Published in Chest on June 01, 2008
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med (2011) 8.73
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med (2010) 6.23
Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology (2006) 5.67
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med (2015) 5.51
Culturally appropriate storytelling to improve blood pressure: a randomized trial. Ann Intern Med (2011) 4.24
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
A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation. J Immunol (2008) 2.58
A critical role for LTA4H in limiting chronic pulmonary neutrophilic inflammation. Science (2010) 2.56
Regulation of chloride channels by protein kinase C in normal and cystic fibrosis airway epithelia. Science (1989) 2.46
Cultural tailoring for mammography and fruit and vegetable intake among low-income African-American women in urban public health centers. Prev Med (2004) 2.46
Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line. Nat Med (1997) 2.24
The ups and downs of peer review. Adv Physiol Educ (2007) 2.04
Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers. J Physiol (2005) 1.97
Microdeletion/microduplication of proximal 15q11.2 between BP1 and BP2: a susceptibility region for neurological dysfunction including developmental and language delay. Hum Genet (2011) 1.94
Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function. Am J Respir Crit Care Med (2013) 1.93
Molecular analysis expands the spectrum of phenotypes associated with GLI3 mutations. Hum Mutat (2010) 1.88
A functional anatomic defect of the cystic fibrosis airway. Am J Respir Crit Care Med (2014) 1.85
Matrix metalloproteinase 9 activity enhances host susceptibility to pulmonary infection with type A and B strains of Francisella tularensis. J Immunol (2007) 1.85
Killing of Klebsiella pneumoniae by human alveolar macrophages. Am J Physiol Lung Cell Mol Physiol (2002) 1.83
A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease. PLoS One (2012) 1.64
Interprofessional simulation training improves knowledge and teamwork in nursing and medical students during internal medicine clerkship. J Hosp Med (2014) 1.62
Potential role of high-mobility group box 1 in cystic fibrosis airway disease. Am J Respir Crit Care Med (2008) 1.62
Endorectal repair of rectocele revisited. Br J Surg (1997) 1.57
Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography. PLoS One (2013) 1.56
Pulmonary transplantation for advanced bronchioloalveolar carcinoma. J Thorac Cardiovasc Surg (2003) 1.54
Sociocultural correlates of breast cancer knowledge and screening in urban African American women. Am J Public Health (2003) 1.53
A(2) adenosine receptors regulate CFTR through PKA and PLA(2). Am J Physiol Lung Cell Mol Physiol (2002) 1.51
N-alpha-PGP and PGP, potential biomarkers and therapeutic targets for COPD. Respir Res (2009) 1.50
Clinically relevant single gene or intragenic deletions encompassing critical neurodevelopmental genes in patients with developmental delay, mental retardation, and/or autism spectrum disorders. Am J Med Genet A (2011) 1.46
Clinician performance and prominence of diagnoses displayed by a clinical diagnostic decision support system. AMIA Annu Symp Proc (2003) 1.45
Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis. J Pediatr (2002) 1.44
Responses to behaviorally vs culturally tailored cancer communication among African American women. Am J Health Behav (2004) 1.42
Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients. Am J Physiol Lung Cell Mol Physiol (2007) 1.41
Intrafamilial variability of noncompaction of the ventricular myocardium. Am Heart J (2006) 1.41
Cystic fibrosis transmembrane conductance regulator facilitates ATP release by stimulating a separate ATP release channel for autocrine control of cell volume regulation. J Biol Chem (2000) 1.40
Genetic testing will bring interesting times to clinical practice. Curr Opin Pediatr (2004) 1.39
Medical Geneticists' duty to warn at-risk relatives for genetic disease. Am J Med Genet A (2003) 1.36
Efficient endocytosis of the cystic fibrosis transmembrane conductance regulator requires a tyrosine-based signal. J Biol Chem (1999) 1.35
Use of computer kiosks for breast cancer education in five community settings. Health Educ Behav (2006) 1.35
Microbiology, safety, and pharmacokinetics of aztreonam lysinate for inhalation in patients with cystic fibrosis. Pediatr Pulmonol (2006) 1.34
A self-propagating matrix metalloprotease-9 (MMP-9) dependent cycle of chronic neutrophilic inflammation. PLoS One (2011) 1.34
Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD. Chest (2013) 1.32
Activation of airway cl- secretion in human subjects by adenosine. Am J Respir Cell Mol Biol (2004) 1.32
The presence of a matrix-derived neutrophil chemoattractant in bronchiolitis obliterans syndrome after lung transplantation. J Immunol (2009) 1.21
Is there a doctor in the house? The downside of 24/7 attending coverage in academic intensive care units. Am J Respir Crit Care Med (2010) 1.18
Role of spirituality in cancer coping among African Americans: a qualitative examination. J Cancer Surviv (2008) 1.17
Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort. Pediatr Pulmonol (2010) 1.16
DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers. Pulm Pharmacol Ther (2010) 1.16
Fibrosis: ultimate and proximate causes. J Clin Invest (2014) 1.15
Amniotic constriction band: a multidisciplinary assessment of etiology and clinical presentation. J Bone Joint Surg Am (2009) 1.14
Understanding the relationship between sweat chloride and lung function in cystic fibrosis. Chest (2013) 1.13
Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry. J Biol Chem (2003) 1.12
Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis. Curr Opin Pulm Med (2010) 1.10
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor. Am J Respir Cell Mol Biol (2014) 1.10
Sociocultural characteristics and responses to cancer education materials among African American women. Cancer Control (2003) 1.09
Neutrophils contain prolyl endopeptidase and generate the chemotactic peptide, PGP, from collagen. J Neuroimmunol (2009) 1.09
No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. Am J Respir Cell Mol Biol (2007) 1.08
Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue. Am J Respir Cell Mol Biol (2009) 1.07
Attitudes of African American premedical students toward genetic testing and screening. Genet Med (2003) 1.07
American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss. Genet Med (2014) 1.06
Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data. J Cyst Fibros (2014) 1.06
An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load. Am J Respir Cell Mol Biol (2014) 1.05
Relationship between religious social support and general social support with health behaviors in a national sample of African Americans. J Behav Med (2011) 1.04
A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis. Hum Gene Ther (2001) 1.04
Matrix metalloproteinase activity in pediatric acute lung injury. Int J Med Sci (2008) 1.01
Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis. Am J Respir Cell Mol Biol (2014) 1.01
Foodborne disease outbreaks in United States schools. Pediatr Infect Dis J (2002) 1.00
Duty to warn at-risk relatives for genetic disease: genetic counselors' clinical experience. Am J Med Genet C Semin Med Genet (2003) 0.99
Attacking the multi-tiered proteolytic pathology of COPD: new insights from basic and translational studies. Pharmacol Ther (2008) 0.99
Plasma TGF-β₁ in pediatric cystic fibrosis: potential biomarker of lung disease and response to therapy. Pediatr Pulmonol (2011) 0.98
Religious involvement measurement model in a national sample of African Americans. J Relig Health (2012) 0.98
Dysmorphic and anthropometric outcomes in 6-year-old prenatally cocaine-exposed children. Neurotoxicol Teratol (2005) 0.97
Quercetin increases cystic fibrosis transmembrane conductance regulator-mediated chloride transport and ciliary beat frequency: therapeutic implications for chronic rhinosinusitis. Am J Rhinol Allergy (2011) 0.97
Human neutrophil elastase-mediated cleavage sites of MMP-9 and TIMP-1: implications to cystic fibrosis proteolytic dysfunction. Mol Med (2010) 0.97
Cigarette smoke and CFTR: implications in the pathogenesis of COPD. Am J Physiol Lung Cell Mol Physiol (2013) 0.96
Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation. Open Respir Med J (2010) 0.96
An international randomized multicenter comparison of nasal potential difference techniques. Chest (2010) 0.96
Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease. PLoS One (2013) 0.95
An aberrant leukotriene A4 hydrolase-proline-glycine-proline pathway in the pathogenesis of chronic obstructive pulmonary disease. Am J Respir Crit Care Med (2014) 0.95
TLR expression on neutrophils at the pulmonary site of infection: TLR1/TLR2-mediated up-regulation of TLR5 expression in cystic fibrosis lung disease. J Immunol (2008) 0.94
Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR. Am J Respir Crit Care Med (1997) 0.94
Early elevation of matrix metalloproteinase-8 and -9 in pediatric ARDS is associated with an increased risk of prolonged mechanical ventilation. PLoS One (2011) 0.94
Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs. Am J Respir Cell Mol Biol (2015) 0.94
Improving the quality of care for patients with cystic fibrosis. Curr Opin Pediatr (2010) 0.93
Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia. Am J Respir Cell Mol Biol (2006) 0.93
Predictors of colorectal cancer knowledge and screening among church-attending African Americans and Whites in the Deep South. J Community Health (2009) 0.93
Adenosine receptors, cystic fibrosis, and airway hydration. Handb Exp Pharmacol (2009) 0.92
Anti-inflammatory mechanisms of apolipoprotein A-I mimetic peptide in acute respiratory distress syndrome secondary to sepsis. PLoS One (2013) 0.92