R B Moss
Affiliation: Stanford University
- Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trialRichard B Moss
Stanford University School of Medicine, Palo Alto, CA, USA Electronic address
Lancet Respir Med 3:524-33. 2015..Ivacaftor has been previously assessed in patients with cystic fibrosis with Gly551Asp-CFTR or other gating mutations. We assessed ivacaftor in patients with Arg117His-CFTR, a residual function mutation...
- The use of biological agents for the treatment of fungal asthma and allergic bronchopulmonary aspergillosisRichard B Moss
Department of Pediatrics, Stanford University School of Medicine, Stanford, California, USA
Ann N Y Acad Sci 1272:49-57. 2012..Controlled trials of Th2-inhibiting biologicals in patients with ABPA and severe asthma with fungal sensitization appear warranted...
- Specific antibodies to recombinant allergens of Aspergillus fumigatus in cystic fibrosis patients with ABPAViswanath P Kurup
Allergy Immunology Division, Medical College of Wisconsin and Research Service, V A Medical Center, 5000 West National Avenue, Milwaukee, WI 53295, USA
Clin Mol Allergy 4:11. 2006..However, this is not true with cystic fibrosis patients where the prevalence of ABPA is fairly high and the morbidity and mortality are significant...
- Randomized, double-blind, placebo-controlled, dose-escalating study of aerosolized interferon gamma-1b in patients with mild to moderate cystic fibrosis lung diseaseRichard B Moss
Department of Pediatrics, Stanford University Medical Center, Palo Alto, California 94304 5786, USA
Pediatr Pulmonol 39:209-18. 2005..Aerosolized IFN-gamma1b did not improve pulmonary function, reduce sputum bacterial density, or affect inflammatory sputum markers in patients with mild-moderate lung disease...
- Pathophysiology and immunology of allergic bronchopulmonary aspergillosisR B Moss
Department of Pediatrics, Division of Pulmonary Medicine, Stanford University, Palo Alto, CA 94304 5786, USA
Med Mycol 43:S203-6. 2005..It is treated with systemic glucocorticoids and azoles. Monitoring clinical, radiographic and serologic responses (especially total IgE) is essential for successful management...
- Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trialRichard B Moss
Department of Pediatrics, Stanford University, Stanford, CA 94305, and Division of Respiratory Diseases, Children s Hospital Boston, MA 02115, USA
Hum Gene Ther 18:726-32. 2007..Because gene transfer is the simplest, most basic way to correct the underlying genetic defect that leads to disease in CF, further research is warranted to develop an effective gene transfer agent for the treatment of CF...
- Allergic bronchopulmonary aspergillosisRichard B Moss
Department of Pediatrics, Stanford University, Palo Alto, CA, USA
Clin Rev Allergy Immunol 23:87-104. 2002..Monitoring of clinical, radiographic and laboratory responses (especially total serum IgE) is essential...
- Long-term benefits of inhaled tobramycin in adolescent patients with cystic fibrosisRichard B Moss
Department of Pediatrics, Stanford University Medical Center, Palo Alto, CA 94304 5786, USA
Chest 121:55-63. 2002..To determine the effect of long-term suppression of Pseudomonas aeruginosa on lung function and other clinical end points in adolescent patients with cystic fibrosis (CF)...
- Administration of aerosolized antibiotics in cystic fibrosis patientsR B Moss
Department of Pediatric Pulmonary Medicine, Stanford University Medical Center, Palo Alto, CA 94304 5786, USA
Chest 120:107S-113S. 2001..Antibiotic susceptibility of the bacterial isolates did not predict clinical response. TSI was safe, well-tolerated, and effective for long-term treatment (96 weeks) of P aeruginosa colonization and infection in CF patients...
- Allergic bronchopulmonary aspergillosis and Aspergillus infection in cystic fibrosisRichard B Moss
Department of Pediatrics, Stanford University, Palo Alto, CA 94304, USA
Curr Opin Pulm Med 16:598-603. 2010..fumigatus airways infection and A. fumigatus allergy without allergic bronchopulmonary aspergillosis (ABPA)...
- Cytokine dysregulation in activated cystic fibrosis (CF) peripheral lymphocytesR B Moss
Department of Paediatrics, Stanford University School of Medicine, Palo Alto, CA 94304 5786, USA
Clin Exp Immunol 120:518-25. 2000..These findings extend defects described in respiratory epithelial cells to circulating immunoregulatory cells, suggesting a link between CF genotype and cytokine dysregulation...
- Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis transmembrane regulator gene transfer to the lungs of patients with cystic fibrosis: a multicenter, double-blind, placebo-controlled trialRichard B Moss
Department of Pediatrics, Stanford University, Stanford, CA, USA
Chest 125:509-21. 2004....
- Sensitization to aztreonam and cross-reactivity with other beta-lactam antibiotics in high-risk patients with cystic fibrosisR B Moss
Department of Pediatrics, Stanford University School of Medicine, Calif
J Allergy Clin Immunol 87:78-88. 1991..However, caution should be exercised with aztreonam in BLA-allergic patients with CF in light of 5% preexisting ST cross-reactivity and 20% sensitization rates found in this study...
- Maxillary sinusitis as a surrogate model for CF gene therapy clinical trials in patients with antrostomiesJ A Wagner
Department of Molecular Pharmacology, Stanford University School of Medicine, CA 94305 5332, USA
J Gene Med 1:13-21. 1999..Using CF sinusitis as a surrogate model for testing clinical efficacy of new treatments is attractive because CF upper respiratory disease is similar to the lower respiratory disease with respect to electrophysiology and microbiology...
- Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: a quantitative analysisTerry E Robinson
Department of Pediatrics Pulmonary, Pediatric Pulmonary Division, Stanford University Medical Center, 701 Welch Rd, Whelan Building, 3328, Palo Alto, CA 94305 5786, USA
Chest 128:2327-35. 2005....
- Diagnostic testing by CFTR gene mutation analysis in a large group of Hispanics: novel mutations and assessment of a population-specific mutation spectrumIris Schrijver
Department of Pathology, L235, Stanford University Medical Center, Stanford, CA 94305, USA
J Mol Diagn 7:289-99. 2005..These results underscore the disparity in mutation identification between Caucasians and Hispanics and show utility for comprehensive diagnostic CFTR mutation analysis in this population...
- Profound functional and signaling changes in viable inflammatory neutrophils homing to cystic fibrosis airwaysRabindra Tirouvanziam
Departments of Pediatrics, Genetics, and Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA
Proc Natl Acad Sci U S A 105:4335-9. 2008..Thus, we identified a subset of neutrophils within CF airways with a viable but dysfunctional phenotype. This subset provides a possible therapeutic target and indicates a need to revisit current paradigms of CF airway disease...
- Allergic bronchopulmonary aspergillosis in cystic fibrosis--state of the art: Cystic Fibrosis Foundation Consensus ConferenceDavid A Stevens
Department of Medicine, Santa Clara Valley Medical Center, Stanford University Medical School, San Jose, California 95128 2699, USA
Clin Infect Dis 37:S225-64. 2003..Diagnostic criteria that could provide a framework for monitoring were adopted, and helpful imaging features were identified. New possibilities in therapy produced plans for managing diverse clinical presentations...
- Cystic fibrosis HRCT scores correlate strongly with Pseudomonas infectionTerry E Robinson
Center of Excellence for Pediatric Cystic Fibrosis and Pulmonary Diseases, Stanford University Medical Center, Palo Alto, California 94305 5715, USA
Pediatr Pulmonol 44:1107-17. 2009....
- Composite spirometric-computed tomography outcome measure in early cystic fibrosis lung diseaseTerry E Robinson
Pediatric Pulmonary Division, Stanford University Medical Center, 701 Welch Road, Whelan Building 3328, Palo Alto, CA 94304 5786
Am J Respir Crit Care Med 168:588-93. 2003....
- A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomiesJohn A Wagner
Department of Molecular Pharmacology, Stanford CCSR Building, Stanford, CA 94305 5174, USA
Hum Gene Ther 13:1349-59. 2002..In summary, this Phase II trial confirms the safety of tgAAVCF but provides little support of its efficacy in the within-patient controlled sinus study. Various potentially confounding factors are discussed...
- Safety, tolerability, and efficacy of high-frequency chest wall oscillation in pediatric patients with cerebral palsy and neuromuscular diseases: an exploratory randomized controlled trialNanci Yuan
Department of Pediatrics, Division of Pulmonology, Stanford University, Palo Alto, California 94304, USA
J Child Neurol 25:815-21. 2010..Our data suggest safety, tolerability, and better compliance with high-frequency chest wall oscillation. Improvement in airway clearance may help prevent hospitalizations. Larger controlled trials are required to confirm these results...
- High-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosisRabindra Tirouvanziam
Department of Genetics, Stanford University School of Medicine, Stanford, CA 94305, USA
Proc Natl Acad Sci U S A 103:4628-33. 2006..032). Thus, high-dose oral N-acetylcysteine has the potential to counter the intertwined redox and inflammatory imbalances in CF...
- Reduced IL-10 secretion by CD4+ T lymphocytes expressing mutant cystic fibrosis transmembrane conductance regulator (CFTR)R B Moss
Department of Pediatrics, Stanford University School of Medicine, CA 94305 5119, USA
Clin Exp Immunol 106:374-88. 1996..It is possible that disruption of IL-10-mediated anti-inflammatory homeostasis may contribute to early onset sustained inflammation in CF airways...
- Deficiency of IgG4 in children: association of isolated IgG4 deficiency with recurrent respiratory tract infectionR B Moss
Allergy Pulmonary Division, Lucille Salter Packard Children s Hospital, Standford, Palo Alto, CA 94304
J Pediatr 120:16-21. 1992..This association suggests a physiologic defense role for mucosal IgG4...
- Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosisT E Robinson
Division of Pediatric Pulmonology, Stanford University Medical Center, Palo Alto 94304-5786, California, USA
J Pediatr 138:553-9. 2001..Total and reversible HRCT scores and percent predicted SVC, FEV1, and FVC were the most sensitive to change. The greatest change was seen in the mucus plugging subcomponent HRCT score...
- Comprehensive mutation screening in a cystic fibrosis centerJ J Wine
Cystic Fibrosis Research Laboratory, Stanford University, Stanford, California, USA
Pediatrics 107:280-6. 2001..We discuss 5 possible explanations that could account for the few remaining undetected mutations...
- Expression of perforin on HIV-1-specific CD8+ lymphocytes after immunization with a gp120-depleted, whole-killed HIV-1 immunogenR B Moss
The Immune Response Corporation, Carlsbad, CA, USA
Clin Exp Immunol 124:248-54. 2001..The induction of such responses with immunization may have direct antiviral consequences and is being studied in ongoing clinical trials...
- Activation of critical, host-induced, metabolic and stress pathways marks neutrophil entry into cystic fibrosis lungsMegha Makam
Department of Pediatrics, Stanford University School of Medicine, Stanford, CA 94305, USA
Proc Natl Acad Sci U S A 106:5779-83. 2009..Taken together, our data demonstrate an early and sustained activation of host-responsive metabolic and stress pathways upon neutrophil entry into CF airways, suggesting potential targets for therapeutic modulation...
- Advances against aspergillosisWilliam J Steinbach
Division of Pediatric Infectious Diseases, Department of Pediatrics, Duke University, Durham, North Carolina, USA
Clin Infect Dis 37:S155-6. 2003
- Fully automated system for three-dimensional bronchial morphology analysis using volumetric multidetector computed tomography of the chestRaman Venkatraman
Department of Pediatric Pulmonology, Stanford University School of Medicine, Stanford, CA 94305, USA
J Digit Imaging 19:132-9. 2006....
- Sputum cathelicidin, urokinase plasminogen activation system components, and cytokines discriminate cystic fibrosis, COPD, and asthma inflammationWei Xiao
Department of Medicine, Shandong University, Jinan, People s Republic of China
Chest 128:2316-26. 2005..We investigated several components of innate immunity in induced sputum of patients with cystic fibrosis (CF), COPD, and asthma, and healthy control subjects...
- Quantitative air-trapping analysis in children with mild cystic fibrosis lung diseaseAnne Sophie Bonnel
Division of Pediatric Respirology, University Hospital, Rouen, France
Pediatr Pulmonol 38:396-405. 2004..Pulmonary density measurements and % AT better discriminated differences between groups than PFTs. Measurements made on expiratory scans near FRC showed significantly higher values for AT than those made near RV...