-
1
-
-
34548512049
-
Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: A randomized placebo-controlled phase 2b trial
-
Moss RB, Milla C, Columbo J, et al. Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2b trial. Hum Gene Ther 2007; 18:726-732.
-
(2007)
Hum Gene Ther
, vol.18
, pp. 726-732
-
-
Moss, R.B.1
Milla, C.2
Columbo, J.3
-
2
-
-
43449111490
-
CpG plasmids confer reduced inflammation and sustained pulmonary gene expression
-
Hyde SC, Pringle IA, Abdullah S, et al. CpG plasmids confer reduced inflammation and sustained pulmonary gene expression. Nat Biotechnol 2008; 26:549-551.
-
(2008)
Nat Biotechnol
, vol.26
, pp. 549-551
-
-
Hyde, S.C.1
Pringle, I.A.2
Abdullah, S.3
-
3
-
-
60149095412
-
On behalf of the UK Cystic Fibrosis Gene Therapy Consortium.Gene transfer to the lung: Lessons learned from more than 2 decades of CF gene therapy
-
Griesenbach U, Alton EWFW, on behalf of the UK Cystic Fibrosis Gene Therapy Consortium. Gene transfer to the lung: lessons learned from more than 2 decades of CF gene therapy. Adv Drug Deliv Rev 2009; 61:128-129.
-
(2009)
Adv Drug Deliv Rev
, vol.61
, pp. 128-129
-
-
Griesenbach, U.1
Ewfw, A.2
-
4
-
-
50149098401
-
Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: A prospective phase II trial
-
Kerem E, Hirawat S, Armoni S, et al. Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial. Lancet 2008; 372:719-727.
-
(2008)
Lancet
, vol.372
, pp. 719-727
-
-
Kerem, E.1
Hirawat, S.2
Armoni, S.3
-
6
-
-
84871966453
-
-
Press Release October 20, 2008. Vertex website [online].
-
Vertex. Press Release October 20, 2008. Vertex website [online]. http:// investors.vrtx.com/releasedetail.cfm?ReleaseID=341350.
-
-
-
-
10
-
-
47049095809
-
Cystic fibrosis: The mechanisms of pathogenesis of an inherited lung disorder
-
Clunes MT, Boucher RC. Cystic fibrosis: the mechanisms of pathogenesis of an inherited lung disorder. Drug Discov Today Dis Mech 2007; 4:63-72.
-
(2007)
Drug Discov Today Dis Mech
, vol.4
, pp. 63-72
-
-
Clunes, M.T.1
Boucher, R.C.2
-
11
-
-
58149512622
-
Impact of Pseudomonas and Staphylococcus infection on inflammation and clinical status in young children with cystic fibrosis
-
Sagel S, Gibson R, Emerson J, et al. Impact of Pseudomonas and Staphylococcus infection on inflammation and clinical status in young children with cystic fibrosis. J Pediatr 2009; 154:183-188.
-
(2009)
J Pediatr
, vol.154
, pp. 183-188
-
-
Sagel, S.1
Gibson, R.2
Emerson, J.3
-
12
-
-
57149116921
-
Lung function in infants with cystic fibrosis diagnosed by newborn screening
-
Linnane BM, Hall GL, Nolan G, et al. Lung function in infants with cystic fibrosis diagnosed by newborn screening. Am J Respir Crit Care Med 2008; 178:1238-1244.
-
(2008)
Am J Respir Crit Care Med
, vol.178
, pp. 1238-1244
-
-
Linnane, B.M.1
Hall, G.L.2
Nolan, G.3
-
13
-
-
67650712233
-
On behalf of AREST for Cystic Fibrosis (AREST-CF). Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening
-
Sly PD, Brennan S, Gangell C, et al. on behalf of AREST for Cystic Fibrosis (AREST-CF). Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med 2009; 180:146-152.
-
(2009)
Am J Respir Crit Care Med
, vol.180
, pp. 146-152
-
-
Sly, P.D.1
Brennan, S.2
Gangell, C.3
-
14
-
-
30944452384
-
Mucus clearance and lung function in cystic fibrosis with hypertonic saline
-
Donaldson SH, Bennett WD, Zeman KL, et al. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 2006; 354:241-250.
-
(2006)
N Engl J Med
, vol.354
, pp. 241-250
-
-
Donaldson, S.H.1
Bennett, W.D.2
Zeman, K.L.3
-
15
-
-
30944466084
-
A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
-
Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006; 354:229-240.
-
(2006)
N Engl J Med
, vol.354
, pp. 229-240
-
-
Elkins, M.R.1
Robinson, M.2
Rose, B.R.3
-
16
-
-
36248953255
-
Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health
-
Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007; 176:957-969.
-
(2007)
Am J Respir Crit Care Med
, vol.176
, pp. 957-969
-
-
Flume, P.A.1
O'Sullivan, B.P.2
Robinson, K.A.3
-
17
-
-
34247880282
-
Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis
-
Subbarao P, Balkovec S, Solomon M, Ratjen F. Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis. Pediatr Pulmonol 2007; 42:417-476.
-
(2007)
Pediatr Pulmonol
, vol.42
, pp. 417-476
-
-
Subbarao, P.1
Balkovec, S.2
Solomon, M.3
Ratjen, F.4
-
18
-
-
57349158832
-
Safety and tolerability of inhaled hypertonic saline in young children with cystic fibrosis
-
Dellon EP, Donaldson SH, Johnson R, Davis SD. Safety and tolerability of inhaled hypertonic saline in young children with cystic fibrosis. Pediatr Pulmonol 2008; 43:1100-1106.
-
(2008)
Pediatr Pulmonol
, vol.43
, pp. 1100-1106
-
-
Dellon, E.P.1
Donaldson, S.H.2
Johnson, R.3
Davis, S.D.4
-
20
-
-
44649166466
-
Inhaled mannitol improves lung function in cystic fibrosis
-
Jaques A, Daviskas E, Turton JA, et al. Inhaled mannitol improves lung function in cystic fibrosis. Chest 2008; 133:1388-1396.
-
(2008)
Chest
, vol.133
, pp. 1388-1396
-
-
Jaques, A.1
Daviskas, E.2
Turton, J.A.3
-
21
-
-
57349176546
-
Bronchial provocation testing with dry powder mannitol in children with cystic fibrosis
-
Minasian C, Wallis C, Metcalfe C, Bush A. Bronchial provocation testing with dry powder mannitol in children with cystic fibrosis. Pediatr Pulmonol 2008; 43:1078-1084.
-
(2008)
Pediatr Pulmonol
, vol.43
, pp. 1078-1084
-
-
Minasian, C.1
Wallis, C.2
Metcalfe, C.3
Bush, A.4
-
22
-
-
70349825055
-
Phase 3 study of denufosol tetrasodium for the treatment of cystic fibrosis
-
Inspire website [online]
-
Moss RB, Anbar R, Wilmott R, et al. Phase 3 study of denufosol tetrasodium for the treatment of cystic fibrosis. Am J Respir Crit Care Med 2009; 179:A1189. Inspire website [online]. http://www.inspirepharm.com/r-and- d/publications.php.
-
(2009)
Am J Respir Crit Care Med
, vol.179
-
-
Moss, R.B.1
Anbar, R.2
Wilmott, R.3
-
25
-
-
57149104917
-
Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in bENaC-overexpressing mice
-
Zhou Z, Treis D, Schubert SC, et al. Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in bENaC-overexpressing mice. Am J Respir Crit Care Med 2008; 178:1245-1256.
-
(2008)
Am J Respir Crit Care Med
, vol.178
, pp. 1245-1256
-
-
Zhou, Z.1
Treis, D.2
Schubert, S.C.3
-
26
-
-
41749115751
-
Development of chronic bronchitis and emphysema in b-epithelial Na+ channel-overexpressing mice
-
Mall MA, Harkema JR, Trojanek JB, et al. Development of chronic bronchitis and emphysema in b-epithelial Na+ channel-overexpressing mice. Am J Respir Crit Care Med 2008; 177:730-742.
-
(2008)
Am J Respir Crit Care Med
, vol.177
, pp. 730-742
-
-
Mall, M.A.1
Harkema, J.R.2
Trojanek, J.B.3
-
28
-
-
41149164824
-
Pharmacological properties of 552-02, a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease
-
Hirsh AJ, Zhang J, Zamurs A, et al. Pharmacological properties of 552-02, a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease. J Pharmacol Exper Ther 2008; 325:77-88.
-
(2008)
J Pharmacol Exper Ther
, vol.325
, pp. 77-88
-
-
Hirsh, A.J.1
Zhang, J.2
Zamurs, A.3
-
29
-
-
59449089139
-
Anti-inflammatory therapies for cystic fibrosis-related lung disease
-
Nichols DP, Konstan MW, Chmiel JF. Anti-inflammatory therapies for cystic fibrosis-related lung disease. Clin Rev Allergy Immunol 2008; 35:135-153.
-
(2008)
Clin Rev Allergy Immunol
, vol.35
, pp. 135-153
-
-
Nichols, D.P.1
Konstan, M.W.2
Chmiel, J.F.3
-
30
-
-
58149394958
-
Inhibition of airway proteases in cystic fibrosis lung disease
-
Griese M, Kappler M, Gaggar A, Hartl D. Inhibition of airway proteases in cystic fibrosis lung disease. Eur Respir J 2008; 32:783-795.
-
(2008)
Eur Respir J
, vol.32
, pp. 783-795
-
-
Griese, M.1
Kappler, M.2
Gaggar, A.3
Hartl, D.4
-
31
-
-
33846907545
-
A1-Antitypsin inhalation reduces airway inflammation in cystic fibrosis patients
-
Griese M, Latzin P, Kappler M, et al. a1-Antitypsin inhalation reduces airway inflammation in cystic fibrosis patients. Eur Respir J 2007; 29:240-250.
-
(2007)
Eur Respir J
, vol.29
, pp. 240-250
-
-
Griese, M.1
Latzin, P.2
Kappler, M.3
-
33
-
-
34548038663
-
High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial
-
Lands LC, Milner R, Cantin AM, et al. High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial. J Pediatr 2007; 151:249-254.
-
(2007)
J Pediatr
, vol.151
, pp. 249-254
-
-
Lands, L.C.1
Milner, R.2
Cantin, A.M.3
-
34
-
-
36848999782
-
Clinical use of ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis
-
Konstan MW, Schluchter MD, Xue W, Davis PB. Clinical use of ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis. Am J Respir Crit Care Med 2007; 176:1084-1089.
-
(2007)
Am J Respir Crit Care Med
, vol.176
, pp. 1084-1089
-
-
Konstan, M.W.1
Schluchter, M.D.2
Xue, W.3
Davis, P.B.4
-
35
-
-
44949151079
-
Oral nonsteroidal anti-inflammatory drug therapy for cystic fibrosis (review)
-
Lands LC, Stanojevic S. Oral nonsteroidal anti-inflammatory drug therapy for cystic fibrosis (review). Cochrane Database Syst Rev 2007:CD001505.
-
(2007)
Cochrane Database Syst Rev
-
-
Lands, L.C.1
Stanojevic, S.2
-
36
-
-
0025868478
-
Prevention of chronic Pseudomonas aeruginosa colonization in cystic fibrosis by early treatment
-
Valerius NH, KochC,Hoiby N. Prevention of chronic Pseudomonas aeruginosa colonization in cystic fibrosis by early treatment. Lancet 1991; 338:725-726.
-
(1991)
Lancet
, vol.338
, pp. 725-726
-
-
Valerius, N.H.1
Kochchoiby, N.2
-
37
-
-
23444434606
-
Early eradication therapy against Pseudomonas aeruginosa in cystic fibrosis patients
-
Tacetti G, Campana S, Festini F, et al. Early eradication therapy against Pseudomonas aeruginosa in cystic fibrosis patients. Eur Respir J 2005; 26:458-461.
-
(2005)
Eur Respir J
, vol.26
, pp. 458-461
-
-
Tacetti, G.1
Campana, S.2
Festini, F.3
-
38
-
-
0030995361
-
Antibiotic treatment of initial colonization with Pseudomonas aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis
-
Frederiksen B, Koch C, Hoiby N. Antibiotic treatment of initial colonization with Pseudomonas aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis. Pediatr Pulmonol 1998; 23:330-335.
-
(1998)
Pediatr Pulmonol
, vol.23
, pp. 330-335
-
-
Frederiksen, B.1
Koch, C.2
Hoiby, N.3
-
39
-
-
66949174609
-
Short and long-term efficacy of inhaled tobramycin in early P. aeruginosa infection: The ELITE study [abstract #334]
-
Ratjen F, Munck A, Kho P. Short and long-term efficacy of inhaled tobramycin in early P. aeruginosa infection: the ELITE study [abstract #334]. Pediatr Pulmonol 2008; 43 (Suppl 31):319.
-
(2008)
Pediatr Pulmonol
, vol.43
, Issue.SUPPL. 31
, pp. 319
-
-
Ratjen, F.1
Munck, A.2
Kho, P.3
-
40
-
-
67349204479
-
EPIC Study Group.Early antipseudomonal acquisition in young patients with cystic fibrosis: Rationale and design of the EPIC clinical trial and observational study
-
Treggiari MM, Rosenfeld M, Mayer-Hamblett N, et al., EPIC Study Group. Early antipseudomonal acquisition in young patients with cystic fibrosis: rationale and design of the EPIC clinical trial and observational study. Contemp Clin Trials 2009; 30:256-268.
-
(2009)
Contemp Clin Trials
, vol.30
, pp. 256-268
-
-
Treggiari, M.M.1
Rosenfeld, M.2
Mayer-Hamblett, N.3
-
41
-
-
50849131275
-
For the investigators and coordinators of the Epidemiologic Study of Cystic Fibrosis (ESCF). Shifting patterns of antibiotic use in cystic fibrosis
-
Moskowitz SM, Silva SJ, Mayer-Hamblett N, et al., for the investigators and coordinators of the Epidemiologic Study of Cystic Fibrosis (ESCF). Shifting patterns of antibiotic use in cystic fibrosis. Pediatr Pulmnol 2008; 43:874-881.
-
(2008)
Pediatr Pulmnol
, vol.43
, pp. 874-881
-
-
Moskowitz, S.M.1
Silva, S.J.2
Mayer-Hamblett, N.3
-
42
-
-
33745686502
-
Microbiology, safety, and pharmacokinetics of aztreonam lysinate for inhalation in patients with cystic fibrosis
-
Gibson RL, Retsch-Bogart GZ, Oermann C, et al. Microbiology, safety, and pharmacokinetics of aztreonam lysinate for inhalation in patients with cystic fibrosis. Pediatr Pulmonol 2006; 41:656-665.
-
(2006)
Pediatr Pulmonol
, vol.41
, pp. 656-665
-
-
Gibson, R.L.1
Retsch-Bogart, G.Z.2
Oermann, C.3
-
43
-
-
49649099230
-
Rapid pulmonary delivery of inhaled tobramycin for Pseudomonas infection in cystic fibrosis: A pilot project
-
Coates AL, Green M, Leung K, et al. Rapid pulmonary delivery of inhaled tobramycin for Pseudomonas infection in cystic fibrosis: a pilot project. Pediatr Pulmonol 2008; 43:753-759.
-
(2008)
Pediatr Pulmonol
, vol.43
, pp. 753-759
-
-
Coates, A.L.1
Green, M.2
Leung, K.3
-
44
-
-
84871943508
-
Effective treatment of chronic Pseudomonas aeruginosa (Pa) infection with tobramycin inhalation powder in cystic fibrosis patients
-
Konstan MW, Geller DE, Minic P, et al. Effective treatment of chronic Pseudomonas aeruginosa (Pa) infection with tobramycin inhalation powder in cystic fibrosis patients. J Cyst Fibros 2009; 8:S27.
-
(2009)
J Cyst Fibros
, vol.8
-
-
Konstan, M.W.1
Geller, D.E.2
Minic, P.3
-
45
-
-
38349058630
-
For the AZLI Phase II Study Group. A phase 2 study of aztreonam lysine for inhalation to treat patients with cystic fibrosis and Pseudomonas aeruginosa infection
-
Retsch-Bogart GZ, Burns JL, Otto KL, et al., for the AZLI Phase II Study Group. A phase 2 study of aztreonam lysine for inhalation to treat patients with cystic fibrosis and Pseudomonas aeruginosa infection. Pediatr Pulmonol 2008; 43:47-58.
-
(2008)
Pediatr Pulmonol
, vol.43
, pp. 47-58
-
-
Retsch-Bogart, G.Z.1
Burns, J.L.2
Otto, K.L.3
-
46
-
-
65949124667
-
Efficacy and safety of inhaled aztreonam lysine in cystic fibrosis
-
Retsch-Bogart GZ, Quittner AL, Gibson RL, et al. Efficacy and safety of inhaled aztreonam lysine in cystic fibrosis. Chest 2009; 135:1223-1232.
-
(2009)
Chest
, vol.135
, pp. 1223-1232
-
-
Retsch-Bogart, G.Z.1
Quittner, A.L.2
Gibson, R.L.3
-
47
-
-
55549137086
-
Inhaled aztreonam lysine is effective in intensively-treated patients with cystic fibrosis
-
McCoy KS, Quittner AL, Oermann CM, et al. Inhaled aztreonam lysine is effective in intensively-treated patients with cystic fibrosis. Am J Respir Crit Care Med 2008; 178:921-928.
-
(2008)
Am J Respir Crit Care Med
, vol.178
, pp. 921-928
-
-
McCoy, K.S.1
Quittner, A.L.2
Oermann, C.M.3
-
48
-
-
66649116259
-
Determination of the minimally important difference scores for the Cystic Fibrosis Questionnaire-Revised respiratory symptom scale in two populations of patients with cystic fibrosis and chronic Pseudomonas aeruginosa airway infection
-
Quittner AL, Modi AC, Wainwright C, et al. Determination of the minimally important difference scores for the Cystic Fibrosis Questionnaire-Revised respiratory symptom scale in two populations of patients with cystic fibrosis and chronic Pseudomonas aeruginosa airway infection. Chest 2009; 135:1610-1618.
-
(2009)
Chest
, vol.135
, pp. 1610-1618
-
-
Quittner, A.L.1
Modi, A.C.2
Wainwright, C.3
-
50
-
-
66949134436
-
A phase 1 open label trial to assess the safety and tolerability of fosfomycin/tobramycin for inhalation (FTI).in subjects with cystic fibrosis (CF) or bronchiectasis (BE)
-
Wilson J, Moorehead L, Montgomery B. A phase 1 open label trial to assess the safety and tolerability of fosfomycin/tobramycin for inhalation (FTI) in subjects with cystic fibrosis (CF) or bronchiectasis (BE). Pediatr Pulmonol 2008; S31:320.
-
(2008)
Pediatr Pulmonol
, vol.S31
, pp. 320
-
-
Wilson, J.1
Moorehead, L.2
Montgomery, B.3
-
51
-
-
59949083248
-
Pharmacokinetics and pharmacodynamics of linezolid in children with cystic fibrosis
-
Santos RB, Prestidge CB, Brown ME, et al. Pharmacokinetics and pharmacodynamics of linezolid in children with cystic fibrosis. Pediatr Pulmonol 2009; 44:148-154.
-
(2009)
Pediatr Pulmonol
, vol.44
, pp. 148-154
-
-
Santos, R.B.1
Prestidge, C.B.2
Brown, M.E.3
|