NACFC Meeting Presentations Print E-mail

North American Cystic Fibrosis Conference

Orlando, FL
October 11-13, 2012

  1. Rowe, S; Tearney, G, J. “Functional anatomic imaging using one micron resolution optical coherence tomography.: Pediatric Pulmonology Supp 35 (Summary S16.3), 2012.
  1. Konstan, M; Accurso, F; De Boeck, K; Kerem, E; Rowe, S; Sermet-Gaudelus, I; Wilschanski, M; Barth, J; Elfring, G; Peltz, S; Ajayi, T. “Targeting class 1 mutations: Update on ataluren as a promising treatment for nonsense mutation cystic fibrosis.” Pediatric Pulmonology Supp 35 (Summary S1.1), 2012.
  1. Thomas, PJ; Schmidt, A; Mendoza, JL; Millen, L; Richardson, J; Fuller, M; Patrick, A; Vetter, A; Karamysheva, Z; Karamyshev, AL. “Mechanism of action and molecular pathology: Matching drugs and mutants.” Pediatric Pulmonology Supp 35 (Summary S8.2), 2012.
  1. Chung, WJ; Goeckeler-Fried, J; Brodsky, J; Havasi, V; Rowe, SM; Piazza, GA; Keeton, AB; Sorscher, EJ. “Availability of F508del CFTR for small molecule correction.”  Pediatric Pulmonology Supp 35 (Abst 4), 2012.
  1. Chung, WJ; Fortenberry, JA; Lukacs, G; Roldan, HA; Kappes, JC; Brouillette, C; DeLucas, LJ; Ray, MJ; Sorscher, EJ; Accavitti-Loper, MA. “Developing antibodies capable of monitorind conformation and maturation of CFTR.” Pediatric Pulmonology Supp 35 (Abst 5), 2012.
  1. Aller, S; Tang, LP; Rajpur, J; Sloane, P; Sorscher, EJ; Rowe, SM. “Identification of efficacious F508del CFTR correctors using a novel homology model.” Pediatric Pulmonology Supp 35 (Abst 12), 2012.
  1. Goeckeler-Fried, J; Chiang, A; Chung, W. J; Havasi, V; Weissman, AJ; Lewis, T; Brodsky, JL. “Synergistic correction of ΔF508-CFTR when an inhibitor of protein ubiquitination is combined with a small molecule corrector.” Pediatric Pulmonology Supp 35 (Abst 33), 2012.
  1. Wang, C; Kota, P; An, J; Protassevitch, I; Dokholyan, NV; Brouillette, C; Hunt, JF. “A robust and efficient visible fluorescence assay for human NBD1 folding and stability designed for mechanistic studies and high-throughput screening for corrector compounds.” Pediatric Pulmonology Supp 35 (Abst 50), 2012.
  1. Hudson, R; Chong, PA; Protasevich, II; Dawson, J; Vernon, R; Noy, E; Bihler, H; An, JL; Kalid, O; Sela-Culang, I; Mense, M; Senderowitz, H; Brouillette, C; Forman-Kay, J. “Direct binding of CFTR modulators to human CFTR NBD1.” Pediatric Pulmonology Supp 35 (Abst 59), 2012.
  1. Yang, Z; Wang, C; Zhou, Q; An, J; Hunt, JF; Brouillette, C. ”The importance of the NBD domains in the selection of detergents for CFTR purification.” Pediatric Pulmonology Supp 35 (Abst 67), 2012.
  1. Rooj, AK; Cormet-Boyaka, E; Clark, EB; Qadri, YJ; Sorscher, EJ; Fuller, CM; Berdiev, BK. “Association of CFTR with ENaC subunits carrying Liddle’s Syndrome mutations.” Pediatric Pulmonology Supp 35 (Abst 68), 2012.
  1. McClure, M; Wilson, L; Ray, M; DeLucas, LJ; Rowe, S; Wu, X; Dai, Q; Hong, J; Sorscher, EJ; Kappes, JC; Barnes, S. “Post-translational modifications of CFTR glycoforms identified by high resolution, high mass accuracy mass spectrometry.” Pediatric Pulmonology Supp 35 (Abst 70), 2012.
  1. McClure, M; Wilson, L; Ray, M; DeLucas, LJ; Rowe, S; Wu, X; Dai, Q; Hong, JS; Kappes, JC; Barnes, S; Sorscher, EJ. “S-palmitoylation mediates trafficking of CFTR to the cell surface.” Pediatric Pulmonology Supp 35 (Abst 71), 2012.
  1. Zhang, S; Skinner, D; Chaaban, M; Sorscher, E; Woodworth, BA. “CFTR activation by the solvent ethanol: Implications for CF drug testing and delivery.” Pediatric Pulmonology Supp 35 (Abst 75), 2012.
  1. DeLucas, LJ; Kappes, JC; Dai, Q; Ray, M; Brackin, W; McCombs, D; Johnson, D; Ding, H; Hildebrandt, E; Purna, B; Riordan, JR; Urbatsch, IL. “Progress toward elucidating the 3D structure of full-length CFTR.” Pediatric Pulmonology Supp 35 (Abst 78), 2012.
  1. Raju, SV; Jackson, PL; McNicholas, CM; Sloane, PA; Sabbatini, GM; Tang, LP; Mazur, M; Liu, B; Clancy, JP; Bowen, L; Accurso, F; Blalock, JE; Dransfield, M; Rowe, SM. “Defects in CFTR activity can be acquired and transmitted by a systemic pathway.” Pediatric Pulmonology Supp 35 (Abst 81), 2012.
  1. Harris, WT; Kelly, D; Hagood, J; Ambalavanan, N; Sorscher, E. “Myofibroblast proliferation and fibrosis in CF lung disease.” Pediatric Pulmonology Supp 35 (Abst 106), 2012.
  1. Szul, T; Gaggar, A; Xu, X. “Expression of prolyl endopeptidase in bronchial epithelial cells: Impact of F508del CFTR.” Pediatric Pulmonology Supp 35 (Abst 110), 2012.
  1. McNicholas, CM; Gaggar, A. “Neutrophil elastase modulates CFTR current in bronchial epithelial cells.” Pediatric Pulmonology Supp 35 (Abst 125), 2012.
  1. Jackson, PL; Xu, X; Davis, V; Jones, C; Okafor, S; Noerager, B. “Modulation of small matrikines by reactive aldehydes in CF.” Pediatric Pulmonology Supp 35 (Abst 150), 2012.
  1. Tuggle, KL; Gamber, K; Cui, X; Ji, D; Chambers, A; Bedwell, DM; Rowe, SM; Sorscher, EJ; Fanucchi, MV. “Generation of a CFTR-knockout rat model for cystic fibrosis.” Pediatric Pulmonology Supp 35 (Abst 162), 2012.
  1. Guo, J; Sorscher, EJ; Hartman, JL. “Advancing a yeast phenomics model for gene interaction network analysis of F508del-CFTR biogenesis.” Pediatric Pulmonology Supp 35 (Abst 184), 2012.
  1. Skinner, D; Zhang, S; Chaaban, M; Sorscher, E; Woodworth, BA. “Primary rabbit nasal septal epithelial cultures for studies of cystic fibrosis sinus disease.” Pediatric Pulmonology Supp 35 (Abst 188), 2012.
  1. Rowe, S; Sermet-Gaudelus, I; Konstan, M; Kerem, E; Wilschanski, M; DeBoeck, K; Accurso, F; Miller, NL; Elfring, GL; Barth, J; Ajayi, T. “Results of the phase 3 study of ataluren in nonsense mutation cystic fibrosis (NMCF).” Pediatric Pulmonology Supp 35 (Abst 193), 2012.
  1. Rowe, SM; Tang, LP; Xue, X; Biswas, S; Du, M; Belakhov, V; Kandasamy, J; Chen, F; Schacht, J; Baasov, T; Bedwell, D. “The synthetic aminoglycoside NB124 suppresses CFTR premature termination codons more effectively than gentamicin and prior synthetic derivatives.” Pediatric Pulmonology Supp 35 (Abst 194), 2012.
  1. Xu, X; Sabbatini, GM; Hathorne, H; Clancy, JP; Gaggar, A. “A randomized double blinded placebo control trial of doxycycline as an adjunctive anti-inflammatory agent during CF exacerbation (DOXY).” Pediatric Pulmonology Supp 35 (Abst 209), 2012.
  1. Clancy, JP; Donaldson, S; Gabriel, S; Hornick, D; Starner, TD; Rowe, S; VanDyke, R; Fenchel, M; Sun, H. “Centralized intestinal current measurements (ICM): CFTR activity in fresh and cold-stored rectal biopsies, and comparison of forceps vs. suction biopsy.” Pediatric Pulmonology Supp 35 (Abst 210), 2012.
  1. Kerem, E; Wilschanski, M; Accurso, F; DeBoeck, K; Konstan, M; Rowe, S; Sermet-Gaudelus, I; Miller, N; Elfring, GL; Barth, J; Ajayi, T. “Natural history of cystic fibrosis in patients with nonsense-mutation-mediated disease.” Pediatric Pulmonology Supp 35 (Abst 252), 2012.
  1. Liu, L; Wilsterman, E; Shastry, S; Houser, G; Smith, A; Boylan, N; Hanes, J; Tearney, G; Rowe, SM. “Measuring mucus viscosity using micro-optical coherence tomography developed microrheology techniques.” Pediatric Pulmonology Supp 35 (Abst 255), 2012.
  1. Kong, M; Peng, N; Clancy, JP; Blalock, E; Gaggar, A; Sorscher, EJ. “”RSV stimulates MMP-9 release from CF epithelial cells and increases MMP activity in human lungs.” Pediatric Pulmonology Supp 35 (Abst 263), 2012.
  1. Dennis, E; Coats, M; Briles, D; Crain, M. “The effects of CFTR and capsular phenotype on susceptibility and innate immune responses in a mouse model of pneumococcal lung disease.” Pediatric Pulmonology Supp 35 (Abst 303), 2012.
  1. Searcy, H; Phillips, S; Benner, K; Britton, LJ; Harris, WT; Hoover, W. “Eradication of Pseudomonas aeruginosa in pediatric patients with cystic fibrosis: A comparison of protocols.” Pediatric Pulmonology Supp 35 (Abst 346), 2012.
  1. Harris, WT; Boyd, JT; VanDyke, R; Fenchel, M; Brody, A; McPhail, GL; Clancy, JP. “CF-related constrictive bronchiolitis in adolescents with refractory pulmonary decline.” Pediatric Pulmonology Supp 35 (Abst 372), 2012.
  1. Searcy, H; Britton, LJ; Askenazi, D; Scott, A; Lyrene, R; Gutierrez, H. “Prevention of acute kidney injury in hospitalized children with cystic fibrosis.” Pediatric Pulmonology Supp 35 (Abst 430), 2012.
  1. Britton, LJ; Mims, C; Harris, WT; Brown, J. “Predictors of parental knowledge and patient outcomes in patients diagnosed by newborn screening.” Pediatric Pulmonology Supp 35 (Abst 436), 2012.
  1. Rodgers, TF; Britton, LJ; Woodridge, N; Stalvey, MS; Gutierrez, H; Hoover, W. “Mesosystems approach to cystic fibrosis related diabetes (CFRD) management.” Pediatric Pulmonology Supp 35 (Abst 477), 2012.
  1. Britton, LJ; Gutierrez, H; Hardy, J. “Patient acceptance and satisfaction with complete segregation while hospitalized.” Pediatric Pulmonology Supp 35 (Abst 490), 2012.
  1. Hoover, W; Phillips, C; Benner, K; Britton, LJ; Harris, WT; Searcy, H. “Effect of proton pump inhibitors on absorption and serum levels of vitamin D in cystic fibrosis patients.” Pediatric Pulmonology Supp 35 (Abst 533), 2012.
  1. Kines, K; Wooldridge, N; Griffin, R; Britton, LJ; Hoover, W; Gutierrez, H. “Nutritional status and disease severity in pre-adolescence and adolescent patients with cystic fibrosis.” Pediatric Pulmonology Supp 35 (Abst 535), 2012.
  1. Stalvey, MS; Clines, KL; Chung, WJ; Clines, GA. “Cystic fibrosis bone disease: Interactions between osteoblasts and osteoclasts may be the key to increased bone loss.” Pediatric Pulmonology Supp 35 (Abst 541), 2012.
  1. Grossoehme, DH; VanDyke, R; Dimitriou, S; Britton, LJ; McPhail, GL; Seid, M. “Airway clearance adherence: Influence of parental spirituality and depression.” Pediatric Pulmonology Supp 35 (Abst 576), 2012.
  1. Loosen, H; Vela, K; Klintworth, T; Lew, J; Lowman, J. “On the Go with ECMO! Preparing Clinical Patients for Transplant with Exercise & Ambulation” (Care) Pediatric Pulmonology Supp 35. 2012.

Anaheim, CA
November 3-5, 2011

1. Kirk, KL. “ATP-free CFTR channel gating: Constitutive mutants, R domain regulation and CF relevance.” Pediatric Pulmonology Supp 34 (Summary S4.4), 2011.

2. Rowe, SM. “Measuring CFTR activity in clinical trials: in vivo assays.” Pediatric Pulmonology Supp 34 (Summary S14.2), 2011.

3. Rowe, SM; Boyle MP. “Combination of correctors and potentiators for F508del CFTR.”  Pediatric Pulmonology Supp 34 (Summary S15.3), 2011.

4. Hudson, R; Chong, P; Dawson, JE; Mense, M; Thomas PJ; Brouillette, C; Forman-Kay, JD “Common conformational changes within NBD1 elicited by CF modulators, an ICL4 peptide and suppressor mutations.” Pediatric Pulmonology Supp 34 (Abst 7), 2011.

5. Chung, W; Fortenberry, JA; Kappes, JC; DeLucas, LJ; Marjorie, RJ; Alverson, P; Sorscher, EJ; Accavitti-Loper, M. “Towards development of conformationally sensitive monoclonal antibodies against CFTR.” Pediatric Pulmonology Supp 34 (Abst 26), 2011.

6. Chung, W; Rowe, SM; Sorscher, EJ; Piazza, GA; Keeton, AB. “High content cell-based imaging to identify small molecule correctors of F508del-CFTR.” Pediatric Pulmonology Supp 34 (Abst 27), 2011.

7. Wang, C; Protassevitch, I; An, J; Kota, P; Dokholyan, N; Brouillette, C; Hunt, J. “Engineering a cys-less human NBD1 for use in visible fluorescence assays of domain stability and intermolecular associations.” Pediatric Pulmonology Supp 34 (Abst 46), 2011.

8. McClure, M; Wilson, L; DeLucas, LJ; Ray, M; Rowe, SM; Wu, X; Dai, Q; Hong, J; Kappes, JC; Sorscher, EJ; Barnes, S. “Identification of CFTR post-translational modification by mass spectrometry.” Pediatric Pulmonology Supp 34 (Abst 64), 2011.

9. McClure, M; Wilson, L; DeLucas, LJ; Ray, M; Rowe, SM; Wu, X; Dai, Q; Hong, JS; Kappes, JC; Barnes, S; Sorscher, EJ. “S-palmitoylation is a regulator of CFTR processing.” Pediatric Pulmonology Supp 34 (Abst 65), 2011.

10. Liu, L; Shastry, S; Sloane, PA; Mazur, M; Parker, S; Grizzle, W; Bouma, BE; Rowe, SM; Tearney, GJ. “Visualizing respiratory ciliary motion and mechanosensitivity of ciliated cells using 1-mm resolution optical coherence tomography (MOCT).” Pediatric Pulmonology Supp 34 (Abst 71), 2011.

11. Maeng, P; Kong, M; Hong, J; Sorscher, E; VanDyke, R; Clancy, J. “RSV infection rapidly disrupts airway cell monolayer integrity in cells expressing F508del CFTR, but not in monolayers expressing wt CFTR.” Pediatric Pulmonology Supp 34 (Abst 78), 2011.

12. Sabbatini, GM; Xu, X; Gaggar, A. “C-reactive protein as a potential biomarker predictive for inpatient exacerbation in cystic fibrosis.” Pediatric Pulmonology Supp 34 (Abst 81), 2011.

13. Sanders, Y; Harris, T; Li, Y; Clancy, JP. “TGF-beta down-regulates CFTR and TMEM16A expression and function in polarized T84 cells.” Pediatric Pulmonology Supp 34 (Abst 93), 2011.

14. Skinner, D; Alexander, NS; Hatch, N; Zhang, S; Fortenberry, J; Sorscher, EJ; Woodworth, BA. “Resveratrol has salutary effects on mucociliary transport and inflammation in sinonasal epithelium.” Pediatric Pulmonology Supp 34 (Abst 108), 2011.

15. Zhang, S; Blount, A; Chestnut, M; Hixon, B; Skinner, D; Sorscher, EJ; Woodworth, BA. “Transepithelial ion transport through CFTR and TMEM16A is suppressed in hypoxic sinonasal epithelium.” Pediatric Pulmonology Supp 34 (Abst 109), 2011.

16. Woodworth, BA; Zhang, S; Skinner, D; Sorscher, EJ; Rowe, SM. “Comparison of CFTR and ciliary beat frequency activation by the CFTR modulators VX-770, VRT532, and UCCF-152 in primary sinonasal epithelial cultures.” Pediatric Pulmonology Supp 34 (Abst 114), 2011.

17. Hong, J; Guo, J; Louie, R; Wen, H; Sorscher, EJ; Miller, E; Hartman, JL. “A pro-biogenesis complex for F508del-CFTR discovered with a yeast model and validated in human cells.” Pediatric Pulmonology Supp 34 (Abst 178), 2011.

18. Harris, WT; Nicola, T; Macewen, M; Li, Y; Ambalavanan, N; Clancy, JP. “TGF-Β production and myofibroblast differentiation in cystic fibrosis.” Pediatric Pulmonology Supp 34 (Abst 184), 2011.

19. Guo, J; Louie, R; Rodgers, JW; White, R; Hong, J; Sorscher, EJ; Bryan, JA; Miller, EA;  Hartman, JL. “A conserved biogenesis network for yeast YOR1-ΔF and CFTR-ΔF.”  Pediatric Pulmonology Supp 34 (Abst 185), 2011.

20. Havasi, V; Buckley-Lanier, J; Childress-Roper, V; Fortenberry, JA; Schoeb, TR; Bedwell, DM; Yoder, BK; Sorscher, EJ. “Developing a CFTR deficient PKD mouse model.” Pediatric Pulmonology Supp 34 (Abst 193), 2011.

21. Clancy, JP; Sun, H; Donaldson, SH; Gabriel, S; Starner, TD; Ahrens, R; Hornick, DB; Liu, B; Rowe, SM. “CF and non-CF intestinal biopsy currents defining CFTR function through ROC curve analyses and ex vivo modulation.” Pediatric Pulmonology Supp 34 (Abst 199), 2011.

22. Clancy, JP; Rowe, SM; Liu, B; Hathorne, H; Dong, Q; Wisseh, S; Ordonez, C. “Variability of nasal potential difference measurements in clinical testing of CFTR modulators.” Pediatric Pulmonology Supp 34 (Abst 202), 2011.

23. Konstan, MW; Accurso, FJ; DeBoeck, K; Kerem, E; Rowe, SM; Sermet-Gaudelus, I; Wilschanski, M; Miller, N; Elfring, GL; Barth, J; Ajayi, T. “Pretreatment data from phase 3 study of ataluren document significant disease burden in a subpopulation of patients with nonsense mutation cystic fibrosis.” Pediatric Pulmonology Supp 34 (Abst 232), 2011.

24. Sabbatini, GM; Frederick, C; Konstan, MW; Rosenbluth, D; Liu, B; Bucur, C; Burks, P; Li, Y;  Lymp, JF; Clancy, JP; Rowe, SM. “Nasal potential difference studies utilizing the CFTR modulator quercetin.” Pediatric Pulmonology Supp 34 (Abst 244), 2011.

25. Sloane, PA; Shastry, S; Wilhelm, A; Courville, C; Tang, l; Mehta, N; Backer, K; Levin, E; Mazur, M; Van Goor, F; Byan-Parker, S; Grizzle, W; Sorscher, EJ; Dransfield, MT; Rowe, SM. Pharmacologic rescue of acquired CFTR dysfunction in chronic obstructive pulmonary disease. Pediatric Pulmonology Supp 34 (Abst 245), 2011.

26. Ballew, B; Troxler, B; Britton, LJ; Hoover, W; Searcey, H; Cates, M. Clinical outcomes in cystic fibrosis patients prescribed thrice weekly azithromycin without chronic infection with Pseudomonas aeruginosa. Pediatric Pulmonology Supp 34 (Abst 348), 2011.

27. Hoover, W; Mims, C; Gamble, S. Sensitivity and specificity of infant pulmonary function testing in sick and well CF infants identified by newborn screening. Pediatric Pulmonology Supp 34 (Abst 374), 2011.

28. Gutierrez, H; Self, S; Gamble, S. Statistical process control (SPC)—validated home spirometry for CF lung disease. Pediatric Pulmonology Supp 34 (Abst 380), 2011.

29. Hathorne, H; Britton, LJ; Reeves, GA; Sabbatini, GM; Tarn, VE. Initiation of quality improvement within the clinical research team. Pediatric Pulmonology Supp 34 (Abst 441), 2011.

30. Grant, AP; Britton, LJ; Wooldridge, N; Gutierrez, H. Room service for the patient with cystic fibrosis: Patient and staff satisfaction survey. Pediatric Pulmonology Supp 34 (Abst 469), 2011.

31. Gutierrez, H; Self, S; Mims, C. Use of checklists to assist patients with early recognition of acute pulmonary exacerbation in cystic fibrosis. Pediatric Pulmonology Supp 34 (Abst 473), 2011.

32. Bailey, J; Wooldridge, N; Redden, D; Britton, LJ; Hoover, W; Gutierrez, H. Predictors of vitamin D insufficiency in pediatric patients with cystic fibrosis. Pediatric Pulmonology Supp 34 (Abst 509), 2011.

Baltimore, Maryland
October 21-23, 2010

1. Pyle LC, Balch W, Lukacs G, Braakman I, Guggino W, Thomas PJ, Penland C, Pollard H, Brodsky J, Frizzell R, Sorscher EJ, Skach WR. "Developing a cellular roadmap for correctors of CFTR misfolding." Pediatric Pulmonology Supp 33 (Abst 7), 2010.

2. Sorscher EJ, Hong JS, Hutt DM, Chalfant M, Roth DM, Balch W, Noel S, Frizzell R, Okiyoneda T, Veit G, Lukacs G, Eidelman O, Jozwik C, Caohuy H, Eudy Y, Pollard H, Guggino B, Tessari MA, Fischer DF. "Screening of candidate adenovirus vectors expressing shRNAs for functional recovery of ΔF508 CFTR." Pediatric Pulmonology Supp 33 (Abst 9), 2010.

3. Protasevich I, Yang Z, Wang C, Atwell S, Zhao X, Emtage S, Wetmore D, Hunt JF, Brouillette C. "Analysis of the thermal unfolding of CFTR nucleotide binding domain 1 suggests a mechanism for the trafficking defect and temperature rescue of (F508del) CFTR." Pediatric Pulmonology Supp 33 (Abst 40), 2010.

4. Chung W, Ehrhardt A, Pyle LC, Wang W, Bear CE, Lukacs G, Kirk KL, Sorscher EJ. "A biochemical assay for discovery of gating modulators based on intramolecular interactions between nucleotide binding domain 1 and the first cytoplasmic loop of CFTR." Pediatric Pulmonology Supp 33 (Abst 47), 2010.

5. Li Y, Clancy JP. "Stability of C Band CFTR in T84 cell lysates with varying low temperature storage conditions." Pediatric Pulmonology Supp 33 (Abst 53), 2010.

6. Sabbatini GM, Donaldson SH, Dellom E, Bendahmane N, Quinney N, Gabriel S, Theresi M, Starner TD, Hornick DB, Ernst S, Karp P, Fan L, Sugandha S, Clancy JP. "Development of ex vivo GI outcome measures for studies of CFTR modulators." Pediatric Pulmonology Supp 33 (Abst 58), 2010.

7. Mitchell LH, Pyle LC, Bolger GB. "Activation of CFTR by the CAMP-specific phosphodiesterase PDE4." Pediatric Pulmonology Supp 33 (Abst 61), 2010.

8. McClure M, DeLucas LJ, Wilson L, Ray M, Hong JS, Kappes JC, Sorscher EJ, Barnes S. "Post-translational modifications of CFTR with relevance to gating and processing." Pediatric Pulmonology Supp 33 (Abst 62), 2010.

9. Harris WT, Grenett HE, Macewan M, Clancy JP. "TGFβ1 activation in cystic fibrosis cell culture model systems." Pediatric Pulmonology Supp 33 (Abst 85), 2010.

10. Solomon GM, Frederick C, Sabbatini G, Gaggar A, Harris T, Woodworth BA, Steele C, Rowe SM. "IP-10 is a potential biomarker of acute pulmonary exacerbations of cystic fibrosis in nasal lavage." Pediatric Pulmonology Supp 33 (Abst 105), 2010.

11. Tuggle KL, Asmellash S, Dickinson DA, Mobley JA, Fanucchi MV. "Early life exposure to ozone alters airway epithelial responses in wild-type and cystic fibrosis transmembrane receptor (CFTR) knockout mice." Pediatric Pulmonology Supp 33 (Abst 133), 2010.

12. Zhang S, Skinner D, Hicks B, Bevensee M, Sorscher EJ, Woodworth BA. "Sinupret® stimulates ciliary beat frequency and transepithelial chloride transport through CFTR dependent and independent mechanisms." Pediatric Pulmonology Supp 33 (Abst 137), 2010.

13. Skinner D, Zhang S, Azbell C, Fortenberry J, Sorscher EJ. "Hesperidin stimulates CFTR-mediated chloride secretion and ciliary beat frequency in sinonasal epithelium." Pediatric Pulmonology Supp 33 (Abst 138), 2010.

14. Guo J, Louie R, Rodgers JW, Miller E, Sorscher EJ, Hartman JL. "YOR1-ΔF as a CFTR-ΔF508-like mutation to identify modulators in S. cerevisiae." Pediatric Pulmonology Supp 33 (Abst 201), 2010.

15. Havasi V, Buckley-Lanier JA, Childress Roper V, Fortenberry JA, Bedwell DM, Sorscher EJ, Yoder BK. "Development of a CFTR deficient ciliopathic mouse model." Pediatric Pulmonology Supp 33 (Abst 215), 2010.

16. Liu L, Shastry S, Sloane PA, Mazur M, Parker SB, Bouma BE, Tearney GJ, Rowe SM. "Modulation of ion transport and mucus clearance in cells and tissues: New insights from video-rate reflectance tomography imaging." Pediatric Pulmonology Supp 33 (Abst 222), 2010.

17. Clancy JP, Rowe SM, Accurso FJ, Ballmann M, Boyle MP, DeBoeck C, Konstan MW, Spencer-Green G. "A Phase II, randomized, placebo-controlled, clinical trial of four doses of VX-809 in CF patients homozygous for the F508del CFTR mutation." Pediatric Pulmonology Supp 33 (Abst 224), 2010.

18. Clancy JP, Rowe SM, Durie PR, Konstan MW, Dunitz J, Hornick DB, Sagel SD, Boyle MP, Uluer AZ, Moss RB, Freedman S, Dong Q, Zha J, Stone A, Olson E, Ordonez C, Campbell P, Ashlock M, Accurso FJ. "Comparison of NPD parameters in a phase IIa study to optimize detection of CFTR modulator bioactivity in clinical trials." Pediatric Pulmonology Supp 33 (Abst 231), 2010.

19. Lui B, Hathorne H, Hill A, Hovick G, Cohen M, Beamer J, Solomon MG, Clancy JP, Rowe SM. "Normative values and receiver operating characteristics of NPD for diagnostic measurements." Pediatric Pulmonology Supp 33 (Abst 247), 2010.

20. Clancy JP, Minic P, Dupont L, Goss CH, Quittner AL, Lymp JF, Burns JL, Govan JR, Ramsey B, Gupta R. "Full analyses of data from two phase II blinded and placebo-controlled studies of nebulized liposomal amikacin for inhalation (arikaceTM) in the treatment of CF patients with Pseudomonas aeruginosa lung infection." Pediatric Pulmonology Supp 33 (Abst 227), 2010.

21. Sloane PA, Tang L, Mazur M, Buckley-Lanier J, Bedwell D, Rowe SM. "Translational readthrough of premature stop codons combined with CFTR potentiation: Potential for combination CFTR therapy." Pediatric Pulmonology Supp 33 (Abst 264), 2010.

22. Kerem E, Wilschanski M, Melotti P, Sermet-Gaudelus I, DeBoeck K, Rowe SM, Konstan MW, Accurso F, Pimentel S, Constantine S, Miller N, Barth J, Miller L, Ajayi T. "Phase 3 study of Ataluren (PTC124®) in nonsense mutation cystic fibrosis (NMCF): Demographic and other baseline data." Pediatric Pulmonology Supp 33 (Abst 266), 2010.

23. Rowe SM, van Goor F, Clancy JP, Durie PR, Konstan MW, Dunitz J, Hornick DB, Sagel SD, Boyle MP, Uluer AZ, Moss RB, Ramsey B, Freedman S, Dong Q, Zha J, Stone A, Olson E, Ordonez C, Campbell P, Ashlock M, Accurso FJ. "Corresponding effects of VX-770 on NPD in vivo and human bronchial epithelial (HBE) cells in vitro." Pediatric Pulmonology Supp 33 (Abst 281), 2010.

24. Merriss M, Thompson P, Britton LJ, Troxler RB, Hoover W. "Burkholderia cepacia eradication in cystic fibrosis patients: A retrospective review." Pediatric Pulmonology Supp 33 (Abst 338), 2010.

25. Maeng P, Li Y, Hong JS, Gaggar A, Clancy JP, Kong M. "RSV infection stimulates MMP-9 expression and release from human airway monolayers, and replication is abrogated by MMP-9 inhibition." Pediatric Pulmonology Supp 33 (Abst 362), 2010.

26. Coats M, Nahm MH, Briles DE, Crain MJ. "Mucoid pneumococci are unusually common in the cystic fibrosis lung." Pediatric Pulmonology Supp 33 (Abst 372), 2010.

27. Zhang S, Skinner D, Sorscher EJ, Woodworth BA. "LPS and Pseudomonas aeruginosa filtrate reduce calcium activated chloride channel transport in primary sinonasal epithelial cultures." Pediatric Pulmonology Supp 33 (Abst 382), 2010.

28. Sabbatini GM, Britton LJ, Hathorne HY, Reeves GA, Lucious TD. "Integration of clinical and research terms improve patient enrollment in clinical trials." Pediatric Pulmonology Supp 33 (Abst 501), 2010.

29. Britton LJ, Boren KE, Hoover W, Rodgers TF, Gutierrez HH. "Assessment of team satisfaction, meeting quality, and team cohesiveness." Pediatric Pulmonology Supp 33 (Abst 517), 2010.

30. Dharmalingam P, Kurundkar A, Harris WT, Clancy JP, Sorscher EJ, Maheshwari A. "Intestinal mucosal inflammation in cystic fibrosis." Pediatric Pulmonology Supp 33 (Abst 572), 2010.

31. Wooldridge N, Anderson V, Gutierrez H, Gardner J, Britton LJ. "The relationship between suboptimal serum 25-hydroxyvitamin D levels and cystic fibrosis related diabetes." Pediatric Pulmonology Supp 33 (Abst 587), 2010.

32. Christian BJ. "Children's perceptions of growing up with cystic fibrosis: Discovering the chronic illness trajectory." Pediatric Pulmonology Supp 33 (Abst 614), 2010.

33. Britton LJ, Mims C, Brown J, Troxler B. "Nursing annual visit questionnaire to assess patient and family knowledge of CF." Pediatric Pulmonology Supp 33 (Abst 636), 2010.

34. Self S, Gutierrez H, Britton LJ, Troxler B. "Evaluation of different education tracks for parents of babies diagnosed with CF through newborn screening." Pediatric Pulmonology Supp 33 (Abst 650), 2010.

Minneapolis, Minnesota
October 15-17, 2009


1. Hutt, DM; Herman, DM; Rodrigues, AP; Noel, S; Gentzsch, M; Pilewski, JM; Riordan, JR; Sorscher, EJ; Kelly, JW; Frizzell, RA; Manning, G; Gottesfeld, JM; Balch, WE. "Restoring ΔF508-CFTR Trafficking and Activity with Epigenetic Modifiers" Pediatric Pulmonology Supp 32 (Abst 8), 2009.

2. Protasevich, I; Yang, Z; Wang, C; Atwell, S; Zhao, X; Emtage, S; Wetmore, D; Hunt, J; Brouillette, C. "Analysis of the Thermal Unfolding of CFTR Nucleotide Binding Domain 1 by Differential Scanning Calorimetry" Pediatric Pulmonology Supp 32 (Abst 41), 2009.

3. Zhang, S; Smith, N; Schuster, D; Sorscher, EJ; Rowe, SM; Woodworth, BA. "Activation of Ciliary Beat Frequency in Primary Murine Nasal Epithelial Cultures by the CFTR Potentiator, Quercetin" Pediatric Pulmonology Supp 32 (Abst 44), 2009.

4. DeLucas, LJ; Kappes, JC; Pruett, P; Ray, M; Dai, Q. "Large-Scale Expression and Purification of CFTR" Pediatric Pulmonology Supp 32 (Abst 56), 2009.

5. Wang, C; Protasevich, I; Yang, Z; Atwell, S; Zhao, X; Emtage, S; Wetmore, D; Brouillette, C; Hunt, J. "Integrated Biophysical Studies of HNBD1 Unfolding" Pediatric Pulmonology Supp 32 (Abst 57), 2009.

6. Harris, WT; Kelly, DR; Hagood, JS. "Interstitial Remodeling: A Potential Link Between TGF-Β1 and CF Lung Disease Histopathology" Pediatric Pulmonology Supp 32 (Abst 92), 2009.

7. Xu, X; Jackson, PL; Luy, G; Tanner, S; Hardison, MT; Blalock, JE; Gaggar, A. "Acetylated PGP Induces Release of MMP-9 from Neutrophils via an ERK-Dependent Pathway" Pediatric Pulmonology Supp 32 (Abst 107), 2009.

8. Liu, L; Bouma, BE; Rowe, SM; Tearney, GJ. "Video-Rate Ultrahigh-Resolution 3D Optical Imaging of the Pulmonary Airways" Pediatric Pulmonology Supp 32 (Abst 120), 2009.

9. Virgin, FW; Wade, MB; St. John, JJ; Rowe, SM; Gaggar, A; Leon, KJ; Young, K; Woodworth, BA "Aggressive Surgical & Postoperative Medical Management for Severe, Recalcitrant Cystic Fibrosis Chronic Rhinosinusitis" Pediatric Pulmonology Supp 32 (Abst 156), 2009.

10. Plyler, ZE; Bernard, KF; Havasi, V; Keiles, SB; Hong, JS; Wen, H; Sorscher, EJ. "Analysis of Synonymous CFTR Polymorphisms as Contributors to CBAVD" Pediatric Pulmonology Supp 32 (Abst 188), 2009.

11. Chung, W; Rowe, SM; Keeton, AB; Shevin, R; Piazza, GA; Sorscher, EJ. "A Novel Cell-Based Imaging Assay for High Throughput Screens to Identify Small Molecule Correctors of ΔF508-CFTR" Pediatric Pulmonology Supp 32 (Abst 192), 2009.

12. Chung, W; Fortenberry, JA; Kappes, JC; Accavitti-Loper, MA; DeLucas, LJ; Ray, MJ; Alverson, P; Sorscher, EJ. "Development of Conformationally Sensitive Monoclonal Antibodies Raised Against Full-Length CFTR" Pediatric Pulmonology Supp 32 (Abst 193), 2009.

13. Clancy, JP; Li, Y; Gentzsch, M. "Relationships Between Corrected ΔF508 and WT CFTR Activity in CFBE41o- Cells in the Presence and Absence of Potentiators" Pediatric Pulmonology Supp 32 (Abst 199), 2009.

14. Clancy, JP; Gabriel, S; Gentzsch, M; Donaldson, S; Hornick, D; Ahrens, R; Lymp, J; Hocevar-Trnka, J. "Development of Multicenter GI Outcome Measures for CFTR Modulator Clinical Trials" Pediatric Pulmonology Supp 32 (Abst 200), 2009.

15. Guo, J; Louie, R; Rodgers, JW; Miller, E; Sorscher, EJ; Hartman, JL. "Genome-Wide Epistasis Analysis of a CFTR-ΔF508-Like Mutation in S. Cerevisiae" Pediatric Pulmonology Supp 32 (Abst 208), 2009.

16. Solomon, GM; Konstan, MW; Wilschanski, M; Billings, J; Sermet, I; Accurso, FJ; Vermeleun, F; Young, H; Reeves, G; Sabbatini, G; Mayer-Hamblett, N; Ashlock, M; Clancy, JP; Rowe, SM. "Universal Protocol for Nasal Potential Difference Studies: Results of an International Multi-Center Clinical Trial" Pediatric Pulmonology Supp 32 (Abst 215), 2009.

17. Rowe, SM; Backer, K; Tang, L; Woodworth, B; Mazur, M; Buckley-Lanier, J; Schwiebert, E; Baasov, T; Bedwell, DM. "Enhanced Activity for Translational Readthrough and Reduced Toxicity Demonstrated by the Synthetic Aminoglycoside NB54" Pediatric Pulmonology Supp 32 (Abst 218), 2009.

18. Clancy, JP; Rowe, SM; Durie, P; Freedman, S; Dong, Q; Ordonez, C; Ashlock, M; Accurso, FJ. "NPD Evaluation of Ion Transport in G551D CF Patients Treated with a CFTR Potentiator" Pediatric Pulmonology Supp 32 (Abst 222), 2009.

19. Pyle, LC; Fan, L; Fortenberry, J; Tang, L; Backer, K; Mazur, M; Clancy, JP; Sorscher, EJ; Rowe, SM. "Comprehensive Spectrum of Activity Analysis for ΔF508-CFTR Correctors" Pediatric Pulmonology Supp 32 (Abst 229), 2009.

20. Goss, CH; Clancy, JP; Nick, JA; Billings, J; Rubenstein, RC; Young, KR; Sannuti, A; Lechtzin, N; Nasr, SZ; Sawicki, GS; Quittner, AL; Lymp, JF; Hamblett, NM; Saiman, L; Burns, JL; Ramsey, B; Gupta, R. "A Phase II Blinded and Placebo-Controlled Study of Nebulized Liposomal Amikacin (ArikaceTM) in the Treatment of CF Patients with Pseudomonas Aeruginosa Lung Infection" Pediatric Pulmonology Supp 32 (Abst 239), 2009.

21. Troxler, RB; Mobley, JA; Bowersock, G; Parrott, M; Roach, M; Sorscher, EJ. "Proteomic Analysis of Salivary Fluid in Cystic Fibrosis" Pediatric Pulmonology Supp 32 (Abst 263), 2009.

22. Zhang, S; Schuster, D; Bedwell, D; Buckley-Lanier, J; Sorscher, EJ; Woodworth, BA. "Marked Repression of CFTR MMA in the Transgenic CFTR^TM1KTH Mouse Model" Pediatric Pulmonology Supp 32 (Abst 270), 2009.

23. Kong, M; Li, Y; Chen, F; Fan, L; Sullender, W; Clancy, JP. "Induction of MMP-9 Expression and Preferential Apical Surface Release Produced by RSV Infection of Human Airway Cell Monolayers" Pediatric Pulmonology Supp 32 (Abst 309), 2009.

24. Troxler, RB; Hoover, WC; Britton, LJ; Gerwin, AM; Rowe, SM. "Eradication of Mucoid Pseudomonas Aeruginosa in Patients with Cystic Fibrosis: A Retrospective Analysis" Pediatric Pulmonology Supp 32 (Abst 330), 2009.

25. Gerwin, AM; Britton, LJ; Benner, K; Tofil, N; Guiterrez, H; Troxler, RB; Hoover, WC. "Pseudomonas Aeruginosa Eradication in Cystic Fibrosis Patients: A Retrospective Review" Pediatric Pulmonology Supp 32 (Abst 365), 2009.

26. Milla, C; Chmiel, JF; Accurso, FJ; McCoy, KS; Billings, JL; Atkinson, JJ; Clancy, JP; Liou, TG; Acton, JD; Lynch, SV; Slusher, N; Burns, JL; Hamblett, NM; Harris, JK; Patel, R; Tremblay, TM; Parli, TJ. "Anti-Inflammatory Effect of KB001, An Antipcrv Antibody Fragment, in CF Patients Chronically Infected with Pseudomonas Aeruginosa" Pediatric Pulmonology Supp 32 (Abst 369), 2009.

27. Britton, LJ; Hoover, W; Mims, C; Gutierrez, HH. "Reorganization of Annual Visits Improves Clinic Efficiency and Satisfaction of Team, Patients and Families" Pediatric Pulmonology Supp 32 (Abst 480), 2009.

28. Seals, NI; Britton, LJ; Gutierrez, H; Gunter, L; Wooldridge, N. " Specialized Nutrition Clinic Enhances Delivery of Nutrition Interventions and Improves Nutritional Status" Pediatric Pulmonology Supp 32 (Abst 515), 2009.

29. Britton, LJ; Moreland, E; Wooldridge, N; Mims, C; Gutierrez, H; Rowe, SM; Hoover, W. Development of a CFRD Clinic Improves Consistency of Care" Pediatric Pulmonology Supp 32 (Abst 571), 2009.
30. Boren, KE; Gunter, LH; Gutierrez, H; Britton, LJ. "Maintaining Professional Boundaries" Pediatric Pulmonology Supp 32 (Abst 608), 2009.