Research Topics
| J C DaviesSummaryAffiliation: Imperial College Country: UK Publications
| Collaborators
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Detail Information
Publications
Modifier genes in cystic fibrosisJane C Davies
Department of Gene Therapy Royal Brompton Hospital and Imperial College, Sydney Street, London, SW3 6NP, UK
Pediatr Pulmonol Suppl 26:86-7. 2004- Emerging and unusual gram-negative infections in cystic fibrosisJane C Davies
Department of Pediatric Respiratory Medicine, Royal Brompton Hospital, and Department of Gene Therapy, Imperial College, London, United Kingdom
Semin Respir Crit Care Med 28:312-21. 2007..cepacia complex, are clearly linked to an adverse prognosis, and both treatment and infection control issues can pose a real challenge... 
Cystic fibrosisJane C Davies
Department of Gene Therapy, Imperial College, London
BMJ 335:1255-9. 2007- Ion transport in lung diseaseJane C Davies
Department of Gene Therapy Royal Brompton Hospital and Imperial College, Sydney Street, London, SW3 6NT, UK
Pediatr Pulmonol Suppl 26:147-8. 2004 - Research applications of bronchoscopyJane Davies
Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, Sydney Street, SW3 6NP, London, UK
Paediatr Respir Rev 4:230-6. 2003..Collaboration between centres should be encouraged to maximise the use of samples obtained... - Cystic fibrosis presenting as acute upper airway obstructionJ Davies
Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London SW3 6LR, UK
Thorax 61:92. 2006 - Gene therapy for cystic fibrosisJ C Davies
Department of Gene Therapy, Imperial College at the National Heart and Lung Institute, London, UK
J Gene Med 3:409-17. 2001..We highlight the problems encountered, and likely future directions of the field... - New therapeutic approaches for cystic fibrosis lung diseaseJane C Davies
Royal Brompton Hospital, Sydney Street, London SW3 6NT, UK
J R Soc Med 95:58-67. 2002 - Prospects for gene therapy in lung diseaseJ C Davies
Imperial College at the National Heart and Lung Institute, London, UK
Curr Opin Pharmacol 1:272-7. 2001..Recently, progress has been made in both laboratory and clinical studies of gene therapy for cystic fibrosis, alpha1-antitrypsin deficiency and lung cancer... - Bone marrow stem cells do not repopulate the healthy upper respiratory tractJane C Davies
Department of Gene Therapy, Imperial College at the National Heart and Lung Institute, London, UK
Pediatr Pulmonol 34:251-6. 2002..In the absence of such conditions, whole bone marrow transplantation is unlikely to provide a route for correction of the CF airway... - Inflammation in cystic fibrosis airways: relationship to increased bacterial adherenceP Scheid
Dept. of Gene Therapy, Imperial College at the National Heart and Lung Institute, London, UK
Eur Respir J 17:27-35. 2001..The authors conclude that the stimulus produced by Pseudomonas aeruginosa is the predominant inflammatory trigger in their models... 
Mannose-binding lectin is present in the infected airway: a possible pulmonary defence mechanismK J Fidler
Infectious Diseases and Microbiology Unit, Institute of Child Health, London, UK
Thorax 64:150-5. 2009..The mechanism is uncertain, and could relate either to systemic or local effects. The aim of this study was to determine, in a large cohort of children, whether MBL is present on the airway surface in health or disease...- Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator functionN J Simmonds
Dept of Cystic Fibrosis, Royal Brompton Hospital, London, SW3 6NP, UK
Eur Respir J 37:1076-82. 2011..5 (80.0-113.5) mmol·L⁻¹; young/mild). Δ Amil is associated with FEV₁ but our findings indicate that long-term survival cannot be explained by residual CFTR function when measurements are taken in later life... - Normal nasal mucociliary clearance in CF children: evidence against a CFTR-related defectD McShane
Dept of Gene Therapy, Imperial College at the National Heart and Lung Institute, London, UK
Eur Respir J 24:95-100. 2004..Studies to explore this mechanism in the distal, more sparsely-ciliated airways could aid an understanding of pathogenesis and the development of new treatments... - Safety of endobronchial biopsy in children with cystic fibrosisA Molina-Teran
Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom
Pediatr Pulmonol 41:1021-4. 2006..Studies of such samples would allow us to determine the early pathological changes in the CF airway and possibly find new treatments to prevent the progression to bronchiectasis and end stage airway destruction... - Pseudomonas aeruginosa, cyanide accumulation and lung function in CF and non-CF bronchiectasis patientsB Ryall
Dept of Life Sciences, Division of Biology, Faculty of Natural Sciences, Imperial College London, Sir Alexander Fleming Building, London, SW7 2AZ, UK
Eur Respir J 32:740-7. 2008..4+/-4.9 versus 60.1+/-7.7%). Cyanide is detectable in sputum from cystic fibrosis and non-cystic fibrosis bronchiectasis patients infected with Pseudomonas aeruginosa, and is also associated with impaired lung function... - Modifier genes in cystic fibrosisJ C Davies
Department of Gene Therapy, National Lung and Heart Institute, Faculty of Medicine, Imperial College, London, UK
Pediatr Pulmonol 39:383-91. 2005..This review will summarize the field to date and discuss some of the methodological issues important in the design and interpretation of such studies... - Tumour necrosis factor gene polymorphisms and childhood wheezingH Bilolikar
Dept of Paediatric Respiratory Medicine, Royal BromptonHospital, Sydney Street, London SW3 6NP, UK
Eur Respir J 26:637-46. 2005..In conclusion, these findings may have implications for future early intervention studies by helping to identify infants at increased risk for wheezing and childhood asthma... - Multiresistant Pseudomonas aeruginosa in a pediatric cystic fibrosis center: natural history and implications for segregationG Davies
Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Trust, London, UK
Pediatr Pulmonol 35:253-6. 2003..The variability with time in isolation of MR strains from individuals demonstrates the potential difficulties in designing segregation policies based on antibiotic sensitivity patterns... - Airway surface pH in subjects with cystic fibrosisD McShane
Dept of Gene Therapy, Imperial College at the National Heart and Lung Institute, London, UK
Eur Respir J 21:37-42. 2003.... - Computed tomography and cystic fibrosis: promises and problemsZ A Aziz
Department of Radiology, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK
Thorax 62:181-6. 2007..This review summarises current concepts relating to the research applications of CT in CF, with particular emphasis on the evidence supporting the use of CT as a surrogate outcome measure in clinical trials... - Impaired pulmonary status in cystic fibrosis adults with two mutated MBL-2 allelesJ C Davies
Dept of Paediatric Respiratory Medicine, Royal Brompton Hospital, Sydney St, London, SW3 6NP, UK
Eur Respir J 24:798-804. 2004.... - Novel antipseudomonal treatment approachesJ C Davies
Dept of Gene Therapy, National Heart and Lung Institute, Imperial College, Emmanuel Kaye Building, Manresa Rd, London SW3 6LR
Arch Pediatr 13:S51-4. 2006.... - Wheezing in infants and pre-school children accounts for a considerable proportion of acute hospital admissionsJane Davies
Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
Paediatr Respir Rev 4:267, 270. 2003 - Early detection of lung disease in preschool children with cystic fibrosisAndrew Bush
Imperial School of Medicine at National Heart and Lung Institute, Royal Brompton Hospital, London, United Kingdom
Curr Opin Pulm Med 11:534-8. 2005..This is particularly urgent to select groups of children for new, phenotype-specific therapies, and monitor their effects... - New tests for cystic fibrosisJane C Davies
Department of Gene Therapy, Imperial College, London, UK
Paediatr Respir Rev 7:S141-3. 2006..Nasal PD can also be helpful in the latter group, although there are a number of caveats to its use and interpretation... - Pseudomonas aeruginosa in cystic fibrosis: pathogenesis and persistenceJane C Davies
Department of Gene Therapy, Imperial College School of Medicine, London, UK
Paediatr Respir Rev 3:128-34. 2002..Understanding the steps involved in both initial infection and in establishing chronicity may help in the development of new treatment strategies... - Increased incidence and severity of the systemic inflammatory response syndrome in patients deficient in mannose-binding lectinKaty J Fidler
Infectious Diseases and Microbiology Unit, Institute of Child Health, 30 Guilford Street, London, WC1 N 1EH, UK
Intensive Care Med 30:1438-45. 2004..To determine whether pediatric PICU patients with mannose-binding lectin (MBL) gene polymorphisms associated with low levels of the functional protein have an increased risk of developing sepsis and SIRS... - Potential difference measurements in the lower airway of children with and without cystic fibrosisJane C Davies
Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, Emmanuel Kaye Building, Manresa Road, London SW3 6LR, UK
Am J Respir Crit Care Med 171:1015-9. 2005..Potential difference measurements could form a useful functional endpoint assay for future studies of either the CFTR gene or protein-based therapies in future trials in the pediatric age group... - Airway gene therapyJane C Davies
Department of Gene Therapy, Imperial College London, London SW3 6LR, United Kingdom
Adv Genet 54:291-314. 2005..This chapter reviews these issues, strategies aimed at overcoming them, and progress into clinical trials with non-viral vectors in a variety of pulmonary diseases... - Bronchoscopy following diagnosis with cystic fibrosisTom N Hilliard
Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
Arch Dis Child 92:898-9. 2007..Lavage culture was positive in eight of 18 children without respiratory symptoms. This highlights the potential of bronchoscopy following diagnosis, even in asymptomatic children... - Quality, size, and composition of pediatric endobronchial biopsies in cystic fibrosisNicolas Regamey
Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK
Chest 131:1710-7. 2007..We prospectively assessed how the quality of biopsy specimens obtained from children with CF compare with those from children with other airway diseases... - Exploring the mechanisms of macrolides in cystic fibrosisAmanda C Equi
Department of Gene Therapy, National Heart and Lung Institute, Imperial College, London, UK
Respir Med 100:687-97. 2006..aeruginosa did not decrease. We conclude that these are unlikely to be significant contributing mechanisms accounting for the consistent beneficial results observed in clinical trials of macrolides in CF... - Biomarkers for cystic fibrosis: are we progressing?Eric W F W Alton
Am J Respir Crit Care Med 175:750-1. 2007 - Gene and cell therapy for cystic fibrosisJane C Davies
Departments of Gene Therapy and Paediatric Respiratory Medicine, Imperial College, National Heart and Lung Institute, Manresa Road, London, SW3 6LR, UK
Paediatr Respir Rev 7:S163-5. 2006..Whether this approach will be able to achieve clinical success remains to be seen... - CpG-free plasmids confer reduced inflammation and sustained pulmonary gene expressionStephen C Hyde
Gene Medicine Research Group, Nuffield Department of Clinical Laboratory Sciences, University of Oxford, Oxford OX3 9DU, UK
Nat Biotechnol 26:549-51. 2008..Using a CpG-free pDNA expression vector, we achieved sustained (>or=56 d) in vivo transgene expression in the absence of lung inflammation... - Nasal abnormalities in cystic fibrosis mice independent of infection and inflammationTom N Hilliard
Department of Gene Therapy, Emmanuel Kaye Building, 1B Manresa Road, London SW3 6LR, UK
Am J Respir Cell Mol Biol 39:19-25. 2008..There are significant histologic changes in the nasal mucosa of adult CF mice, not associated with increased lumenal inflammation or bacterial content, and which are not present perinatally. These may be novel therapeutic targets... - Lung clearance index in CF: a sensitive marker of lung disease severityJane C Davies
Thorax 63:96-7. 2008 - Increased airway smooth muscle mass in children with asthma, cystic fibrosis, and non-cystic fibrosis bronchiectasisNicolas Regamey
Department of Gene Therapy, National Heart and Lung Institute, 1B Manresa Road, London SW3 6LR, UK
Am J Respir Crit Care Med 177:837-43. 2008..Structural alterations to airway smooth muscle (ASM) are a feature of asthma and cystic fibrosis (CF) in adults... - Endobronchial biopsy in childhoodNicolas Regamey
Chest 133:312; author reply 313. 2008 - Measurement of tobramycin and gentamicin in saliva is not suitable for therapeutic drug monitoring of patients with cystic fibrosisHelen Spencer
J Cyst Fibros 4:209. 2005 - Detection of antibodies to Pseudomonas aeruginosa in serum and oral fluid from patients with cystic fibrosisAbbie M Weisner
Laboratory of Healthcare Associated Infections, Centre for Infections, Health Protection Agency, 61 Colindale Avenue, London NW9 5EQ, UK
J Med Microbiol 56:670-4. 2007..All serum samples from the CF patients were positive by both methods. Thus this is a sensitive procedure for the detection of antibodies to A-band LPS of P. aeruginosa in oral fluid and serum from patients with CF... - Airway remodelling in children with cystic fibrosisTom N Hilliard
Department of Gene Therapy, National Heart and Lung Institute, Imperial College, London, UK
Thorax 62:1074-80. 2007..A study was undertaken to determine changes in airway remodelling in children with CF compared with appropriate disease and healthy controls...