PubRank

Michael W Konstan

Author PubWeight™ 102.52‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011 8.73
2 Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 2010 6.23
3 Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med 2005 5.51
4 Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med 2015 5.51
5 Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax 2011 2.84
6 Factors influencing outcomes in cystic fibrosis: a center-based analysis. Chest 2003 2.54
7 Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med 2007 2.03
8 Jointly modelling the relationship between survival and pulmonary function in cystic fibrosis patients. Stat Med 2002 1.88
9 The role of inflammation in the pathophysiology of CF lung disease. Clin Rev Allergy Immunol 2002 1.85
10 Pulmonary outcome prediction (POP) tools for cystic fibrosis patients. Pediatr Pulmonol 2010 1.71
11 Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med 2002 1.70
12 Association between respiratory tract methicillin-resistant Staphylococcus aureus and survival in cystic fibrosis. JAMA 2010 1.69
13 Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis. Pediatr Pulmonol 2002 1.67
14 Presence of methicillin resistant Staphylococcus aureus in respiratory cultures from cystic fibrosis patients is associated with lower lung function. Pediatr Pulmonol 2007 1.67
15 Pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol 2004 1.65
16 Standardized procedure for measurement of nasal potential difference: an outcome measure in multicenter cystic fibrosis clinical trials. Pediatr Pulmonol 2004 1.64
17 Classifying severity of cystic fibrosis lung disease using longitudinal pulmonary function data. Am J Respir Crit Care Med 2006 1.63
18 Relationship between inhaled corticosteroid therapy and rate of lung function decline in children with cystic fibrosis. J Pediatr 2008 1.60
19 Sputum biomarkers of inflammation in cystic fibrosis lung disease. Proc Am Thorac Soc 2007 1.49
20 Novel tobramycin inhalation powder in cystic fibrosis subjects: pharmacokinetics and safety. Pediatr Pulmonol 2007 1.46
21 Measuring and improving respiratory outcomes in cystic fibrosis lung disease: opportunities and challenges to therapy. J Cyst Fibros 2009 1.35
22 Association of socioeconomic status with the use of chronic therapies and healthcare utilization in children with cystic fibrosis. J Pediatr 2009 1.31
23 Shifting patterns of inhaled antibiotic use in cystic fibrosis. Pediatr Pulmonol 2008 1.30
24 Inflammation and anti-inflammatory therapies for cystic fibrosis. Clin Chest Med 2007 1.29
25 Emergence of linezolid-resistant Staphylococcus aureus after prolonged treatment of cystic fibrosis patients in Cleveland, Ohio. Antimicrob Agents Chemother 2011 1.26
26 Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national sample. Qual Life Res 2011 1.21
27 Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis. Pediatr Pulmonol 2007 1.19
28 The efficacy and safety of meropenem and tobramycin vs ceftazidime and tobramycin in the treatment of acute pulmonary exacerbations in patients with cystic fibrosis. Chest 2005 1.13
29 Prolonged inflammatory response to acute Pseudomonas challenge in interleukin-10 knockout mice. Am J Respir Crit Care Med 2002 1.08
30 Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data. J Cyst Fibros 2014 1.06
31 Coefficients of fat and nitrogen absorption in healthy subjects and individuals with cystic fibrosis. J Pediatr Pharmacol Ther 2007 1.04
32 A pipeline of therapies for cystic fibrosis. Semin Respir Crit Care Med 2009 1.03
33 Treatment complexity in cystic fibrosis: trends over time and associations with site-specific outcomes. J Cyst Fibros 2013 1.00
34 Year-to-year changes in lung function in individuals with cystic fibrosis. J Cyst Fibros 2010 1.00
35 Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis. Pediatr Pulmonol 2010 0.99
36 Socioeconomic status and the likelihood of antibiotic treatment for signs and symptoms of pulmonary exacerbation in children with cystic fibrosis. J Pediatr 2011 0.99
37 Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis. J Cyst Fibros 2012 0.98
38 Trends in the clinical characteristics of the U.S. cystic fibrosis patient population from 1995 to 2005. Pediatr Pulmonol 2008 0.97
39 Impact of pregnancy on women with cystic fibrosis. Chest 2006 0.97
40 An international randomized multicenter comparison of nasal potential difference techniques. Chest 2010 0.96
41 Infant care patterns at epidemiologic study of cystic fibrosis sites that achieve superior childhood lung function. Pediatrics 2007 0.95
42 Lung function decline from adolescence to young adulthood in cystic fibrosis. Pediatr Pulmonol 2011 0.95
43 Assessing time to pulmonary function benefit following antibiotic treatment of acute cystic fibrosis exacerbations. Respir Res 2010 0.94
44 A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis. Pediatr Pulmonol 2002 0.94
45 Erratum to: Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national, US sample. Qual Life Res 2012 0.93
46 Oral, inhaled, and intravenous antibiotic choice for treating pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol 2012 0.92
47 Multiple antibiotic-resistant Pseudomonas aeruginosa and lung function decline in patients with cystic fibrosis. J Cyst Fibros 2012 0.92
48 Anti-inflammatory medications for cystic fibrosis lung disease: selecting the most appropriate agent. Treat Respir Med 2005 0.92
49 Anti-PcrV antibody in cystic fibrosis: a novel approach targeting Pseudomonas aeruginosa airway infection. Pediatr Pulmonol 2013 0.91
50 Inflammatory mediators in CF patients. Methods Mol Med 2002 0.90
51 Study of a novel pancreatic enzyme replacement therapy in pancreatic insufficient subjects with cystic fibrosis. J Pediatr 2006 0.89
52 Anti-inflammatory therapies for cystic fibrosis-related lung disease. Clin Rev Allergy Immunol 2008 0.89
53 Pulmonary exacerbations in cystic fibrosis: young children with characteristic signs and symptoms. Pediatr Pulmonol 2012 0.88
54 Murine models of CF airway infection and inflammation. Methods Mol Med 2002 0.87
55 Safety and preliminary clinical activity of a novel pancreatic enzyme preparation in pancreatic insufficient cystic fibrosis patients. Pancreas 2006 0.87
56 Changing thresholds and incidence of antibiotic treatment of cystic fibrosis pulmonary exacerbations, 1995-2005. J Cyst Fibros 2012 0.86
57 Risk factors for onset of persistent respiratory symptoms in children with cystic fibrosis. Pediatr Pulmonol 2012 0.86
58 Relation of sweat chloride concentration to severity of lung disease in cystic fibrosis. Pediatr Pulmonol 2004 0.86
59 Probability of treatment following acute decline in lung function in children with cystic fibrosis is related to baseline pulmonary function. J Pediatr 2013 0.86
60 Beta 2 adrenergic receptor polymorphisms in cystic fibrosis. Pediatr Pulmonol 2005 0.85
61 Antibiotic and anti-inflammatory therapies for cystic fibrosis. Cold Spring Harb Perspect Med 2013 0.84
62 Early childhood wheezing is associated with lower lung function in cystic fibrosis. Pediatr Pulmonol 2013 0.83
63 Non-viral gene transfer therapy for cystic fibrosis. Expert Opin Biol Ther 2003 0.83
64 Implementation of the first worldwide quality assurance program for cystic fibrosis multiple mutation detection in population-based screening. Clin Chim Acta 2011 0.82
65 Normalized T1 magnetic resonance imaging for assessment of regional lung function in adult cystic fibrosis patients--a cross-sectional study. PLoS One 2013 0.81
66 Inhaled hypertonic saline in infants and young children with cystic fibrosis. JAMA 2012 0.80
67 The role of inhaled corticosteroids in the management of cystic fibrosis. Paediatr Drugs 2009 0.80
68 Standard care versus protocol based therapy for new onset Pseudomonas aeruginosa in cystic fibrosis. Pediatr Pulmonol 2013 0.79
69 Location and duration of treatment of cystic fibrosis respiratory exacerbations. Am J Respir Crit Care Med 2011 0.78
70 A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatr Pulmonol 2011 0.78
71 Tobramycin inhalation powder manufactured by improved process in cystic fibrosis: the randomized EDIT trial. Curr Med Res Opin 2013 0.77
72 Long-term effects of pregnancy and motherhood on disease outcomes of women with cystic fibrosis. Ann Am Thorac Soc 2013 0.77
73 Antibiotic treatment of signs and symptoms of pulmonary exacerbations: a comparison by care site. Pediatr Pulmonol 2014 0.75
74 Rapid intravenous desensitization to colistin. Ann Allergy Asthma Immunol 2012 0.75
75 Liver involvement in the Hispanic population of North America with cystic fibrosis. J Pediatr Gastroenterol Nutr 2014 0.75