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Scott D Sagel

TitleProfessor
InstitutionUniversity of Colorado Denver - Anschutz Medical Campus
DepartmentSOM-PEDS
Phone720/777-6181

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    K23RR018611     (SAGEL, SCOTT D)Aug 1, 2003 - Jul 31, 2009
    NIH
    Noninvasive biomarkers of proteolytic activity in CF
    Role: Principal Investigator

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    Collapse selected publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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    1. Kaspy KR, Dell SD, Davis SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla C, Olivier KN, Barber AT, Wee W, Lin FC, Li L, Rampakakis E, Zariwala MA, Knowles MR, Leigh MW, Shapiro AJ. Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia. Chest. 2023 Dec 09. PMID: 38072392.
      View in: PubMed
    2. Gardner RA, Sagel SD, Knowles MR, Ferkol TW, Davis SD, Leigh MW, Zariwala MA. Decoding negative genetic panels in primary ciliary dyskinesia. Pediatr Pulmonol. 2024 Mar; 59(3):784-787. PMID: 38051289.
      View in: PubMed
    3. Wagner BD, Zemanick ET, Sagel SD, Robertson CE, Stevens MJ, Mayer-Hamblett N, Retsch-Bogart G, Ramsey BW, Harris JK. Limited effects of azithromycin on the oropharyngeal microbiome in children with CF and early pseudomonas infection. BMC Microbiol. 2023 10 27; 23(1):312. PMID: 37891457.
      View in: PubMed
    4. Gill ER, Goss CH, Sagel SD, Wright ML, Horner SD, Zu?iga JA. Predicting return of lung function after a pulmonary exacerbation using the cystic fibrosis respiratory symptom diary-chronic respiratory infection symptom scale. Res Sq. 2023 Sep 13. PMID: 37790510.
      View in: PubMed
    5. Martiniano SL, Caceres SM, Poch K, Rysavy NM, Lovell VK, Armantrout E, Jones M, Anthony M, Keck A, Nichols DP, Vandalfsen JM, Sagel SD, Wagner B, Xie J, Weaver K, Heltshe SL, Daley CL, Davidson RM, Nick JA. Prospective evaluation of nontuberculous mycobacteria disease in cystic fibrosis: The design of the PREDICT study. J Cyst Fibros. 2024 Jan; 23(1):50-57. PMID: 37666709.
      View in: PubMed
    6. Finn E, Severn C, Pyle L, Garrish J, Vigers T, Behn CGD, Zeitler PS, Sagel SD, Nadeau KJ, Chan CL. Hypoglycemia in children and young adults with cystic fibrosis during oral glucose tolerance testing vs. continuous glucose monitoring. Pediatr Pulmonol. 2023 09; 58(9):2495-2504. PMID: 37350354.
      View in: PubMed
    7. Kinghorn B, Rosenfeld M, Sullivan E, Onchiri F, Ferkol TW, Sagel SD, Dell SD, Milla C, Shapiro AJ, Sullivan KM, Zariwala MA, Pittman JE, Mollica F, Tiddens HAWM, Kemner-van de Corput M, Knowles MR, Davis SD, Leigh MW. Airway Disease in Children with Primary Ciliary Dyskinesia: Impact of Ciliary Ultrastructure Defect and Genotype. Ann Am Thorac Soc. 2023 04; 20(4):539-547. PMID: 36442147.
      View in: PubMed
    8. Barber AT, Shapiro AJ, Davis SD, Ferkol TW, Atkinson JJ, Sagel SD, Dell SD, Olivier KN, Milla CE, Rosenfeld M, Li L, Lin FC, Sullivan KM, Capps NA, Zariwala MA, Knowles MR, Leigh MW. Laterality Defects in Primary Ciliary Dyskinesia: Relationship to Ultrastructural Defect or Genotype. Ann Am Thorac Soc. 2023 03; 20(3):397-405. PMID: 36342963.
      View in: PubMed
    9. Sagel SD, Kupfer O, Wagner BD, Davis SD, Dell SD, Ferkol TW, Hoppe JE, Rosenfeld M, Sullivan KM, Tiddens HAWM, Knowles MR, Leigh MW. Airway Inflammation in Children with Primary Ciliary Dyskinesia. Ann Am Thorac Soc. 2023 01; 20(1):67-74. PMID: 35984413.
      View in: PubMed
    10. Wee WB, Leigh MW, Davis SD, Rosenfeld M, Sullivan KM, Sawras MG, Ferkol TW, Knowles MR, Milla C, Sagel SD, Zariwala MA, Pullenayegum E, Dell SD. Association of Neonatal Hospital Length of Stay with Lung Function in Primary Ciliary Dyskinesia. Ann Am Thorac Soc. 2022 11; 19(11):1865-1870. PMID: 35657736.
      View in: PubMed
    11. Schwarzenberg SJ, Vu PT, Skalland M, Hoffman LR, Pope C, Gelfond D, Narkewicz MR, Nichols DP, Heltshe SL, Donaldson SH, Frederick CA, Kelly A, Pittman JE, Ratjen F, Rosenfeld M, Sagel SD, Solomon GM, Stalvey MS, Clancy JP, Rowe SM, Freedman SD. Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of promise-GI. J Cyst Fibros. 2023 03; 22(2):282-289. PMID: 36280527.
      View in: PubMed
    12. Gabel ME, Wang H, Gelfond D, Roach C, Rowe SM, Clancy JP, Sagel SD, Borowitz D. Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated With 1 Month of Lumacaftor/Ivacaftor. J Pediatr Gastroenterol Nutr. 2022 07 01; 75(1):42-47. PMID: 35442228.
      View in: PubMed
    13. Hoppe JE, Wagner BD, Kirk Harris J, Rowe SM, Heltshe SL, DeBoer EM, Sagel SD. Effects of ivacaftor on systemic inflammation and the plasma proteome in people with CF and G551D. J Cyst Fibros. 2022 11; 21(6):950-958. PMID: 35440409.
      View in: PubMed
    14. Zhai J, Emond MJ, Spangenberg A, Stern DA, Vasquez MM, Blue EE, Buckingham KJ, Sherrill DL, Halonen M, Gibson RL, Rosenfeld M, Sagel SD, Bamshad MJ, Morgan WJ, Guerra S. Club cell secretory protein and lung function in children with cystic fibrosis. J Cyst Fibros. 2022 09; 21(5):811-820. PMID: 35367162.
      View in: PubMed
    15. Pittman JE, Skalland MS, Sagel SD, Ramsey BW, Mayer-Hamblett N, Retsch-Bogart GZ. Impact of azithromycin on serum inflammatory markers in children with cystic fibrosis and new Pseudomonas. J Cyst Fibros. 2022 11; 21(6):946-949. PMID: 35260354.
      View in: PubMed
    16. Nichols DP, Paynter AC, Heltshe SL, Donaldson SH, Frederick CA, Freedman SD, Gelfond D, Hoffman LR, Kelly A, Narkewicz MR, Pittman JE, Ratjen F, Rosenfeld M, Sagel SD, Schwarzenberg SJ, Singh PK, Solomon GM, Stalvey MS, Clancy JP, Kirby S, Van Dalfsen JM, Kloster MH, Rowe SM. Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial. Am J Respir Crit Care Med. 2022 03 01; 205(5):529-539. PMID: 34784492.
      View in: PubMed
    17. Davidson RM, Hasan NA, Epperson LE, Benoit JB, Kammlade SM, Levin AR, Calado de Moura V, Hunkins J, Weakly N, Beagle S, Sagel SD, Martiniano SL, Salfinger M, Daley CL, Nick JA, Strong M. Population Genomics of Mycobacterium abscessus from U.S. Cystic Fibrosis Care Centers. Ann Am Thorac Soc. 2021 12; 18(12):1960-1969. PMID: 33856965.
      View in: PubMed
    18. Poore TS, Meier M, Towler E, Martiniano SL, Brinton JT, DeBoer EM, Sagel SD, Wagner BD, Zemanick ET. Clinical characteristics of people with cystic fibrosis and frequent fungal infection. Pediatr Pulmonol. 2022 01; 57(1):152-161. PMID: 34687280.
      View in: PubMed
    19. Hasan NA, Davidson RM, Epperson LE, Kammlade SM, Beagle S, Levin AR, de Moura VC, Hunkins JJ, Weakly N, Sagel SD, Martiniano SL, Salfinger M, Daley CL, Nick JA, Strong M. Population Genomics and Inference of Mycobacterium avium Complex Clusters in Cystic Fibrosis Care Centers, United States. Emerg Infect Dis. 2021 11; 27(11):2836-2846. PMID: 34670648.
      View in: PubMed
    20. Martiniano SL, Croak K, Bonn G, Sontag MK, Sagel SD. Improving outcomes for Colorado's IRT-IRT-DNA cystic fibrosis newborn screening algorithm by implementing floating cutoffs. Mol Genet Metab. 2021 Sep-Oct; 134(1-2):65-67. PMID: 34489170.
      View in: PubMed
    21. Donaldson SH, Laube BL, Mogayzel P, Corcoran TE, Pilewski JM, Ceppe A, Wu J, Bhambhvani PG, Ratjen F, Sagel SD, Clancy JP, Rowe SM, Bennett WD. Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study. J Cyst Fibros. 2022 01; 21(1):143-145. PMID: 34083123.
      View in: PubMed
    22. Martiniano SL, Wagner BD, Brennan L, Wempe MF, Anderson PL, Daley CL, Anthony M, Nick JA, Sagel SD. Pharmacokinetics of oral antimycobacterials and dosing guidance for Mycobacterium avium complex treatment in cystic fibrosis. J Cyst Fibros. 2021 09; 20(5):772-778. PMID: 34030986.
      View in: PubMed
    23. Nichols DP, Donaldson SH, Frederick CA, Freedman SD, Gelfond D, Hoffman LR, Kelly A, Narkewicz MR, Pittman JE, Ratjen F, Sagel SD, Rosenfeld M, Schwarzenberg SJ, Singh PK, Solomon GM, Stalvey MS, Kirby S, VanDalfsen JM, Clancy JP, Rowe SM. PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy. J Cyst Fibros. 2021 03; 20(2):205-212. PMID: 33619012.
      View in: PubMed
    24. Sagel SD, Khan U, Heltshe SL, Clancy JP, Borowitz D, Gelfond D, Donaldson SH, Moran A, Ratjen F, VanDalfsen JM, Rowe SM. Clinical Effectiveness of Lumacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for F508del-CFTR. A Clinical Trial. Ann Am Thorac Soc. 2021 01; 18(1):75-83. PMID: 32644818.
      View in: PubMed
    25. Guimbellot JS, Baines A, Paynter A, Heltshe SL, VanDalfsen J, Jain M, Rowe SM, Sagel SD. Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation. J Cyst Fibros. 2021 03; 20(2):213-219. PMID: 33249004.
      View in: PubMed
    26. Moheet A, Beisang D, Zhang L, Sagel SD, VanDalfsen JM, Heltshe SL, Frederick C, Mann M, Antos N, Billings J, Rowe SM, Moran A. Lumacaftor/ivacaftor therapy fails to increase insulin secretion in F508del/F508del CF patients. J Cyst Fibros. 2021 03; 20(2):333-338. PMID: 32917547.
      View in: PubMed
    27. Lenhart-Pendergrass PM, Caverly LJ, Wagner BD, Sagel SD, Nick JA, LiPuma JJ, Martiniano SL. Clinical characteristics and outcomes associated with Inquilinus infection in cystic fibrosis. J Cyst Fibros. 2021 03; 20(2):310-315. PMID: 32747193.
      View in: PubMed
    28. Jain R, Baines A, Khan U, Wagner BD, Sagel SD. Evaluation of airway and circulating inflammatory biomarkers for cystic fibrosis drug development. J Cyst Fibros. 2021 01; 20(1):50-56. PMID: 32622665.
      View in: PubMed
    29. Shaw M, Khan U, Clancy JP, Donaldson SH, Sagel SD, Rowe SM, Ratjen F. Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study. J Cyst Fibros. 2020 11; 19(6):931-933. PMID: 32513528.
      View in: PubMed
    30. Secunda KE, Guimbellot JS, Jovanovic B, Heltshe SL, Sagel SD, Rowe SM, Jain M. Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared with Males. Am J Respir Crit Care Med. 2020 04 15; 201(8):996-998. PMID: 31841644.
      View in: PubMed
    31. Harris JK, Wagner BD, Zemanick ET, Robertson CE, Stevens MJ, Heltshe SL, Rowe SM, Sagel SD. Changes in Airway Microbiome and Inflammation with Ivacaftor Treatment in Patients with Cystic Fibrosis and the G551D Mutation. Ann Am Thorac Soc. 2020 02; 17(2):212-220. PMID: 31604026.
      View in: PubMed
    32. Sagel SD, Wagner BD, Ziady A, Kelley T, Clancy JP, Narvaez-Rivas M, Pilewski J, Joseloff E, Sha W, Zelnick L, Setchell KDR, Heltshe SL, Muhlebach MS. Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity. J Cyst Fibros. 2020 07; 19(4):632-640. PMID: 31870630.
      View in: PubMed
    33. Vece TJ, Sagel SD, Zariwala MA, Sullivan KM, Burns KA, Dutcher SK, Yusupov R, Leigh MW, Knowles MR. Cytoplasmic "ciliary inclusions" in isolation are not sufficient for the diagnosis of primary ciliary dyskinesia. Pediatr Pulmonol. 2020 01; 55(1):130-135. PMID: 31549486.
      View in: PubMed
    34. Bhatt JM, Muhonen EG, Meier M, Sagel SD, Chan KH. Rhinosinusitis in Pediatric Primary Ciliary Dyskinesia: Impact of Disease. Otolaryngol Head Neck Surg. 2019 11; 161(5):877-880. PMID: 31500503.
      View in: PubMed
    35. Hoppe JE, Sagel SD. Shifting Landscape of Airway Infection in Early Cystic Fibrosis. Am J Respir Crit Care Med. 2019 Sep 01; 200(5):528-529. PMID: 30875233.
      View in: PubMed
    36. Liou TG, Adler FR, Argel N, Asfour F, Brown PS, Chatfield BA, Daines CL, Durham D, Francis JA, Glover B, Heynekamp T, Hoidal JR, Jensen JL, Keogh R, Kopecky CM, Lechtzin N, Li Y, Lysinger J, Molina O, Nakamura C, Packer KA, Poch KR, Quittner AL, Radford P, Redway AJ, Sagel SD, Sprandel S, Taylor-Cousar JL, Vroom JB, Yoshikawa R, Clancy JP, Elborn JS, Olivier KN, Cox DR. 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 Med Res Methodol. 2019 04 26; 19(1):88. PMID: 31027503.
      View in: PubMed
    37. Davis SD, Rosenfeld M, Lee HS, Ferkol TW, Sagel SD, Dell SD, Milla C, Pittman JE, Shapiro AJ, Sullivan KM, Nykamp KR, Krischer JP, Zariwala MA, Knowles MR, Leigh MW. Primary Ciliary Dyskinesia: Longitudinal Study of Lung Disease by Ultrastructure Defect and Genotype. Am J Respir Crit Care Med. 2019 01 15; 199(2):190-198. PMID: 30067075.
      View in: PubMed
    38. DeBoer EM, Wagner BD, Popler J, Harris JK, Zemanick ET, Accurso FJ, Sagel SD, Deterding RR. Novel Application of Aptamer Proteomic Analysis in Cystic Fibrosis Bronchoalveolar Lavage Fluid. Proteomics Clin Appl. 2019 05; 13(3):e1800085. PMID: 30431231.
      View in: PubMed
    39. Hoch H, Sontag MK, Scarbro S, Juarez-Colunga E, McLean C, Kempe A, Sagel SD. Clinical outcomes in U.S. infants with cystic fibrosis from 2001 to 2012. Pediatr Pulmonol. 2018 11; 53(11):1492-1497. PMID: 30259702.
      View in: PubMed
    40. Sagel SD, Khan U, Jain R, Graff G, Daines CL, Dunitz JM, Borowitz D, Orenstein DM, Abdulhamid I, Noe J, Clancy JP, Slovis B, Rock MJ, McCoy KS, Strausbaugh S, Livingston FR, Papas KA, Shaffer ML. Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial. Am J Respir Crit Care Med. 2018 09 01; 198(5):639-647. PMID: 29688760.
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    41. Ratjen F, Klingel M, Black P, Powers MR, Grasemann H, Solomon M, Sagel SD, Donaldson SH, Rowe SM, Rosenfeld M. Changes in Lung Clearance Index in Preschool-aged Patients with Cystic Fibrosis Treated with Ivacaftor (GOAL): A Clinical Trial. Am J Respir Crit Care Med. 2018 08 15; 198(4):526-528. PMID: 29614238.
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    42. Li A, Vigers T, Pyle L, Zemanick E, Nadeau K, Sagel SD, Chan CL. Continuous glucose monitoring in youth with cystic fibrosis treated with lumacaftor-ivacaftor. J Cyst Fibros. 2019 01; 18(1):144-149. PMID: 30104123.
      View in: PubMed
    43. Hoppe JE, Wagner BD, Accurso FJ, Zemanick ET, Sagel SD. Characteristics and outcomes of oral antibiotic treated pulmonary exacerbations in children with cystic fibrosis. J Cyst Fibros. 2018 11; 17(6):760-768. PMID: 29921503.
      View in: PubMed
    44. Shapiro AJ, Davis SD, Polineni D, Manion M, Rosenfeld M, Dell SD, Chilvers MA, Ferkol TW, Zariwala MA, Sagel SD, Josephson M, Morgan L, Yilmaz O, Olivier KN, Milla C, Pittman JE, Daniels MLA, Jones MH, Janahi IA, Ware SM, Daniel SJ, Cooper ML, Nogee LM, Anton B, Eastvold T, Ehrne L, Guadagno E, Knowles MR, Leigh MW, Lavergne V. Diagnosis of Primary Ciliary Dyskinesia. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2018 06 15; 197(12):e24-e39. PMID: 29905515.
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    45. Mueller JL, Muller P, Mellenthin M, Murthy R, Capps M, Alsaker M, Deterding R, Sagel SD, DeBoer E. Estimating regions of air trapping from electrical impedance tomography data. Physiol Meas. 2018 05 31; 39(5):05NT01. PMID: 29726838.
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    46. Saavedra MT, Quon BS, Faino A, Caceres SM, Poch KR, Sanders LA, Malcolm KC, Nichols DP, Sagel SD, Taylor-Cousar JL, Leach SM, Strand M, Nick JA. Whole Blood Gene Expression Profiling Predicts Severe Morbidity and Mortality in Cystic Fibrosis: A 5-Year Follow-Up Study. Ann Am Thorac Soc. 2018 05; 15(5):589-598. PMID: 29425066.
      View in: PubMed
    47. Muller PA, Mueller JL, Mellenthin M, Murthy R, Capps M, Wagner BD, Alsaker M, Deterding R, Sagel SD, Hoppe J. Evaluation of surrogate measures of pulmonary function derived from electrical impedance tomography data in children with cystic fibrosis. Physiol Meas. 2018 04 26; 39(4):045008. PMID: 29565263.
      View in: PubMed
    48. Guimbellot J, Solomon GM, Baines A, Heltshe SL, VanDalfsen J, Joseloff E, Sagel SD, Rowe SM. Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations. J Cyst Fibros. 2019 01; 18(1):102-109. PMID: 29685811.
      View in: PubMed
    49. Chan CL, Vigers T, Pyle L, Zeitler PS, Sagel SD, Nadeau KJ. Continuous glucose monitoring abnormalities in cystic fibrosis youth correlate with pulmonary function decline. J Cyst Fibros. 2018 11; 17(6):783-790. PMID: 29580828.
      View in: PubMed
    50. Goutaki M, Halbeisen FS, Spycher BD, Maurer E, Belle F, Amirav I, Behan L, Boon M, Carr S, Casaulta C, Clement A, Crowley S, Dell S, Ferkol T, Haarman EG, Karadag B, Knowles M, Koerner-Rettberg C, Leigh MW, Loebinger MR, Mazurek H, Morgan L, Nielsen KG, Phillipsen M, Sagel SD, Santamaria F, Schwerk N, Yiallouros P, Lucas JS, Kuehni CE. Growth and nutritional status, and their association with lung function: a study from the international Primary Ciliary Dyskinesia Cohort. Eur Respir J. 2017 12; 50(6). PMID: 29269581.
      View in: PubMed
    51. Hoppe JE, Wagner BD, Sagel SD, Accurso FJ, Zemanick ET. Pulmonary exacerbations and clinical outcomes in a longitudinal cohort of infants and preschool children with cystic fibrosis. BMC Pulm Med. 2017 Dec 11; 17(1):188. PMID: 29228933.
      View in: PubMed
    52. Zemanick ET, Wagner BD, Robertson CE, Ahrens RC, Chmiel JF, Clancy JP, Gibson RL, Harris WT, Kurland G, Laguna TA, McColley SA, McCoy K, Retsch-Bogart G, Sobush KT, Zeitlin PL, Stevens MJ, Accurso FJ, Sagel SD, Harris JK. Airway microbiota across age and disease spectrum in cystic fibrosis. Eur Respir J. 2017 11; 50(5). PMID: 29146601.
      View in: PubMed
    53. Knight-Perry J, Branchford BR, Thornhill D, Martiniano SL, Sagel SD, Wang M. Venous thromboembolism in children with cystic fibrosis: Retrospective incidence and intrapopulation risk factors. Thromb Res. 2017 Oct; 158:161-166. PMID: 28934665.
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    54. Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, Ladores S, Rubenstein RC, Sagel SD, Weiner DJ, Pilewski JM, Orenstein DM, Borrero S. Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis. J Cyst Fibros. 2018 01; 17(1):64-70. PMID: 28866451.
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    55. Kazmerski TM, Sawicki GS, Miller E, Jones KA, Abebe KZ, Tuchman LK, Ladores S, Rubenstein RC, Sagel SD, Weiner DJ, Pilewski JM, Orenstein DM, Borrero S. Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis. J Cyst Fibros. 2018 01; 17(1):57-63. PMID: 28774749.
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    56. DeBoer EM, Kroehl ME, Wagner BD, Accurso FJ, Harris JK, Lynch DA, Sagel SD, Deterding RR. Proteomic profiling identifies novel circulating markers associated with bronchiectasis in cystic fibrosis. Proteomics Clin Appl. 2017 09; 11(9-10). PMID: 28452194.
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    57. Pittman JE, Noah H, Calloway HE, Davis SD, Leigh MW, Drumm M, Sagel SD, Accurso FJ, Knowles MR, Sontag MK. Early childhood lung function is a stronger predictor of adolescent lung function in cystic fibrosis than early Pseudomonas aeruginosa infection. PLoS One. 2017; 12(5):e0177215. PMID: 28505188.
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    58. Martiniano SL, Wagner BD, Levin A, Nick JA, Sagel SD, Daley CL. Safety and Effectiveness of Clofazimine for Primary and Refractory Nontuberculous Mycobacterial Infection. Chest. 2017 10; 152(4):800-809. PMID: 28483608.
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    59. Muhlebach MS, Clancy JP, Heltshe SL, Ziady A, Kelley T, Accurso F, Pilewski J, Mayer-Hamblett N, Joseloff E, Sagel SD. Biomarkers for cystic fibrosis drug development. J Cyst Fibros. 2016 11; 15(6):714-723. PMID: 28215711.
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    60. Leigh MW, Ferkol TW, Davis SD, Lee HS, Rosenfeld M, Dell SD, Sagel SD, Milla C, Olivier KN, Sullivan KM, Zariwala MA, Pittman JE, Shapiro AJ, Carson JL, Krischer J, Hazucha MJ, Knowles MR. Clinical Features and Associated Likelihood of Primary Ciliary Dyskinesia in Children and Adolescents. Ann Am Thorac Soc. 2016 08; 13(8):1305-13. PMID: 27070726.
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    61. Martiniano SL, Sagel SD, Zemanick ET. Cystic fibrosis: a model system for precision medicine. Curr Opin Pediatr. 2016 06; 28(3):312-7. PMID: 27031658.
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    62. Sontag MK, Lee R, Wright D, Freedenberg D, Sagel SD. Improving the Sensitivity and Positive Predictive Value in a Cystic Fibrosis Newborn Screening Program Using a Repeat Immunoreactive Trypsinogen and Genetic Analysis. J Pediatr. 2016 08; 175:150-158.e1. PMID: 27131402.
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    63. Moss A, Juarez-Colunga E, Nathoo F, Wagner B, Sagel S. A comparison of change point models with application to longitudinal lung function measurements in children with cystic fibrosis. Stat Med. 2016 05 30; 35(12):2058-73. PMID: 27118629.
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    64. Shapiro AJ, Zariwala MA, Ferkol T, Davis SD, Sagel SD, Dell SD, Rosenfeld M, Olivier KN, Milla C, Daniel SJ, Kimple AJ, Manion M, Knowles MR, Leigh MW. Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatr Pulmonol. 2016 Feb; 51(2):115-32. PMID: 26418604.
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    65. Sagel SD, Thompson V, Chmiel JF, Montgomery GS, Nasr SZ, Perkett E, Saavedra MT, Slovis B, Anthony MM, Emmett P, Heltshe SL. Effect of treatment of cystic fibrosis pulmonary exacerbations on systemic inflammation. Ann Am Thorac Soc. 2015 May; 12(5):708-17. PMID: 25714657.
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    66. Heltshe SL, Goss CH, Thompson V, Sagel SD, Sanders DB, Marshall BC, Flume PA. Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis. Thorax. 2016 Mar; 71(3):223-9. PMID: 25911223.
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    67. Davis SD, Ferkol TW, Rosenfeld M, Lee HS, Dell SD, Sagel SD, Milla C, Zariwala MA, Pittman JE, Shapiro AJ, Carson JL, Krischer JP, Hazucha MJ, Cooper ML, Knowles MR, Leigh MW. Clinical features of childhood primary ciliary dyskinesia by genotype and ultrastructural phenotype. Am J Respir Crit Care Med. 2015 Feb 01; 191(3):316-24. PMID: 25493340.
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    68. Zemanick ET, Wagner BD, Robertson CE, Stevens MJ, Szefler SJ, Accurso FJ, Sagel SD, Harris JK. Assessment of airway microbiota and inflammation in cystic fibrosis using multiple sampling methods. Ann Am Thorac Soc. 2015 Feb; 12(2):221-9. PMID: 25474078.
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    69. Hoppe JE, Towler E, Wagner BD, Accurso FJ, Sagel SD, Zemanick ET. Sputum induction improves detection of pathogens in children with cystic fibrosis. Pediatr Pulmonol. 2015 Jul; 50(7):638-46. PMID: 25565628.
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    70. Sagel SD. The challenges of developing effective anti-inflammatory agents in cystic fibrosis. J Cyst Fibros. 2015 Mar; 14(2):164-6. PMID: 25576392.
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    71. Tiddens HAWM, Puderbach M, Venegas JG, Ratjen F, Donaldson SH, Davis SD, Rowe SM, Sagel SD, Higgins M, Waltz DA. Novel outcome measures for clinical trials in cystic fibrosis. Pediatr Pulmonol. 2015 Mar; 50(3):302-315. PMID: 25641878.
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    72. Shapiro AJ, Davis SD, Ferkol T, Dell SD, Rosenfeld M, Olivier KN, Sagel SD, Milla C, Zariwala MA, Wolf W, Carson JL, Hazucha MJ, Burns K, Robinson B, Knowles MR, Leigh MW. Laterality defects other than situs inversus totalis in primary ciliary dyskinesia: insights into situs ambiguus and heterotaxy. Chest. 2014 Nov; 146(5):1176-1186. PMID: 24577564.
      View in: PubMed
    73. Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, Sagel SD, Khan U, Mayer-Hamblett N, Van Dalfsen JM, Joseloff E, Ramsey BW. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Am J Respir Crit Care Med. 2014 Jul 15; 190(2):175-84. PMID: 24927234.
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    74. Martiniano SL, Hoppe JE, Sagel SD, Zemanick ET. Advances in the diagnosis and treatment of cystic fibrosis. Adv Pediatr. 2014 Aug; 61(1):225-43. PMID: 25037130.
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    75. Knowles MR, Ostrowski LE, Leigh MW, Sears PR, Davis SD, Wolf WE, Hazucha MJ, Carson JL, Olivier KN, Sagel SD, Rosenfeld M, Ferkol TW, Dell SD, Milla CE, Randell SH, Yin W, Sannuti A, Metjian HM, Noone PG, Noone PJ, Olson CA, Patrone MV, Dang H, Lee HS, Hurd TW, Gee HY, Otto EA, Halbritter J, Kohl S, Kircher M, Krischer J, Bamshad MJ, Nickerson DA, Hildebrandt F, Shendure J, Zariwala MA. Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype. Am J Respir Crit Care Med. 2014 Mar 15; 189(6):707-17. PMID: 24568568.
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    76. DeBoer EM, Swiercz W, Heltshe SL, Anthony MM, Szefler P, Klein R, Strain J, Brody AS, Sagel SD. Automated CT scan scores of bronchiectasis and air trapping in cystic fibrosis. Chest. 2014 Mar 01; 145(3):593-603. PMID: 24114359.
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    77. Martiniano SL, Sontag MK, Daley CL, Nick JA, Sagel SD. Clinical significance of a first positive nontuberculous mycobacteria culture in cystic fibrosis. Ann Am Thorac Soc. 2014 Jan; 11(1):36-44. PMID: 24251858.
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    78. Leigh MW, Hazucha MJ, Chawla KK, Baker BR, Shapiro AJ, Brown DE, Lavange LM, Horton BJ, Qaqish B, Carson JL, Davis SD, Dell SD, Ferkol TW, Atkinson JJ, Olivier KN, Sagel SD, Rosenfeld M, Milla C, Lee HS, Krischer J, Zariwala MA, Knowles MR. Standardizing nasal nitric oxide measurement as a test for primary ciliary dyskinesia. Ann Am Thorac Soc. 2013 Dec; 10(6):574-81. PMID: 24024753.
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    79. Austin-Tse C, Halbritter J, Zariwala MA, Gilberti RM, Gee HY, Hellman N, Pathak N, Liu Y, Panizzi JR, Patel-King RS, Tritschler D, Bower R, O'Toole E, Porath JD, Hurd TW, Chaki M, Diaz KA, Kohl S, Lovric S, Hwang DY, Braun DA, Schueler M, Airik R, Otto EA, Leigh MW, Noone PG, Carson JL, Davis SD, Pittman JE, Ferkol TW, Atkinson JJ, Olivier KN, Sagel SD, Dell SD, Rosenfeld M, Milla CE, Loges NT, Omran H, Porter ME, King SM, Knowles MR, Drummond IA, Hildebrandt F. Zebrafish Ciliopathy Screen Plus Human Mutational Analysis Identifies C21orf59 and CCDC65 Defects as Causing Primary Ciliary Dyskinesia. Am J Hum Genet. 2013 Oct 03; 93(4):672-86. PMID: 24094744.
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    80. Knowles MR, Ostrowski LE, Loges NT, Hurd T, Leigh MW, Huang L, Wolf WE, Carson JL, Hazucha MJ, Yin W, Davis SD, Dell SD, Ferkol TW, Sagel SD, Olivier KN, Jahnke C, Olbrich H, Werner C, Raidt J, Wallmeier J, Pennekamp P, Dougherty GW, Hjeij R, Gee HY, Otto EA, Halbritter J, Chaki M, Diaz KA, Braun DA, Porath JD, Schueler M, Baktai G, Griese M, Turner EH, Lewis AP, Bamshad MJ, Nickerson DA, Hildebrandt F, Shendure J, Omran H, Zariwala MA. Mutations in SPAG1 cause primary ciliary dyskinesia associated with defective outer and inner dynein arms. Am J Hum Genet. 2013 Oct 03; 93(4):711-20. PMID: 24055112.
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    81. Zariwala MA, Gee HY, Kurkowiak M, Al-Mutairi DA, Leigh MW, Hurd TW, Hjeij R, Dell SD, Chaki M, Dougherty GW, Adan M, Spear PC, Esteve-Rudd J, Loges NT, Rosenfeld M, Diaz KA, Olbrich H, Wolf WE, Sheridan E, Batten TF, Halbritter J, Porath JD, Kohl S, Lovric S, Hwang DY, Pittman JE, Burns KA, Ferkol TW, Sagel SD, Olivier KN, Morgan LC, Werner C, Raidt J, Pennekamp P, Sun Z, Zhou W, Airik R, Natarajan S, Allen SJ, Amirav I, Wieczorek D, Landwehr K, Nielsen K, Schwerk N, Sertic J, K?hler G, Washburn J, Levy S, Fan S, Koerner-Rettberg C, Amselem S, Williams DS, Mitchell BJ, Drummond IA, Otto EA, Omran H, Knowles MR, Hildebrandt F. ZMYND10 is mutated in primary ciliary dyskinesia and interacts with LRRC6. Am J Hum Genet. 2013 Aug 08; 93(2):336-45. PMID: 23891469.
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    82. Nick JA, Sanders LA, Ickes B, Briones NJ, Caceres SM, Malcolm KC, Brayshaw SJ, Chacon CS, Barboa CM, Jones MC, St Clair C, Taylor-Cousar JL, Nichols DP, Sagel SD, Strand M, Saavedra MT. Blood mRNA biomarkers for detection of treatment response in acute pulmonary exacerbations of cystic fibrosis. Thorax. 2013 Oct; 68(10):929-37. PMID: 23783371.
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    83. Zemanick ET, Harris JK, Wagner BD, Robertson CE, Sagel SD, Stevens MJ, Accurso FJ, Laguna TA. Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations. PLoS One. 2013; 8(4):e62917. PMID: 23646159.
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    84. Malcolm KC, Nichols EM, Caceres SM, Kret JE, Martiniano SL, Sagel SD, Chan ED, Caverly L, Solomon GM, Reynolds P, Bratton DL, Taylor-Cousar JL, Nichols DP, Saavedra MT, Nick JA. Mycobacterium abscessus induces a limited pattern of neutrophil activation that promotes pathogen survival. PLoS One. 2013; 8(2):e57402. PMID: 23451220.
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    85. Antony D, Becker-Heck A, Zariwala MA, Schmidts M, Onoufriadis A, Forouhan M, Wilson R, Taylor-Cox T, Dewar A, Jackson C, Goggin P, Loges NT, Olbrich H, Jaspers M, Jorissen M, Leigh MW, Wolf WE, Daniels ML, Noone PG, Ferkol TW, Sagel SD, Rosenfeld M, Rutman A, Dixit A, O'Callaghan C, Lucas JS, Hogg C, Scambler PJ, Emes RD, Chung EM, Shoemark A, Knowles MR, Omran H, Mitchison HM. Mutations in CCDC39 and CCDC40 are the major cause of primary ciliary dyskinesia with axonemal disorganization and absent inner dynein arms. Hum Mutat. 2013 Mar; 34(3):462-72. PMID: 23255504.
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    86. Knowles MR, Leigh MW, Ostrowski LE, Huang L, Carson JL, Hazucha MJ, Yin W, Berg JS, Davis SD, Dell SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla CE, Olivier KN, Turner EH, Lewis AP, Bamshad MJ, Nickerson DA, Shendure J, Zariwala MA. Exome sequencing identifies mutations in CCDC114 as a cause of primary ciliary dyskinesia. Am J Hum Genet. 2013 Jan 10; 92(1):99-106. PMID: 23261302.
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    87. Ratjen F, Saiman L, Mayer-Hamblett N, Lands LC, Kloster M, Thompson V, Emmett P, Marshall B, Accurso F, Sagel S, Anstead M. Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa. Chest. 2012 Nov; 142(5):1259-1266. PMID: 22595153.
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    88. Rowe SM, Borowitz DS, Burns JL, Clancy JP, Donaldson SH, Retsch-Bogart G, Sagel SD, Ramsey BW. Progress in cystic fibrosis and the CF Therapeutics Development Network. Thorax. 2012 Oct; 67(10):882-90. PMID: 22960984.
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    89. Sagel SD, Wagner BD, Anthony MM, Emmett P, Zemanick ET. Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis. Am J Respir Crit Care Med. 2012 Nov 01; 186(9):857-65. PMID: 22904182.
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    90. Laguna TA, Wagner BD, Starcher B, Luckey Tarro HK, Mann SA, Sagel SD, Accurso FJ. Urinary desmosine: a biomarker of structural lung injury during CF pulmonary exacerbation. Pediatr Pulmonol. 2012 Sep; 47(9):856-63. PMID: 22431382.
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    91. Knowles MR, Leigh MW, Carson JL, Davis SD, Dell SD, Ferkol TW, Olivier KN, Sagel SD, Rosenfeld M, Burns KA, Minnix SL, Armstrong MC, Lori A, Hazucha MJ, Loges NT, Olbrich H, Becker-Heck A, Schmidts M, Werner C, Omran H, Zariwala MA. Mutations of DNAH11 in patients with primary ciliary dyskinesia with normal ciliary ultrastructure. Thorax. 2012 May; 67(5):433-41. PMID: 22184204.
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    92. Stillwell PC, Wartchow EP, Sagel SD. Primary Ciliary Dyskinesia in Children: A Review for Pediatricians, Allergists, and Pediatric Pulmonologists. Pediatr Allergy Immunol Pulmonol. 2011 Dec; 24(4):191-196. PMID: 22276227.
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    93. Sagel SD, Davis SD, Campisi P, Dell SD. Update of respiratory tract disease in children with primary ciliary dyskinesia. Proc Am Thorac Soc. 2011 Sep; 8(5):438-43. PMID: 21926396.
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    94. Zemanick ET, Sagel SD, Harris JK. The airway microbiome in cystic fibrosis and implications for treatment. Curr Opin Pediatr. 2011 Jun; 23(3):319-24. PMID: 21494150.
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    95. Sagel SD. Nasal nitric oxide: diagnostic value and physiological significance in primary ciliary dyskinesia. J Pediatr. 2011 Sep; 159(3):363-5. PMID: 21612792.
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    96. Olin JT, Burns K, Carson JL, Metjian H, Atkinson JJ, Davis SD, Dell SD, Ferkol TW, Milla CE, Olivier KN, Rosenfeld M, Baker B, Leigh MW, Knowles MR, Sagel SD. Diagnostic yield of nasal scrape biopsies in primary ciliary dyskinesia: a multicenter experience. Pediatr Pulmonol. 2011 May; 46(5):483-8. PMID: 21284095.
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    97. Zemanick ET, Wagner BD, Sagel SD, Stevens MJ, Accurso FJ, Harris JK. Reliability of quantitative real-time PCR for bacterial detection in cystic fibrosis airway specimens. PLoS One. 2010 Nov 30; 5(11):e15101. PMID: 21152087.
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    98. Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordo?ez CL, Campbell PW, Ashlock MA, Ramsey BW. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med. 2010 Nov 18; 363(21):1991-2003. PMID: 21083385.
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    99. Sagel SD, Sontag MK, Anthony MM, Emmett P, Papas KA. Effect of an antioxidant-rich multivitamin supplement in cystic fibrosis. J Cyst Fibros. 2011 Jan; 10(1):31-6. PMID: 20961818.
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    100. Zemanick ET, Wagner BD, Harris JK, Wagener JS, Accurso FJ, Sagel SD. Pulmonary exacerbations in cystic fibrosis with negative bacterial cultures. Pediatr Pulmonol. 2010 Jun; 45(6):569-77. PMID: 20503282.
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    101. Laguna TA, Wagner BD, Luckey HK, Mann SA, Sagel SD, Regelmann W, Accurso FJ. Sputum desmosine during hospital admission for pulmonary exacerbation in cystic fibrosis. Chest. 2009 Dec; 136(6):1561-1568. PMID: 19567495.
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    102. Sontag MK, Wright D, Beebe J, Accurso FJ, Sagel SD. A new cystic fibrosis newborn screening algorithm: IRT/IRT1 upward arrow/DNA. J Pediatr. 2009 Nov; 155(5):618-22. PMID: 19540513.
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    103. Sagel SD, Sontag MK, Accurso FJ. Relationship between antimicrobial proteins and airway inflammation and infection in cystic fibrosis. Pediatr Pulmonol. 2009 Apr; 44(4):402-9. PMID: 19283840.
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    104. Sagel SD, Gibson RL, Emerson J, McNamara S, Burns JL, Wagener JS, Ramsey BW. Impact of Pseudomonas and Staphylococcus infection on inflammation and clinical status in young children with cystic fibrosis. J Pediatr. 2009 Feb; 154(2):183-8. PMID: 18822427.
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    105. Saavedra MT, Hughes GJ, Sanders LA, Carr M, Rodman DM, Coldren CD, Geraci MW, Sagel SD, Accurso FJ, West J, Nick JA. Circulating RNA transcripts identify therapeutic response in cystic fibrosis lung disease. Am J Respir Crit Care Med. 2008 Nov 01; 178(9):929-38. PMID: 18723435.
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    106. Laguna TA, Sagel SD, Sontag MK, Accurso FJ. The clinical course of a Mexican female with cystic fibrosis and the novel genotype S531P/S531P. J Cyst Fibros. 2008 Sep; 7(5):454-6. PMID: 18463004.
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    107. Harris JK, De Groote MA, Sagel SD, Zemanick ET, Kapsner R, Penvari C, Kaess H, Deterding RR, Accurso FJ, Pace NR. Molecular identification of bacteria in bronchoalveolar lavage fluid from children with cystic fibrosis. Proc Natl Acad Sci U S A. 2007 Dec 18; 104(51):20529-33. PMID: 18077362.
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    108. Kang JB, Rothenberg SS, Krawiec ME, Sagel SD. Diagnosis of a symptomatic tracheal pouch by means of bronchoscopy after neonatal repair of a tracheoesophageal fistula. J Allergy Clin Immunol. 2007 Sep; 120(3):541-3. PMID: 17765755.
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    109. Sagel SD, Chmiel JF, Konstan MW. Sputum biomarkers of inflammation in cystic fibrosis lung disease. Proc Am Thorac Soc. 2007 Aug 01; 4(4):406-17. PMID: 17652508.
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    110. Papas KA, Sontag MK, Pardee C, Sokol RJ, Sagel SD, Accurso FJ, Wagener JS. A pilot study on the safety and efficacy of a novel antioxidant rich formulation in patients with cystic fibrosis. J Cyst Fibros. 2008 Jan; 7(1):60-7. PMID: 17569601.
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    111. Mayer-Hamblett N, Aitken ML, Accurso FJ, Kronmal RA, Konstan MW, Burns JL, Sagel SD, Ramsey BW. Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med. 2007 Apr 15; 175(8):822-8. PMID: 17234902.
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    112. Montgomery GS, Sagel SD, Taylor AL, Abman SH. Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease. Pediatr Pulmonol. 2006 Apr; 41(4):383-5. PMID: 16479610.
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    113. Swiercz W, Cios KJ, Staley K, Kurgan L, Accurso F, Sagel S. A new synaptic plasticity rule for networks of spiking neurons. IEEE Trans Neural Netw. 2006 Jan; 17(1):94-105. PMID: 16526479.
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    114. Brody AS, Tiddens HA, Castile RG, Coxson HO, de Jong PA, Goldin J, Huda W, Long FR, McNitt-Gray M, Rock M, Robinson TE, Sagel SD. Computed tomography in the evaluation of cystic fibrosis lung disease. Am J Respir Crit Care Med. 2005 Nov 15; 172(10):1246-52. PMID: 16100011.
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    115. Sagel SD, Kapsner RK, Osberg I. Induced sputum matrix metalloproteinase-9 correlates with lung function and airway inflammation in children with cystic fibrosis. Pediatr Pulmonol. 2005 Mar; 39(3):224-32. PMID: 15635615.
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    116. Sagel SD. Identifying novel endpoints for cystic fibrosis clinical trials. Adv Pediatr. 2005; 52:115-27. PMID: 16124338.
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    117. Wagener JS, Sontag MK, Sagel SD, Accurso FJ. Update on newborn screening for cystic fibrosis. Curr Opin Pulm Med. 2004 Nov; 10(6):500-4. PMID: 15510057.
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    118. Sagel SD. Noninvasive biomarkers of airway inflammation in cystic fibrosis. Curr Opin Pulm Med. 2003 Nov; 9(6):516-21. PMID: 14534405.
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    119. Sagel SD, Accurso FJ. Monitoring inflammation in CF. Cytokines. Clin Rev Allergy Immunol. 2002 Aug; 23(1):41-57. PMID: 12162105.
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    120. Sagel SD, Dowell EB, Accurso FJ. Bacterial colonization and infection in the CF lung. Methods Mol Med. 2002; 70:433-46. PMID: 11917541.
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    121. Sagel SD, Kapsner R, Osberg I, Sontag MK, Accurso FJ. Airway inflammation in children with cystic fibrosis and healthy children assessed by sputum induction. Am J Respir Crit Care Med. 2001 Oct 15; 164(8 Pt 1):1425-31. PMID: 11704590.
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    122. Sagel SD, Cohen H, Townsend SF. Neonatal Hirschsprung disease, dysautonomia, and central hypoventilation. Obstet Gynecol. 1999 May; 93(5 Pt 2):834-6. PMID: 10912412.
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