Published in Curr Pharm Des on January 01, 2012
Microbiota and metabolite profiling reveal specific alterations in bacterial community structure and environment in the cystic fibrosis airway during exacerbation. PLoS One (2013) 0.97
L-ornithine derived polyamines in cystic fibrosis airways. PLoS One (2012) 0.95
Mature cystic fibrosis airway neutrophils suppress T cell function: evidence for a role of arginase 1 but not programmed death-ligand 1. J Immunol (2015) 0.81
Multitracer stable isotope quantification of arginase and nitric oxide synthase activity in a mouse model of pseudomonas lung infection. Mediators Inflamm (2014) 0.79
Neutrophil plasticity enables the development of pathological microenvironments: implications for cystic fibrosis airway disease. Mol Cell Pediatr (2016) 0.77
Effect of arginase inhibition on pulmonary L-arginine metabolism in murine Pseudomonas pneumonia. PLoS One (2014) 0.77
Oscillatory shear stress-induced arginase activity may explain reduced exhaled nitric oxide levels after vest chest physiotherapy in cystic fibrosis. Pulm Med (2014) 0.75
New insights into upper airway innate immunity. Am J Rhinol Allergy (2016) 0.75
CFTR Modulator Therapies in Pediatric Cystic Fibrosis: Focus on Ivacaftor. Expert Opin Orphan Drugs (2016) 0.75
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med (2011) 8.73
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med (2015) 5.51
Consensus statement for inert gas washout measurement using multiple- and single- breath tests. Eur Respir J (2013) 3.34
Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA (2010) 2.75
Exhaled nitric oxide in pulmonary diseases: a comprehensive review. Chest (2010) 2.29
Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis. Thorax (2013) 2.20
Open-label, follow-on study of azithromycin in pediatric patients with CF uninfected with Pseudomonas aeruginosa. Pediatr Pulmonol (2012) 2.08
Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function. Thorax (2010) 1.97
Endothelial nitric oxide synthase variants in cystic fibrosis lung disease. Am J Respir Crit Care Med (2002) 1.87
Stenotrophomonas maltophilia in cystic fibrosis: serologic response and effect on lung disease. Am J Respir Crit Care Med (2010) 1.82
Diagnostic value of nasal nitric oxide measured with non-velum closure techniques for children with primary ciliary dyskinesia. J Pediatr (2011) 1.81
Lung clearance index as an outcome measure for clinical trials in young children with cystic fibrosis. A pilot study using inhaled hypertonic saline. Am J Respir Crit Care Med (2013) 1.76
Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein. Nat Biotechnol (2012) 1.73
Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia. Pediatr Res (2011) 1.70
Sputum induction in routine clinical care of children with cystic fibrosis. J Pediatr (2010) 1.67
Effect of dornase alfa on inflammation and lung function: potential role in the early treatment of cystic fibrosis. J Cyst Fibros (2011) 1.65
The effect of chronic infection with Aspergillus fumigatus on lung function and hospitalization in patients with cystic fibrosis. Chest (2009) 1.56
beta2 adrenoceptor gene polymorphisms in cystic fibrosis lung disease. Pharmacogenetics (2002) 1.54
Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis. Eur Respir J (2013) 1.53
Effect of pulmonary exacerbations on long-term lung function decline in cystic fibrosis. Eur Respir J (2011) 1.31
Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA (2012) 1.25
Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis. Am J Respir Crit Care Med (2003) 1.24
Increased arginase activity in cystic fibrosis airways. Am J Respir Crit Care Med (2005) 1.21
Plastic bronchitis as an unusual cause of mucus plugging in cystic fibrosis. Pediatr Pulmonol (2009) 1.19
Restoring airway surface liquid in cystic fibrosis. N Engl J Med (2006) 1.14
Inhalation of Moli1901 in patients with cystic fibrosis. Chest (2007) 1.13
Alveolar inflammation in cystic fibrosis. J Cyst Fibros (2010) 1.11
Nitrogen redox balance in the cystic fibrosis airway: effects of antipseudomonal therapy. Am J Respir Crit Care Med (2002) 1.09
Multiple breath nitrogen washout: a feasible alternative to mass spectrometry. PLoS One (2013) 1.07
Improvement of alveolar glutathione and lung function but not oxidative state in cystic fibrosis. Am J Respir Crit Care Med (2004) 1.05
Inhaled L-arginine improves exhaled nitric oxide and pulmonary function in patients with cystic fibrosis. Am J Respir Crit Care Med (2006) 1.05
Reliability and validity of the habitual activity estimation scale (HAES) in patients with cystic fibrosis. Pediatr Pulmonol (2008) 1.04
Pulmonary surfactant, lung function, and endobronchial inflammation in cystic fibrosis. Am J Respir Crit Care Med (2004) 0.99
Mucolytics in cystic fibrosis. Paediatr Respir Rev (2007) 0.99
A multicenter, randomized, double-blind, placebo-controlled trial to evaluate the metabolic and respiratory effects of growth hormone in children with cystic fibrosis. Pediatrics (2007) 0.98
Genetic variations in inflammatory mediators influence lung disease progression in cystic fibrosis. Pediatr Pulmonol (2008) 0.98
Asymmetric dimethylarginine contributes to airway nitric oxide deficiency in patients with cystic fibrosis. Am J Respir Crit Care Med (2011) 0.98
Exercise and physical activity in children with cystic fibrosis. Paediatr Respir Rev (2009) 0.97
Cystic Fibrosis Foundation pulmonary guideline. pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection. Ann Am Thorac Soc (2014) 0.97
Multidrug-resistant organisms in cystic fibrosis: management and infection-control issues. Expert Rev Anti Infect Ther (2006) 0.97
Chronic Stenotrophomonas maltophilia infection and exacerbation outcomes in cystic fibrosis. J Cyst Fibros (2011) 0.96
Decreased systemic bioavailability of L-arginine in patients with cystic fibrosis. Respir Res (2006) 0.96
Influence of interleukin-10 on Aspergillus fumigatus infection in patients with cystic fibrosis. J Infect Dis (2005) 0.96
Emerging therapies for cystic fibrosis lung disease. Expert Opin Emerg Drugs (2010) 0.95
L-ornithine derived polyamines in cystic fibrosis airways. PLoS One (2012) 0.95
Chronic Stenotrophomonas maltophilia infection and mortality or lung transplantation in cystic fibrosis patients. J Cyst Fibros (2013) 0.94
Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis. Pediatr Pulmonol (2007) 0.93
The approach to Pseudomonas aeruginosa in cystic fibrosis. Semin Respir Crit Care Med (2009) 0.92
Early intervention studies in infants and preschool children with cystic fibrosis: are we ready? Eur Respir J (2013) 0.90
Update in cystic fibrosis 2012. Am J Respir Crit Care Med (2013) 0.89
The effect of early Pseudomonas aeruginosa treatment on lung function in pediatric cystic fibrosis. Pediatr Pulmonol (2011) 0.89
Effects of sex and of gene variants in constitutive nitric oxide synthases on exhaled nitric oxide. Am J Respir Crit Care Med (2003) 0.89
P2Y2 receptor polymorphisms and haplotypes in cystic fibrosis and their impact on Ca2+ influx. Pharmacogenet Genomics (2006) 0.88
Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis. Cochrane Database Syst Rev (2013) 0.88
Ancestral haplotype 8.1 and lung disease severity in European cystic fibrosis patients. J Cyst Fibros (2011) 0.88
Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis. Cochrane Database Syst Rev (2012) 0.87
Nebulized and oral thiol derivatives for pulmonary disease in cystic fibrosis. Cochrane Database Syst Rev (2009) 0.87
Cystic fibrosis: detecting changes in airway inflammation with FDG PET/CT. Radiology (2012) 0.84
Reduced lung function in cystic fibrosis: a primary or secondary phenotype? Am J Respir Crit Care Med (2008) 0.84
The cystic fibrosis gender gap: potential roles of estrogen. Pediatr Pulmonol (2013) 0.84
Nasal Staphylococcus aureus carriage is not a risk factor for lower-airway infection in young cystic fibrosis patients. J Clin Microbiol (2007) 0.83
Inhaled hypertonic saline in infants and toddlers with cystic fibrosis: short-term tolerability, adherence, and safety. Pediatr Pulmonol (2011) 0.83
Emerging drugs for cystic fibrosis. Expert Opin Emerg Drugs (2014) 0.82
Pulmonary exacerbations and parent-reported outcomes in children <6 years with cystic fibrosis. Pediatr Pulmonol (2014) 0.81
Epidemiology of borderline oxacillin-resistant Staphylococcus aureus in pediatric cystic fibrosis. Pediatr Pulmonol (2011) 0.81
Sequential analysis of surfactant, lung function and inflammation in cystic fibrosis patients. Respir Res (2005) 0.81
Pulmonary function after early vs late lobectomy during childhood: a preliminary study. J Pediatr Surg (2009) 0.80
Pharmacokinetic modelling of a once-daily dosing regimen for intravenous tobramycin in paediatric cystic fibrosis patients. J Antimicrob Chemother (2007) 0.80
Inhaled liposomal amikacin. Expert Rev Respir Med (2014) 0.80
Bronchoalveolar lavage protein patterns in children with malignancies, immunosuppression, fever and pulmonary infiltrates. Proteomics (2002) 0.79
A pilot study to compare tobramycin 80 mg injectable preparation with 300 mg solution for inhalation in cystic fibrosis patients. Can Respir J (2008) 0.79
Serum galactomannan in cystic fibrosis patients colonized with Aspergillus species. Med Mycol (2012) 0.79
Human adenosine 2B receptor: SNP discovery and evaluation of expression in patients with cystic fibrosis. Pharmacogenet Genomics (2005) 0.79
Standard versus biofilm antimicrobial susceptibility testing to guide antibiotic therapy in cystic fibrosis. Cochrane Database Syst Rev (2015) 0.79
Effect of endoscopic sinus surgery on pulmonary function and microbial pathogens in a pediatric population with cystic fibrosis. Arch Otolaryngol Head Neck Surg (2011) 0.78
Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas aeruginosa in cystic fibrosis. Cochrane Database Syst Rev (2008) 0.78
Airway nitric oxide in Duchenne muscular dystrophy. J Pediatr (2002) 0.78
Assessment of body composition in pediatric patients with cystic fibrosis. Pediatr Pulmonol (2008) 0.78
Factors influencing the acquisition of Stenotrophomonas maltophilia infection in cystic fibrosis patients. J Cyst Fibros (2013) 0.78
Brain arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia: clinical presentation and anatomical distribution. Pediatr Neurol (2013) 0.78
Exhaled nitric oxide in children after accidental exposure to chlorine gas. Inhal Toxicol (2007) 0.78
Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis. Cochrane Database Syst Rev (2014) 0.78
Effect of equipment dead space on multiple breath washout measures. Respirology (2015) 0.78
A comparison of amount and speed of deposition between the PARI LC STAR® jet nebulizer and an investigational eFlow® nebulizer. J Aerosol Med Pulm Drug Deliv (2011) 0.78
Rapid pulmonary delivery of inhaled tobramycin for Pseudomonas infection in cystic fibrosis: a pilot project. Pediatr Pulmonol (2008) 0.78
Lack of association of small-colony-variant Staphylococcus aureus strains with long-term use of azithromycin in patients with cystic fibrosis. J Clin Microbiol (2011) 0.78
Symptomatic liver involvement in neonatal hereditary hemorrhagic telangiectasia. Pediatrics (2011) 0.77
Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas aeruginosa in cystic fibrosis. Cochrane Database Syst Rev (2015) 0.77
Does earlier lobectomy result in better long-term pulmonary function in children with congenital lung anomalies? A prospective study. J Pediatr Surg (2012) 0.77
Treatment of Aspergillus fumigatus in patients with cystic fibrosis: a randomized, placebo-controlled pilot study. PLoS One (2012) 0.77
Cystic fibrosis: a review of pulmonary and nutritional therapies. Adv Pediatr (2008) 0.77
Standard versus biofilm antimicrobial susceptibility testing to guide antibiotic therapy in cystic fibrosis. Cochrane Database Syst Rev (2012) 0.77
Prevalence of pulmonary arteriovenous malformations in children versus adults with hereditary hemorrhagic telangiectasia. J Pediatr (2013) 0.77
Impact of acute antibiotic therapy on the pulmonary exacerbation endpoint in cystic fibrosis clinical trials. Contemp Clin Trials (2013) 0.76
Physiological correlates of pulmonary function in children with cystic fibrosis. Pediatr Pulmonol (2013) 0.76
The prevalence of asthma in Canadian children of South Asian descent. Pediatr Pulmonol (2013) 0.76
Chemical pleurodesis versus surgical intervention for persistent and recurrent pneumothoraces in cystic fibrosis. Cochrane Database Syst Rev (2012) 0.76
Beta2-agonists for asthma: the pediatric perspective. Clin Rev Allergy Immunol (2006) 0.76
Aquagenic wrinkling of the palms in a patient with cystic fibrosis. N Engl J Med (2013) 0.75
Pediatric Respiratory Assembly. Mini symposium on lung inflammation. Can Respir J (2010) 0.75