Published in N Engl J Med on May 23, 2013
Air trapping and airflow obstruction in newborn cystic fibrosis piglets. Am J Respir Crit Care Med (2013) 1.65
Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants. Thorax (2013) 1.58
miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production. Am J Respir Crit Care Med (2014) 1.45
Radiological diagnosis in lung disease: factoring treatment options into the choice of diagnostic modality. Dtsch Arztebl Int (2014) 1.29
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure. J Clin Invest (2014) 1.26
Cystic fibrosis: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases. Ann Am Thorac Soc (2014) 0.92
Release of cystic fibrosis airway inflammatory markers from Pseudomonas aeruginosa-stimulated human neutrophils involves NADPH oxidase-dependent extracellular DNA trap formation. J Immunol (2014) 0.91
Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF. Pediatr Pulmonol (2013) 0.90
Host-pathogen interplay in the respiratory environment of cystic fibrosis. J Cyst Fibros (2015) 0.89
New and emerging targeted therapies for cystic fibrosis. BMJ (2016) 0.87
Thrombospondin-1 restrains neutrophil granule serine protease function and regulates the innate immune response during Klebsiella pneumoniae infection. Mucosal Immunol (2014) 0.83
Identifying the origins of cystic fibrosis lung disease. N Engl J Med (2013) 0.83
Airway, but not serum or urinary, levels of YKL-40 reflect inflammation in early cystic fibrosis lung disease. BMC Pulm Med (2014) 0.82
Neutrophil elastase and matrix metalloproteinase 12 in cystic fibrosis lung disease. Mol Cell Pediatr (2016) 0.82
2-O, 3-O-desulfated heparin inhibits neutrophil elastase-induced HMGB-1 secretion and airway inflammation. Am J Respir Cell Mol Biol (2014) 0.82
Showing Value in Newborn Screening: Challenges in Quantifying the Effectiveness and Cost-Effectiveness of Early Detection of Phenylketonuria and Cystic Fibrosis. Healthcare (Basel) (2015) 0.82
Pulmonary exacerbations and parent-reported outcomes in children <6 years with cystic fibrosis. Pediatr Pulmonol (2014) 0.81
Free DNA in cystic fibrosis airway fluids correlates with airflow obstruction. Mediators Inflamm (2015) 0.81
Quantitative proteomics reveals an altered cystic fibrosis in vitro bronchial epithelial secretome. Am J Respir Cell Mol Biol (2015) 0.81
The activin A antagonist follistatin inhibits cystic fibrosis-like lung inflammation and pathology. Immunol Cell Biol (2015) 0.81
Genetic Deletion and Pharmacological Inhibition of PI3K γ Reduces Neutrophilic Airway Inflammation and Lung Damage in Mice with Cystic Fibrosis-Like Lung Disease. Mediators Inflamm (2015) 0.80
Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age? Thorax (2013) 0.80
Acute administration of ivacaftor to people with cystic fibrosis and a G551D-CFTR mutation reveals smooth muscle abnormalities. JCI Insight (2016) 0.80
Hypoxic epithelial necrosis triggers neutrophilic inflammation via IL-1 receptor signaling in cystic fibrosis lung disease. Am J Respir Crit Care Med (2015) 0.79
An interactive network of elastase, secretases, and PAR-2 protein regulates CXCR1 receptor surface expression on neutrophils. J Biol Chem (2014) 0.78
The Evolution of Cystic Fibrosis Care. Chest (2015) 0.78
What did we learn from two decades of chest computed tomography in cystic fibrosis? Pediatr Radiol (2014) 0.78
Cystic Fibrosis: Microbiology and Host Response. Pediatr Clin North Am (2016) 0.78
Changes in structural lung disease in cystic fibrosis children over 4 years as evaluated by high-resolution computed tomography. Eur Radiol (2015) 0.78
Hypoxia upregulates neutrophil degranulation and potential for tissue injury. Thorax (2016) 0.78
Neutrophil plasticity enables the development of pathological microenvironments: implications for cystic fibrosis airway disease. Mol Cell Pediatr (2016) 0.77
Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease. Ann Am Thorac Soc (2016) 0.77
Year in review 2013: Lung cancer, respiratory infections, tuberculosis, cystic fibrosis, pleural diseases, bronchoscopic intervention and imaging. Respirology (2014) 0.77
Refining the continuum of CFTR-associated disorders in the era of newborn screening. Clin Genet (2016) 0.77
NETs and CF Lung Disease: Current Status and Future Prospects. Antibiotics (Basel) (2015) 0.77
Cholesteryl esters are elevated in the lipid fraction of bronchoalveolar lavage fluid collected from pediatric cystic fibrosis patients. PLoS One (2015) 0.77
A secretory leukocyte protease inhibitor variant with improved activity against lung infection. Mucosal Immunol (2015) 0.76
Early Childhood Risk Factors for Decreased FEV1 at Age Six to Seven Years in Young Children with Cystic Fibrosis. Ann Am Thorac Soc (2015) 0.76
Interactions between Neutrophils and Pseudomonas aeruginosa in Cystic Fibrosis. Pathogens (2017) 0.75
Infant lung function tests as endpoints in the ISIS multicenter clinical trial in cystic fibrosis. J Cyst Fibros (2015) 0.75
The development of bronchiectasis on chest computed tomography in children with cystic fibrosis: can pre-stages be identified? Eur Radiol (2016) 0.75
Novel outcome measures for clinical trials in cystic fibrosis. Pediatr Pulmonol (2014) 0.75
Lessons learned from metabolomics in cystic fibrosis. Mol Cell Pediatr (2015) 0.75
Respiratory infection rates differ between geographically distant paediatric cystic fibrosis cohorts. ERJ Open Res (2016) 0.75
Sputum club cell protein concentration is associated with pulmonary exacerbation in cystic fibrosis. J Cyst Fibros (2014) 0.75
CFTR Modulator Therapies in Pediatric Cystic Fibrosis: Focus on Ivacaftor. Expert Opin Orphan Drugs (2016) 0.75
Biomarkers for cystic fibrosis drug development. J Cyst Fibros (2016) 0.75
Inhaled hypertonic saline for cystic fibrosis: Reviewing the potential evidence for modulation of neutrophil signalling and function. World J Crit Care Med (2015) 0.75
Neutrophil extracellular trap release driven by bacterial motility: Relevance to cystic fibrosis lung disease. Commun Integr Biol (2017) 0.75
Background and Epidemiology. Pediatr Clin North Am (2016) 0.75
Hypertonic Saline as a Useful Tool for Sputum Induction and Pathogen Detection in Cystic Fibrosis. Lung (2017) 0.75
Neutrophil Membrane Cholesterol Content is a Key Factor in Cystic Fibrosis Lung Disease. EBioMedicine (2017) 0.75
Neutrophil Elastase Activity is Associated with Exacerbations and Lung Function Decline in Bronchiectasis. Am J Respir Crit Care Med (2016) 0.75
Consensus statement for inert gas washout measurement using multiple- and single- breath tests. Eur Respir J (2013) 3.34
PRAGMA-CF. A Quantitative Structural Lung Disease Computed Tomography Outcome in Young Children with Cystic Fibrosis. Am J Respir Crit Care Med (2015) 2.70
Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax (2011) 2.58
Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr (2009) 2.31
Vitamin D deficiency at 16 to 20 weeks' gestation is associated with impaired lung function and asthma at 6 years of age. Ann Am Thorac Soc (2014) 2.14
Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med (2009) 1.87
Intrinsic biochemical and functional differences in bronchial epithelial cells of children with asthma. Am J Respir Crit Care Med (2006) 1.84
Induction of epithelial-mesenchymal transition in primary airway epithelial cells from patients with asthma by transforming growth factor-beta1. Am J Respir Crit Care Med (2009) 1.83
Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med (2008) 1.79
Innate inflammatory responses of pediatric cystic fibrosis airway epithelial cells: effects of nonviral and viral stimulation. Am J Respir Cell Mol Biol (2011) 1.70
Definition of cutoff values for the hypoxia test used for preflight testing in young children with neonatal chronic lung disease. Chest (2007) 1.64
Early respiratory infection is associated with reduced spirometry in children with cystic fibrosis. Am J Respir Crit Care Med (2014) 1.60
Isotonic versus hypotonic solutions for maintenance intravenous fluid administration in children. Cochrane Database Syst Rev (2014) 1.49
Impact of intensive upper limb rehabilitation on quality of life: a randomized trial in children with unilateral cerebral palsy. Dev Med Child Neurol (2012) 1.46
Functional performance in young Australian children with achondroplasia. Dev Med Child Neurol (2011) 1.41
Predicting functional communication ability in children with cerebral palsy at school entry. Dev Med Child Neurol (2014) 1.40
Development in children with achondroplasia: a prospective clinical cohort study. Dev Med Child Neurol (2012) 1.40
Mastery motivation as a predictor of occupational performance following upper limb intervention for school-aged children with congenital hemiplegia. Dev Med Child Neurol (2014) 1.40
Spontaneous chylothorax in a 2-year-old child. Med J Aust (2009) 1.39
Mastery motivation in children with congenital hemiplegia: individual and environmental associations. Dev Med Child Neurol (2013) 1.39
Number and order of whole cell pertussis vaccines in infancy and disease protection. JAMA (2012) 1.37
Validity and reproducibility of measures of oropharyngeal dysphagia in preschool children with cerebral palsy. Dev Med Child Neurol (2014) 1.37
Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests. Thorax (2006) 1.37
Infection, inflammation, and lung function decline in infants with cystic fibrosis. Am J Respir Crit Care Med (2011) 1.33
Lung function in infants with cystic fibrosis diagnosed by newborn screening. Am J Respir Crit Care Med (2008) 1.31
Characterization of side population cells from human airway epithelium. Stem Cells (2008) 1.25
Chloral hydrate sedation for infant pulmonary function testing. Pediatr Pulmonol (2014) 1.19
A prospective randomised longitudinal MRI study of left ventricular adaptation to endurance and resistance exercise training in humans. J Physiol (2011) 1.16
Decreased fibronectin production significantly contributes to dysregulated repair of asthmatic epithelium. Am J Respir Crit Care Med (2010) 1.14
Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screening. PLoS One (2011) 1.08
Mock MRI: reducing the need for anaesthesia in children. Pediatr Radiol (2010) 1.06
Validation of the research capacity and culture (RCC) tool: measuring RCC at individual, team and organisation levels. Aust J Prim Health (2012) 1.06
Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis. Clin Infect Dis (2011) 1.05
DNA methylation profiles of airway epithelial cells and PBMCs from healthy, atopic and asthmatic children. PLoS One (2012) 1.04
Targeting the oligomerization domain of ETO interferes with RUNX1/ETO oncogenic activity in t(8;21)-positive leukemic cells. Cancer Res (2007) 1.02
Associations of television viewing time with excess body weight among urban and rural high-school students in regional mainland China. Public Health Nutr (2007) 1.01
Measuring exhaled nitric oxide levels in adults: the importance of atopy and airway responsiveness. Chest (2004) 1.00
Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis. Thorax (2011) 1.00
Observational Research in Childhood Infectious Diseases (ORChID): a dynamic birth cohort study. BMJ Open (2012) 0.97
Better understanding the influence of cigarette smoking and indoor air pollution on chronic obstructive pulmonary disease: a case-control study in Mainland China. Respirology (2007) 0.97
Move it to improve it (Mitii): study protocol of a randomised controlled trial of a novel web-based multimodal training program for children and adolescents with cerebral palsy. BMJ Open (2013) 0.96
The role of patient activation in frequent attendance at primary care: a population-based study of people with chronic disease. Patient Educ Couns (2010) 0.96
Protocol and baseline data from The Inala Chronic Disease Management Service evaluation study: a health services intervention study for diabetes care. BMC Health Serv Res (2010) 0.95
Identifying peroxidases and their oxidants in the early pathology of cystic fibrosis. Free Radic Biol Med (2010) 0.95
Nasal swab samples and real-time polymerase chain reaction assays in community-based, longitudinal studies of respiratory viruses: the importance of sample integrity and quality control. BMC Infect Dis (2014) 0.95
Evaluating a team-based approach to research capacity building using a matched-pairs study design. BMC Fam Pract (2012) 0.94
Does aberrant activation of the epithelial-mesenchymal trophic unit play a key role in asthma or is it an unimportant sideshow? Curr Opin Pharmacol (2004) 0.94
Acquisition of human polyomaviruses in the first 18 months of life. Emerg Infect Dis (2015) 0.94
Diabetic retinopathy screening and monitoring of early stage disease in general practice: design and methods. Contemp Clin Trials (2012) 0.93
Childhood pneumonia: a neglected, climate-sensitive disease? Lancet (2010) 0.93
An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age. Ann Am Thorac Soc (2013) 0.92
Incidence of basal cell carcinoma multiplicity and detailed anatomic distribution: longitudinal study of an Australian population. J Invest Dermatol (2008) 0.92
Deaths due to suicide: the effects of certification and coding practices in Australia. Aust N Z J Public Health (2008) 0.92
Psychiatric care of adults with intellectual disabilities: changing perceptions over a decade. Aust N Z J Psychiatry (2008) 0.92
Early intervention studies in infants and preschool children with cystic fibrosis: are we ready? Eur Respir J (2013) 0.90
A school-based comprehensive lifestyle intervention among Chinese kids against Obesity (CLICK-Obesity) in Nanjing City, China: the baseline data. Asia Pac J Clin Nutr (2014) 0.89
A new model of integrated primary-secondary care for complex diabetes in the community: study protocol for a randomised controlled trial. Trials (2013) 0.89
Role of high-resolution computed tomography in the detection of early cystic fibrosis lung disease. Paediatr Respir Rev (2008) 0.89
Distribution of early structural lung changes due to cystic fibrosis detected with chest computed tomography. J Pediatr (2013) 0.89
Monte Carlo simulation of the cost-effectiveness of sample size maintenance programs revealed the need to consider substitution sampling. J Clin Epidemiol (2012) 0.89
A school-based comprehensive lifestyle intervention among chinese kids against obesity (CLICK-Obesity): rationale, design and methodology of a randomized controlled trial in Nanjing city, China. BMC Public Health (2012) 0.88
Sevenfold rise in likelihood of pertussis test requests in a stable set of Australian general practice encounters, 2000-2011. Med J Aust (2013) 0.88
Coding external causes of injuries: problems and solutions. Accid Anal Prev (2007) 0.88
Bronchiolitis obliterans syndrome in lung transplant recipients: can thin-section CT findings predict disease before its clinical appearance? Radiology (2004) 0.88
Pleural fluid nucleic acid testing enhances pneumococcal surveillance in children. Respirology (2012) 0.88
Bacterial causes of empyema in children, Australia, 2007-2009. Emerg Infect Dis (2011) 0.87
Transcription factor p63 regulates key genes and wound repair in human airway epithelial basal cells. Am J Respir Cell Mol Biol (2013) 0.87
Impact of tactile dysfunction on upper-limb motor performance in children with unilateral cerebral palsy. Arch Phys Med Rehabil (2012) 0.87
Multi-site videoconference tutorials for medical students in Australia. ANZ J Surg (2012) 0.87
Assessment of early bronchiectasis in young children with cystic fibrosis is dependent on lung volume. Chest (2013) 0.87
Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening. Pediatrics (2012) 0.87
Effect of a telephone-delivered coronary heart disease secondary prevention program (proactive heart) on quality of life and health behaviours: primary outcomes of a randomised controlled trial. Int J Behav Med (2013) 0.86
Associations of residential density with adolescents' physical activity in a rapidly urbanizing area of Mainland China. J Urban Health (2009) 0.85
Which health conditions impact on productivity in working Australians? J Occup Environ Med (2011) 0.85
Value of serology in predicting Pseudomonas aeruginosa infection in young children with cystic fibrosis. Thorax (2010) 0.85
Characterization of maximal respiratory pressures in healthy children. Respiration (2012) 0.84
Health-related productivity losses increase when the health condition is co-morbid with psychological distress: findings from a large cross-sectional sample of working Australians. BMC Public Health (2011) 0.84
Interactions between airway epithelial cells and dendritic cells: implications for the regulation of airway inflammation. Curr Drug Targets (2006) 0.84
Airway sizes and proportions in children quantified by a video-bronchoscopic technique. BMC Pulm Med (2006) 0.84
Reproducibility of tactile assessments for children with unilateral cerebral palsy. Phys Occup Ther Pediatr (2012) 0.84
Factors influencing child abuse and neglect recognition and reporting by nurses: a multivariate analysis. Int J Nurs Stud (2009) 0.84
The endothelin axis and gelatinase activity in alveolar macrophages after brain-stem death injury: a pilot study. J Heart Lung Transplant (2007) 0.84
Assessment of bronchodilator responsiveness in preschool children using forced oscillations. Thorax (2007) 0.83
Exciting new clinical trials in cystic fibrosis: infants need not apply. Am J Respir Crit Care Med (2011) 0.83
Inhaled fluticasone dipropionate decreases levels of nitric oxide in recurrenty wheezy infants. Pediatr Pulmonol (2004) 0.83
Early bronchiectasis in cystic fibrosis detected by surveillance CT. Respirology (2010) 0.82
Airway, but not serum or urinary, levels of YKL-40 reflect inflammation in early cystic fibrosis lung disease. BMC Pulm Med (2014) 0.82
The value of FeNO measurement in asthma management: the motion against FeNO to help manage childhood asthma--reality bites. Paediatr Respir Rev (2008) 0.82
Medical management of children with achondroplasia: evaluation of an Australasian cohort aged 0-5 years. J Paediatr Child Health (2011) 0.82