-
1
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Kommens JM, Kerem B, Alon N, Rozmahel R, Grzelezak Z, Zielenski J, Lok S, Plavsic N, Chou JL, et al: Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 1989; 245:1066-1073.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Kommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelezak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
2
-
-
0004482287
-
ASL in normal and CF subjects is isotonic: Evidence for the isotonic volume absorption/mucus clearance hypothesis for the pathogencsis of early CF lung disease
-
Boucher RC: ASL in normal and CF subjects is isotonic: Evidence for the isotonic volume absorption/mucus clearance hypothesis for the pathogencsis of early CF lung disease. Pediatr Pulmonol Suppl 1998;17:132.
-
(1998)
Pediatr Pulmonol Suppl
, vol.17
, pp. 132
-
-
Boucher, R.C.1
-
3
-
-
0026270058
-
Early respiratory course in infants with cystic fibrosis: Relevance to newborn screening
-
Accurso FJ, Sokol RJ, Hammond KB, Abman SH: Early respiratory course in infants with cystic fibrosis: Relevance to newborn screening. Pediatr Pulmonol Suppl 1991;7:42-45.
-
(1991)
Pediatr Pulmonol Suppl
, vol.7
, pp. 42-45
-
-
Accurso, F.J.1
Sokol, R.J.2
Hammond, K.B.3
Abman, S.H.4
-
4
-
-
0025281628
-
Increased levels of interleukin-1 in bronchoalveolar washings from children with bacterial pulmonary infections
-
Wilmott RW, Kassab JT, Kilian PL, Benjamin WR, Douglas SD, Wood RE: Increased levels of interleukin-1 in bronchoalveolar washings from children with bacterial pulmonary infections. Am Rev Respir Dis 1990;142:365-368.
-
(1990)
Am Rev Respir Dis
, vol.142
, pp. 365-368
-
-
Wilmott, R.W.1
Kassab, J.T.2
Kilian, P.L.3
Benjamin, W.R.4
Douglas, S.D.5
Wood, R.E.6
-
5
-
-
0028015883
-
Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild disease suggest ongoing infection and inflammation
-
Konstan MW, Hilliard KA, Norvel TM, Berger M: Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild disease suggest ongoing infection and inflammation. Am J Respir Crit Care Mcd 1994;150: 448-454.
-
(1994)
Am J Respir Crit Care Mcd
, vol.150
, pp. 448-454
-
-
Konstan, M.W.1
Hilliard, K.A.2
Norvel, T.M.3
Berger, M.4
-
6
-
-
0028240276
-
Protease antiprotease inbalance in the lungs of children with cystic fibrosis
-
Birrer P, McElvaney NG, Rudeberg A, Sommer CW, Liechti-Gallati S, Kraemer R, Hubbard R, Crystal RG: Protease antiprotease inbalance in the lungs of children with cystic fibrosis. Am J Respir Crit Care Med 1994;150: 207-213.
-
(1994)
Am J Respir Crit Care Med
, vol.150
, pp. 207-213
-
-
Birrer, P.1
McElvaney, N.G.2
Rudeberg, A.3
Sommer, C.W.4
Liechti-Gallati, S.5
Kraemer, R.6
Hubbard, R.7
Crystal, R.G.8
-
7
-
-
0029010510
-
Lower respiratory tract infection and inflammation in infants with newly diagnosed cystic fibrosis
-
Armstrong DS, Grimwood K, Carzino R, Carlin JB, Olinsky A, Phelan PD: Lower respiratory tract infection and inflammation in infants with newly diagnosed cystic fibrosis. BMJ 1995;310:1571-1572.
-
(1995)
BMJ
, vol.310
, pp. 1571-1572
-
-
Armstrong, D.S.1
Grimwood, K.2
Carzino, R.3
Carlin, J.B.4
Olinsky, A.5
Phelan, P.D.6
-
8
-
-
0028914085
-
Early pulmonary inflammation in infants with cystic fibrosis
-
Khan TZ, Wagener JS, Bost T, Martinez J, Accurso FJ, Riches DW: Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995;151:1075-1082.
-
(1995)
Am J Respir Crit Care Med
, vol.151
, pp. 1075-1082
-
-
Khan, T.Z.1
Wagener, J.S.2
Bost, T.3
Martinez, J.4
Accurso, F.J.5
Riches, D.W.6
-
9
-
-
0032081281
-
Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells
-
DiMango E, Ratner AJ, Bryan R, Tabibi S, Prince A: Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J Clin Invest 1998;101:2598-2605.
-
(1998)
J Clin Invest
, vol.101
, pp. 2598-2605
-
-
DiMango, E.1
Ratner, A.J.2
Bryan, R.3
Tabibi, S.4
Prince, A.5
-
10
-
-
0022485439
-
Lysosomal enzymes from polymorphonuclear leukocytes and proteinase inhibitors in patients with cystic fibrosis
-
Goldstein W, Doring G: Lysosomal enzymes from polymorphonuclear leukocytes and proteinase inhibitors in patients with cystic fibrosis. Am Rev Respir Dis 1986;134:49-56.
-
(1986)
Am Rev Respir Dis
, vol.134
, pp. 49-56
-
-
Goldstein, W.1
Doring, G.2
-
11
-
-
0027682586
-
Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis
-
Konstan MW, Walenga RW, Hilliard KS, Hilliard JB: Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis. Am Rev Respir Dis 1993;148:896-901.
-
(1993)
Am Rev Respir Dis
, vol.148
, pp. 896-901
-
-
Konstan, M.W.1
Walenga, R.W.2
Hilliard, K.S.3
Hilliard, J.B.4
-
12
-
-
0027477775
-
Sputum tumour necrosis factor-alpha and leukotriene concentrations in cystic fibrosis
-
Greally P, Hussein MJ, Cook AJ, Sampson AP, Piper PJ, Price JF: Sputum tumour necrosis factor-alpha and leukotriene concentrations in cystic fibrosis. Arch Dis Child 1993;68:389-392.
-
(1993)
Arch Dis Child
, vol.68
, pp. 389-392
-
-
Greally, P.1
Hussein, M.J.2
Cook, A.J.3
Sampson, A.P.4
Piper, P.J.5
Price, J.F.6
-
13
-
-
0030768159
-
Lower airway inflammation in infants and young children with cystic fibrosis
-
Armstrong DS, Grimwood K, Carlin JB, Carzino R, Gutierrez JP, Hull J, Olinsky A, Phelan EM, Robertson CF, Phelan PD: Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 1997;156:1197-1204.
-
(1997)
Am J Respir Crit Care Med
, vol.156
, pp. 1197-1204
-
-
Armstrong, D.S.1
Grimwood, K.2
Carlin, J.B.3
Carzino, R.4
Gutierrez, J.P.5
Hull, J.6
Olinsky, A.7
Phelan, E.M.8
Robertson, C.F.9
Phelan, P.D.10
-
14
-
-
0029286816
-
Cystic fibrosis lung inflammation: Early, sustained, and severe
-
Cantin A: Cystic fibrosis lung inflammation: Early, sustained, and severe. Am J Respir Crit Care Med 1995;151:939-941.
-
(1995)
Am J Respir Crit Care Med
, vol.151
, pp. 939-941
-
-
Cantin, A.1
-
15
-
-
0029372481
-
Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis
-
Bonfield TL, Konstan WM, Burfeind P, Panuska JR, Hilliard JB, Berger M: Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis. Am J Respir Cell Mol Biol 1995;13:257-261.
-
(1995)
Am J Respir Cell Mol Biol
, vol.13
, pp. 257-261
-
-
Bonfield, T.L.1
Konstan, W.M.2
Burfeind, P.3
Panuska, J.R.4
Hilliard, J.B.5
Berger, M.6
-
16
-
-
0025966879
-
Aerosol alpha 1-antitrypsin treatment for cystic fibrosis
-
McElvaney NG, Hubbard RC, Birrer P, Chernick MS, Caplan DB, Frank MM, Crystal RG: Aerosol alpha 1-antitrypsin treatment for cystic fibrosis. Lancet 1991;337:392-394.
-
(1991)
Lancet
, vol.337
, pp. 392-394
-
-
McElvaney, N.G.1
Hubbard, R.C.2
Birrer, P.3
Chernick, M.S.4
Caplan, D.B.5
Frank, M.M.6
Crystal, R.G.7
-
17
-
-
0027521818
-
Transgenic livestock as bioreactors: Stable expression of human alpha-1-antitrypsin by a flock of sheep
-
Carver AS, Dalrymple MA, Wright G, Cotton DS, Reeves DB, Gibson YH, Keenan IL, Barrass JD, Scott AR, Colman A, et al: Transgenic livestock as bioreactors: Stable expression of human alpha-1-antitrypsin by a flock of sheep. Biotechnology (NY) 1993;11:1263-1270.
-
(1993)
Biotechnology (NY)
, vol.11
, pp. 1263-1270
-
-
Carver, A.S.1
Dalrymple, M.A.2
Wright, G.3
Cotton, D.S.4
Reeves, D.B.5
Gibson, Y.H.6
Keenan, I.L.7
Barrass, J.D.8
Scott, A.R.9
Colman, A.10
-
18
-
-
0029821647
-
Secretory leukoprotease inhibitor: Partnering alpha 1-proteinase inhibitor to combat pulmonary inflammation
-
Bingle L, Tetley TD: Secretory leukoprotease inhibitor: Partnering alpha 1-proteinase inhibitor to combat pulmonary inflammation. Thorax 1996;51:1273-1274.
-
(1996)
Thorax
, vol.51
, pp. 1273-1274
-
-
Bingle, L.1
Tetley, T.D.2
-
19
-
-
0027495712
-
Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis
-
McElvaney NG, Doujaiji B, Moan MJ, Burnham MR, Wu MC, Crystal RG: Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis. Am Rev Respir Dis 1993;148:1056-1060.
-
(1993)
Am Rev Respir Dis
, vol.148
, pp. 1056-1060
-
-
McElvaney, N.G.1
Doujaiji, B.2
Moan, M.J.3
Burnham, M.R.4
Wu, M.C.5
Crystal, R.G.6
-
20
-
-
0026474443
-
Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor
-
McElvaney NG, Nakamura H, Birrer P, Hebert CA, Wong WL, Alphonso M, Baker JB, Catalano MA, Crystal RG: Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest 1992;90:1296-1301.
-
(1992)
J Clin Invest
, vol.90
, pp. 1296-1301
-
-
McElvaney, N.G.1
Nakamura, H.2
Birrer, P.3
Hebert, C.A.4
Wong, W.L.5
Alphonso, M.6
Baker, J.B.7
Catalano, M.A.8
Crystal, R.G.9
-
21
-
-
0031888040
-
Right and left ventricular dysfunction in patients with severe pulmonary disease
-
Vizza CD, Lynch JP, Ochua LL, Richardson G, Trulock EP: Right and left ventricular dysfunction in patients with severe pulmonary disease. Chest 1998;113:576-583.
-
(1998)
Chest
, vol.113
, pp. 576-583
-
-
Vizza, C.D.1
Lynch, J.P.2
Ochua, L.L.3
Richardson, G.4
Trulock, E.P.5
-
23
-
-
0024502869
-
Pneumothorax in cystic fibrosis: A 26-year experience
-
Spector M, Stern R: Pneumothorax in cystic fibrosis: A 26-year experience. Ann Thorac Surg 1989;47:204-207.
-
(1989)
Ann Thorac Surg
, vol.47
, pp. 204-207
-
-
Spector, M.1
Stern, R.2
-
24
-
-
0343463604
-
Allergy and infection in cystic fibrosis
-
Milner AD. Martin RJ (eds): London, Butterworths, chap 10
-
Wilmott RW: Allergy and infection in cystic fibrosis; in Milner AD. Martin RJ (eds): Neonatal and Paediatric Respiratory Medicine. London, Butterworths, 1985, chap 10, pp 190-210.
-
(1985)
Neonatal and Paediatric Respiratory Medicine
, pp. 190-210
-
-
Wilmott, R.W.1
-
25
-
-
0029874303
-
Annual review or continuous assessment
-
Carr SD, Dinwiddie R: Annual review or continuous assessment. J R Soc Med 1996;89 (suppl 27):3-7.
-
(1996)
J R Soc Med
, vol.89
, Issue.SUPPL. 27
, pp. 3-7
-
-
Carr, S.D.1
Dinwiddie, R.2
-
26
-
-
0023775479
-
Role of respiratory syncytial virus in early hospitalizations for respiratory distress of young infants with cystic fibrosis
-
Abman SH, Ogle JW, Butler-Simon N, Rumack CM, Accurso FJ: Role of respiratory syncytial virus in early hospitalizations for respiratory distress of young infants with cystic fibrosis. J Pediatr 1988;113:826-830.
-
(1988)
J Pediatr
, vol.113
, pp. 826-830
-
-
Abman, S.H.1
Ogle, J.W.2
Butler-Simon, N.3
Rumack, C.M.4
Accurso, F.J.5
-
27
-
-
0029027898
-
Effect of respiratory virus infections including rhinovirus on clinical status in cystic fibrosis
-
Smyth AR, Smyth RL, Tong CY, Hart CA, Heaf DP: Effect of respiratory virus infections including rhinovirus on clinical status in cystic fibrosis. Arch Dis Child 1995;73:117-120.
-
(1995)
Arch Dis Child
, vol.73
, pp. 117-120
-
-
Smyth, A.R.1
Smyth, R.L.2
Tong, C.Y.3
Hart, C.A.4
Heaf, D.P.5
-
28
-
-
0023101960
-
Pseudomonas aeruginosa adhesins for tracheobronchial mucin
-
Ramphal R, Guay C, Pier GB: Pseudomonas aeruginosa adhesins for tracheobronchial mucin. Infect Immun 1987;55:600-603.
-
(1987)
Infect Immun
, vol.55
, pp. 600-603
-
-
Ramphal, R.1
Guay, C.2
Pier, G.B.3
-
29
-
-
0025881366
-
Airway adherence of Pseudomonas aeruginosa: Mucoexopolysaccharide binding to human and bovine airway proteins
-
Hata JS, Pick RB Jr: Airway adherence of Pseudomonas aeruginosa: Mucoexopolysaccharide binding to human and bovine airway proteins. J Lab Clin Med 1991;117:410-422.
-
(1991)
J Lab Clin Med
, vol.117
, pp. 410-422
-
-
Hata, J.S.1
Pick R.B., Jr.2
-
30
-
-
0031660253
-
Burkholderia cepacia. Management issues and new insights
-
LiPuma JJ: Burkholderia cepacia. Management issues and new insights. Clin Chest Med 1998;19:473-486.
-
(1998)
Clin Chest Med
, vol.19
, pp. 473-486
-
-
LiPuma, J.J.1
-
31
-
-
0031457408
-
Immunologic aspects of lung diseases and cystic fibrosis
-
Greenberger PA: Immunologic aspects of lung diseases and cystic fibrosis. JAMA 1997;278: 1924-1930.
-
(1997)
JAMA
, vol.278
, pp. 1924-1930
-
-
Greenberger, P.A.1
-
32
-
-
0030881846
-
Current understanding of the inflammation process in cystic fibrosis: Onset and etiology
-
Konstan MW, Berger M: Current understanding of the inflammation process in cystic fibrosis: Onset and etiology. Pediatr Pulmonol 1997;24:137-142.
-
(1997)
Pediatr Pulmonol
, vol.24
, pp. 137-142
-
-
Konstan, M.W.1
Berger, M.2
|