-
1
-
-
0001752544
-
Cystic fibrosis
-
In: Scriver CR, Beaudet AL, Valle D, Sly WS, editors New York: McGraw Hill
-
Welsh MJ, Ramsey BW, Accurso FJ, Cutting GR. Cystic fibrosis. In: Scriver CR, Beaudet AL, Valle D, Sly WS, editors. The metabolic and molecular bases of inherited disease, 8th ed. New York: McGraw Hill; 2001. pp. 521-588.
-
(2001)
The Metabolic and Molecular Bases of Inherited Disease, 8th Ed.
, pp. 521-588
-
-
Welsh, M.J.1
Ramsey, B.W.2
Accurso, F.J.3
Cutting, G.R.4
-
2
-
-
25844491194
-
Genetic modifiers of lung disease in cystic fibrosis
-
Drumm ML, Konstan MW, Schluchter MD, Handler A, Pace R, Zou F, Zariwala M, Fargo D, Xu A, Dunn JM, et al. Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med 2005;353:1443-1453.
-
(2005)
N Engl J Med
, vol.353
, pp. 1443-1453
-
-
Drumm, M.L.1
Konstan, M.W.2
Schluchter, M.D.3
Handler, A.4
Pace, R.5
Zou, F.6
Zariwala, M.7
Fargo, D.8
Xu, A.9
Dunn, J.M.10
-
3
-
-
0028015883
-
Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation
-
Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med 1994;150:448-454.
-
(1994)
Am J Respir Crit Care Med
, vol.150
, pp. 448-454
-
-
Konstan, M.W.1
Hilliard, K.A.2
Norvell, T.M.3
Berger, M.4
-
4
-
-
0035888715
-
Airway inflammation in children with cystic fibrosis and healthy children assessed by sputum induction
-
Sagel SD, Kapsner R, Osberg I, Sontag MK, Accurso FJ. Airway inflammation in children with cystic fibrosis and healthy children assessed by sputum induction. Am J Respir Crit Care Med 2001; 164:1425-1431.
-
(2001)
Am J Respir Crit Care Med
, vol.164
, pp. 1425-1431
-
-
Sagel, S.D.1
Kapsner, R.2
Osberg, I.3
Sontag, M.K.4
Accurso, F.J.5
-
5
-
-
0021783739
-
Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibrosis
-
Bruce MC, Poncz L, Klinger JD, Stern RC, Tomashefski JF Jr, Dearborn DG. Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibrosis. Am Rev Respir Dis 1985; 132:529-535.
-
(1985)
Am Rev Respir Dis
, vol.132
, pp. 529-535
-
-
Bruce, M.C.1
Poncz, L.2
Klinger, J.D.3
Stern, R.C.4
Tomashefski Jr., J.F.5
Dearborn, D.G.6
-
6
-
-
0029082706
-
Elastin and collagen degradation products in urine of patients with cystic fibrosis
-
Stone PJ, Konstan MW, Berger M, Dorkin HL, Franzblau C, Snider GL. Elastin and collagen degradation products in urine of patients with cystic fibrosis. Am J Respir Crit Care Med 1995;152:157-162.
-
(1995)
Am J Respir Crit Care Med
, vol.152
, pp. 157-162
-
-
Stone, P.J.1
Konstan, M.W.2
Berger, M.3
Dorkin, H.L.4
Franzblau, C.5
Snider, G.L.6
-
7
-
-
0036907325
-
Induced sputum inflammatory measures correlate with lung function in children with cystic fibrosis
-
Sagel SD, Sontag MK, Wagener JS, Kapsner RK, Osberg I, Accurso FJ. Induced sputum inflammatory measures correlate with lung function in children with cystic fibrosis. J Pediatr 2002;141:811-817.
-
(2002)
J Pediatr
, vol.141
, pp. 811-817
-
-
Sagel, S.D.1
Sontag, M.K.2
Wagener, J.S.3
Kapsner, R.K.4
Osberg, I.5
Accurso, F.J.6
-
8
-
-
16244368754
-
Induced sputum matrix metalloproteinase-9 correlates with lung function and airway inflammation in children with cystic fibrosis
-
Sagel SD, Kapsner RK, Osberg I. Induced sputum matrix metalloproteinase-9 correlates with lung function and airway inflammation in children with cystic fibrosis. Pediatr Pulmonol 2005;39:224-232.
-
(2005)
Pediatr Pulmonol
, vol.39
, pp. 224-232
-
-
Sagel, S.D.1
Kapsner, R.K.2
Osberg, I.3
-
9
-
-
0022642346
-
Levels of free granulocyte elastase in bronchial secre-tions from patients with cystic fibrosis: Effect of antimicrobial treatment against Pseudomonas aeruginosa
-
Suter S, Schaad UB, Tegner H, Ohlsson K, Desgrandchamps D, Waldvogel FA. Levels of free granulocyte elastase in bronchial secre-tions from patients with cystic fibrosis: effect of antimicrobial treatment against Pseudomonas aeruginosa. J Infect Dis 1986;153:902-909.
-
(1986)
J Infect Dis
, vol.153
, pp. 902-909
-
-
Suter, S.1
Schaad, U.B.2
Tegner, H.3
Ohlsson, K.4
Desgrandchamps, D.5
Waldvogel, F.A.6
-
10
-
-
0027131471
-
Alpha 1-proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis
-
O'Connor CM, Gaffney K, Keane J, Southey A, Byrne N, O'Mahoney S, FitzGerald MX. Alpha 1-proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis. Am Rev Respir Dis 1993;148: 1665-1670.
-
(1993)
Am Rev Respir Dis
, vol.148
, pp. 1665-1670
-
-
O'Connor, C.M.1
Gaffney, K.2
Keane, J.3
Southey, A.4
Byrne, N.5
O'Mahoney, S.6
Fitzgerald, M.X.7
-
11
-
-
0029188241
-
Sputum peroxidase activity correlates with the severity of lung disease in cystic fibrosis
-
Regelmann WE, Siefferman CM, Herron JM, Elliott GR, Clawson CC, Gray BH. Sputum peroxidase activity correlates with the severity of lung disease in cystic fibrosis. Pediatr Pulmonol 1995;19:1-9.
-
(1995)
Pediatr Pulmonol
, vol.19
, pp. 1-9
-
-
Regelmann, W.E.1
Siefferman, C.M.2
Herron, J.M.3
Elliott, G.R.4
Clawson, C.C.5
Gray, B.H.6
-
12
-
-
34247390062
-
Association between pul-monary function and sputum biomarkers in cystic fibrosis
-
Mayer-Hamblett N, Aitken ML, Accurso FJ, Kronmal RA, Konstan MW, Burns JL, Sagel SD, Ramsey BW. Association between pul-monary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med 2007;175:822-828.
-
(2007)
Am J Respir Crit Care Med
, vol.175
, pp. 822-828
-
-
Mayer-Hamblett, N.1
Aitken, M.L.2
Accurso, F.J.3
Kronmal, R.A.4
Konstan, M.W.5
Burns, J.L.6
Sagel, S.D.7
Ramsey, B.W.8
-
13
-
-
34447530328
-
Risk fac-tors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis
-
Konstan MW, Morgan WJ, Butler SM, Pasta DJ, Craib ML, Silva SJ, Stokes DC, Wohl ME, Wagener JS, Regelmann WE, et al. Risk fac-tors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. J Pediatr 2007;151:134-139.
-
(2007)
J Pediatr
, vol.151
, pp. 134-139
-
-
Konstan, M.W.1
Morgan, W.J.2
Butler, S.M.3
Pasta, D.J.4
Craib, M.L.5
Silva, S.J.6
Stokes, D.C.7
Wohl, M.E.8
Wagener, J.S.9
Regelmann, W.E.10
-
14
-
-
84868279821
-
Longitudinal assessment of sputum biomarkers in school age children with cystic fibrosis
-
abstract
-
Sagel SD, Anthony MM, Emmett P, Wagner BD. Longitudinal assessment of sputum biomarkers in school age children with cystic fibrosis. Am J Respir Crit Care Med 2011;183:A6118. (abstract).
-
(2011)
Am J Respir Crit Care Med
, vol.183
-
-
Sagel, S.D.1
Anthony, M.M.2
Emmett, P.3
Wagner, B.D.4
-
15
-
-
0029090616
-
Standardization of spirometry: 1994 update
-
American Thoracic Society
-
American Thoracic Society. Standardization of spirometry: 1994 update. Am J Respir Crit Care Med 1995;152:1107-1136.
-
(1995)
Am J Respir Crit Care Med
, vol.152
, pp. 1107-1136
-
-
-
16
-
-
0027551290
-
Pulmonary function between 6 and 18 years of age
-
Wang X, Dockery DW, Wypij D, Fay ME, Ferris BG Jr. Pulmonary function between 6 and 18 years of age. Pediatr Pulmonol 1993;15: 75-88.
-
(1993)
Pediatr Pulmonol
, vol.15
, pp. 75-88
-
-
Wang, X.1
Dockery, D.W.2
Wypij, D.3
Fay, M.E.4
Ferris Jr., B.G.5
-
17
-
-
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
-
18
-
-
0020333131
-
Random-effects models for longitudinal data
-
Laird NM, Ware JH. Random-effects models for longitudinal data. Biometrics 1982;38:963-974.
-
(1982)
Biometrics
, vol.38
, pp. 963-974
-
-
Laird, N.M.1
Ware, J.H.2
-
19
-
-
0001025775
-
Analysis of left-censored longitudinal data with application to viral load in HIV infection
-
Jacqmin-Gadda H, Thiebaut R, Chene G, Commenges D. Analysis of left-censored longitudinal data with application to viral load in HIV infection. Biostatistics 2000;1:355-368.
-
(2000)
Biostatistics
, vol.1
, pp. 355-368
-
-
Jacqmin-Gadda, H.1
Thiebaut, R.2
Chene, G.3
Commenges, D.4
-
20
-
-
2342617437
-
Mixed models for longitudinal left-censored repeated measures
-
Thiebaut R, Jacqmin-Gadda H. Mixed models for longitudinal left-censored repeated measures. Comput Methods Programs Biomed 2004;74:255-260.
-
(2004)
Comput Methods Programs Biomed
, vol.74
, pp. 255-260
-
-
Thiebaut, R.1
Jacqmin-Gadda, H.2
-
21
-
-
33646521973
-
Pulmonary function is negatively correlated with sputum inflammatory markers and cough clearability in subjects with cystic fibrosis but not those with chronic bronchitis
-
Kim JS, Okamoto K, Rubin BK. Pulmonary function is negatively correlated with sputum inflammatory markers and cough clearability in subjects with cystic fibrosis but not those with chronic bronchitis. Chest 2006;129:1148-1154.
-
(2006)
Chest
, vol.129
, pp. 1148-1154
-
-
Kim, J.S.1
Okamoto, K.2
Rubin, B.K.3
-
22
-
-
10344250594
-
Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis
-
Paul K, Rietschel E, Ballmann M, Griese M, Worlitzsch D, Shute J, Chen C, Schink T, Doring G, Van Koningsbruggen S, et al. Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis. Am J Respir Crit Care Med 2004;169:719-725.
-
(2004)
Am J Respir Crit Care Med
, vol.169
, pp. 719-725
-
-
Paul, K.1
Rietschel, E.2
Ballmann, M.3
Griese, M.4
Worlitzsch, D.5
Shute, J.6
Chen, C.7
Schink, T.8
Doring, G.9
Van Koningsbruggen, S.10
-
23
-
-
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
-
25
-
-
33645229803
-
High-dose oral N-acetylcysteine, a glutathione pro-drug, modulates inflammation in cystic fibrosis
-
Tirouvanziam R, Conrad CK, Bottiglieri T, Herzenberg LA, Moss RB, Herzenberg LA. High-dose oral N-acetylcysteine, a glutathione pro-drug, modulates inflammation in cystic fibrosis. Proc Natl Acad Sci USA 2006;103:4628-4633.
-
(2006)
Proc Natl Acad Sci USA
, vol.103
, pp. 4628-4633
-
-
Tirouvanziam, R.1
Conrad, C.K.2
Bottiglieri, T.3
Herzenberg, L.A.4
Moss, R.B.5
Herzenberg, L.A.6
-
26
-
-
31544470902
-
Safety and efficacy of recombinant alpha (1)-antitrypsin therapy in cystic fi-brosis
-
Martin SL, Downey D, Bilton D, Keogan MT, Edgar J, Elborn JS. Safety and efficacy of recombinant alpha(1)-antitrypsin therapy in cystic fi-brosis. Pediatr Pulmonol 2006;41:177-183.
-
(2006)
Pediatr Pulmonol
, vol.41
, pp. 177-183
-
-
Martin, S.L.1
Downey, D.2
Bilton, D.3
Keogan, M.T.4
Edgar, J.5
Elborn, J.S.6
-
27
-
-
33846907545
-
Alpha1-antitrypsin inhalation reduces airway inflamma-tion in cystic fibrosis patients
-
Griese M, Latzin P, Kappler M, Weckerle K, Heinzlmaier T, Bernhardt T, Hartl D. Alpha1-antitrypsin inhalation reduces airway inflamma-tion 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
Weckerle, K.4
Heinzlmaier, T.5
Bernhardt, T.6
Hartl, D.7
-
28
-
-
0031471090
-
Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis
-
Corey M, Edwards L, Levison H, Knowles M. Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis. J Pediatr 1997;131:809-814.
-
(1997)
J Pediatr
, vol.131
, pp. 809-814
-
-
Corey, M.1
Edwards, L.2
Levison, H.3
Knowles, M.4
-
29
-
-
0036112237
-
Predictors of de-terioration of lung function in cystic fibrosis
-
Schaedel C, de Monestrol I, Hjelte L, Johannesson M, Kornfalt R, Lindblad A, Strandvik B, Wahlgren L, Holmberg L. Predictors of de-terioration of lung function in cystic fibrosis. Pediatr Pulmonol 2002;33: 483-491.
-
(2002)
Pediatr Pulmonol
, vol.33
, pp. 483-491
-
-
Schaedel, C.1
De Monestrol, I.2
Hjelte, L.3
Johannesson, M.4
Kornfalt, R.5
Lindblad, A.6
Strandvik, B.7
Wahlgren, L.8
Holmberg, L.9
-
30
-
-
0033827478
-
Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: Analysis of the Cystic Fibrosis Foundation National CF Patient Registry
-
Zemel BS, Jawad AF, FitzSimmons S, Stallings VA. Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: analysis of the Cystic Fibrosis Foundation National CF Patient Registry. J Pediatr 2000;137:374-380.
-
(2000)
J Pediatr
, vol.137
, pp. 374-380
-
-
Zemel, B.S.1
Jawad, A.F.2
Fitzsimmons, S.3
Stallings, V.A.4
-
31
-
-
0029016495
-
Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection
-
Demko CA, Byard PJ, Davis PB. Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection. J Clin Epidemiol 1995;48:1041-1049.
-
(1995)
J Clin Epidemiol
, vol.48
, pp. 1041-1049
-
-
Demko, C.A.1
Byard, P.J.2
Davis, P.B.3
-
33
-
-
78449303522
-
Sputum induction in routine clinical care of children with cystic fibrosis
-
Al Saleh S, Dell SD, Grasemann H, Yau YC, Waters V, Martin S, Ratjen F. Sputum induction in routine clinical care of children with cystic fibrosis. J Pediatr 2010;157:1006-1011.
-
(2010)
J Pediatr
, vol.157
, pp. 1006-1011
-
-
Al Saleh, S.1
Dell, S.D.2
Grasemann, H.3
Yau, Y.C.4
Waters, V.5
Martin, S.6
Ratjen, F.7
-
34
-
-
34548282581
-
Sputum biomarkers of inflammation in cystic fibrosis lung disease
-
Sagel SD, Chmiel JF, Konstan MW. Sputum biomarkers of inflammation in cystic fibrosis lung disease. Proc Am Thorac Soc 2007;4:406-417.
-
(2007)
Proc Am Thorac Soc
, vol.4
, pp. 406-417
-
-
Sagel, S.D.1
Chmiel, J.F.2
Konstan, M.W.3
-
35
-
-
54049139979
-
Potential role of high-mobility group box 1 in cystic fibrosis airway disease
-
Rowe SM, Jackson PL, Liu G, Hardison M, Livraghi A, Solomon GM, McQuaid DB, Noerager BD, Gaggar A, Clancy JP, et al. Potential role of high-mobility group box 1 in cystic fibrosis airway disease. Am J Respir Crit Care Med 2008;178:822-831.
-
(2008)
Am J Respir Crit Care Med
, vol.178
, pp. 822-831
-
-
Rowe, S.M.1
Jackson, P.L.2
Liu, G.3
Hardison, M.4
Livraghi, A.5
Solomon, G.M.6
McQuaid, D.B.7
Noerager, B.D.8
Gaggar, A.9
Clancy, J.P.10
-
36
-
-
77953350894
-
Sputum and serum calprotectin are useful biomarkers during CF ex-acerbation
-
Gray RD, Imrie M, Boyd AC, Porteous D, Innes JA, Greening AP. Sputum and serum calprotectin are useful biomarkers during CF ex-acerbation. J Cyst Fibros 2010;9:193-198.
-
(2010)
J Cyst Fibros
, vol.9
, pp. 193-198
-
-
Gray, R.D.1
Imrie, M.2
Boyd, A.C.3
Porteous, D.4
Innes, J.A.5
Greening, A.P.6
-
37
-
-
57449104934
-
17beta-estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia
-
Coakley RD, Sun H, Clunes LA, Rasmussen JE, Stackhouse JR, Okada SF, Fricks I, Young SL, Tarran R. 17beta-estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia. J Clin Invest 2008;118:4025-4035.
-
(2008)
J Clin Invest
, vol.118
, pp. 4025-4035
-
-
Coakley, R.D.1
Sun, H.2
Clunes, L.A.3
Rasmussen, J.E.4
Stackhouse, J.R.5
Okada, S.F.6
Fricks, I.7
Young, S.L.8
Tarran, R.9
|