Michael W Konstan

Summary

Affiliation: Case Western Reserve University
Country: USA

Publications

  1. doi request reprint Ibuprofen therapy for cystic fibrosis lung disease: revisited
    Michael W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine and Rainbow Babies and Children s Hospital, Cleveland, Ohio 44106, USA
    Curr Opin Pulm Med 14:567-73. 2008
  2. pmc Clinical use of Ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis
    Michael W Konstan
    Rainbow Babies and Children s Hospital, 11100 Euclid Avenue, Cleveland, OH 44106, USA
    Am J Respir Crit Care Med 176:1084-9. 2007
  3. ncbi request reprint Ultrase MT12 and Ultrase MT20 in the treatment of exocrine pancreatic insufficiency in cystic fibrosis: safety and efficacy
    M W Konstan
    Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
    Aliment Pharmacol Ther 20:1365-71. 2004
  4. ncbi request reprint Characterizing aggressiveness and predicting future progression of CF lung disease
    Michael W Konstan
    Department of Pediatrics, Rainbow Babies and Children s Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    J Cyst Fibros 8:S15-9. 2009
  5. ncbi request reprint Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis
    Michael W Konstan
    Department of Pediatrics, Rainbow Babies and Children s Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    J Pediatr 151:134-9, 139.e1. 2007
  6. ncbi request reprint Trends in the use of routine therapies in cystic fibrosis: 1995-2005
    Michael W Konstan
    Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 45:1167-72. 2010
  7. ncbi request reprint Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis
    Michael W Konstan
    Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 46:545-53. 2011
  8. pmc Risk factors for rate of decline in FEV1 in adults with cystic fibrosis
    Michael W Konstan
    Case Western Reserve University School of Medicine and Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
    J Cyst Fibros 11:405-11. 2012
  9. ncbi request reprint Pulmonary exacerbations in cystic fibrosis: young children with characteristic signs and symptoms
    Warren E Regelmann
    Department of Pediatrics, University of Minnesota, Minneapolis, MN 55455, USA
    Pediatr Pulmonol 48:649-57. 2013
  10. ncbi request reprint Pulmonary outcome prediction (POP) tools for cystic fibrosis patients
    Donald R VanDevanter
    Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 45:1156-66. 2010

Detail Information

Publications66

  1. doi request reprint Ibuprofen therapy for cystic fibrosis lung disease: revisited
    Michael W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine and Rainbow Babies and Children s Hospital, Cleveland, Ohio 44106, USA
    Curr Opin Pulm Med 14:567-73. 2008
    ..Additional clinical studies were advocated to better assess the risk-benefit profile. The results of several studies have been published within the last couple of years...
  2. pmc Clinical use of Ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis
    Michael W Konstan
    Rainbow Babies and Children s Hospital, 11100 Euclid Avenue, Cleveland, OH 44106, USA
    Am J Respir Crit Care Med 176:1084-9. 2007
    ..High-dose ibuprofen in a 4-year controlled trial slowed FEV(1) decline in young subjects with cystic fibrosis, but the effectiveness of ibuprofen has not been assessed in a large group of patients treated clinically with this therapy...
  3. ncbi request reprint Ultrase MT12 and Ultrase MT20 in the treatment of exocrine pancreatic insufficiency in cystic fibrosis: safety and efficacy
    M W Konstan
    Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
    Aliment Pharmacol Ther 20:1365-71. 2004
    ..It is recognized that patients experience variations in clinical response to different brands of enzymes. This has prompted the US Food and Drug Administration to require that enzyme supplements be subjected to New Drug Applications...
  4. ncbi request reprint Characterizing aggressiveness and predicting future progression of CF lung disease
    Michael W Konstan
    Department of Pediatrics, Rainbow Babies and Children s Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    J Cyst Fibros 8:S15-9. 2009
    ....
  5. ncbi request reprint Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis
    Michael W Konstan
    Department of Pediatrics, Rainbow Babies and Children s Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    J Pediatr 151:134-9, 139.e1. 2007
    ..To characterize the rate of decline of forced expiratory volume in 1 second (FEV(1)) in children and adolescents with cystic fibrosis and to identify and compare risk factors associated with FEV(1) decline...
  6. ncbi request reprint Trends in the use of routine therapies in cystic fibrosis: 1995-2005
    Michael W Konstan
    Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 45:1167-72. 2010
    ..Notable increases in use of therapies, particularly of inhaled therapies, suggest that overall patient treatment burden must have risen correspondingly...
  7. ncbi request reprint Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis
    Michael W Konstan
    Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 46:545-53. 2011
    ..We assessed the relationship of dornase alpha use and FEV(1) decline using the Epidemiologic Study of Cystic Fibrosis (ESCF)...
  8. pmc Risk factors for rate of decline in FEV1 in adults with cystic fibrosis
    Michael W Konstan
    Case Western Reserve University School of Medicine and Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
    J Cyst Fibros 11:405-11. 2012
    ..Previously we assessed risk factors for FEV(1) decline in children and adolescents using the Epidemiologic Study of Cystic Fibrosis (J Pediatr 2007;151:134-139); the current study assessed risk factors in adults...
  9. ncbi request reprint Pulmonary exacerbations in cystic fibrosis: young children with characteristic signs and symptoms
    Warren E Regelmann
    Department of Pediatrics, University of Minnesota, Minneapolis, MN 55455, USA
    Pediatr Pulmonol 48:649-57. 2013
    ....
  10. ncbi request reprint Pulmonary outcome prediction (POP) tools for cystic fibrosis patients
    Donald R VanDevanter
    Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 45:1156-66. 2010
    ..Loss of lung function in patients with cystic fibrosis (CF) is associated with increased mortality and varies between individuals and over time. Predicting this decline could improve patient management...
  11. doi request reprint Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort
    Margaret Rosenfeld
    Department of Pediatrics, Seattle Children s Hospital and University of Washington, Seattle, Washington, USA
    Pediatr Pulmonol 45:934-44. 2010
    ..The EPIC Observational Study is an ongoing prospective cohort study investigating risk factors for and clinical outcomes associated with early Pseudomonas aeruginosa (Pa) acquisition in young children with cystic fibrosis (CF)...
  12. ncbi request reprint Inflammation and anti-inflammatory therapies for cystic fibrosis
    James F Chmiel
    Division of Pediatric Pulmonology, Department of Pediatrics, Case Western Reserve University School of Medicine, 11100 Euclid Avenue, Cleveland, OH 44106, USA
    Clin Chest Med 28:331-46. 2007
    ..This article reviews the current state of the art of anti-inflammatory therapy in cystic fibrosis and introduces clinical trials that are underway...
  13. ncbi request reprint Presence of methicillin resistant Staphylococcus aureus in respiratory cultures from cystic fibrosis patients is associated with lower lung function
    Clement L Ren
    Department of Pediatrics, University of Rochester, Rochester, New York 14642, USA
    Pediatr Pulmonol 42:513-8. 2007
    ..Similar results were seen in patients >or=18 years old. The results of our study highlight the growing clinical impact of MRSA infections in CF patients...
  14. ncbi request reprint Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution
    Michael W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA
    Hum Gene Ther 15:1255-69. 2004
    ..In conclusion, compacted DNA nanoparticles can be safely administered to the nares of CF subjects, with evidence of vector gene transfer and partial NPD correction...
  15. ncbi request reprint Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis
    Ronald L Gibson
    Department of Pediatrics, University of Washington Children s Hospital and Regional Medical Center, Seattle, WA 98105 0371, USA
    Pediatr Pulmonol 42:610-23. 2007
    ..Early intervention strategies include tobramycin solution for inhalation (TSI), which can eradicate lower airway Pa from cultures obtained at the end of 28 days of treatment in young children...
  16. doi request reprint Relationship between inhaled corticosteroid therapy and rate of lung function decline in children with cystic fibrosis
    Clement L Ren
    Department of Pediatrics, University of Rochester, Rochester, NY, USA
    J Pediatr 153:746-51. 2008
    ....
  17. doi request reprint Shifting patterns of inhaled antibiotic use in cystic fibrosis
    Samuel M Moskowitz
    Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington 98195 7740, USA
    Pediatr Pulmonol 43:874-81. 2008
    ..Our working hypothesis was that a shift from acute to chronic use of inhaled antibiotics has coincided with increased prevalence of use among CF patients...
  18. ncbi request reprint Genetic modifiers of lung disease in cystic fibrosis
    Mitchell L Drumm
    Department of Pediatrics, Case Western Reserve University, Cleveland, USA
    N Engl J Med 353:1443-53. 2005
    ..Polymorphisms in genes other than the cystic fibrosis transmembrane conductance regulator (CFTR) gene may modify the severity of pulmonary disease in patients with cystic fibrosis...
  19. ncbi request reprint The efficacy and safety of meropenem and tobramycin vs ceftazidime and tobramycin in the treatment of acute pulmonary exacerbations in patients with cystic fibrosis
    Jeffrey L Blumer
    Rainbow Babies and Children s Hospital, Case Western Reserve University School of Medicine, Cleveland, OH, USA
    Chest 128:2336-46. 2005
    ....
  20. ncbi request reprint Infant care patterns at epidemiologic study of cystic fibrosis sites that achieve superior childhood lung function
    Raj Padman
    Department of Pediatrics, Alfred I duPont Hospital for Children, Nemours Children s Clinic, Wilmington, DE 19899, USA
    Pediatrics 119:e531-7. 2007
    ..The aim of this study was to assess whether patterns of care for infants at cystic fibrosis sites with superior average lung function in 6- to 12-year-old children showed any differences from those at the lowest outcome sites...
  21. pmc Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial
    Michael W Konstan
    Rainbow Babies and Children s Hospital, Case Western Reserve University School of Medicine, Cleveland, OH, USA
    J Cyst Fibros 10:54-61. 2011
    ....
  22. pmc Antibiotic and anti-inflammatory therapies for cystic fibrosis
    James F Chmiel
    Department of Pediatrics, Case Western Reserve University School of Medicine, Rainbow Babies and Children s Hospital, Cleveland, Ohio 44106
    Cold Spring Harb Perspect Med 3:a009779. 2013
    ..It is likely that antibiotics and anti-inflammatory drugs will remain an important part of the maintenance regimen for CF in the foreseeable future. Current and future antibiotic and anti-inflammatory therapies for CF are reviewed. ..
  23. ncbi request reprint Prolonged inflammatory response to acute Pseudomonas challenge in interleukin-10 knockout mice
    James F Chmiel
    Department of Pediatrics, Rainbow Babies and Children s Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    Am J Respir Crit Care Med 165:1176-81. 2002
    ..These data suggest that IL-10 deficiency contributes to prolonged inflammatory responses early in CF, when infection may be transient...
  24. pmc Association between pulmonary function and sputum biomarkers in cystic fibrosis
    Nicole Mayer-Hamblett
    Department of Pediatrics, University of Washington, 4800 Sand Point Way N E, Box 5371, Seattle, WA 98105 0371, USA
    Am J Respir Crit Care Med 175:822-8. 2007
    ..Validation of these biomarkers as correlates of disease severity is a key step for their application...
  25. pmc Classifying severity of cystic fibrosis lung disease using longitudinal pulmonary function data
    Mark D Schluchter
    Department of Pediatrics, Case Western Reserve University, Cleveland, OH 44106 4945, USA
    Am J Respir Crit Care Med 174:780-6. 2006
    ..One type of genetic association study design compares polymorphisms in patients at extremes of phenotype, requiring accurate classification of pulmonary disease at varying ages...
  26. ncbi request reprint Effect of ibuprofen on neutrophil migration in vivo in cystic fibrosis and healthy subjects
    Michael W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine, Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
    J Pharmacol Exp Ther 306:1086-91. 2003
    ....
  27. pmc Measuring and improving respiratory outcomes in cystic fibrosis lung disease: opportunities and challenges to therapy
    Edith T Zemanick
    Department of Pediatrics, University of Colorado Denver, 13123 E 16th Avenue, Aurora, CO 80045, USA
    J Cyst Fibros 9:1-16. 2010
    ..This review highlights emerging treatments, obstacles to optimizing outcomes, and key future directions for research...
  28. pmc Assessing time to pulmonary function benefit following antibiotic treatment of acute cystic fibrosis exacerbations
    Donald R VanDevanter
    Case Western Reserve University School of Medicine, Cleveland, OH, USA
    Respir Res 11:137. 2010
    ..However, no prospective studies have identified an optimal antibiotic treatment duration and this lack of objective data has been identified as an area of concern and interest...
  29. ncbi request reprint Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis
    Ronald L Gibson
    Department of Pediatrics, Children s Hospital and Regional Medical Center University of Washington, Seattle, Washington 98105 0371, USA
    Am J Respir Crit Care Med 167:841-9. 2003
    ..We conclude that 28 days of tobramycin solution for inhalation of 300 mg twice daily is safe and effective for significant reduction of lower airway Pa density in young children with cystic fibrosis...
  30. pmc Sputum biomarkers of inflammation in cystic fibrosis lung disease
    Scott D Sagel
    Department of Pediatrics, The Children s Hospital and University of Colorado at Denver and Health Sciences Center, Denver, Colorado 80218, USA
    Proc Am Thorac Soc 4:406-17. 2007
    ..In the future, pulmonary biomarkers will likely be useful in predicting disease progression, indicating the onset and resolution of a pulmonary exacerbation, and assessing response to current therapies or candidate therapeutics...
  31. pmc 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
    J P Clancy
    Department of Pediatrics, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA
    Thorax 67:12-8. 2012
    ..VX-809, a cystic fibrosis transmembrane conductance regulator (CFTR) modulator, has been shown to increase the cell surface density of functional F508del-CFTR in vitro...
  32. pmc Effect of dornase alfa on inflammation and lung function: potential role in the early treatment of cystic fibrosis
    Michael W Konstan
    Case Western Reserve University School of Medicine and Rainbow Babies and Children s Hospital, 11100 Euclid Avenue, Cleveland, OH 44106, USA
    J Cyst Fibros 11:78-83. 2012
    ..These encouraging results, possibly linked to indirect effects on inflammation, suggest a greater role for dornase alfa therapy in the early treatment of CF, where it may help preserve lung function and potentially extend survival...
  33. ncbi request reprint The role of inflammation in the pathophysiology of CF lung disease
    James F Chmiel
    Department of Pediatrics, Case Western Reserve University School of Medicine, Division of Pediatric Pulmonology, Rainbow Babies and Children s Hospital, Cleveland, OH, USA
    Clin Rev Allergy Immunol 23:5-27. 2002
    ....
  34. ncbi request reprint Jointly modelling the relationship between survival and pulmonary function in cystic fibrosis patients
    Mark D Schluchter
    Department of Pediatrics, Rainbow Babies and Children s Hospital, Case Western Reserve University, Cleveland, OH, USA
    Stat Med 21:1271-87. 2002
    ..s.a...
  35. ncbi request reprint Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis
    Michael W Konstan
    Department of Pediatrics, Rainbow Babies and Children s Hospital and Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    J Pediatr 142:624-30. 2003
    ..To determine the relation of growth and nutritional status to pulmonary function in young children with cystic fibrosis (CF)...
  36. doi request reprint Anti-inflammatory therapies for cystic fibrosis-related lung disease
    David P Nichols
    Department of Pediatrics, Case Western Reserve University School of Medicine, Rainbow Babies and Children s Hospital, Cleveland, OH, USA
    Clin Rev Allergy Immunol 35:135-53. 2008
    ..Available data from studies commenced over the last two decades, which have generated both encouraging and disappointing results, are reviewed below...
  37. ncbi request reprint Relation of sweat chloride concentration to severity of lung disease in cystic fibrosis
    Pamela B Davis
    Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA
    Pediatr Pulmonol 38:204-9. 2004
    ..These data suggest that, by itself, sweat chloride concentration does not necessarily predict a milder pulmonary course in patients with cystic fibrosis...
  38. ncbi request reprint Changing thresholds and incidence of antibiotic treatment of cystic fibrosis pulmonary exacerbations, 1995-2005
    Donald R VanDevanter
    Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA
    J Cyst Fibros 12:332-7. 2013
    ..Increased chronic therapy use and improved cystic fibrosis (CF) patient health should be accompanied by reduced pulmonary exacerbation-associated antibiotic treatment incidence...
  39. ncbi request reprint Beta 2 adrenergic receptor polymorphisms in cystic fibrosis
    Meeghan A Hart
    Department of Pediatrics, Case Western Reserve University and Rainbow Babies and Children s Hospital, Cleveland, Ohio 44106, USA
    Pediatr Pulmonol 39:544-50. 2005
    ..These data are consistent with variants of the ADRB2 gene having different responses to bronchodilator, but the long-term effects, if any, are not apparent over a 5-year period...
  40. ncbi request reprint Anti-inflammatory medications for cystic fibrosis lung disease: selecting the most appropriate agent
    James F Chmiel
    Department of Pediatrics, Case Western Reserve University School of Medicine, Rainbow Babies and Children s Hospital, Cleveland, OH 44106, USA
    Treat Respir Med 4:255-73. 2005
    ..Until those therapies aimed at repairing the basic defect are realized, limiting the effects of the inflammatory process will be important in slowing the decline in lung function and thus prolonging survival in patients with CF...
  41. pmc The role of inhaled corticosteroids in the management of cystic fibrosis
    Kristie R Ross
    Department of Pediatrics, Division of Pulmonology, Case Western Reserve University School of Medicine, Rainbow Babies and Children s Hospital, Cleveland, Ohio, USA
    Paediatr Drugs 11:101-13. 2009
    ..This review provides an overview of the inflammatory response in CF, the mechanisms of action of corticosteroids, the safety of ICS, and the literature relevant to the use of ICS in CF...
  42. pmc Clinical use of tobramycin inhalation solution (TOBIĀ®) shows sustained improvement in FEV1 in cystic fibrosis
    Michael W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine, Rainbow Babies and Children s Hospital, Cleveland, Ohio
    Pediatr Pulmonol 49:529-36. 2014
    ..Using data from the Epidemiologic Study of CF (ESCF), we assessed the change in level and trend of FEV1 % predicted (pred) over a 2-year period associated with initiation of TIS during routine clinical practice...
  43. ncbi request reprint Tobramycin inhalation powder for P. aeruginosa infection in cystic fibrosis: the EVOLVE trial
    Michael W Konstan
    Rainbow Babies and Children s Hospital, and Case Western Reserve University School of Medicine, Cleveland, Ohio
    Pediatr Pulmonol 46:230-8. 2011
    ..In conclusion, tobramycin inhalation powder was effective and well tolerated in CF patients, and may offer an important treatment option to decrease the treatment burden of CF pseudomonas lung infections...
  44. ncbi request reprint Pharmacological approaches for the discovery and development of new anti-inflammatory agents for the treatment of cystic fibrosis
    Michael W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine and Rainbow Babies and Children s Hospital, 11100 Euclid Avenue, Cleveland, OH 44106, USA
    Adv Drug Deliv Rev 54:1409-23. 2002
    ....
  45. ncbi request reprint Association between respiratory tract methicillin-resistant Staphylococcus aureus and survival in cystic fibrosis
    Elliott C Dasenbrook
    Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, Case Western Reserve University School of Medicine and University Hospitals Case Medical Center, Cleveland, Ohio 44106 5067, USA
    JAMA 303:2386-92. 2010
    ..Because the time between infection with bacteria in CF and death can be decades, observational studies with long periods of follow-up are well suited to address the current gap in knowledge...
  46. ncbi request reprint Inflammatory mediators in CF patients
    Jay B Hilliard
    Department of Pediatrics, Rainbow Babies and Children s Hospital, Case Western Reserve University School of Medicine, Cleveland, OH, USA
    Methods Mol Med 70:409-31. 2002
  47. ncbi request reprint Non-viral gene transfer therapy for cystic fibrosis
    Assem G Ziady
    Department of Pediatrics, Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USA
    Expert Opin Biol Ther 3:449-58. 2003
    ..modifying the plasmid DNA to reduce inflammatory CpG sequences and enhance intensity, duration and tissue specificity of expression, and iv). modification of the complexes to improve nuclear access...
  48. pmc Efficacy and Safety of a New Formulation of Pancrelipase (Ultrase MT20) in the Treatment of Malabsorption in Exocrine Pancreatic Insufficiency in Cystic Fibrosis
    Michael W Konstan
    Cystic Fibrosis Center, Rainbow Babies and Children s Hospital and Case Western Reserve University School of Medicine, 11100 Euclid Avenue, Cleveland, OH 44106, USA
    Gastroenterol Res Pract 2010:898193. 2010
    ..Conclusions. This pancrelipase is a safe and effective treatment for malabsorption associated with exocrine PI in patients with CF...
  49. pmc Emergence of linezolid-resistant Staphylococcus aureus after prolonged treatment of cystic fibrosis patients in Cleveland, Ohio
    Andrea Endimiani
    Department of Medicine, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Antimicrob Agents Chemother 55:1684-92. 2011
    ..Emergence of LRSA is a serious concern for CF patients who undergo prolonged courses of LZD therapy...
  50. ncbi request reprint Safety and early treatment effects of the CXCR2 antagonist SB-656933 in patients with cystic fibrosis
    Richard B Moss
    Center for Excellence in Pulmonary Biology, Department of Pediatrics, Stanford University, Palo Alto, CA, USA
    J Cyst Fibros 12:241-8. 2013
    ..The objective of this study was to evaluate the safety, tolerability and pharmacodynamics of SB-656933, an oral CXCR2 antagonist...
  51. pmc Normalized T1 magnetic resonance imaging for assessment of regional lung function in adult cystic fibrosis patients--a cross-sectional study
    Elliott C Dasenbrook
    Department of Pediatrics, Case Western Reserve University School of Medicine, University Hospitals Case Medical Center and Rainbow Babies and Children s Hospital, Cleveland, Ohio, United States of America Department of Medicine, Case Western Reserve University School of Medicine, University Hospitals Case Medical Center and Rainbow Babies and Children s Hospital, Cleveland, Ohio, United States of America
    PLoS ONE 8:e73286. 2013
    ..We developed a rapid normalized T1 MRI technique to detect regional lung disease in early-stage CF patients...
  52. ncbi request reprint Murine models of CF airway infection and inflammation
    James F Chmiel
    Department of Pediatrics, Rainbow Babies and Children s Hospital, Case Western Reserve University School of Medicine, Cleveland, OH, USA
    Methods Mol Med 70:495-515. 2002
  53. ncbi request reprint Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis
    Richard C Ahrens
    Department of Pediatrics, University of Iowa, Iowa City, Iowa 52242 1083, USA
    Pediatr Pulmonol 33:142-50. 2002
    ..Standardization of measurement protocols, careful operator training and certification, and ongoing monitoring of individual operator performance may help to improve reliability in multicenter trials...
  54. ncbi request reprint Efficacy of the Flutter device for airway mucus clearance in patients with cystic fibrosis
    M W Konstan
    Leroy W Matthews Cystic Fibrosis Center, Rainbow Babies and Children s Hospital, Cleveland, OH 44106
    J Pediatr 124:689-93. 1994
    ..For hospitalized patients, elimination of the need for a therapist could reduce health care costs. Long-term studies of the use of the Flutter seem justified to determine its effects on pulmonary function and outcome...
  55. ncbi request reprint The use of anti-inflammatory medications in cystic fibrosis: trends and physician attitudes
    C M Oermann
    Department of Pediatrics, Baylor College of Medicine, Houston, TX 77030, USA
    Chest 115:1053-8. 1999
    ..Inhaled corticosteroids (ICS) have not been adequately studied. Little is known regarding use trends and physician attitudes toward these drugs...
  56. ncbi request reprint Patterns of medical practice in cystic fibrosis: part I. Evaluation and monitoring of health status of patients. Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis
    M W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 28:242-7. 1999
    ..An increase in the utilization of healthcare resources was observed in patients with more severe disease. This information will help to establish benchmarks for future quality assessment programs...
  57. ncbi request reprint Patterns of medical practice in cystic fibrosis: part II. Use of therapies. Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis
    M W Konstan
    Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA
    Pediatr Pulmonol 28:248-54. 1999
    ..However, there was no gender difference for this group of therapies on pulmonary or nutritional status...
  58. ncbi request reprint Study of a novel pancreatic enzyme replacement therapy in pancreatic insufficient subjects with cystic fibrosis
    Drucy Borowitz
    Women and Children s Hospital of Buffalo, Division of Pediatric Pulmonology, State University of New York at Buffalo, Buffalo, NY 14222, USA
    J Pediatr 149:658-662. 2006
    ....
  59. ncbi request reprint Safety and preliminary clinical activity of a novel pancreatic enzyme preparation in pancreatic insufficient cystic fibrosis patients
    Drucy Borowitz
    CF Therapeutics Development Network, Seattle, Wash, USA
    Pancreas 32:258-63. 2006
    ..We conducted a phase 1 study of a novel pancreatic enzyme product, TheraCLEC-Total (TCT), a proprietary formulation of microbial-derived lipase, protease, and amylase, to determine its safety and preliminary efficacy in cystic fibrosis...
  60. ncbi request reprint A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis
    Nael A McCarty
    Department of Physiology, Emory University, Atlanta, Georgia, USA
    Pediatr Pulmonol 33:90-8. 2002
    ..The positive safety and pharmacokinetic findings of this study support continued development of CPX as a potential therapeutic for CF...
  61. ncbi request reprint Pulmonary exacerbations in cystic fibrosis
    Harvey R Rabin
    Foothills Medical Center and University of Calgary, Calgary, Alberta, Canada
    Pediatr Pulmonol 37:400-6. 2004
    ..The reproducibility of the model over time was confirmed by application to a subsequent set of data. This model has potential for use as an outcome measure in clinical trials, and to assist in treatment decisions for individual patients...
  62. doi request reprint Trends in the clinical characteristics of the U.S. cystic fibrosis patient population from 1995 to 2005
    Donald R VanDevanter
    ICON Clinical Research, San Francisco, California, USA
    Pediatr Pulmonol 43:739-44. 2008
    ..Additionally, changes in these variables over the past decade may provide insight into the improving health of the CF population...
  63. ncbi request reprint Impact of pregnancy on women with cystic fibrosis
    Ann H McMullen
    University of Rochester School of Nursing, 601 Elmwood Avenue, Rochester, NY 14642, USA
    Chest 129:706-11. 2006
    ..Concern exists among care providers about the impact of pregnancy on the health of women with CF...
  64. ncbi request reprint Standardized procedure for measurement of nasal potential difference: an outcome measure in multicenter cystic fibrosis clinical trials
    Thomas A Standaert
    University of Washington, Seattle, Washington, USA
    Pediatr Pulmonol 37:385-92. 2004
    ..Valid and consistent results can be attained with trained operators and attention to technical details. These data demonstrate the procedure to be sufficient for multicenter studies in the CF Foundation network...
  65. ncbi request reprint Factors influencing outcomes in cystic fibrosis: a center-based analysis
    Charles Johnson
    Genentech, 1 DNA Way, Mail Stop 59, South San Francisco, CA 94080, USA
    Chest 123:20-7. 2003
    ..Guidelines for managing cystic fibrosis (CF) patients have been widely circulated, but little is known about the variations in practice between sites and their association with outcomes...
  66. ncbi request reprint Novel tobramycin inhalation powder in cystic fibrosis subjects: pharmacokinetics and safety
    David E Geller
    Nemours Children s Clinic, Orlando, Florida 32806, USA
    Pediatr Pulmonol 42:307-13. 2007
    ..The development of dry powder inhaled antibiotics may represent an important advance in the treatment of chronic lung infections...