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Changing Lives Many at a Time: Research in the CF Center

We are a CF Foundation Therapeutic Development Network Center. We were involved in the clinical research for most of the medications drug pipeline for CF.


We initiate and collaborate with others to accomplish research projects that have changed our knowledge of CF and the way that CF Care is provided in Utah and around the world. Here is a sampling of interesting scientific papers involving patients with CF from our Center and the University of Utah over the last 30 years.

1. White R, Woodward S, Leppert M, O’Connell P, Hoff M, Herbst J, Lalouel JM, Dean M, Vande Woude G. A closely linked genetic marker for cystic fibrosis. Nature. 1985 Dec 28;318(6044):382–384. PMID: 3906407

2. White R, Leppert M, O’Connell P, Nakamura Y, Woodward S, Hoff M, Herbst J, Dean M, Woude GV, Lathrop GM, Lalouel J-M. Further linkage data on cystic fibrosis: The Utah study. Am J Hum Genet. 1986 Dec;39(6):694–698. PMCID: PMC1684126

3. Bosso JA, Saxon BA, Matsen JM. In vitro activity of aztreonam combined with tobramycin and gentamicin against clinical isolates of Pseudomonas aeruginosa and Pseudomonas cepacia from patients with cystic fibrosis. Antimicrob Agents Chemother. 1987 Sep;31(9):1403–1405. PMCID: PMC174950

4. Wittwer CT, Marshall BC, Reed GH, Cherry JL. Rapid cycle allele-specific amplification: studies with the cystic fibrosis delta F508 locus. Clin Chem. 1993 May;39(5):804–809. PMID: 7683581

5. Pfeffer KD, Huecksteadt TP, Hoidal JR. Expression and regulation of tumor necrosis factor in macrophages from cystic fibrosis patients. Am J Respir Cell Mol Biol. 1993 Nov;9(5):511–519. PMID: 8217192

6. Mortensen LA, Chan GM, Alder SC, Marshall BC. Bone mineral status in prepubertal children with cystic fibrosis. J Pediatr. 2000 May;136(5):648–652. PMID: 10802498

7. Marshall BC, Liou TG. Elusiveness of ideal approach to Pseudomonas aeruginosa infection complicating cystic fibrosis. Lancet. 2000 Aug 19;356(9230):613–4. PMID: 10968431

8. Liou TG, Adler FR, Fitzsimmons SC, Cahill BC, Hibbs JR, Marshall BC. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol. 2001 Feb 15;153(4):345–52. PMID: 11207152

9. Marshall BC, Butler SM, Stoddard M, Moran AM, Liou TG, Morgan WJ. Epidemiology of cystic fibrosis-related diabetes. J Pediatr. 2005 May;146(5):681–7. PMID: 15870674

10. Liou TG, Adler FR, Cox DR, Cahill BC. Lung transplantation and survival in children with cystic fibrosis. N Engl J Med. 2007 Nov 22;357(21):2143–52. PMID: 18032764

11. Adler FR, Aurora P, Barker DH, Barr ML, Blackwell LS, Bosma OH, Brown S, Cox DR, Jensen JL, Kurland G, Nossent GD, Quittner AL, Robinson WM, Romero SL, Spencer H, Sweet SC, van der Bij W, Vermeulen J, Verschuuren EAM, Vrijlandt EJLE, Walsh W, Woo MS, Liou TG. Lung transplantation for cystic fibrosis. Proc Am Thorac Soc. 2009 Dec;6(8):619–633. PMCID: PMC2797068

12. Flume PA, Liou TG, Borowitz DS, Li H, Yen K, Ordoñez CL, Geller DE. Ivacaftor in Subjects with Cystic Fibrosis who are Homozygous for the F508del-CFTR Mutation. Chest. 2012 Sep;142(3):718–724. PMCID: PMC3435140

13. Liou TG, Adler FR, Keogh RH, Li Y, Jensen JL, Walsh W, Packer K, Clark T, Carveth H, Chen J, Rogers SL, Lane C, Moore J, Sturrock A, Paine R 3rd, Cox DR, Hoidal JR. Sputum biomarkers and the prediction of clinical outcomes in patients with cystic fibrosis. PLoS ONE. 2012;7(8):e42748. PMCID: PMC3416785

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