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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. Pediatr Pulmonol. 2005 Apr;39(4):339-48. Deterding R, Retsch-Bogart G, Milgram L, Gibson R, Daines C, Zeitlin PL, Milla C, Marshall B, Lavange L, Engels J, Mathews D, Gorden J, Schaberg A, Williams J, Ramsey B.This study demonstrated that the inhaled drug denufosol was safe in CF patients. Download the PDF file of the above article. (140 KB) 
Randomized, double-blind, placebo-controlled, dose-escalating study of aerosolized interferon gamma-1b in patients with mild to moderate cystic fibrosis lung disease. Pediatr Pulmonol. 2005 Mar;39(3):209-18. Moss RB, Mayer-Hamblett N, Wagener J, Daines C, Hale K, Ahrens R, Gibson RL, Anderson P, Retsch-Bogart G, Nasr SZ, Noth I, Waltz D, Zeitlin P, Ramsey B, Starko K. This study demonstrated that the inhaled drug interferon gamma-1b was safe in CF patients. Download the PDF file of the above article. (138 KB)
Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis
transmembrane regulator gene transfer to the lungs of patients with cystic
fibrosis: a multicenter, double-blind, placebo-controlled trial. Chest. 2004. Feb;125(2):509-21. Moss RB, Rodman D, Spencer LT, Aitken ML, Zeitlin PL, Waltz D, Milla C, Brody AS, Clancy JP, Ramsey B, Hamblett N, Heald AE.
This study demonstrated that repeat dosing of the AAV gene therapy vectors was safe and may improve lung function. Download the PDF file of the above article. (728 KB)
Phenotypic and genetic characterization of patients with features of "nonclassic" forms of cystic fibrosis. J Pediatr. 2005 May;146(5):675-80. Groman JD, Karczeski B, Sheridan M, Robinson TE, Fallin MD, Cutting GR. This study characterized the features of CF patients with atypical presentations. Download the PDF file of the above article. (223 KB)
Failure of high-dose ergocalciferol to correct vitamin D deficiency in adults with cystic fibrosis. Am J Respir Crit Care Med. 2005 Jul 15;172(2):212-7. Boyle MP, Noschese ML, Watts SL, Davis ME, Stenner SE, Lechtzin N. This study demonstrated that the current recommendations for vitamin D supplementation are not effective in adults with CF. Download the PDF of the above article. (456 KB)
Outcomes of Adults with Cystic Fibrosis Infected with Antibiotic-Resistant Pseudomonas aeruginosa. Respiration. 2005 Aug 17.Lechtzin N, John M, Irizarry R, Merlo C, Diette GB, Boyle MP. This study showed that infection with Pseudomonas aeruginosa that is resistant to multiple antibiotics is associated with a more rapid decline in lung function. Download the PDF file of the above article. (108 KB)
Gene Modifier Study Group. Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med. 2005 Oct 6;353(14):1443-53. Drumm ML, Konstan MW, Schluchter MD, Handler A, Pace R, Zou F, Zariwala M, Fargo D, Xu A, Dunn JM, Darrah RJ, Dorfman R, Sandford AJ, Corey M, Zielenski J, Durie P, Goddard K, Yankaskas JR, Wright FA, Knowles MR.This large multi-center study determined that variations in the TGF-beta gene were associated with lung function in CF patients. Download the PDF file of the above article (133 KB)
Calcium kinetics are altered in clinically stable girls with cystic fibrosis. J Clin Endocrinol Metab. 2004 Jul;89(7):3385-91. Schulze KJ, O'Brien KO, Germain-Lee EL, Booth SL, Leonard A, Rosenstein BJ. This study found that calcium metabolism was abnormal in girls with CF. Download the PDF file of the above article (164 KB)
A phase I trial of intranasal Moli1901 for cystic fibrosis. Chest. 2004 Jan;125(1):143-9. Zeitlin PL, Boyle MP, Guggino WB, Molina L. This study showed that Moli1901 was safe in CF patients. Download the PDF file of the above article (300 KB)
Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate. Mol Ther. 2002 Jul;6(1):119-26. Zeitlin PL, Diener-West M, Rubenstein RC, Boyle MP, Lee CK, Brass-Ernst L. This trial showed that 4-phenylbutyrate could increase CFTR function in patients with CF who have two ?F08 muations. Download the PDF file of the above article (136 KB) 
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