Cori L Daines
- Professor, Pediatrics - (Clinical Scholar Track)
- M.D. Medicines
- St. Louis University, St. Louis, Missouri, United States
- Cincinnati Children's Hospital (1999 - 2006)
- America’s Best Doctors
- Spring 2019
- Spring 2018
- Spring 2017
- Spring 2016
- CFF Quality Award
- Cystic Fibrosis Foundation, Fall 2016
Licensure & Certification
- Board-Certified, Pediatric Pulmonary Medicine, American Board of Pediatrics (2001)
- Board-Certified, Pediatrics, American Board of Pediatrics (1996)
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- Daines, C. L. (2020). Cystic Fibrosis. In Conn's Current Therapy 2020.
- Daines, C. L. (2018). Aspiration Syndromes. In Rudolph's Pediatrics, 23rd ed.
- Daines, C. L. (2011). Aspiration Syndroms. In Rudolph's Pediatrics(pp 1958-1962). McGraw-Hill.
- Asseri, A. A., Zeng, Y., & Daines, C. L. (2019). Acute pulmonary embolism in a child with ANCA-negative Idiopathic Pulmonary Capillaritis. Saudi medical journal, 40(6), 610-613.More infoDiffuse alveolar hemorrhage is an uncommon and often fatal condition in children that is characterized by distinct histopathological etiologies. Herein, we discuss the case of an 11-year-old girl who presented with acute worsening of hypoxia and left-sided chest pain. The patient had lung biopsy-proven idiopathic pulmonary capillaritis and was being treated with prednisolone every alternate day, azathioprine, and hydroxychloroquine. A contrast-computed tomography (CT) scan of the chest showed an acute left lower-lobe pulmonary embolism. Negative results were obtained on a test for thrombophilia. In children, pulmonary embolism with anti-neutrophil cytoplasmic antibody-negative idiopathic pulmonary capillaritis is a rare clinical condition. The exact cause of thrombus formation in this case is unknown; however, obesity, immobility, and chronic systemic corticosteroid therapy probably played a role.
- Balakrishnan, K., Sidell, D. R., Bauman, N. M., Bellia-Munzon, G. F., Boesch, R. P., Bromwich, M., Cofer, S. A., Daines, C., de Alarcon, A., Garabedian, N., Hart, C. K., Ida, J. B., Leboulanger, N., Manning, P. B., Mehta, D. K., Monnier, P., Myer, C. M., Prager, J. D., Preciado, D., , Propst, E. J., et al. (2019). Outcome measures for pediatric laryngotracheal reconstruction: International consensus statement. The Laryngoscope, 129(1), 244-255.More infoDevelop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies.
- Hobart, C. B., Daines, C. L., & Phan, H. (2019). Developing Future Clinical Pharmacy Leaders in the Interprofessional Care of Children with Special Health Care Needs and Medical Complexity (CSHCN-CMC) in a Pediatric Pulmonary Center. Children (Basel, Switzerland), 6(12).More infoThe health care needs of children with special health care needs and medical complexity (CSHCN-CMC) are multifaceted and often require the expertise of various disciplines. The medication-related needs of this population can be further complicated with off-label medication use, polypharmacy, and vulnerability to medication errors. Although clinical pharmacists are increasingly becoming a common part of inpatient, pediatric interprofessional patient care teams, their presence remains lacking in the outpatient or ambulatory care realm. Pediatric clinical pharmacists in the ambulatory care setting have the potential to help optimize medication use and safety through collaborative efforts as part of the interprofessional team. Since the late 1960s, Pediatric Pulmonary Centers (PPCs) provide training programs designed to develop interprofessional leaders who will improve the health status of CSHCN-CMC, specifically those with chronic respiratory and sleep-related conditions. The addition of pharmacists not only provides a more comprehensive care model for CSHCN-CMC, it creates an avenue to encourage the career paths of pediatric pharmacists in the ambulatory care setting. Here, we describe the addition of clinical pharmacy as part of an interprofessional patient care team and the development and implementation of a maternal child health (MCH) pharmacy discipline training model designed to mentor future pharmacist leaders in the care of CSHCN-CMC.
- Liou, T. G., Adler, F. R., Argel, N., Asfour, F., Brown, P. S., Chatfield, B. A., Daines, C. L., Durham, D., Francis, J. A., Glover, B., Heynekamp, T., Hoidal, J. R., Jensen, J. L., Keogh, R., Kopecky, C. M., Lechtzin, N., Li, Y., Lysinger, J., Molina, O., , Nakamura, C., et al. (2019). Prospective multicenter randomized patient recruitment and sample collection to enable future measurements of sputum biomarkers of inflammation in an observational study of cystic fibrosis. BMC medical research methodology, 19(1), 88.More infoBiomarkers of inflammation predictive of cystic fibrosis (CF) disease outcomes would increase the power of clinical trials and contribute to better personalization of clinical assessments. A representative patient cohort would improve searching for believable, generalizable, reproducible and accurate biomarkers.
- Martiniano, S. L., Daines, C. L., Dellon, E. P., Esther, C. R., Muhlebach, M. S., Ong, T., Rabinowitz, E. C., Toprak, D., & Zemanick, E. T. (2019). Highlights from the 2018 North American cystic fibrosis conference. Pediatric pulmonology, 54(7), 941-948.More infoThe 32nd annual North American Cystic Fibrosis Conference was held in Denver, CO on Oct. 18 to 20, 2018. This review highlights presentations in several topic areas, including the pathophysiology and basic science of cystic fibrosis lung disease, clinical trials, clinical care, and quality improvement. Citations from the conference are by first author and abstract or symposium number, as designated in the previously published supplement.
- Wheatley, C. M., Baker, S. E., Daines, C. M., Phan, H., Martinez, M. G., Morgan, W. J., & Snyder, E. M. (2019). Influence of the Vibralung Acoustical Percussor on pulmonary function and sputum expectoration in individuals with cystic fibrosis. Therapeutic advances in respiratory disease, 12, 1753466618770997.More infoThe Vibralung Acoustical Percussor is a new airway clearance therapy (ACT) utilizing intrapulmonary sound waves in addition to positive expiratory pressure (PEP). We evaluated the safety of the Vibralung and collected preliminary data on its ability to mediate sputum expectoration in individuals with cystic fibrosis (CF).
- Boesch, R. P., Balakrishnan, K., Acra, S., Benscoter, D. T., Cofer, S. A., Collaco, J. M., Dahl, J. P., Daines, C. L., DeAlarcon, A., DeBoer, E. M., Deterding, R. R., Friedlander, J. A., Gold, B. D., Grothe, R. M., Hart, C. K., Kazachkov, M., Lefton-Greif, M. A., Miller, C. K., Moore, P. E., , Pentiuk, S., et al. (2018). Structure and Functions of Pediatric Aerodigestive Programs: A Consensus Statement. Pediatrics, 141(3).More infoAerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.
- Keating, D., Marigowda, G., Burr, L., Daines, C., Mall, M. A., McKone, E. F., Ramsey, B. W., Rowe, S. M., Sass, L. A., Tullis, E., McKee, C. M., Moskowitz, S. M., Robertson, S., Savage, J., Simard, C., Van Goor, F., Waltz, D., Xuan, F., Young, T., , Taylor-Cousar, J. L., et al. (2018). VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. The New England journal of medicine, 379(17), 1612-1620.More infoVX-445 is a next-generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore Phe508del CFTR protein function in patients with cystic fibrosis when administered with tezacaftor and ivacaftor (VX-445-tezacaftor-ivacaftor).
- Martiniano, S. L., Toprak, D., Ong, T., Zemanick, E. T., Daines, C. L., Muhlebach, M. S., Esther, C. R., & Dellon, E. P. (2018). Highlights from the 2017 North American Cystic Fibrosis Conference. Pediatric pulmonology, 53(7), 979-986.More infoThe 31st annual North American Cystic Fibrosis Conference (NACFC) was held in Indianapolis, IN on November 2-4, 2017. Abstracts of presentations from the conference were published in a supplement to Pediatric Pulmonology [2017; Pediatr Pulmonol Suppl. 52: S1-S776]. The current review summarizes several major topic areas addressed at the conference: the pathophysiology and basic science of cystic fibrosis (CF) lung disease, clinical trials, clinical management issues, and quality improvement (QI). In this review, we describe emerging concepts in several areas of CF research and care.
- Sagel, S. D., Khan, U., Jain, R., Graff, G., Daines, C. L., Dunitz, J. M., Borowitz, D., Orenstein, D. M., Abdulhamid, I., Noe, J., Clancy, J. P., Slovis, B., Rock, M. J., McCoy, K. S., Strausbaugh, S., Livingston, F. R., Papas, K. A., & Shaffer, M. L. (2018). Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial. American journal of respiratory and critical care medicine, 198(5), 639-647.More infoCystic fibrosis (CF) is characterized by dietary antioxidant deficiencies, which may contribute to an oxidant-antioxidant imbalance and oxidative stress.
- Wheatley, C. M., Baker, S. E., Daines, C. L., Phan, H., Martinez, M. G., Morgan, W. J., & Snyder, E. M. (2018). Influence of the Vibralung Acoustical Percussor on pulmonary function and sputum expectoration in individuals with cystic fibrosis.. Ther Adv Respir Dis., 12, 1-15. doi:10.1177/1753466618770997
- Rowe, S. M., Daines, C., Ringshausen, F. C., Kerem, E., Wilson, J., Tullis, E., Nair, N., Simard, C., Han, L., Ingenito, E. P., McKee, C., Lekstrom-Himes, J., & Davies, J. C. (2017). Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis. The New England journal of medicine, 377(21), 2024-2035.More infoCystic fibrosis is an autosomal recessive disease caused by mutations in the CFTR gene that lead to progressive respiratory decline. Some mutant CFTR proteins show residual function and respond to the CFTR potentiator ivacaftor in vitro, whereas ivacaftor alone does not restore activity to Phe508del mutant CFTR.
- Sanders, D. B., Solomon, G. M., Beckett, V. V., West, N. E., Daines, C. L., Heltshe, S. L., VanDevanter, D. R., Spahr, J. E., Gibson, R. L., Nick, J. A., Marshall, B. C., Flume, P. A., Goss, C. H., & , S. S. (2017). Standardized Treatment of Pulmonary Exacerbations (STOP) study: Observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 16(5), 592-599.More infoThe Standardized Treatment of Pulmonary Exacerbations (STOP) program has the intent of defining best practices in the treatment of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF). The objective of this analysis was to describe the clinical presentations of patients admitted for intravenous (IV) antibiotics and enrolled in a prospective observational PEx study as well as to understand physician treatment goals at the start of the intervention.
- VanDevanter, D. R., Heltshe, S. L., Spahr, J., Beckett, V. V., Daines, C. L., Dasenbrook, E. C., Gibson, R. L., Jain, R., Sanders, D. B., Goss, C. H., Flume, P. A., & , S. S. (2017). Rationalizing endpoints for prospective studies of pulmonary exacerbation treatment response in cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 16(5), 607-615.More infoGiven the variability in pulmonary exacerbation (PEx) management within and between Cystic Fibrosis (CF) Care Centers, it is possible that some approaches may be superior to others. A challenge with comparing different PEx management approaches is lack of a community consensus with respect to treatment-response metrics. In this analysis, we assess the feasibility of using different response metrics in prospective randomized studies comparing PEx treatment protocols.
- Zemanick, E. T., Daines, C. L., Dellon, E. P., Esther, C. R., Kinghorn, B., Ong, T., & Muhlebach, M. S. (2017). Highlights from the 2016 North American Cystic Fibrosis Conference. Pediatric pulmonology, 52(8), 1103-1110.More infoThe 30th annual North American Cystic Fibrosis Conference (NACFC) was held in Orlando, FL, on October 27-29, 2016. Abstracts were published in a supplement to Pediatric Pulmonology. This review summarizes several major topic areas addressed at the conference: the pathophysiology of cystic fibrosis (CF) lung disease, clinical trials, clinical management issues, and quality improvement. We sought to provide an overview of emerging concepts in several areas of CF research and care, rather than a comprehensive review of the conference. Citations from the conference are by first author and abstract number or symposium number, as designated in the supplement.
- Daines, C. L., & Morgan, W. J. (2016). Planning the future of newborn screening for cystic fibrosis. Pediatric pulmonology, 51(9), 883-5.
- Fitzpatrick, A. M., Jackson, D. J., Mauger, D. T., Boehmer, S. J., Phipatanakul, W., Sheehan, W. J., Moy, J. N., Paul, I. M., Bacharier, L. B., Cabana, M. D., Covar, R., Holguin, F., Lemanske, R. F., Martinez, F. D., Pongracic, J. A., Beigelman, A., Baxi, S. N., Benson, M., Blake, K., , Chmiel, J. F., et al. (2016). Individualized therapy for persistent asthma in young children. The Journal of allergy and clinical immunology, 138(6), 1608-1618.e12.More infoPhenotypic presentations in young children with asthma are varied and might contribute to differential responses to asthma controller medications.
- Sheehan, W. J., Mauger, D. T., Paul, I. M., Moy, J. N., Boehmer, S. J., Szefler, S. J., Fitzpatrick, A. M., Jackson, D. J., Bacharier, L. B., Cabana, M. D., Covar, R., Holguin, F., Lemanske, R. F., Martinez, F. D., Pongracic, J. A., Beigelman, A., Baxi, S. N., Benson, M., Blake, K., , Chmiel, J. F., et al. (2016). Acetaminophen versus Ibuprofen in Young Children with Mild Persistent Asthma. The New England journal of medicine, 375(7), 619-30.More infoStudies have suggested an association between frequent acetaminophen use and asthma-related complications among children, leading some physicians to recommend that acetaminophen be avoided in children with asthma; however, appropriately designed trials evaluating this association in children are lacking.
- Zemanick, E. T., Ong, T., Daines, C. L., Dellon, E. P., Muhlebach, M. S., & Esther, C. R. (2016). Highlights from the 2015 North American Cystic Fibrosis Conference. Pediatric pulmonology, 51(6), 650-7.More infoThe 29th Annual North American Cystic Fibrosis Conference was held in Phoenix, Arizona on October 8-10, 2015. Abstracts were published in a supplement to Pediatric Pulmonology.(1) In this review, we summarize presentations in several of the topic areas addressed at the conference. Our goal is to provide an overview of presentations with relevance to emerging or changing concepts in several areas rather than a comprehensive review. Citations from the conference are by first author and abstract number or symposium number, as designated in the supplement. Pediatr Pulmonol. 2016;51:650-657. © 2016 Wiley Periodicals, Inc.
- Faro, A., Wood, R. E., Schechter, M. S., Leong, A. B., Wittkugel, E., Abode, K., Chmiel, J. F., Daines, C., Davis, S., Eber, E., Huddleston, C., Kilbaugh, T., Kurland, G., Midulla, F., Molter, D., Montgomery, G. S., Retsch-Bogart, G., Rutter, M. J., Visner, G., , Walczak, S. A., et al. (2015). Official American Thoracic Society technical standards: flexible airway endoscopy in children. American journal of respiratory and critical care medicine, 191(9), 1066-80.More infoFlexible airway endoscopy (FAE) is an accepted and frequently performed procedure in the evaluation of children with known or suspected airway and lung parenchymal disorders. However, published technical standards on how to perform FAE in children are lacking.
- Powers, S. W., Stark, L. J., Chamberlin, L. A., Filigno, S. S., Sullivan, S. M., Lemanek, K. L., Butcher, J. L., Driscoll, K. A., Daines, C. L., Brody, A. S., Schindler, T., Konstan, M. W., McCoy, K. S., Nasr, S. Z., Castile, R. G., Acton, J. D., Wooldridge, J. L., Ksenich, R. A., Szczesniak, R. D., , Rausch, J. R., et al. (2015). Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial. JAMA pediatrics, 169(5), e150636.More infoEvidence-based treatments that achieve optimal energy intake and improve growth in preschool-aged children with cystic fibrosis (CF) are a critical need.
- Schechter, M. S., Trueman, D., Farquharson, R., Higuchi, K., & Daines, C. L. (2015). Inhaled aztreonam lysine versus inhaled tobramycin in cystic fibrosis. An economic evaluation. Annals of the American Thoracic Society, 12(7), 1030-1038.
- Schechter, M. S., Trueman, D., Farquharson, R., Higuchi, K., & Daines, C. L. (2015). Inhaled aztreonam lysine versus inhaled tobramycin in cystic fibrosis. An economic evaluation. Annals of the American Thoracic Society, 12(7), 1030-8.More infoPseudomonas aeruginosa infection is a significant cause of morbidity and mortality in patients with cystic fibrosis and is associated with a high economic burden. A recently published comparator trial demonstrated that outcomes in patients with cystic fibrosis with chronic P. aeruginosa infections switched from tobramycin solution for inhalation to aztreonam lysine for inhalation were better than those of patients who continued on tobramycin.
- Wheatley, C. M., Baker, S. E., Daines, C. M., Phan, H., Martinez, M. G., Morgan, W. J., & Snyder, E. M. (2015). Influence of the Vibralung Acoustical Percussor on pulmonary function and sputum expectoration in individuals with cystic fibrosis. Therapeutic advances in respiratory disease, 12, 1753466618770997.More infoThe Vibralung Acoustical Percussor is a new airway clearance therapy (ACT) utilizing intrapulmonary sound waves in addition to positive expiratory pressure (PEP). We evaluated the safety of the Vibralung and collected preliminary data on its ability to mediate sputum expectoration in individuals with cystic fibrosis (CF).
- Daines, C., VanDeVanter, D., Khan, U., Emerson, J., Heltshe, S., McNamara, S., Anstead, M., Langkamp, M., Doring, G., Ratjen, F., Ramsey, B., Gibson, R. L., Morgan, W., Rosenfeld, M., & , E. I. (2014). Serology as a diagnostic tool for predicting initialPseudomonas aeruginosa acquisition in children with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 13(5), 542-9.More infoPseudomonas aeruginosa (Pa) serology could potentially be a useful adjunct to respiratory culture methods for the detection of initial or early Pa infection in patients with cystic fibrosis (CF).
- Nevin, M. A., Daines, C. L., Redding, G. J., Ren, C. L., Ratjen, F. A., Shah, A. V., Hiranrattana, A., Alexiou, S., Bansal, M., Pizarro-Gamboa, M. E., & Panitch, H. B. (2014). ATS core curriculum 2014: part IV. Pediatric pulmonary medicine. Annals of the American Thoracic Society, 11(10), 1633-9.
- Wheatley, C. M., Morgan, W. J., Cassuto, N. A., Foxx-Lupo, W. T., Daines, C. L., Morgan, M. A., Phan, H., & Snyder, E. M. (2013). Exhaled breath condensate detects baseline reductions in chloride and increases in response to albuterol in cystic fibrosis patients. Clinical medicine insights. Circulatory, respiratory and pulmonary medicine, 7, 79-90.More infoImpaired ion regulation and dehydration is the primary pathophysiology in cystic fibrosis (CF) lung disease. A potential application of exhaled breath condensate (EBC) collection is to assess airway surface liquid ionic composition at baseline and in response to pharmacological therapy in CF. Our aims were to determine if EBC could detect differences in ion regulation between CF and healthy and measure the effect of the albuterol on EBC ions in these populations. Baseline EBC Cl(-), DLCO and SpO2 were lower in CF (n = 16) compared to healthy participants (n = 16). EBC Cl(-) increased in CF subjects, while there was no change in DLCO or membrane conductance, but a decrease in pulmonary-capillary blood volume in both groups following albuterol. This resulted in an improvement in diffusion at the alveolar-capillary unit, and removal of the baseline difference in SpO2 by 90-minutes in CF subjects. These results demonstrate that EBC detects differences in ion regulation between healthy and CF individuals, and that albuterol mediates increases in Cl(-) in CF, suggesting that the benefits of albuterol extend beyond simple bronchodilation.
- Daines, C., & Morgan, W. (2011). The importance of imaging in cystic fibrosis. American journal of respiratory and critical care medicine, 184(7), 751-2.
- Traylor, B. R., Wheatley, C. M., Skrentny, T. T., Foxx-Lupo, W. T., Phan, H., Patanwala, A. E., Morgan, W. J., Daines, C. L., Sprissler, R., & Snyder, E. M. (2011). Influence of genetic variation of the β2-adrenergic receptor on lung diffusion in patients with cystic fibrosis. Pulmonary pharmacology & therapeutics, 24(5), 610-6.More infoCystic fibrosis (CF) is a disease that adversely affects the lung resulting in a reduction in lung diffusion. Stimulation of the β(2)-adrenergic receptors mediates mucociliary clearance and bronchodilation. We sought to determine the influence of an inhaled β-agonist on the diffusing capacity of the lungs for carbon monoxide (DLCO), alveolar-capillary membrane conductance (D(M)), pulmonary capillary blood volume (Vc), and peripheral oxygen saturation (SaO(2)) in subjects with CF, when compared to matched healthy subjects, according to genetic variation of the β(2)-adrenergic receptor (ADRB2).
- Wheatley, C. M., Foxx-Lupo, W. T., Cassuto, N. A., Wong, E. C., Daines, C. L., Morgan, W. J., & Snyder, E. M. (2011). Impaired lung diffusing capacity for nitric oxide and alveolar-capillary membrane conductance results in oxygen desaturation during exercise in patients with cystic fibrosis. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 10(1), 45-53.More infoExercise has been shown to be beneficial for patients with cystic fibrosis (CF), but for some CF patients there is a risk of desaturation, although the predicting factors are not conclusive or reliable. We sought to determine the relationship between the diffusion capacity of the lungs for nitric oxide and carbon monoxide (DLNO and DLCO) and the components of DLCO: alveolar-capillary membrane conductance (D(M)), and pulmonary capillary blood volume (V(C)) on peripheral oxygen saturation (SaO(2)) at rest and during exercise in CF.
- Daines, C. L., Wood, R. E., & Boesch, R. P. (2008). Foreign body aspiration: an important etiology of respiratory symptoms in children. The Journal of allergy and clinical immunology, 121(5), 1297-8.
- Daines, M. O., Chen, W., Tabata, Y., Walker, B. A., Gibson, A. M., Masino, J. A., Warrier, M. R., Daines, C. L., Wenzel, S. E., & Hershey, G. K. (2007). Allergen-dependent solubilization of IL-13 receptor alpha2 reveals a novel mechanism to regulate allergy. The Journal of allergy and clinical immunology, 119(2), 375-83.More infoAllergic sensitization affects half of western populations and often precedes the development of allergic disorders including asthma. Despite the critical role of allergens in the pathogenesis of these disorders, little is known about how allergens modulate the immune response. IL-13 receptor alpha2 (IL-13Ralpha2) is a decoy receptor for IL-13.
- Boesch, R. P., Daines, C., Willging, J. P., Kaul, A., Cohen, A. P., Wood, R. E., & Amin, R. S. (2006). Advances in the diagnosis and management of chronic pulmonary aspiration in children. The European respiratory journal, 28(4), 847-61.More infoChronic pulmonary aspiration (CPA) in children is an important cause of recurrent pneumonia, progressive lung injury, respiratory disability and death. It is sporadic, intermittent and variable, and often occurs in children with complicated underlying medical conditions and syndromes that produce symptoms indistinguishable from CPA. For most types of aspiration there is no gold-standard diagnostic test. The diagnosis of CPA is currently made clinically with some supporting diagnostic evaluations, but often not until significant lung injury has been sustained. Despite multiple diagnostic techniques, the diagnosis or exclusion of CPA in children is challenging. This is of particular concern given the outcome of unrecognised progressive lung injury and the invasiveness of definitive therapies. Although new techniques have been introduced since the 1990s and significant advances in the understanding of dysphagia and gastro-oesophageal reflux have been made, characterisation of the aspirating child remains elusive.
- Gibson, R. L., Retsch-Bogart, G. Z., Oermann, C., Milla, C., Pilewski, J., Daines, C., Ahrens, R., Leon, K., Cohen, M., McNamara, S., Callahan, T. L., Markus, R., & Burns, J. L. (2006). Microbiology, safety, and pharmacokinetics of aztreonam lysinate for inhalation in patients with cystic fibrosis. Pediatric pulmonology, 41(7), 656-65.More infoAztreonam lysinate for inhalation (AI) is a novel monobactam formulation being investigated for pulmonary Pseudomonas aeruginosa infections in patients with cystic fibrosis (CF).
- Piazza-Waggoner, C., Ferguson, K. S., Daines, C., Acton, J. D., & Powers, S. W. (2006). Case study: providing evidence-based behavioral and nutrition treatment to a toddler with cystic fibrosis and multiple food allergies via telehealth. Pediatric pulmonology, 41(10), 1001-4.More infoBarriers to successful outcome for cystic fibrosis (CF) therapies can include distance from a CF care center, co-morbid conditions that require individualized alterations to the prescribed treatment, and patient-provider interactions, among others. We present the case of a 21-month-old female with CF for whom modifications of an efficacious behavioral and nutrition treatment were made due to food allergies and distance from the CF care center. She was classified as at-risk nutritionally. Following treatment a significant increase in energy intake (calories) was observed in addition to her meeting weight and height growth rates for a child of this age and gender who is growing normally at the 50th percentile.
- Powers, S. W., Piazza-Waggoner, C., Jones, J. S., Ferguson, K. S., Daines, C., & Acton, J. D. (2006). Examining clinical trial results with single-subject analysis: an example involving behavioral and nutrition treatment for young children with cystic fibrosis. Journal of pediatric psychology, 31(6), 574-81.More infoTo examine the process of change in a clinical trial of behavioral and nutrition treatment for children age 18-48 months with cystic fibrosis (CF) using single-subject analysis.
- Deterding, R., Retsch-Bogart, G., Milgram, L., Gibson, R., Daines, C., Zeitlin, P. L., Milla, C., Marshall, B., Lavange, L., Engels, J., Mathews, D., Gorden, J., Schaberg, A., Williams, J., Ramsey, B., & , C. F. (2005). Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis. Pediatric pulmonology, 39(4), 339-48.More infoDenufosol tetrasodium (INS37217) is a selective P2Y(2) agonist that stimulates ciliary beat frequency and Cl(-) secretion in normal and cystic fibrosis (CF) airway epithelia, and is being investigated as an inhaled treatment for CF. The Cl(-) secretory response is mediated via a non-CFTR pathway, and the driving force for Cl(-) secretion is enhanced by the effect of P2Y(2) activation to also inhibit epithelial Na(+) transport. Denufosol is metabolically more stable and better tolerated, and may enhance mucociliary clearance for a longer period of time than previously investigated P2Y(2) agonists. The goal of this phase 1/phase 2 study was to assess the safety and tolerability of single and repeated doses of aerosolized denufosol in subjects with CF. The study was a double-blind, placebo-controlled, multicenter comparison of ascending single doses of denufosol (10, 20, 40, and 60 mg, administered by inhalation via the Pari LC Star nebulizer) vs. placebo (normal saline), followed by a comparison of twice-daily administration of the maximum tolerated dose (MTD) of denufosol or placebo for 5 days. Thirty-seven adult (18 years of age or older) and 24 pediatric (5-17 years of age) subjects with CF were evaluated in five cohorts. Subjects were randomized in a 3:1 ratio to receive either denufosol or placebo within each cohort. The percent of subjects experiencing adverse events was similar between the denufosol and placebo groups. The most common adverse event in subjects receiving denufosol was chest tightness in adult subjects (39%) and cough in pediatric subjects (56%). Three (7%) subjects receiving denufosol and one (7%) subject receiving placebo experienced a serious adverse event. Forced expiratory volume in 1 sec (FEV(1)) profiles following dosing were similar across treatment groups, with some acute, reversible decline seen in both groups, most notably in subjects with lower lung function at baseline. In conclusion, doses up to 60 mg of denufosol inhalation solution were well-tolerated in most subjects. Some intolerability was noted among subjects with lower baseline lung function. Based on the results of this phase 1/phase 2 study, the Therapeutics Development Network (TDN) of the Cystic Fibrosis Foundation (CFF) and Inspire Pharmaceuticals, Inc., recently completed a multicenter, 28-day, phase 2 safety and efficacy clinical trial of denufosol inhalation solution in CF subjects with mild lung disease.
- Moss, R. B., Mayer-Hamblett, N., Wagener, J., Daines, C., Hale, K., Ahrens, R., Gibson, R. L., Anderson, P., Retsch-Bogart, G., Nasr, S. Z., Noth, I., Waltz, D., Zeitlin, P., Ramsey, B., & Starko, K. (2005). Randomized, double-blind, placebo-controlled, dose-escalating study of aerosolized interferon gamma-1b in patients with mild to moderate cystic fibrosis lung disease. Pediatric pulmonology, 39(3), 209-18.More infoInterferon gamma-1b (IFN-gamma1b) is a pleiotropic cytokine with immunomodulatory activities that could decrease bacterial burden, inflammation, and obstruction in patients with CF. Patients with CF (> or =12 years old, FEV1 > or =40% predicted) were randomly assigned to sequential dose cohorts inhaling 500 microg IFN-gamma1b, 1,000 microg IFN-gamma1b, or placebo by Respirgard II nebulizer thrice weekly for 12 weeks. Sputum bacterial density and spirometry were measured. Safety, antibiotic use, hospitalization, and sputum neutrophils, elastase, DNA, IL-8, and myeloperoxidase were also evaluated. Sixty-six patients (mean age, 24 years, with mean baseline FEV1 of 74 +/- 20 (SD) percent predicted) were studied. One patient had bronchospasm after the first dose of IFN-gamma1b; the overall withdrawal rate was 15% (5 in the placebo group, 2 in the 500-microg IFN-gamma1b group, and 3 in the 1,000 microg IFN-gamma1b group). The 500-microg IFN-gamma1b dose was well-tolerated, but the 1,000-mug dose cohort, who had a higher baseline bacterial density than placebo patients (mean difference, 1.2 log(10) CFU/g sputum, 95% confidence interval (CI), 0.1,2.8, P=0.04), had 24% more hospitalizations for exacerbation than placebo patients (95% CI, 2,45%, P=0.05). There was a 0.12-l difference between the 500-microg IFN-gamma1b and placebo groups with respect to the 12-week change in FEV1 (active group minus placebo group, 95% CI, -0.03,0.26, P=0.11), as compared to a 0.01-l difference between the 1,000-microg IFN-gamma1b and placebo groups (95% CI, -0.16,0.17, P=0.96). No effects of IFN-gamma1b were seen in sputum bacterial density or inflammatory biomarkers at 12 weeks. Aerosolized IFN-gamma1b did not improve pulmonary function, reduce sputum bacterial density, or affect inflammatory sputum markers in patients with mild-moderate lung disease.
- Powers, S. W., Jones, J. S., Ferguson, K. S., Piazza-Waggoner, C., Daines, C., & Acton, J. D. (2005). Randomized clinical trial of behavioral and nutrition treatment to improve energy intake and growth in toddlers and preschoolers with cystic fibrosis. Pediatrics, 116(6), 1442-50.More infoTo conduct a randomized clinical trial comparing a behavioral and nutrition intervention (BEH) with a usual care control condition (CTL) for children (ages 18 months to 4 years) with cystic fibrosis (CF) and pancreatic insufficiency. This trial was designed to (1) evaluate a randomized comparison of BEH with CTL over 8 weeks, (2) provide a replication of the impact of BEH by inviting the CTL group to receive BEH after 8 weeks, and (3) examine the maintenance of BEH at 3- and 12-month follow-up.
- White, D. R., Giambra, B. K., Hopkin, R. J., Daines, C. L., & Rutter, M. J. (2005). Aspiration in children with CHARGE syndrome. International journal of pediatric otorhinolaryngology, 69(9), 1205-9.More infoPatients with Coloboma, Heart defect, choanal Atresia, Retarded development, Genitorenal and Ear abnormalities (CHARGE) syndrome have been reported to be at high risk for aspiration and swallowing difficulties. Aspiration has been implicated as the most common cause of mortality in these patients. To date, however, aspiration and swallowing disorders in CHARGE patients have not been independently studied.
- Ordoñez, C. L., Henig, N. R., Mayer-Hamblett, N., Accurso, F. J., Burns, J. L., Chmiel, J. F., Daines, C. L., Gibson, R. L., McNamara, S., Retsch-Bogart, G. Z., Zeitlin, P. L., & Aitken, M. L. (2003). Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis. American journal of respiratory and critical care medicine, 168(12), 1471-5.More infoInduced sputum has been used to study airway inflammation. We sought to determine whether markers of infection and inflammation in induced sputum were a useful and safe outcome measure in cystic fibrosis. We hypothesized that bacterial density and inflammatory content of induced sputum would decrease after antibiotic therapy. Induced sputum was assayed for bacterial density, cell count, and differential and inflammatory markers before and after treatment with intravenous antibiotics. Fifty-five of the 72 subjects enrolled (mean age +/- SD 18.2 +/- 7.9 years) completed the study. FEV1 increased by an average 0.3 +/- 0.3 L (10.4 +/- 8.7% predicted FEV1), p
- Phan, H., Sawicki, G. S., Quittner, A. L., Daines, C. L., Nasr, S. Z., Butcher, J. L., Psoter, K., & Riekert, K. A. (2018, Oct). Medication beliefs among adolescent and adult patients prescribed cystic fibrosis transmembrane protein regulator (CFTR) modulators.. 2018 North American Cystic Fibrosis Conference. Denver, CO: Cystic Fibrosis Foundation.More infoPhan H, Sawicki GS, Quittner AL, Daines CL, Nasr SZ, Butcher JL, Psoter K, Riekert KA. Medication beliefs among adolescent and adult patients prescribed cystic fibrosis transmembrane protein regulator (CFTR) modulators. Pediatr Pulmonol. 2018;53: 425.
- Repholz, A., Matthias, K. R., Sivinski, J., Lew, D., Tien, Q., Daines, C. L., & Phan, H. (2017, November). Extended Infusion of Beta-Lactams in the Treatment of Acute Pulmonary Exacerbations in Cystic Fibrosis: A Pilot Evaluation of Safety and Efficiacy Outcomes. 2017 North American Cystic Fibrosis Conference. Indianapolis, IN: Cystic Fibrosis Foundation.
- Daines, C. L. (2016, October). Depression and Anxiety Associate with Pain and Dyspnea but not FEV1 in the Mountain West. North American Cystic Fibrosis Conference.
- Daines, C. L. (2016, October). Effects of an Antioxidant-Enriched Multivitamin Supplement on Inflammation and Oxidative Stress in CF. North American Cystic Fibrosis Conference.
- Daines, C. L. (2016, October). Mountain West CF Consortium Sputum Biomarkers Study. North American Cystic Fibrosis Conference.
- Daines, C. L. (2016, October). Personalizing Cystic Fibrosis: A Longitudinal Analysis of the Microbiome From CF Patients. North American Cystic Fibrosis Conference.
- Daines, C. L. (2016, October). Redesigning Clinic Workflow to Improve Outpatient Cystic Fibrosis Care. North American Cystic Fibrosis Conference.
- Zahedieh, S., Seckeler, M., Andrews, J., Klewer, S. E., Scherer, K., Daines, C. L., & Barber, B. J. (2015, Fall). Regional and Racial Variation in Hospitalization Costs in Patients with Duchenne Muscular Dystrophy. American Academy of Pediatrics National Conference and Exhibition. Washington, DC: AAP.
- Phan, H., Cruz, V., Drake, G., McGuire, M. T., Werchan, B., Settle, P., Werchan, A., Campion, J. M., & Daines, C. L. (2016, October). Redesigning clinic workflow to improve outpatient cystic fibrosis care (published abstract and invited project platform presentation). Pediatric Pulmonology.More infoPublished Abstract for Platform Presentation, North American Cystic Fibrosis Conference