Michael P Boyle

Summary

Affiliation: Johns Hopkins University
Country: USA

Publications

  1. ncbi request reprint A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial
    Michael P Boyle
    Johns Hopkins Medical Institutions, Baltimore, MD, USA Electronic address
    Lancet Respir Med 2:527-38. 2014
  2. doi request reprint A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect
    Michael P Boyle
    Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care Medicine, Baltimore, MD, USA Electronic address
    Lancet Respir Med 1:158-63. 2013
  3. pmc Assessing disease disclosure in adults with cystic fibrosis: the Adult Data for Understanding Lifestyle and Transitions (ADULT) survey Disclosure of disease in adults with cystic fibrosis
    Avani C Modi
    Cincinnati Children s Hospital Medical Center, Center for the Promotion of the Treatment Adherence and Self Management, 3333 Burnet Ave, MLC 7039, Cincinnati, OH, 45229, USA
    BMC Pulm Med 10:46. 2010
  4. ncbi request reprint Adult cystic fibrosis
    Michael P Boyle
    Johns Hopkins Adult Cystic Fibrosis Program, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    JAMA 298:1787-93. 2007
  5. pmc Strategies for identifying modifier genes in cystic fibrosis
    Michael P Boyle
    Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Proc Am Thorac Soc 4:52-7. 2007
  6. ncbi request reprint Update on maintaining bone health in cystic fibrosis
    Michael P Boyle
    The Johns Hopkins University School of Medicine, 1830 E Monument Street, 5th Floor, Baltimore, MD 21205, USA
    Curr Opin Pulm Med 12:453-8. 2006
  7. pmc Membrane-associated heparan sulfate is not required for rAAV-2 infection of human respiratory epithelia
    Michael P Boyle
    Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore MD 21205, USA
    Virol J 3:29. 2006
  8. pmc Failure of high-dose ergocalciferol to correct vitamin D deficiency in adults with cystic fibrosis
    Michael P Boyle
    Johns Hopkins Adult CF Program, 1830 East Monument Street, 5th Floor, Baltimore, MD 21205, USA
    Am J Respir Crit Care Med 172:212-7. 2005
  9. ncbi request reprint Nonclassic cystic fibrosis and CFTR-related diseases
    Michael P Boyle
    Johns Hopkins Adult CF Program, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Curr Opin Pulm Med 9:498-503. 2003
  10. ncbi request reprint A multicenter study of the effect of solution temperature on nasal potential difference measurements
    Michael P Boyle
    Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Chest 124:482-9. 2003

Research Grants

  1. Genetic Modifiers of Cystic Fibrosis
    Michael Boyle; Fiscal Year: 2007

Detail Information

Publications38

  1. ncbi request reprint A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial
    Michael P Boyle
    Johns Hopkins Medical Institutions, Baltimore, MD, USA Electronic address
    Lancet Respir Med 2:527-38. 2014
    ....
  2. doi request reprint A new era in the treatment of cystic fibrosis: correction of the underlying CFTR defect
    Michael P Boyle
    Johns Hopkins University School of Medicine, Division of Pulmonary and Critical Care Medicine, Baltimore, MD, USA Electronic address
    Lancet Respir Med 1:158-63. 2013
    ..With analysis of continuing trials and available patient registries, here we assess mutation types and the number and geographical distribution of patients who are likely to benefit from CFTR-correcting treatment. ..
  3. pmc Assessing disease disclosure in adults with cystic fibrosis: the Adult Data for Understanding Lifestyle and Transitions (ADULT) survey Disclosure of disease in adults with cystic fibrosis
    Avani C Modi
    Cincinnati Children s Hospital Medical Center, Center for the Promotion of the Treatment Adherence and Self Management, 3333 Burnet Ave, MLC 7039, Cincinnati, OH, 45229, USA
    BMC Pulm Med 10:46. 2010
    ..This study assessed rates of disclosure and its perceived impact on relationships using the Adult Data for Understanding Lifestyle and Transitions (ADULT) online survey...
  4. ncbi request reprint Adult cystic fibrosis
    Michael P Boyle
    Johns Hopkins Adult Cystic Fibrosis Program, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    JAMA 298:1787-93. 2007
    ....
  5. pmc Strategies for identifying modifier genes in cystic fibrosis
    Michael P Boyle
    Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Proc Am Thorac Soc 4:52-7. 2007
    ..These include single- and multicenter studies, twin and sib studies, microarray approaches, and whole genome association studies...
  6. ncbi request reprint Update on maintaining bone health in cystic fibrosis
    Michael P Boyle
    The Johns Hopkins University School of Medicine, 1830 E Monument Street, 5th Floor, Baltimore, MD 21205, USA
    Curr Opin Pulm Med 12:453-8. 2006
    ..This review summarizes the current best practices for optimizing bone health in cystic fibrosis and highlight recent findings that provide insight into the etiology of cystic fibrosis-related bone disease...
  7. pmc Membrane-associated heparan sulfate is not required for rAAV-2 infection of human respiratory epithelia
    Michael P Boyle
    Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore MD 21205, USA
    Virol J 3:29. 2006
    ..The aim of this investigation was to determine the necessity of cell membrane HSPG for efficient infection by rAAV-2...
  8. pmc Failure of high-dose ergocalciferol to correct vitamin D deficiency in adults with cystic fibrosis
    Michael P Boyle
    Johns Hopkins Adult CF Program, 1830 East Monument Street, 5th Floor, Baltimore, MD 21205, USA
    Am J Respir Crit Care Med 172:212-7. 2005
    ..Treatment guidelines for vitamin D monitoring and supplementation in cystic fibrosis (CF) have recently been developed and published by a consensus committee, but have not been prospectively tested...
  9. ncbi request reprint Nonclassic cystic fibrosis and CFTR-related diseases
    Michael P Boyle
    Johns Hopkins Adult CF Program, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Curr Opin Pulm Med 9:498-503. 2003
    ..To review the spectrum of disease caused by mutations in the cystic fibrosis (CF) gene...
  10. ncbi request reprint A multicenter study of the effect of solution temperature on nasal potential difference measurements
    Michael P Boyle
    Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Chest 124:482-9. 2003
    ..Because small changes in nasal PD-measured chloride transport are often an important component in evaluating new CF therapies, techniques to maximize sensitivity and reproducibility are essential...
  11. ncbi request reprint Effect of adeno-associated virus-specific immunoglobulin G in human amniotic fluid on gene transfer
    Michael P Boyle
    Department of Medicine, The Johns Hopkins University School of Medicine, Jefferson B1 170, 600 North Wolfe Street, Baltimore, MD 21287 8922, USA
    Hum Gene Ther 14:365-73. 2003
    ..These studies demonstrate that AF from some individuals contains AAV-specific IgG that can inhibit gene transfer...
  12. pmc N-acetylcysteine enhances cystic fibrosis sputum penetration and airway gene transfer by highly compacted DNA nanoparticles
    Jung Soo Suk
    Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21231, USA
    Mol Ther 19:1981-9. 2011
    ..Our findings suggest that a promising synthetic nanoparticle gene carrier may transfer genes substantially more effectively to lungs of CF patients if administered following adjuvant mucolytic therapy with NAC or NAC + rhDNase...
  13. pmc Highly compacted DNA nanoparticles with low MW PEG coatings: in vitro, ex vivo and in vivo evaluation
    Nicholas J Boylan
    Department of Chemical and Biomolecular Engineering, The Johns Hopkins University, 3400 N Charles St, Baltimore, MD 21218, USA
    J Control Release 157:72-9. 2012
    ..However, all DNA nanoparticle formulations were immobilized in freshly expectorated human CF sputum, likely due to inadequate PEG surface coverage...
  14. pmc Common gene therapy viral vectors do not efficiently penetrate sputum from cystic fibrosis patients
    Kaoru Hida
    Department of Biomedical Engineering, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States of America
    PLoS ONE 6:e19919. 2011
    ..Nevertheless, poor penetration of CF sputum is likely a major contributor to the ineffectiveness of viral vector based gene therapy in the lungs of CF patients observed to date...
  15. pmc Rapid transport of muco-inert nanoparticles in cystic fibrosis sputum treated with N-acetyl cysteine
    Jung Soo Suk
    Department of Biomedical Engineering, The Johns Hopkins University School of Medicine, MD, USA
    Nanomedicine (Lond) 6:365-75. 2011
    ..Here, we sought to investigate whether a common mucolytic, N-acetyl cysteine (NAC), in combination with dense PEG coatings on particles, can synergistically enhance particle penetration across fresh undiluted CF sputum...
  16. 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...
  17. ncbi request reprint A phase I trial of intranasal Moli1901 for cystic fibrosis
    Pamela L Zeitlin
    Departments of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA
    Chest 125:143-9. 2004
    ..The peptide drug Moli1901 activates an alternative chloride channel that is present in cystic fibrosis (CF) nasal and airway epithelia. Doing so bypasses the dysfunctional CF transmembrane regulator...
  18. ncbi request reprint Incidence and risk factors for multiple antibiotic-resistant Pseudomonas aeruginosa in cystic fibrosis
    Christian A Merlo
    The Johns Hopkins University School of Medicine, 1830 E Monument St Fifth Floor, Baltimore, MD 21205, USA
    Chest 132:562-8. 2007
    ..The main objective of the study was to estimate the incidence and identify risk factors for the acquisition of MARPA among individuals with CF...
  19. pmc Biodegradable polymer nanoparticles that rapidly penetrate the human mucus barrier
    Benjamin C Tang
    Department of Chemical and Biomolecular Engineering, The Johns Hopkins University, Baltimore, MD 21218, USA
    Proc Natl Acad Sci U S A 106:19268-73. 2009
    ..Biodegradable polymeric nanoparticles capable of overcoming human mucus barriers and providing sustained drug release open significant opportunities for improved drug and gene delivery at mucosal surfaces...
  20. ncbi request reprint Patients with mutations in Gsalpha have reduced activation of a downstream target in epithelial tissues due to haploinsufficiency
    Stephanie C Hsu
    Department of Pediatric Endocrinology, Johns Hopkins University, 733 North Broadway, Suite 551, Baltimore, Maryland 21205, USA
    J Clin Endocrinol Metab 92:3941-8. 2007
    ..The mechanism by which these mutations lead to the AHO phenotype has been difficult to establish due to the inaccessibility of the affected tissues...
  21. ncbi request reprint Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax
    Patrick A Flume
    Department of Medicine, Medical University of South Carolina, Charleston, SC 29425, USA
    Am J Respir Crit Care Med 182:298-306. 2010
    ..There may be intermittent pulmonary exacerbations or acute worsening of infection and obstruction, which require more intensive therapies. Hemoptysis and pneumothorax are complications commonly reported in patients with cystic fibrosis...
  22. doi request reprint Appropriate goal level for 25-hydroxyvitamin D in cystic fibrosis
    Natalie E West
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA
    Chest 140:469-74. 2011
    ..We sought to determine the appropriate goal 25OHD level in patients with CF by identifying the level below which the risk of PTH > 50 pg/mL begins to increase...
  23. pmc Lung gene therapy with highly compacted DNA nanoparticles that overcome the mucus barrier
    Jung Soo Suk
    The Center for Nanomedicine, The Johns Hopkins University School of Medicine, 400 North Broadway, Baltimore 21231, USA Department of Ophthalmology, The Johns Hopkins University School of Medicine, 400 North Broadway, Baltimore 21231, USA Department of Biomedical Engineering, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore 21205, USA
    J Control Release 178:8-17. 2014
    ..Highly compacted mucus penetrating DNA nanoparticles hold promise for lung gene therapy. ..
  24. ncbi request reprint Prevalence and risk factors for recovery of filamentous fungi in individuals with cystic fibrosis
    Christopher R Sudfeld
    Department of Epidemiology, The Johns Hopkins Bloomberg School of Public Health, 615 N Wolfe Street, 6th Floor, Baltimore, MD 21205, United States
    J Cyst Fibros 9:110-6. 2010
    ..Filamentous fungi are frequently recovered from respiratory cultures of individuals with CF...
  25. doi request reprint Persistent methicillin-resistant Staphylococcus aureus and rate of FEV1 decline in cystic fibrosis
    Elliott C Dasenbrook
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, The Johns Hopkins University School of Medicine, 1830 E Monument Street, 5th Floor, Baltimore, MD 21205, USA
    Am J Respir Crit Care Med 178:814-21. 2008
    ..The prevalence in cystic fibrosis (CF) of respiratory cultures with methicillin-resistant Staphylococcus aureus (MRSA) has dramatically increased over the last 10 years, but the effect of MRSA on FEV(1) decline in CF is unknown...
  26. pmc Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease
    Jerry M Wright
    Department of Physiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Am J Respir Cell Mol Biol 35:327-36. 2006
    ....
  27. pmc Levofloxacin pharmacokinetics in adult cystic fibrosis
    Carlton K K Lee
    Pediatrics, Pediatric Infectious Diseases, Johns Hopkins University, 600 North Wolfe St, Carnegie 180, Baltimore, MD 21287 6180, USA
    Chest 131:796-802. 2007
    ..We studied the preliminary pharmacokinetic and pharmacodynamic (PK/PD) relationship of levofloxacin in CF...
  28. pmc Variants in mannose-binding lectin and tumour necrosis factor alpha affect survival in cystic fibrosis
    Kitti Buranawuti
    McKusick Nathans Institute of Genetic Medicine, Johns Hopkins University School of Medicine Baltimore, Maryland, USA
    J Med Genet 44:209-14. 2007
    ..Patients with cystic fibrosis with the same mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene differ widely in survival suggesting other factors have a substantial role in mortality...
  29. ncbi request reprint Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate
    Pamela L Zeitlin
    Departments of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    Mol Ther 6:119-26. 2002
    ..This short-term phase I/II study demonstrates proof of principle that modulation of deltaF508 CFTR biosynthesis and trafficking is a viable therapeutic approach for cystic fibrosis...
  30. pmc Longitudinal association between medication adherence and lung health in people with cystic fibrosis
    Michelle N Eakin
    Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, MD 21224, USA
    J Cyst Fibros 10:258-64. 2011
    ..This study examined the relationship of medication adherence to frequency of pulmonary exacerbation and rate of decline in FEV(1)% predicted (FEV(1))...
  31. ncbi request reprint Modifier genes in cystic fibrosis lung disease
    Christian A Merlo
    Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Jefferson B1 170, 600 N Wolfe Street, Baltimore, MD 21205, USA
    J Lab Clin Med 141:237-41. 2003
    ..Ongoing studies designed to identify genetic modifiers of CF pulmonary phenotype may offer new insights into the pathophysiology of CF lung disease and provide leads for new CF therapeutic interventions...
  32. pmc The penetration of fresh undiluted sputum expectorated by cystic fibrosis patients by non-adhesive polymer nanoparticles
    Jung Soo Suk
    Department of Biomedical Engineering, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205, USA
    Biomaterials 30:2591-7. 2009
    ..The results also offer hope that desperately needed sputum-penetrating drug- and gene-carrier nanoparticles can be developed for CF...
  33. ncbi request reprint The association between depression, lung function, and health-related quality of life among adults with cystic fibrosis
    Kristin A Riekert
    Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 5501 Hopkins Bayview Circle, JHAAC Room 3B 35, Baltimore, MD 21224, USA
    Chest 132:231-7. 2007
    ..The objective of this study was to evaluate the relationships among lung function (ie, FEV(1) percent predicted), depressive symptoms, and HRQoL among adults with CF...
  34. ncbi request reprint Outcomes of adults with cystic fibrosis infected with antibiotic-resistant Pseudomonas aeruginosa
    Noah Lechtzin
    Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Respiration 73:27-33. 2006
    ....
  35. pmc Eradication strategy for persistent methicillin-resistant Staphylococcus aureus infection in individuals with cystic fibrosis-the PMEP trial: study protocol for a randomized controlled trial
    Mark T Jennings
    Johns Hopkins Medical Institutions, 1830 E, Monument Street, 5th Floor, Baltimore, Maryland 21205, USA
    Trials 15:223. 2014
    ..Currently, there are no conclusive studies demonstrating an effective and safe treatment protocol for persistent MRSA respiratory infection in CF...
  36. doi request reprint Key findings of the US Cystic Fibrosis Foundation's clinical practice benchmarking project
    Michael P Boyle
    Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    BMJ Qual Saf 23:i15-i22. 2014
    ..In summary, assessment of practice patterns at CF care centres with top-quintile pulmonary and nutritional outcomes provides insight into characteristic practices that may aid in optimising patient outcomes. ..
  37. ncbi request reprint So many drugs, so little time: the future challenge of cystic fibrosis care
    Michael P Boyle
    Chest 123:3-5. 2003
  38. ncbi request reprint Distal intestinal obstruction syndrome after surgery in cystic fibrosis
    Michael P Boyle
    Chest 124:2408-9. 2003

Research Grants1

  1. Genetic Modifiers of Cystic Fibrosis
    Michael Boyle; Fiscal Year: 2007
    ..Last, we will evaluate three of the strongest current CF candidate modifier genes to determine if the distribution of their functional alleles segregates with severity of lung disease in our CF clinic population. ..