Research Topics
Species | Frank AccursoSummaryAffiliation: University of Colorado Country: USA Publications
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Publications
Update on newborn screening for cystic fibrosisJeffrey S Wagener
University of Colorado Medical School, Department of Pediatrics, Denver, Colorado, USA
Curr Opin Pulm Med 10:500-4. 2004..In this review we discuss the information during the year leading up to this meeting as well as publications since the meeting...
Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus reportPhilip M Farrell
Department of Pediatrics and Population Health Sciences, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA
J Pediatr 153:S4-S14. 2008..Their recommendations, presented herein, involve a combination of clinical presentation, laboratory testing, and genetics to confirm a diagnosis of CF...- Update in cystic fibrosis 2006Frank J Accurso
Department of Pediatrics, University of Colorado Denver and Health Sciences, Denver, Colorado, USA
Am J Respir Crit Care Med 175:754-7. 2007 - Update in cystic fibrosis 2005Frank J Accurso
Children's Hospital, 1056 East 19th Avenue, Denver, CO 80218, USA
Am J Respir Crit Care Med 173:944-7. 2006 
Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung functionFrank J Accurso
University of Colorado Denver, 13123 East 16th Avenue, B395, Aurora, CO 80045
Am J Respir Crit Care Med 183:627-34. 2011....- Update in cystic fibrosis 2007Frank J Accurso
Department of Pediatrics, University of Colorado Denver, Denver, Colorado, USA
Am J Respir Crit Care Med 177:1058-61. 2008 - Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutationFrank J Accurso
University of Colorado Denver and Children s Hospital, Aurora, CO 80045, USA
N Engl J Med 363:1991-2003. 2010..VX-770, a CFTR potentiator, has been shown to increase the activity of wild-type and defective cell-surface CFTR in vitro... - Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohortMargaret 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)... - Association between pulmonary function and sputum biomarkers in cystic fibrosisNicole 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... - Late diagnosis defines a unique population of long-term survivors of cystic fibrosisDavid M Rodman
Center for Genetic Lung Disease, University of Colorado Health Sciences Center, 4200 East Ninth Avenue, Denver, CO 80262, USA
Am J Respir Crit Care Med 171:621-6. 2005..This is the largest cohort of older patients with CF described to date, and our findings indicate that patients diagnosed as adults differ distinctly from survivors of long-term CF diagnosed as children... - Circulating RNA transcripts identify therapeutic response in cystic fibrosis lung diseaseMilene T Saavedra
Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado School of Medicine, Denver, Colorado 80262, USA
Am J Respir Crit Care Med 178:929-38. 2008..Although the standard assessment of pulmonary treatment response is FEV(1), a measure of airflow limitation, the lack of systemic markers to reflect changes in lung inflammation critically limits the testing of proposed therapeutics... - Complications associated with symptomatic diagnosis in infants with cystic fibrosisFrank J Accurso
Department of Pediatrics, University of Colorado Health Sciences Center, The Children's Hospital, Denver, Colorado 80218, USA
J Pediatr 147:S37-41. 2005..01). CONCLUSIONS: SYMP of CF is associated with increased complication rates throughout infancy, childhood, and adolescence when compared with NBS diagnosis... - Sputum desmosine during hospital admission for pulmonary exacerbation in cystic fibrosisTheresa A Laguna
Department of Pediatrics, University of Minnesota Medical School, Minneapolis, MN 55455, USA
Chest 136:1561-8. 2009.... - 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... - Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomesMarci K Sontag
Department of Preventive Medicine and Biometrics and the Department of Pediatrics, University of Colorado Health Sciences Center, Denver, CO 80218, USA
J Pediatr 147:S83-8. 2005..To examine immunoreactive trypsinogen (IRT)-based screening for cystic fibrosis (CF) for recall rate, genotype distribution, and "borderline" sweat test results... - Genetic and physiologic correlates of longitudinal immunoreactive trypsinogen decline in infants with cystic fibrosis identified through newborn screeningMarci K Sontag
Department of Preventive Medicine and Biometrics, University of Colorado Health Sciences Center, Denver, Colorado, USA
J Pediatr 149:650-657. 2006..To characterize the time course and physiologic significance of decline in serum immunoreactive trypsinogen (IRT) levels in infants with cystic fibrosis (CF) by mode of diagnosis and genotype, and to examine IRT heritability... - Gene modifiers in cystic fibrosisFrank J Accurso
Department of Pediatrics, Mike McMorris Cystic Fibrosis Care and Research Center, Children s Hospital, University of Colorado, Aurora, Colorado 80045, USA
J Clin Invest 118:839-41. 2008.... - The clinical course of a Mexican female with cystic fibrosis and the novel genotype S531P/S531PTheresa A Laguna
Department of Pediatrics, University of Colorado School of Medicine and The Children s Hospital, 13123 E 16th Ave, B 395, Aurora, CO 80045, USA
J Cyst Fibros 7:454-6. 2008..This report illustrates the need for better characterization of CFTR mutations in a Hispanic population to aid in clinical care... - Relationship between antimicrobial proteins and airway inflammation and infection in cystic fibrosisScott D Sagel
Department of Pediatrics, The Children s Hospital and University of Colorado Denver School of Medicine, Aurora, Colorado 80045, USA
Pediatr Pulmonol 44:402-9. 2009.... - A new cystic fibrosis newborn screening algorithm: IRT/IRT1 upward arrow/DNAMarci K Sontag
Department of Epidemiology, Colorado School of Public Health, University of Colorado Denver, Aurora, CO, USA
J Pediatr 155:618-22. 2009..To evaluate an immunoreactive trypsinogen (IRT) IRT/IRT1 upward arrow/DNA algorithm, aimed at improving sensitivity while decreasing cystic fibrosis (CF) carrier identification... - Reliability of quantitative real-time PCR for bacterial detection in cystic fibrosis airway specimensEdith T Zemanick
Department of Pediatrics, Colorado School of Public Health, University of Colorado Denver, Aurora, Colorado, United States of America
PLoS ONE 5:e15101. 2010..influenzae from CF airway samples. By reliably quantifying fastidious airway bacteria, qPCR may improve our understanding of polymicrobial CF lung infections, progression of lung disease and ultimately improve antimicrobial treatments... - Monitoring inflammation in CF. CytokinesScott D Sagel
Department of Pediatrics, Children s Hospital, University of Colorado Health Sciences Center, 1056 E 19th Ave, Box B395, Denver, CO 80218, USA
Clin Rev Allergy Immunol 23:41-57. 2002..In addition, cytokines may eventually serve as targets for novel anti-inflammatory therapies in CF... - Pulmonary exacerbations in cystic fibrosis with negative bacterial culturesEdith T Zemanick
Department of Pediatrics, University of Colorado Denver, Aurora, Colorado 80045, USA
Pediatr Pulmonol 45:569-77. 2010..Exacerbations are often associated with characteristic airway bacteria [CF related bacteria (CFRB)]. However, some patients do not have CFRB detected by culture during exacerbations... - Risk factors for initial acquisition of Pseudomonas aeruginosa in children with cystic fibrosis identified by newborn screeningJudith H Maselli
Department of Preventive Medicine and Biometrics, University of Colorado Health Sciences Center, Denver, Colorado 80218, USA
Pediatr Pulmonol 35:257-62. 2003..We conclude that female gender, homozygous DeltaF508 mutation, and S. aureus isolations are important risk factors for early P. aeruginosa detection in children with CF identified through newborn screening... - Newborn screening for cystic fibrosisJeffrey S Wagener
Department of Pediatrics, Pediatric Pulmonary Section, University of Colorado School of Medicine and The Children s Hospital, Denver 80218, USA
Curr Opin Pediatr 15:309-15. 2003..In this review, we examine the many advances in CF newborn screening and early care that were reported during the last few years... - Measuring and improving respiratory outcomes in cystic fibrosis lung disease: opportunities and challenges to therapyEdith 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... - Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trialLisa Saiman
Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NY 10032 3784, USA
JAMA 290:1749-56. 2003..Treatment strategies for cystic fibrosis (CF) lung disease include antibiotics, mucolytics, and anti-inflammatory therapies. Increasing evidence suggests that macrolide antibiotics might be beneficial in patients with CF... - Cystic fibrosis, pediatrics, control of breathing, pulmonary physiology and anatomy, and surfactant biology in AJRCCM in 2004Andrew Bush
Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, 4200 East 9th Avenue, Box C272, Room 5503, Denver, CO 80262-0001, USA
Am J Respir Crit Care Med 171:545-53. 2005 - Decreased total serum coenzyme-Q10 concentrations: a longitudinal study in children with cystic fibrosisTheresa A Laguna
Department of Pediatrics, University of Colorado, Denver, School of Medicine, Aurora, CO, USA
J Pediatr 153:402-7. 2008..To assess total serum levels of coenzyme Q(10) (Co-Q(10)), an important antioxidant, in children with cystic fibrosis (CF) and to investigate an association between Co-Q(10) level and clinical outcome... - Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosisClaudia L Ordonez
Department of Pediatrics, Harvard Medical School, Boston, MA 02215, USA
Am J Respir Crit Care Med 168:1471-5. 2003..Seven of 127 (6%) sputum induction procedures showed a decrease in FEV1 of 20% or more. We conclude that markers in induced sputum may be useful, noninvasive outcome measures to assess response to therapies in cystic fibrosis studies... - Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosisJerry A Nick
Adult Cystic Fibrosis Program, National Jewish Health, 1400 Jackson Street, Denver, CO 80206, USA
Am J Respir Crit Care Med 182:614-26. 2010..Little is known concerning disease progression and outcomes in these cohorts... - Induced sputum inflammatory measures correlate with lung function in children with cystic fibrosisScott D Sagel
Department of Pediatrics, University of Colorado Health Sciences Center, Denver, USA
J Pediatr 141:811-7. 2002.... - The applicability of urinary creatinine as a method of specimen normalization in the cystic fibrosis populationBrandie D Wagner
Department of Biostatistics and Informatics, Colorado School of Public Health, University of Colorado Denver, 13001 E 17th Place, B119, Aurora, CO, 80045, USA
J Cyst Fibros 9:212-6. 2010..Urinary creatinine (UCr) is often used to adjust for urine analyte concentration. We aim to explore the applicability of UCr as a normalization method in a cystic fibrosis (CF) population during hospitalization... - Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosisRonald 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... - Brain natriuretic peptide levels in managing pediatric patients with pulmonary arterial hypertensionAnna Bernus
Pediatric Cardiology, University of Colorado Denver School of Medicine, The Children s Hospital, 13123 East 16th Ave, Aurora, CO 80045, USA
Chest 135:745-51. 2009..In this study, we aimed to investigate the value of brain natriuretic peptide (BNP) in a cohort of children with PAH, with respect to monitoring disease severity as assessed by hemodynamic and echocardiographic parameters... - Frequency of ASCA seropositivity in children with cystic fibrosisAdria A Condino
The Children's Hospital, University of Colorado Health Sciences Center, Department of Pediatrics, Denver, USA
J Pediatr Gastroenterol Nutr 41:23-6. 2005..When evaluating CF patients for Crohn disease, ASCA should be used with caution. The reasons for higher ASCA seropositivity in CF patients are unknown, but may include exposure to fungal organisms via intestinal or pulmonary sources... - A new synaptic plasticity rule for networks of spiking neuronsWaldemar Swiercz
Department of Neurology, Health Sciences Center, University of Colorado at Denver, Denver, CO 80262, USA
IEEE Trans Neural Netw 17:94-105. 2006..We decided to validate the SAPR by using it in a network of spiking neurons for image segmentation because it is easy to visually assess the results. An important thing is that image segmentation is done in an entirely unsupervised way... - Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosisRonald 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... - Thioredoxin and dihydrolipoic acid inhibit elastase activity in cystic fibrosis sputumRees L Lee
Department of Pediatrics, National Jewish Medical and Research Center, Denver, CO 80206, USA
Am J Physiol Lung Cell Mol Physiol 289:L875-82. 2005..By contrast, the elastase activity after DNase treatment increased by 190%. The ability of Trx and DHLA to limit elastase activity combined with their mucolytic effects makes these compounds potential therapies for CF... - Gene modifiers in pediatrics: application to cystic fibrosisMarci K Sontag
The Mike McMorris Cystic Fibrosis Research and Treatment Center, Department of Pediatrics, The Children's Hospital, University of Colorado Health Sciences Center, Denver, USA
Adv Pediatr 51:5-36. 2004..To date, only a few gene modifiers in pediatric disease have been conclusively identified, but we believe that future research will open the doors for new knowledge and treatment... - NO pathway in CF and non-CF childrenJamie L Wooldridge
Department of Pediatrics, University of Colorado Health Sciences Center, Denver, Colorado, USA
Pediatr Pulmonol 37:338-50. 2004..We conclude that lower airway NO, nitrate levels, and NOS II expression are not different in young children with CF and disease control patients, but that NOS II expression decreases in CF as airway inflammation increases... - Impaired clearance of apoptotic cells from cystic fibrosis airwaysR William Vandivier
University of Colorado Health Sciences Center, Denver, CO, USA
Chest 121:89S. 2002 - Nebulized hypertonic saline attenuates acute lung injury following trauma and hemorrhagic shock via inhibition of matrix metalloproteinase-13Max Wohlauer
Department of Surgery, University of Colorado Denver, CO, USA
Crit Care Med 40:2647-53. 2012..We hypothesized that aerosolized inhaled hypertonic saline given at the onset of resuscitation will decrease acute lung injury following hemorrhagic shock, by inhibiting the release of epithelial derived proinflammatory mediators... - Curcumin and cystic fibrosisFrank Accurso
Pediatric Pulmonary Medicine, Cystic Fibrosis Center, University of Colorado Health, Sciences Center and The Children's Hospital, Denver, CO, USA
J Pediatr Gastroenterol Nutr 39:235. 2004 - Reduced thioredoxin increases proinflammatory cytokines and neutrophil influx in rat airways: modulation by airway mucusRaymond C Rancourt
Department of Pediatrics, National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206, USA
Free Radic Biol Med 42:1441-53. 2007..In parallel studies, increased amounts of the p65 subunit of NF-kappaB were present in nuclear extracts from rat lungs administered reduced rhTrx, suggesting a role for NF-kappaB in these proinflammatory responses... - Thioredoxin liquefies and decreases the viscoelasticity of cystic fibrosis sputumRaymond C Rancourt
National Jewish Medical and Research Center, 1400 Jackson St, Denver, CO 80206, USA
Am J Physiol Lung Cell Mol Physiol 286:L931-8. 2004..Recognizing that mucins are the major gel-forming glycoproteins in mucus, we suggest that Trx alters sputum rheology by enzymatic reduction of glycoprotein polymers present in sputum... - Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosisMarci K Sontag
Department of Epidemiology, Colorado School of Public Health, University of Colorado Denver, 13001 E 17th, Box B119, Aurora, CO 80045, USA
Pediatr Pulmonol 45:291-300. 2010.... - Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasisR William Vandivier
Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Health Sciences Center, Denver, Colorado, USA
J Clin Invest 109:661-70. 2002..Therefore, defective airway clearance of apoptotic cells in cystic fibrosis and bronchiectasis may be due to elastase-mediated cleavage of phosphatidylserine receptor on phagocytes and may contribute to ongoing airway inflammation... - Bacterial colonization and infection in the CF lungScott D Sagel
Department of Pediatrics, Children's Hospital, University of Colorado Health Sciences Center, Denver, CO, USA
Methods Mol Med 70:433-46. 2002 - Interleukin-18 expression in cystic fibrosis lungsEdward D Chan
Division of Infectious Diseases, University of Colorado Health Sciences Center, Denver, 80262, USA
Chest 121:84S-85S. 2002 - Proteomics in pediatric research and practiceStephen W Hunsucker
Department of Pediatrics, School of Medicine, University of Colorado at Denver and Health Sciences Center, 12801 East 17th Avenue, Aurora, CO 80010, USA
Adv Pediatr 54:9-28. 2007 - Case report: skin discoloration following administration of colloidal silver in cystic fibrosisChristopher D Baker
Department of Pediatrics, The Children s Hospital, 1056 E 19th Ave, Box B 395, Denver, CO 80218, USA
Curr Opin Pediatr 19:733-5. 2007..In the following case, an adjunctive modality posed a significant health risk to the patient... - Impairment of macrophage survival by NaCl: implications for early pulmonary inflammation in cystic fibrosisGwendolyn S Kerby
Program in Cell Biology, Department of Pediatrics, National Jewish Medical and Research Center, Denver, CO 80206, USA
Am J Physiol Lung Cell Mol Physiol 283:L188-97. 2002..We speculate that augmentation of macrophage apoptosis in CF airways may result in decreased clearance of neutrophils and in deficiencies in the elimination of common CF pathogens... - Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop reportAnne Marie Comeau
New England Newborn Screening Program and Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts, USA
Pediatrics 119:e495-518. 2007.... - Seeking modifier genes in cystic fibrosisFrank J Accurso
Am J Respir Crit Care Med 167:289-90. 2003 - Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airwayChristopher L Karp
Division of Molecular Immunology, Cincinnati Children s Hospital Medical Center, and University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
Nat Immunol 5:388-92. 2004..These findings suggest that there is a pathophysiologically important defect in lipoxin-mediated anti-inflammatory activity in the cystic fibrosis lung and that lipoxins have therapeutic potential in this lethal autosomal disease... - A pilot study on the safety and efficacy of a novel antioxidant rich formulation in patients with cystic fibrosisKonstantinos A Papas
Yasoo Health, Johnson City, TN, USA
J Cyst Fibros 7:60-7. 2008.... - Patterns of neutrophil serine protease-dependent cleavage of surfactant protein D in inflammatory lung diseaseJessica Cooley
Immune Disease Institute, Harvard Medical School, Boston, MA 02115, USA
J Leukoc Biol 83:946-55. 2008..The findings support a pre-eminent role for neutrophil elastase as the critical protease responsible for SP-D depletion in inflammatory lung disease... - Molecular identification of bacteria in bronchoalveolar lavage fluid from children with cystic fibrosisJ Kirk Harris
Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Boulder, CO 80309, USA
Proc Natl Acad Sci U S A 104:20529-33. 2007..These results show that molecular techniques provide a broader perspective on airway bacteria than do routine clinical cultures and thus can identify targets for further clinical evaluation...
Research Grants
- Determinants of early pancreatic injury in cystic fibro*Frank Accurso; Fiscal Year: 2004..Our long-term objectives are to find new approaches to the early treatment of CF in order to delay pancreatic injury and the development of lung disease. ..
- Clinical Proteomics Center in Lung DiseaseFrank Accurso; Fiscal Year: 2007..Establish a national resource for investigators interested in developing protein biomarkers of lung disease. ..