-
1
-
-
0142043977
-
Pathophysiology and management of pulmonary infections in cystic fibrosis
-
Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 2003;168:918-951.
-
(2003)
Am J Respir Crit Care Med
, vol.168
, pp. 918-951
-
-
Gibson, R.L.1
Burns, J.L.2
Ramsey, B.W.3
-
2
-
-
0028129568
-
Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
-
Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey BW, Rosenstein BJ, Smith AL, Wohl ME. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N Engl J Med 1994;331:637-642.
-
(1994)
N Engl J Med
, vol.331
, pp. 637-642
-
-
Fuchs, H.J.1
Borowitz, D.S.2
Christiansen, D.H.3
Morris, E.M.4
Nash, M.L.5
Ramsey, B.W.6
Rosenstein, B.J.7
Smith, A.L.8
Wohl, M.E.9
-
3
-
-
0033531143
-
Intermittent administration of inhaled tobramycin in patients with cystic fibrosis
-
Ramsey BW, Pepe MS, Quan JM, Otto KL, Montgomery AB, Williams-Warren J, Vasiljev KM, Borowitz D, Bowman CM, Marshall BC, et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. N Engl J Med 1999;340:23-30.
-
(1999)
N Engl J Med
, vol.340
, pp. 23-30
-
-
Ramsey, B.W.1
Pepe, M.S.2
Quan, J.M.3
Otto, K.L.4
Montgomery, A.B.5
Williams-Warren, J.6
Vasiljev, K.M.7
Borowitz, D.8
Bowman, C.M.9
Marshall, B.C.10
-
4
-
-
33745686502
-
Microbiology, safety, and pharmacokinetics of aztreonam lysinate for inhalation in patients with cystic fibrosis
-
Gibson RL, Retsch-Bogart GZ, Oermann C, Milla C, Pilewski J, Daines C, Ahrens R, Leon K, Cohen M, McNamara S, 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
Milla, C.4
Pilewski, J.5
Daines, C.6
Ahrens, R.7
Leon, K.8
Cohen, M.9
McNamara, S.10
-
5
-
-
20144363443
-
2 receptor agonist: Results of a phase 1/2 multicenter study in mild to moderate cystic fibrosis
-
2 receptor agonist: results of a phase 1/2 multicenter study in mild to moderate cystic fibrosis. Pediatr Pulmonol 2005;39:339-348.
-
(2005)
Pediatr Pulmonol
, vol.39
, pp. 339-348
-
-
Deterding, R.1
Retsch-Bogart, G.2
Milgram, L.3
Gibson, R.4
Daines, C.5
Zeitlin, P.L.6
Milla, C.7
Marshall, B.8
Lavange, L.9
Engels, J.10
-
6
-
-
33744831154
-
Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
-
Van Goor F, Straley KS, Cao D, Gonzalez J, Hadida S, Hazlewood A, Joubran J, Knapp T, Makings LR, Miller M, et al. Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 2006;290:L1117-L1130.
-
(2006)
Am J Physiol Lung Cell Mol Physiol
, vol.290
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
Gonzalez, J.4
Hadida, S.5
Hazlewood, A.6
Joubran, J.7
Knapp, T.8
Makings, L.R.9
Miller, M.10
-
7
-
-
0141863491
-
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations
-
Wilschanski M, Yahav Y, Yaacov Y, Blau H, Bentur L, Rivlin J, Aviram M, Bdolah-Abram T, Bebok Z, Shushi L, et al. Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations. N Engl J Med 2003;349:1433-1441.
-
(2003)
N Engl J Med
, vol.349
, pp. 1433-1441
-
-
Wilschanski, M.1
Yahav, Y.2
Yaacov, Y.3
Blau, H.4
Bentur, L.5
Rivlin, J.6
Aviram, M.7
Bdolah-Abram, T.8
Bebok, Z.9
Shushi, L.10
-
8
-
-
33750999611
-
Advances in cystic fibrosis therapies
-
Rowe SM, Clancy JP. Advances in cystic fibrosis therapies. Curr Opin Pediatr 2006;18:604-613.
-
(2006)
Curr Opin Pediatr
, vol.18
, pp. 604-613
-
-
Rowe, S.M.1
Clancy, J.P.2
-
10
-
-
0031021662
-
Identifying treatments that halt progression of pulmonary disease in cystic fibrosis
-
Davis PB, Byard PJ, Konstan MW. Identifying treatments that halt progression of pulmonary disease in cystic fibrosis. Pediatr Res 1997;41:161-165.
-
(1997)
Pediatr Res
, vol.41
, pp. 161-165
-
-
Davis, P.B.1
Byard, P.J.2
Konstan, M.W.3
-
11
-
-
0028091157
-
Outcome measures for clinical trials in cystic fibrosis: Summary of a Cystic Fibrosis Foundation consensus conference
-
Ramsey BW, Boat TF. Outcome measures for clinical trials in cystic fibrosis: summary of a Cystic Fibrosis Foundation consensus conference. J Pediatr 1994;124:177-192.
-
(1994)
J Pediatr
, vol.124
, pp. 177-192
-
-
Ramsey, B.W.1
Boat, T.F.2
-
12
-
-
30344465311
-
Raised volume forced expirations in infants: Guidelines for current practice [ATS/ERS statement]
-
American Thoracic Society, European Respiratory Society
-
American Thoracic Society, European Respiratory Society. Raised volume forced expirations in infants: guidelines for current practice [ATS/ERS statement]. Am J Respir Crit Care Med 2005;172:1463-1471.
-
(2005)
Am J Respir Crit Care Med
, vol.172
, pp. 1463-1471
-
-
-
13
-
-
2142771098
-
Pulmonary disease assessment in cystic fibrosis: Comparison of CT scoring systems and value of bronchial and arterial dimension measurements
-
de Jong PA, Ottink MD, Robben SG, Lequin MH, Hop WC, Hendriks JJ, Pare PD, Tiddens HA. Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 2004;231:434-439.
-
(2004)
Radiology
, vol.231
, pp. 434-439
-
-
de Jong, P.A.1
Ottink, M.D.2
Robben, S.G.3
Lequin, M.H.4
Hop, W.C.5
Hendriks, J.J.6
Pare, P.D.7
Tiddens, H.A.8
-
14
-
-
0037027942
-
The Cystic Fibrosis Therapeutics Development Network (CF TDN): A paradigm of a clinical trials network for genetic and orphan diseases
-
Goss CH, Hamblett N, Kronmal RA, Ramsey BW. The Cystic Fibrosis Therapeutics Development Network (CF TDN): a paradigm of a clinical trials network for genetic and orphan diseases. Adv Drug Deliv Rev 2002;54:1505-1528.
-
(2002)
Adv Drug Deliv Rev
, vol.54
, pp. 1505-1528
-
-
Goss, C.H.1
Hamblett, N.2
Kronmal, R.A.3
Ramsey, B.W.4
-
15
-
-
0031900652
-
Cystic Fibrosis Foundation Consensus Panel. The diagnosis of cystic fibrosis: A consensus statement
-
Rosenstein BJ, Cutting GR; Cystic Fibrosis Foundation Consensus Panel. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr 1998;132:589-595.
-
(1998)
J Pediatr
, vol.132
, pp. 589-595
-
-
Rosenstein, B.J.1
Cutting, G.R.2
-
16
-
-
33749446633
-
Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials
-
Wilschanski M, Dupuis A, Ellis L, Jarvi K, Zielenski J, Tullis E, Martin S, Corey M, Tsui LC, Durie P. Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials. Am J Respir Crit Care Med 2006;174:787-794.
-
(2006)
Am J Respir Crit Care Med
, vol.174
, pp. 787-794
-
-
Wilschanski, M.1
Dupuis, A.2
Ellis, L.3
Jarvi, K.4
Zielenski, J.5
Tullis, E.6
Martin, S.7
Corey, M.8
Tsui, L.C.9
Durie, P.10
-
17
-
-
0032433707
-
Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
-
Matsui H, Grubb BR, Tarran R, Randell SH, Gatzy JT, Davis CW, Boucher RC. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998;95:1005-1015.
-
(1998)
Cell
, vol.95
, pp. 1005-1015
-
-
Matsui, H.1
Grubb, B.R.2
Tarran, R.3
Randell, S.H.4
Gatzy, J.T.5
Davis, C.W.6
Boucher, R.C.7
-
18
-
-
30944452384
-
Mucus clearance and lung function in cystic fibrosis with hypertonic saline
-
Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. 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
Knowles, M.R.4
Tarran, R.5
Boucher, R.C.6
-
19
-
-
30944466084
-
National Hypertonic Saline in Cystic Fibrosis Study G: A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
-
Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB, Belousova EG, Xuan W, Bye PT. National Hypertonic Saline in Cystic Fibrosis Study G: 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
Harbour, C.4
Moriarty, C.P.5
Marks, G.B.6
Belousova, E.G.7
Xuan, W.8
Bye, P.T.9
-
20
-
-
21744460232
-
American Thoracic Society, European Respiratory Society. Coming together: The ATS/ERS consensus on clinical pulmonary function testing
-
Brusasco V, Crapo R, Viegi G; American Thoracic Society, European Respiratory Society. Coming together: the ATS/ERS consensus on clinical pulmonary function testing. Eur Respir J 2005;26:1-2.
-
(2005)
Eur Respir J
, vol.26
, pp. 1-2
-
-
Brusasco, V.1
Crapo, R.2
Viegi, G.3
-
21
-
-
0031874764
-
Microbiology of sputum from patients at cystic fibrosis centers in the United States
-
Burns JL, Emerson J, Stapp JR, Yim DL, Krzewinski J, Louden L, Ramsey BW, Clausen CR. Microbiology of sputum from patients at cystic fibrosis centers in the United States. Clin Infect Dis 1998;27:158-163.
-
(1998)
Clin Infect Dis
, vol.27
, pp. 158-163
-
-
Burns, J.L.1
Emerson, J.2
Stapp, J.R.3
Yim, D.L.4
Krzewinski, J.5
Louden, L.6
Ramsey, B.W.7
Clausen, C.R.8
-
22
-
-
34548228486
-
Monitoring cystic fibrosis lung disease in clinical trials: Is it time for a change?
-
Tiddens HAWM, Brody AS. Monitoring cystic fibrosis lung disease in clinical trials: Is it time for a change? Proc Am Thorac Soc 2007;4:297-298.
-
(2007)
Proc Am Thorac Soc
, vol.4
, pp. 297-298
-
-
Tiddens, H.A.W.M.1
Brody, A.S.2
-
23
-
-
34548255391
-
Patient reported outcomes in cystic fibrosis
-
Goss CH, Quittner AL. Patient reported outcomes in cystic fibrosis. Proc Am Thorac Soc 2007;4:378-386.
-
(2007)
Proc Am Thorac Soc
, vol.4
, pp. 378-386
-
-
Goss, C.H.1
Quittner, A.L.2
-
24
-
-
34548273107
-
Advancing outcome measures for the new era of drug development in cystic fibrosis
-
Mayer-Hamblett N, Ramsey BW, Kronmal RA. Advancing outcome measures for the new era of drug development in cystic fibrosis. Proc Am Thorac Soc 2007;4:370-377.
-
(2007)
Proc Am Thorac Soc
, vol.4
, pp. 370-377
-
-
Mayer-Hamblett, N.1
Ramsey, B.W.2
Kronmal, R.A.3
-
25
-
-
34548224990
-
Endpoints for clinical trials in young children with cystic fibrosis
-
Davis SD, Brody AS, Emond MJ, Brumback LC, Rosenfeld M. Endpoints for clinical trials in young children with cystic fibrosis. Proc Am Thorac Soc 2007;4:418-430.
-
(2007)
Proc Am Thorac Soc
, vol.4
, pp. 418-430
-
-
Davis, S.D.1
Brody, A.S.2
Emond, M.J.3
Brumback, L.C.4
Rosenfeld, M.5
-
26
-
-
0034821607
-
Defining a pulmonary exacerbation in cystic fibrosis
-
Rosenfeld M, Emerson J, Williams-Warren J, Pepe M, Smith A, Montgomery AB, Ramsey B. Defining a pulmonary exacerbation in cystic fibrosis. J Pediatr 2001;139:359-365.
-
(2001)
J Pediatr
, vol.139
, pp. 359-365
-
-
Rosenfeld, M.1
Emerson, J.2
Williams-Warren, J.3
Pepe, M.4
Smith, A.5
Montgomery, A.B.6
Ramsey, B.7
-
27
-
-
2442480846
-
Epidemiologic Study of Cystic Fibrosis. Pulmonary exacerbations in cystic fibrosis
-
Rabin HR, Butler SM, Wohl ME, Geller DE, Colin AA, Schidlow DV, Johnson CA, Konstan MW, Regelmann WE; Epidemiologic Study of Cystic Fibrosis. Pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol 2004;37:400-406.
-
(2004)
Pediatr Pulmonol
, vol.37
, pp. 400-406
-
-
Rabin, H.R.1
Butler, S.M.2
Wohl, M.E.3
Geller, D.E.4
Colin, A.A.5
Schidlow, D.V.6
Johnson, C.A.7
Konstan, M.W.8
Regelmann, W.E.9
-
28
-
-
27144479200
-
The efficacy and safety of meropenem and tobramycin vs ceftazidime and tobramycin in the treatment of acute pulmonary exacerbations in patients with cystic fibrosis
-
Blumer JL, Saiman L, Konstan MW, Melnick D. The efficacy and safety of meropenem and tobramycin vs ceftazidime and tobramycin in the treatment of acute pulmonary exacerbations in patients with cystic fibrosis. Chest 2005;128:2336-2346.
-
(2005)
Chest
, vol.128
, pp. 2336-2346
-
-
Blumer, J.L.1
Saiman, L.2
Konstan, M.W.3
Melnick, D.4
-
30
-
-
34147215995
-
Exacerbation in cystic fibrosis. 1. Epidemiology and pathogenesis
-
Goss CH, Burns JL. Exacerbation in cystic fibrosis. 1. Epidemiology and pathogenesis. Thorax 2007;62:360-367.
-
(2007)
Thorax
, vol.62
, pp. 360-367
-
-
Goss, C.H.1
Burns, J.L.2
-
31
-
-
1642488950
-
Pulmonary exacerbations in cystic fibrosis: It's time to be explicit!
-
Marshall BC. Pulmonary exacerbations in cystic fibrosis: it's time to be explicit! Am J Respir Crit Care Med 2004;169:781-782.
-
(2004)
Am J Respir Crit Care Med
, vol.169
, pp. 781-782
-
-
Marshall, B.C.1
|