeruginosa reveals greater between-patient than within-patient diversity (2017) (0)
87 Pseudomonas aeruginosa from CF patients form larger biofilms in vitro than those from subjects with bronchiectasis (2017) (0)
S60 Upper vs. lower airway microbiological culture in children with respiratory symptoms (2019) (0)
Pseudomonas aeruginosa in the CF Airway: Does it Deserve its Reputation as a Predatory 'Bully'? (2020) (0)
The New York Heat Wave Revisited- Unusual Presentation of Atypical Cystic Fibrosis in an Adult (2019) (0)
ePS6.10 A real-world study evaluating the impact of elexacaftor/tezacaftor/ivacaftor treatment on medication adherence in cystic fibrosis (2022) (0)
Perspective: Care in the age of COVID-19 (2020) (0)
A systematic review of lung clearance index in non-cystic fibrosis, non-primary ciliary dyskinesia bronchiectasis. (2022) (0)
Real world health outcomes in people with cystic fibrosis after initiation of a new combination treatment (2020) (0)
A Systematic Review of Treatment Patterns in Patients with Recurrent Glioblastoma in the Real World (2016) (0)
Systematic review of lung clearance index (LCI) in non-cystic fibrosis (CF), non-primary ciliary dyskinesia (PCD) bronchiectasis (Bx) (2021) (0)
P63 Levelling the playing field: improving access to cystic fibrosis clinical trials for a large, regional population. Lessons learned from the London network of the UK clinical trials accelerator platform (2022) (0)
ePS6.09 A discrete choice experiment to quantify the influence of trial features on patients’ decisions to join cystic fibrosis clinical trials (2022) (0)
P155 Paws for thought: sniffer dogs for infection surveillance in non-sputum-producing people with cystic fibrosis (2022) (0)
Chronic infection by controlling inflammation (2019) (0)
Longitudinal development of the airway microbiota in infants with cystic fibrosis (2019) (0)
University of Birmingham Comparison of the upper and lower airway microbiota in children with chronic lung diseases (2018) (0)
Reply to migration is not the perfect answer: Optimized methodology to assess LCI agreement between corrected legacy multiple breath nitrogen washout data and that directly collected on updated software. (2023) (0)
166 Measuring adherence to chronic therapies over the first year of treatment with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis (2022) (0)
169 A phase 4, fully decentralized clinical trial to evaluate physical activity and cough frequency in patients with cystic fibrosis using wearable technology (2022) (0)
283 Expectations and experiences of adolescents with cystic fibrosis: Influence of elexacaftor/tezacaftor/ivacaftor on treatment adherence and identity (2022) (0)
157 A randomized controlled trial to determine the most sensitive outcome measure of airway clearance in cystic fibrosis (2022) (0)
141 Oxygen-enhanced magnetic resonance imaging and multiple-breath washout with or without short extension as novel functional assessments of cystic fibrosis lung health (2022) (0)
165 Hypoxic challenge testing and air travel: the Welsh experience (2012) (0)
616 Cystic fibrosis gene therapy: Moving forward (2022) (0)
P087 CTN – Lung Clearance Index core facility: quality improvement exercise (2022) (0)
S68 Towards a first-in-human trial with a pseudotyped lentivirus (2021) (0)

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