-
1
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak 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
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
3
-
-
84906952377
-
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis
-
Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, Sagel SD, Khan U, Mayer-Hamblett N, Van Dalfsen JM, et al.; GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Am J Respir Crit Care Med 2014; 190:175-184.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 175-184
-
-
Rowe, S.M.1
Heltshe, S.L.2
Gonska, T.3
Donaldson, S.H.4
Borowitz, D.5
Gelfond, D.6
Sagel, S.D.7
Khan, U.8
Mayer-Hamblett, N.9
Van Dalfsen, J.M.10
-
4
-
-
50649123290
-
CFTR function and prospects for therapy
-
Riordan JR. CFTR function and prospects for therapy. Annu Rev Biochem 2008;77:701-726.
-
(2008)
Annu Rev Biochem
, vol.77
, pp. 701-726
-
-
Riordan, J.R.1
-
5
-
-
2442718786
-
Increased airway epithelial Na1 absorption produces cystic fibrosis-like lung disease in mice
-
Mall M, Grubb BR, Harkema JR, O'Neal WK, Boucher RC. Increased airway epithelial Na1 absorption produces cystic fibrosis-like lung disease in mice. Nat Med 2004;10:487-493.
-
(2004)
Nat Med
, vol.10
, pp. 487-493
-
-
Mall, M.1
Grubb, B.R.2
Harkema, J.R.3
O'Neal, W.K.4
Boucher, R.C.5
-
6
-
-
78649735324
-
Airway mucus function and dysfunction
-
Fahy JV, Dickey BF. Airway mucus function and dysfunction. N Engl J Med 2010;363:2233-2247.
-
(2010)
N Engl J Med
, vol.363
, pp. 2233-2247
-
-
Fahy, J.V.1
Dickey, B.F.2
-
7
-
-
4544353992
-
Submucosal glands and airway defense
-
Wine JJ, Joo NS. Submucosal glands and airway defense. Proc Am Thorac Soc 2004;1:47-53.
-
(2004)
Proc Am Thorac Soc
, vol.1
, pp. 47-53
-
-
Wine, J.J.1
Joo, N.S.2
-
9
-
-
34047166052
-
Airway surface dehydration in cystic fibrosis: Pathogenesis and therapy
-
Boucher RC. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med 2007;58:157-170.
-
(2007)
Annu Rev Med
, vol.58
, pp. 157-170
-
-
Boucher, R.C.1
-
10
-
-
84884284486
-
Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease?
-
Hobbs CA, Da Tan C, Tarran R. Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease? J Physiol 2013;591:4377-4387.
-
(2013)
J Physiol
, vol.591
, pp. 4377-4387
-
-
Hobbs, C.A.1
Da Tan, C.2
Tarran, R.3
-
12
-
-
84893830319
-
Assessing mucociliary transport of single particles in vivo shows variable speed and preference for the ventral trachea in newborn pigs
-
Hoegger MJ, Awadalla M, Namati E, Itani OA, Fischer AJ, Tucker AJ, Adam RJ, McLennan G, Hoffman EA, Stoltz DA, et al. Assessing mucociliary transport of single particles in vivo shows variable speed and preference for the ventral trachea in newborn pigs. Proc Natl Acad Sci USA 2014;111:2355-2360.
-
(2014)
Proc Natl Acad Sci USA
, vol.111
, pp. 2355-2360
-
-
Hoegger, M.J.1
Awadalla, M.2
Namati, E.3
Itani, O.A.4
Fischer, A.J.5
Tucker, A.J.6
Adam, R.J.7
McLennan, G.8
Hoffman, E.A.9
Stoltz, D.A.10
-
13
-
-
84895512997
-
Lung phenotype of juvenile and adult cystic fibrosis transmembrane conductance regulator-knockout ferrets
-
Sun X, Olivier AK, Liang B, Yi Y, Sui H, Evans TI, Zhang Y, Zhou W, Tyler SR, Fisher JT, et al. Lung phenotype of juvenile and adult cystic fibrosis transmembrane conductance regulator-knockout ferrets. Am J Respir Cell Mol Biol 2014;50:502-512.
-
(2014)
Am J Respir Cell Mol Biol
, vol.50
, pp. 502-512
-
-
Sun, X.1
Olivier, A.K.2
Liang, B.3
Yi, Y.4
Sui, H.5
Evans, T.I.6
Zhang, Y.7
Zhou, W.8
Tyler, S.R.9
Fisher, J.T.10
-
14
-
-
84906089250
-
Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis
-
Hoegger MJ, Fischer AJ, McMenimen JD, Ostedgaard LS, Tucker AJ, Awadalla MA, Moninger TO, Michalski AS, Hoffman EA, Zabner J, et al. Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis. Science 2014;345:818-822.
-
(2014)
Science
, vol.345
, pp. 818-822
-
-
Hoegger, M.J.1
Fischer, A.J.2
McMenimen, J.D.3
Ostedgaard, L.S.4
Tucker, A.J.5
Awadalla, M.A.6
Moninger, T.O.7
Michalski, A.S.8
Hoffman, E.A.9
Zabner, J.10
-
15
-
-
84907833958
-
A functional anatomic defect of the cystic fibrosis airway
-
Birket SE, Chu KK, Liu L, Houser GH, Diephuis BJ, Wilsterman EJ, Dierksen G, Mazur M, Shastry S, Li Y, et al. A functional anatomic defect of the cystic fibrosis airway. Am J Respir Crit Care Med 2014; 190:421-432.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 421-432
-
-
Birket, S.E.1
Chu, K.K.2
Liu, L.3
Houser, G.H.4
Diephuis, B.J.5
Wilsterman, E.J.6
Dierksen, G.7
Mazur, M.8
Shastry, S.9
Li, Y.10
-
16
-
-
84907815404
-
Measuring mucociliary transport and mucus properties in multiple regions of airway epithelial surfaces helps clarify cystic fibrosis defects
-
Wine JJ. Measuring mucociliary transport and mucus properties in multiple regions of airway epithelial surfaces helps clarify cystic fibrosis defects. Am J Respir Crit Care Med 2014;190:364-365.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 364-365
-
-
Wine, J.J.1
-
17
-
-
84924565068
-
PH modulates the activity and synergism of the airway surface liquid antimicrobials b-defensin-3 and LL-37
-
Abou Alaiwa MH, Reznikov LR, Gansemer ND, Sheets KA, Horswill AR, Stoltz DA, Zabner J, Welsh MJ. pH modulates the activity and synergism of the airway surface liquid antimicrobials b-defensin-3 and LL-37. Proc Natl Acad Sci USA 2014;111: 18703-18708.
-
(2014)
Proc Natl Acad Sci USA
, vol.111
, pp. 18703-18708
-
-
Abou Alaiwa, M.H.1
Reznikov, L.R.2
Gansemer, N.D.3
Sheets, K.A.4
Horswill, A.R.5
Stoltz, D.A.6
Zabner, J.7
Welsh, M.J.8
-
18
-
-
84863476402
-
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
-
Pezzulo AA, Tang XX, Hoegger MJ, Alaiwa MH, Ramachandran S, Moninger TO, Karp PH, Wohlford-Lenane CL, Haagsman HP, van Eijk M, et al. Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature 2012;487:109-113.
-
(2012)
Nature
, vol.487
, pp. 109-113
-
-
Pezzulo, A.A.1
Tang, X.X.2
Hoegger, M.J.3
Alaiwa, M.H.4
Ramachandran, S.5
Moninger, T.O.6
Karp, P.H.7
Wohlford-Lenane, C.L.8
Haagsman, H.P.9
Van Eijk, M.10
-
19
-
-
48149090717
-
Cystic fibrosis: Impaired bicarbonate secretion and mucoviscidosis
-
Quinton PM. Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis. Lancet 2008;372:415-417.
-
(2008)
Lancet
, vol.372
, pp. 415-417
-
-
Quinton, P.M.1
-
20
-
-
84911909342
-
Expanding our understanding of respiratory microbiota in cystic fibrosis
-
LiPuma JJ. Expanding our understanding of respiratory microbiota in cystic fibrosis. Ann Am Thorac Soc 2014;11:1084-1085.
-
(2014)
Ann Am Thorac Soc
, vol.11
, pp. 1084-1085
-
-
LiPuma, J.J.1
-
21
-
-
84859576702
-
Decade-long bacterial community dynamics in cystic fibrosis airways
-
Zhao J, Schloss PD, Kalikin LM, Carmody LA, Foster BK, Petrosino JF, Cavalcoli JD, VanDevanter DR, Murray S, Li JZ, et al. Decade-long bacterial community dynamics in cystic fibrosis airways. Proc Natl Acad Sci USA 2012;109:5809-5814.
-
(2012)
Proc Natl Acad Sci USA
, vol.109
, pp. 5809-5814
-
-
Zhao, J.1
Schloss, P.D.2
Kalikin, L.M.3
Carmody, L.A.4
Foster, B.K.5
Petrosino, J.F.6
Cavalcoli, J.D.7
VanDevanter, D.R.8
Murray, S.9
Li, J.Z.10
-
22
-
-
84880913324
-
Changes in cystic fibrosis airway microbiota at pulmonary exacerbation
-
Carmody LA, Zhao J, Schloss PD, Petrosino JF, Murray S, Young VB, Li JZ, LiPuma JJ. Changes in cystic fibrosis airway microbiota at pulmonary exacerbation. Ann Am Thorac Soc 2013;10:179-187.
-
(2013)
Ann Am Thorac Soc
, vol.10
, pp. 179-187
-
-
Carmody, L.A.1
Zhao, J.2
Schloss, P.D.3
Petrosino, J.F.4
Murray, S.5
Young, V.B.6
Li, J.Z.7
LiPuma, J.J.8
-
23
-
-
0035002870
-
Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis
-
Nixon GM, Armstrong DS, Carzino R, Carlin JB, Olinsky A, Robertson CF, Grimwood K. Clinical outcome after early Pseudomonas aeruginosa infection in cystic fibrosis. J Pediatr 2001;138: 699-704.
-
(2001)
J Pediatr
, vol.138
, pp. 699-704
-
-
Nixon, G.M.1
Armstrong, D.S.2
Carzino, R.3
Carlin, J.B.4
Olinsky, A.5
Robertson, C.F.6
Grimwood, K.7
-
24
-
-
34447530328
-
Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis
-
e1
-
Konstan MW, Morgan WJ, Butler SM, Pasta DJ, Craib ML, Silva SJ, Stokes DC, Wohl ME, Wagener JS, Regelmann WE, et al.; Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. J Pediatr 2007;51: 134-139, e1.
-
(2007)
J Pediatr
, vol.51
, 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
-
25
-
-
80052511405
-
Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis
-
Treggiari MM, Retsch-Bogart G, Mayer-Hamblett N, Khan U, Kulich M, Kronmal R, Williams J, Hiatt P, Gibson RL, Spencer T, et al.; Early Pseudomonas Infection Control (EPIC) Investigators. Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis. Arch Pediatr Adolesc Med 2011;165:847-856.
-
(2011)
Arch Pediatr Adolesc Med
, vol.165
, pp. 847-856
-
-
Treggiari, M.M.1
Retsch-Bogart, G.2
Mayer-Hamblett, N.3
Khan, U.4
Kulich, M.5
Kronmal, R.6
Williams, J.7
Hiatt, P.8
Gibson, R.L.9
Al Et, S.T.10
-
26
-
-
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
-
27
-
-
84911906189
-
Antibiotic management of lung infections in cystic fibrosis. I. The microbiome, methicillin-resistant Staphylococcus aureus, gram-negative bacteria, and multiple infections
-
Chmiel JF, Aksamit TR, Chotirmall SH, Dasenbrook EC, Elborn JS, LiPuma JJ, Ranganathan SC, Waters VJ, Ratjen FA. Antibiotic management of lung infections in cystic fibrosis. I. The microbiome, methicillin-resistant Staphylococcus aureus, gram-negative bacteria, and multiple infections. Ann Am Thorac Soc 2014;11:1120-1129.
-
(2014)
Ann Am Thorac Soc
, vol.11
, pp. 1120-1129
-
-
Chmiel, J.F.1
Aksamit, T.R.2
Chotirmall, S.H.3
Dasenbrook, E.C.4
Elborn, J.S.5
LiPuma, J.J.6
Ranganathan, S.C.7
Waters, V.J.8
Ratjen, F.A.9
-
28
-
-
84907296644
-
Pseudomonas aeruginosa in vitro phenotypes distinguish cystic fibrosis infection stages and outcomes
-
Mayer-Hamblett N, Rosenfeld M, Gibson RL, Ramsey BW, Kulasekara HD, Retsch-Bogart GZ, Morgan W, Wolter DJ, Pope CE, Houston LS, et al. Pseudomonas aeruginosa in vitro phenotypes distinguish cystic fibrosis infection stages and outcomes. Am J Respir Crit Care Med 2014;190:289-297.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 289-297
-
-
Mayer-Hamblett, N.1
Rosenfeld, M.2
Gibson, R.L.3
Ramsey, B.W.4
Kulasekara, H.D.5
Retsch-Bogart, G.Z.6
Morgan, W.7
Wolter, D.J.8
Pope, C.E.9
Houston, L.S.10
-
29
-
-
84897445133
-
Conditions associated with the cystic fibrosis defect promote chronic Pseudomonas aeruginosa infection
-
Staudinger BJ, Muller JF, Halldórsson S, Boles B, Angermeyer A, Nguyen D, Rosen H, Baldursson O, Gottfreosson M, Guomundsson GH, et al. Conditions associated with the cystic fibrosis defect promote chronic Pseudomonas aeruginosa infection. Am J Respir Crit Care Med 2014;189:812-824.
-
(2014)
Am J Respir Crit Care Med
, vol.189
, pp. 812-824
-
-
Staudinger, B.J.1
Muller, J.F.2
Halldórsson, S.3
Boles, B.4
Angermeyer, A.5
Nguyen, D.6
Rosen, H.7
Baldursson, O.8
Gottfreosson, M.9
Guomundsson, G.H.10
-
30
-
-
84907293414
-
Phenotypes that matter: Pseudomonas aeruginosa and progression of cystic fibrosis lung disease
-
Sanders DB. Phenotypes that matter: Pseudomonas aeruginosa and progression of cystic fibrosis lung disease. Am J Respir Crit Care Med 2014;190:245-246.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 245-246
-
-
Sanders, D.B.1
-
31
-
-
84897397172
-
Cystic fibrosis transmembrane conductance regulator and pseudomonas
-
Zemanick ET, Accurso FJ. Cystic fibrosis transmembrane conductance regulator and pseudomonas. Am J Respir Crit Care Med 2014;189: 763-765.
-
(2014)
Am J Respir Crit Care Med
, vol.189
, pp. 763-765
-
-
Zemanick, E.T.1
Accurso, F.J.2
-
32
-
-
84903759350
-
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure
-
Henderson AG, Ehre C, Button B, Abdullah LH, Cai LH, Leigh MW, DeMaria GC, Matsui H, Donaldson SH, Davis CW, et al. Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure. J Clin Invest 2014;124:3047-3060.
-
(2014)
J Clin Invest
, vol.124
, pp. 3047-3060
-
-
Henderson, A.G.1
Ehre, C.2
Button, B.3
Abdullah, L.H.4
Cai, L.H.5
Leigh, M.W.6
DeMaria, G.C.7
Matsui, H.8
Donaldson, S.H.9
Davis, C.W.10
-
33
-
-
84912080439
-
Antibiotic management of lung infections in cystic fibrosis. II. Nontuberculous mycobacteria, anaerobic bacteria, and fungi
-
Chmiel JF, Aksamit TR, Chotirmall SH, Dasenbrook EC, Elborn JS, LiPuma JJ, Ranganathan SC, Waters VJ, Ratjen FA. Antibiotic management of lung infections in cystic fibrosis. II. Nontuberculous mycobacteria, anaerobic bacteria, and fungi. Ann Am Thorac Soc 2014;11:1298-1306.
-
(2014)
Ann Am Thorac Soc
, vol.11
, pp. 1298-1306
-
-
Chmiel, J.F.1
Aksamit, T.R.2
Chotirmall, S.H.3
Dasenbrook, E.C.4
Elborn, J.S.5
LiPuma, J.J.6
Ranganathan, S.C.7
Waters, V.J.8
Ratjen, F.A.9
-
34
-
-
76749097178
-
Chronic Mycobacterium abscessus infection and lung function decline in cystic fibrosis
-
Esther CR Jr, Esserman DA, Gilligan P, Kerr A, Noone PG. Chronic Mycobacterium abscessus infection and lung function decline in cystic fibrosis. J Cyst Fibros 2010;9:117-123.
-
(2010)
J Cyst Fibros
, vol.9
, pp. 117-123
-
-
Esther, C.R.1
Esserman, D.A.2
Gilligan, P.3
Kerr, A.4
Noone, P.G.5
-
35
-
-
84911897085
-
Environmental risks for nontuberculous mycobacteria: Individual exposures and climatic factors in the cystic fibrosis population
-
Prevots DR, Adjemian J, Fernandez AG, Knowles MR, Olivier KN. Environmental risks for nontuberculous mycobacteria: individual exposures and climatic factors in the cystic fibrosis population. Ann Am Thorac Soc 2014;11:1032-1038.
-
(2014)
Ann Am Thorac Soc
, vol.11
, pp. 1032-1038
-
-
Prevots, D.R.1
Adjemian, J.2
Fernandez, A.G.3
Knowles, M.R.4
Olivier, K.N.5
-
36
-
-
14944364000
-
Identification of Mycobacterium species by secA1 sequences
-
Zelazny AM, Calhoun LB, Li L, Shea YR, Fischer SH. Identification of Mycobacterium species by secA1 sequences. J Clin Microbiol 2005; 43:1051-1058.
-
(2005)
J Clin Microbiol
, vol.43
, pp. 1051-1058
-
-
Zelazny, A.M.1
Calhoun, L.B.2
Li, L.3
Shea, Y.R.4
Fischer, S.H.5
-
37
-
-
33847050904
-
An official ATS/IDSA statement: Diagnosis treatment and prevention of nontuberculous mycobacterial diseases
-
[ Published erratum appears in Am J Respir Crit Care Med 175:744-745.]
-
Griffith DE, Aksamit T, Brown-Elliott BA, Catanzaro A, Daley C, Gordin F, Holland SM, Horsburgh R, Huitt G, Iademarco MF, et al.; ATS Mycobacterial Diseases Subcommittee; American Thoracic Society; Infectious Disease Society of America. An official ATS/IDSA statement: diagnosis, treatment, and prevention of nontuberculous mycobacterial diseases. Am J Respir Crit Care Med 2007;175: 367-416. [Published erratum appears in Am J Respir Crit Care Med 175:744-745.]
-
(2007)
Am J Respir Crit Care Med
, vol.175
, pp. 367-416
-
-
Griffith, D.E.1
Aksamit, T.2
Brown-Elliott, B.A.3
Catanzaro, A.4
Daley, C.5
Gordin, F.6
Holland, S.M.7
Horsburgh, R.8
Huitt, G.9
Iademarco, M.F.10
-
38
-
-
84875829440
-
Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health
-
Mogayzel PJ Jr, Naureckas ET, Robinson KA, Mueller G, Hadjiliadis D, Hoag JB, Lubsch L, Hazle L, Sabadosa K, Marshall B; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2013;187:680-689.
-
(2013)
Am J Respir Crit Care Med
, vol.187
, pp. 680-689
-
-
Mogayzel, P.J.1
Naureckas, E.T.2
Robinson, K.A.3
Mueller, G.4
Hadjiliadis, D.5
Hoag, J.B.6
Lubsch, L.7
Hazle, L.8
Sabadosa, K.9
Marshall, B.10
-
39
-
-
84908146256
-
Nontuberculous mycobacteria among patients with cystic fibrosis in the United States: Screening practices and environmental risk
-
Adjemian J, Olivier KN, Prevots DR. Nontuberculous mycobacteria among patients with cystic fibrosis in the United States: screening practices and environmental risk. Am J Respir Crit Care Med 2014; 190:581-586.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 581-586
-
-
Adjemian, J.1
Olivier, K.N.2
Prevots, D.R.3
-
40
-
-
84855995836
-
Respiratory outbreak of Mycobacterium abscessus subspecies massiliense in a lung transplant and cystic fibrosis center
-
Aitken ML, Limaye A, Pottinger P, Whimbey E, Goss CH, Tonelli MR, Cangelosi GA, Dirac MA, Olivier KN, Brown-Elliott BA, et al. Respiratory outbreak of Mycobacterium abscessus subspecies massiliense in a lung transplant and cystic fibrosis center. Am J Respir Crit Care Med 2012;185:231-232.
-
(2012)
Am J Respir Crit Care Med
, vol.185
, pp. 231-232
-
-
Aitken, M.L.1
Limaye, A.2
Pottinger, P.3
Whimbey, E.4
Goss, C.H.5
Tonelli, M.R.6
Cangelosi, G.A.7
Dirac, M.A.8
Olivier, K.N.9
Brown-Elliott, B.A.10
-
41
-
-
84877584233
-
Whole-genome sequencing to identify transmission of Mycobacterium abscessus between patients with cystic fibrosis: A retrospective cohort study
-
Bryant JM, Grogono DM, Greaves D, Foweraker J, Roddick I, Inns T, Reacher M, Haworth CS, Curran MD, Harris SR, et al. Whole-genome sequencing to identify transmission of Mycobacterium abscessus between patients with cystic fibrosis: a retrospective cohort study. Lancet 2013;381:1551-1560.
-
(2013)
Lancet
, vol.381
, pp. 1551-1560
-
-
Bryant, J.M.1
Grogono, D.M.2
Greaves, D.3
Foweraker, J.4
Roddick, I.5
Inns, T.6
Reacher, M.7
Haworth, C.S.8
Curran, M.D.9
Harris, S.R.10
-
43
-
-
80051572617
-
Infection inflammation and lung function decline in infants with cystic fibrosis
-
Pillarisetti N, Williamson E, Linnane B, Skoric B, Robertson CF, Robinson P, Massie J, Hall GL, Sly P, Stick S, et al.; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF). Infection, inflammation, and lung function decline in infants with cystic fibrosis. Am J Respir Crit Care Med 2011;184:75-81.
-
(2011)
Am J Respir Crit Care Med
, vol.184
, pp. 75-81
-
-
Pillarisetti, N.1
Williamson, E.2
Linnane, B.3
Skoric, B.4
Robertson, C.F.5
Robinson, P.6
Massie, J.7
Hall, G.L.8
Sly, P.9
Stick, S.10
-
44
-
-
84878000440
-
Risk factors for bronchiectasis in children with cystic fibrosis
-
Sly PD, Gangell CL, Chen L, Ware RS, Ranganathan S, Mott LS, Murray CP, Stick SM, Investigators AC; AREST CF Investigators. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med 2013;368:1963-1970.
-
(2013)
N Engl J Med
, vol.368
, pp. 1963-1970
-
-
Sly, P.D.1
Gangell, C.L.2
Chen, L.3
Ware, R.S.4
Ranganathan, S.5
Mott, L.S.6
Murray, C.P.7
Stick, S.M.8
-
45
-
-
84928559004
-
Acute pulmonary exacerbation and lung function decline in patients with cystic fibrosis: High-mobility group box 1 (HMGB1) between inflammation and infection
-
Chirico V, Lacquaniti A, Leonardi S, Grasso L, Rotolo N, Romano C, Di Dio G, Lionetti E, David A, Arrigo T, et al. Acute pulmonary exacerbation and lung function decline in patients with cystic fibrosis: high-mobility group box 1 (HMGB1) between inflammation and infection. Clin Microbiol Infect 2015;21:e1-e9.
-
(2015)
Clin Microbiol Infect
, vol.21
, pp. e1-e9
-
-
Chirico, V.1
Lacquaniti, A.2
Leonardi, S.3
Grasso, L.4
Rotolo, N.5
Romano, C.6
Di Dio, G.7
Lionetti, E.8
David, A.9
Arrigo, T.10
-
46
-
-
84920102880
-
Identification of neutrophil activation markers as novel surrogate markers of CF lung disease
-
Rath T, Zwaschka L, Hage L, Kügler M, Menendez K, Naehrlich L, Schulz R, Roderfeld M, Roeb E. Identification of neutrophil activation markers as novel surrogate markers of CF lung disease. PLoS One 2014;9:e115847.
-
(2014)
PLoS One
, vol.9
, pp. e115847
-
-
Rath, T.1
Zwaschka, L.2
Hage, L.3
Kügler, M.4
Menendez, K.5
Naehrlich, L.6
Schulz, R.7
Roderfeld, M.8
Roeb, E.9
-
47
-
-
36048972322
-
Airway remodelling in children with cystic fibrosis
-
Hilliard TN, Regamey N, Shute JK, Nicholson AG, Alton EW, Bush A, Davies JC. Airway remodelling in children with cystic fibrosis. Thorax 2007;62:1074-1080.
-
(2007)
Thorax
, vol.62
, pp. 1074-1080
-
-
Hilliard, T.N.1
Regamey, N.2
Shute, J.K.3
Nicholson, A.G.4
Alton, E.W.5
Bush, A.6
Davies, J.C.7
-
49
-
-
84872502797
-
Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo
-
Le Gars M, Descamps D, Roussel D, Saussereau E, Guillot L, Ruffin M, Tabary O, Hong SS, Boulanger P, Paulais M, et al. Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo. Am J Respir Crit Care Med 2013;187:170-179.
-
(2013)
Am J Respir Crit Care Med
, vol.187
, pp. 170-179
-
-
Le Gars, M.1
Descamps, D.2
Roussel, D.3
Saussereau, E.4
Guillot, L.5
Ruffin, M.6
Tabary, O.7
Hong, S.S.8
Boulanger, P.9
Paulais, M.10
-
50
-
-
84899791723
-
Lack of neutrophil elastase reduces inflammation, mucus hypersecretion, and emphysema, but not mucus obstruction, in mice with cystic fibrosis-like lung disease
-
Gehrig S, Duerr J, Weitnauer M, Wagner CJ, Graeber SY, Schatterny J, Hirtz S, Belaaouaj A, Dalpke AH, Schultz C, et al. Lack of neutrophil elastase reduces inflammation, mucus hypersecretion, and emphysema, but not mucus obstruction, in mice with cystic fibrosis-like lung disease. Am J Respir Crit Care Med 2014;189: 1082-1092.
-
(2014)
Am J Respir Crit Care Med
, vol.189
, pp. 1082-1092
-
-
Gehrig, S.1
Duerr, J.2
Weitnauer, M.3
Wagner, C.J.4
Graeber, S.Y.5
Schatterny, J.6
Hirtz, S.7
Belaaouaj, A.8
Dalpke, A.H.9
Schultz, C.10
-
51
-
-
80053063761
-
The role of matrix metalloproteinases in cystic fibrosis lung disease
-
Gaggar A, Hector A, Bratcher PE, Mall MA, Griese M, Hartl D. The role of matrix metalloproteinases in cystic fibrosis lung disease. Eur Respir J 2011;38:721-727.
-
(2011)
Eur Respir J
, vol.38
, pp. 721-727
-
-
Gaggar, A.1
Hector, A.2
Bratcher, P.E.3
Mall, M.A.4
Griese, M.5
Hartl, D.6
-
52
-
-
0037768888
-
Inactivation of human beta-defensins 2 and 3 by elastolytic cathepsins
-
Taggart CC, Greene CM, Smith SG, Levine RL, McCray PB Jr, O'Neill S, McElvaney NG. Inactivation of human beta-defensins 2 and 3 by elastolytic cathepsins. J Immunol 2003;171:931-937.
-
(2003)
J Immunol
, vol.171
, pp. 931-937
-
-
Taggart, C.C.1
Greene, C.M.2
Smith, S.G.3
Levine, R.L.4
McCray, P.B.5
O'Neill, S.6
McElvaney, N.G.7
-
53
-
-
84906924544
-
A new player in the game: Epithelial cathepsin S in early cystic fibrosis lung disease
-
Mall MA, Schultz C. A new player in the game: epithelial cathepsin S in early cystic fibrosis lung disease. Am J Respir Crit Care Med 2014; 190:126-127.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 126-127
-
-
Mall, M.A.1
Schultz, C.2
-
54
-
-
84906922269
-
MiR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production
-
Weldon S, McNally P, McAuley DF, Oglesby IK, Wohlford-Lenane CL, Bartlett JA, Scott CJ, McElvaney NG, Greene CM, McCray PB Jr, et al. miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production. Am J Respir Crit Care Med 2014;190:165-174.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 165-174
-
-
Weldon, S.1
McNally, P.2
McAuley, D.F.3
Oglesby, I.K.4
Wohlford-Lenane, C.L.5
Bartlett, J.A.6
Scott, C.J.7
McElvaney, N.G.8
Greene, C.M.9
McCray, P.B.10
-
55
-
-
84878970875
-
Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation
-
Davies JC, Wainwright CE, Canny GJ, Chilvers MA, Howenstine MS, Munck A, Mainz JG, Rodriguez S, Li H, Yen K, et al.; VX08-770-103 (ENVISION) Study Group. Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation. Am J Respir Crit Care Med 2013;187:1219-1225.
-
(2013)
Am J Respir Crit Care Med
, vol.187
, pp. 1219-1225
-
-
Davies, J.C.1
Wainwright, C.E.2
Canny, G.J.3
Chilvers, M.A.4
Howenstine, M.S.5
Munck, A.6
Mainz, J.G.7
Rodriguez, S.8
Li, H.9
Yen, K.10
-
56
-
-
84918774941
-
Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: A phase 3 open-label extension study (PERSIST)
-
McKone EF, Borowitz D, Drevinek P, Griese M, Konstan MW, Wainwright C, Ratjen F, Sermet-Gaudelus I, Plant B, Munck A, et al.; VX08-770-105 (PERSIST) Study Group. Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp-CFTR mutation: a phase 3, open-label extension study (PERSIST). Lancet Respir Med 2014;2:902-910.
-
(2014)
Lancet Respir Med
, vol.2
, pp. 902-910
-
-
McKone, E.F.1
Borowitz, D.2
Drevinek, P.3
Griese, M.4
Konstan, M.W.5
Wainwright, C.6
Ratjen, F.7
Sermet-Gaudelus, I.8
Plant, B.9
Munck, A.10
-
57
-
-
80455162465
-
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
-
Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, D?revínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, et al.; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011;365:1663-1672.
-
(2011)
N Engl J Med
, vol.365
, pp. 1663-1672
-
-
Ramsey, B.W.1
Davies, J.2
McElvaney, N.G.3
Tullis, E.4
Bell, S.C.5
Drevínek, P.6
Griese, M.7
McKone, E.F.8
Wainwright, C.E.9
Konstan, M.W.10
-
58
-
-
84906929662
-
Ivacaftor: From bench to bedside. and back again
-
Molloy K, McElvaney NG. Ivacaftor: from bench to bedside. and back again. Am J Respir Crit Care Med 2014;190:128-129.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 128-129
-
-
Molloy, K.1
McElvaney, N.G.2
-
60
-
-
84864313785
-
Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype
-
Gustafsson JK, Ermund A, Ambort D, Johansson ME, Nilsson HE, Thorell K, Hebert H, Sjövall H, Hansson GC. Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype. J Exp Med 2012;209: 1263-1272.
-
(2012)
J Exp Med
, vol.209
, pp. 1263-1272
-
-
Gustafsson, J.K.1
Ermund, A.2
Ambort, D.3
Johansson, M.E.4
Nilsson, H.E.5
Thorell, K.6
Hebert, H.7
Sjövall, H.8
Hansson, G.C.9
-
61
-
-
78649647597
-
Role of epithelial HCO3? transport in mucin secretion: Lessons from cystic fibrosis
-
Quinton PM. Role of epithelial HCO3? transport in mucin secretion: lessons from cystic fibrosis. Am J Physiol Cell Physiol 2010;299: C1222-C1233.
-
(2010)
Am J Physiol Cell Physiol
, vol.299
, pp. C1222-C1233
-
-
Quinton, P.M.1
-
62
-
-
84876999613
-
Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations
-
Zemanick ET, Harris JK, Wagner BD, Robertson CE, Sagel SD, Stevens MJ, Accurso FJ, Laguna TA. Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations. PLoS One 2013;8:e62917.
-
(2013)
PLoS One
, vol.8
, pp. e62917
-
-
Zemanick, E.T.1
Harris, J.K.2
Wagner, B.D.3
Robertson, C.E.4
Sagel, S.D.5
Stevens, M.J.6
Accurso, F.J.7
Laguna, T.A.8
-
63
-
-
84890435909
-
Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function
-
Van Goor F, Yu H, Burton B, Hoffman BJ. Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function. J Cyst Fibros 2014;13:29-36.
-
(2014)
J Cyst Fibros
, vol.13
, pp. 29-36
-
-
Van Goor, F.1
Yu, H.2
Burton, B.3
Hoffman, B.J.4
-
64
-
-
84911493956
-
Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation
-
De Boeck K, Munck A, Walker S, Faro A, Hiatt P, Gilmartin G, Higgins M. Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation. J Cyst Fibros 2014;13:674-680.
-
(2014)
J Cyst Fibros
, vol.13
, pp. 674-680
-
-
De Boeck, K.1
Munck, A.2
Walker, S.3
Faro, A.4
Hiatt, P.5
Gilmartin, G.6
Higgins, M.7
-
65
-
-
84865853894
-
Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation
-
Flume PA, Liou TG, Borowitz DS, Li H, Yen K, Ordoñez CL, Geller DE, Group VXS; VX 08-770-104 Study Group. Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation. Chest 2012;142:718-724.
-
(2012)
Chest
, vol.142
, pp. 718-724
-
-
Flume, P.A.1
Liou, T.G.2
Borowitz, D.S.3
Li, H.4
Yen, K.5
Ordoñez, C.L.6
Geller, D.E.7
Vxs, G.8
-
66
-
-
81755163563
-
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
-
Van Goor F, Hadida S, Grootenhuis PD, Burton B, Stack JH, Straley KS, Decker CJ, Miller M, McCartney J, Olson ER, et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci USA 2011;108:18843-18848.
-
(2011)
Proc Natl Acad Sci USA
, vol.108
, pp. 18843-18848
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.3
Burton, B.4
Stack, J.H.5
Straley, K.S.6
Decker, C.J.7
Miller, M.8
McCartney, J.9
Olson, E.R.10
-
67
-
-
84855202429
-
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
-
Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax 2012;67:12-18.
-
(2012)
Thorax
, vol.67
, pp. 12-18
-
-
Clancy, J.P.1
Rowe, S.M.2
Accurso, F.J.3
Aitken, M.L.4
Amin, R.S.5
Ashlock, M.A.6
Ballmann, M.7
Boyle, M.P.8
Bronsveld, I.9
Campbell, P.W.10
-
68
-
-
84904002908
-
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: A phase 2 randomised controlled trial
-
Boyle MP, Bell SC, Konstan MW, McColley SA, Rowe SM, Rietschel E, Huang X, Waltz D, Patel NR, Rodman D; VX09-809-102 study group. A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial. Lancet Respir Med 2014;2:527-538.
-
(2014)
Lancet Respir Med
, vol.2
, pp. 527-538
-
-
Boyle, M.P.1
Bell, S.C.2
Konstan, M.W.3
McColley, S.A.4
Rowe, S.M.5
Rietschel, E.6
Huang, X.7
Waltz, D.8
Patel, N.R.9
Rodman, D.10
-
69
-
-
84907223425
-
Potentiator ivacaftor abrogates pharmacological correction of DeltaF508 CFTR in cystic fibrosis
-
Cholon DM, Quinney NL, Fulcher ML, Esther CR Jr, Das J, Dokholyan NV, Randell SH, Boucher RC, Gentzsch M. Potentiator ivacaftor abrogates pharmacological correction of DeltaF508 CFTR in cystic fibrosis. Sci Transl Med 2014;6:246ra296.
-
(2014)
Sci Transl Med
, vol.6
, pp. 246ra296
-
-
Cholon, D.M.1
Quinney, N.L.2
Fulcher, M.L.3
Esther, C.R.4
Das, J.5
Dokholyan, N.V.6
Randell, S.H.7
Boucher, R.C.8
Gentzsch, M.9
-
70
-
-
84907211027
-
Some gating potentiators, including VX-770, diminish DeltaF508-CFTR functional expression
-
Veit G, Avramescu RG, Perdomo D, Phuan PW, Bagdany M, Apaja PM, Borot F, Szollosi D, Wu YS, Finkbeiner WE, et al. Some gating potentiators, including VX-770, diminish DeltaF508-CFTR functional expression. Sci Transl Med 2014;6:246ra297.
-
(2014)
Sci Transl Med
, vol.6
, pp. 246ra297
-
-
Veit, G.1
Avramescu, R.G.2
Perdomo, D.3
Phuan, P.W.4
Bagdany, M.5
Apaja, P.M.6
Borot, F.7
Szollosi, D.8
Wu, Y.S.9
Finkbeiner, W.E.10
-
71
-
-
84922042138
-
Pulmonary hypertension in cystic fibrosis with advanced lung disease
-
Hayes D Jr, Tobias JD, Mansour HM, Kirkby S, McCoy KS, Daniels CJ, Whitson BA. Pulmonary hypertension in cystic fibrosis with advanced lung disease. Am J Respir Crit Care Med 2014;190: 898-905.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 898-905
-
-
Hayes, D.1
Tobias, J.D.2
Mansour, H.M.3
Kirkby, S.4
McCoy, K.S.5
Daniels, C.J.6
Whitson, B.A.7
-
72
-
-
84903830061
-
Oxidation contributes to low glutathione in the airways of children with cystic fibrosis
-
Kettle AJ, Turner R, Gangell CL, Harwood DT, Khalilova IS, Chapman AL, Winterbourn CC, Sly PD, Arest CF; AREST CF. Oxidation contributes to low glutathione in the airways of children with cystic fibrosis. Eur Respir J 2014;44:122-129.
-
(2014)
Eur Respir J
, vol.44
, pp. 122-129
-
-
Kettle, A.J.1
Turner, R.2
Gangell, C.L.3
Harwood, D.T.4
Khalilova, I.S.5
Chapman, A.L.6
Winterbourn, C.C.7
Sly, P.D.8
Arest, C.F.9
Arest, C.F.10
-
73
-
-
57149116921
-
Lung function in infants with cystic fibrosis diagnosed by newborn screening
-
Linnane BM, Hall GL, Nolan G, Brennan S, Stick SM, Sly PD, Robertson CF, Robinson PJ, Franklin PJ, Turner SW, et al.; AREST-CF. 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
Brennan, S.4
Stick, S.M.5
Sly, P.D.6
Robertson, C.F.7
Robinson, P.J.8
Franklin, P.J.9
Turner, S.W.10
-
74
-
-
67650712233
-
Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening
-
Sly PD, Brennan S, Gangell C, de Klerk N, Murray C, Mott L, Stick SM, Robinson PJ, Robertson CF, Ranganathan SC; Australian Respiratory Early Surveillance Team 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
De Klerk, N.4
Murray, C.5
Mott, L.6
Stick, S.M.7
Robinson, P.J.8
Robertson, C.F.9
Ranganathan, S.C.10
-
75
-
-
70349831005
-
Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening
-
e1
-
Stick SM, Brennan S, Murray C, Douglas T, von Ungern-Sternberg BS, Garratt LW, Gangell CL, De Klerk N, Linnane B, Ranganathan S, et al.; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST-CF). Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr 2009;155:623-628, e1.
-
(2009)
J Pediatr
, vol.155
, pp. 623-628
-
-
Stick, S.M.1
Brennan, S.2
Murray, C.3
Douglas, T.4
Von Ungern-Sternberg, B.S.5
Garratt, L.W.6
Gangell, C.L.7
De Klerk, N.8
Linnane, B.9
Ranganathan, S.10
-
76
-
-
84923930953
-
Early respiratory infection is associated with reduced spirometry in children with cystic fibrosis
-
Ramsey KA, Ranganathan S, Park J, Skoric B, Adams AM, Simpson SJ, Robins-Browne RM, Franklin PJ, de Klerk NH, Sly PD, et al.; AREST CF. Early respiratory infection is associated with reduced spirometry in children with cystic fibrosis. Am J Respir Crit Care Med 2014;190:1111-1116.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 1111-1116
-
-
Ramsey, K.A.1
Ranganathan, S.2
Park, J.3
Skoric, B.4
Adams, A.M.5
Simpson, S.J.6
Robins-Browne, R.M.7
Franklin, P.J.8
De Klerk, N.H.9
Sly, P.D.10
-
77
-
-
84928987855
-
Reply: Cumulative radiation exposure to abdominal organs in patients with cystic fibrosis should not be forgotten
-
Kuo W, Ciet P, Tiddens H, Zhang W, Guillerman RP, van Straten M. Reply: cumulative radiation exposure to abdominal organs in patients with cystic fibrosis should not be forgotten. Am J Respir Crit Care Med 2014;190:962.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 962
-
-
Kuo, W.1
Ciet, P.2
Tiddens, H.3
Zhang, W.4
Guillerman, R.P.5
Van Straten, M.6
-
78
-
-
84898994097
-
Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease
-
Wielpütz MO, Puderbach M, Kopp-Schneider A, Stahl M, Fritzsching E, Sommerburg O, Ley S, Sumkauskaite M, Biederer J, Kauczor HU, et al. Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease. Am J Respir Crit Care Med 2014;189: 956-965.
-
(2014)
Am J Respir Crit Care Med
, vol.189
, pp. 956-965
-
-
Wielpütz, M.O.1
Puderbach, M.2
Kopp-Schneider, A.3
Stahl, M.4
Fritzsching, E.5
Sommerburg, O.6
Ley, S.7
Sumkauskaite, M.8
Biederer, J.9
Kauczor, H.U.10
-
79
-
-
84907807886
-
In vivo X-ray imaging reveals improved airway surface hydration after a therapy designed for cystic fibrosis
-
Morgan KS, Donnelley M, Farrow N, Fouras A, Yagi N, Suzuki Y, Takeuchi A, Uesugi K, Boucher RC, Siu KK, et al. In vivo X-ray imaging reveals improved airway surface hydration after a therapy designed for cystic fibrosis. Am J Respir Crit Care Med 2014;190: 469-471.
-
(2014)
Am J Respir Crit Care Med
, vol.190
, pp. 469-471
-
-
Morgan, K.S.1
Donnelley, M.2
Farrow, N.3
Fouras, A.4
Yagi, N.5
Suzuki, Y.6
Takeuchi, A.7
Uesugi, K.8
Boucher, R.C.9
Siu, K.K.10
-
80
-
-
84903556678
-
Tracking extended mucociliary transport activity of individual deposited particles: Longitudinal synchrotron X-ray imaging in live mice
-
Donnelley M, Morgan KS, Siu KK, Fouras A, Farrow NR, Carnibella RP, Parsons DW. Tracking extended mucociliary transport activity of individual deposited particles: longitudinal synchrotron X-ray imaging in live mice. J Synchrotron Radiat 2014;21: 768-773.
-
(2014)
J Synchrotron Radiat
, vol.21
, pp. 768-773
-
-
Donnelley, M.1
Morgan, K.S.2
Siu, K.K.3
Fouras, A.4
Farrow, N.R.5
Carnibella, R.P.6
Parsons, D.W.7
-
81
-
-
84907343152
-
Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the Cystic Fibrosis Foundation patient registry
-
MacKenzie T, Gifford AH, Sabadosa KA, Quinton HB, Knapp EA, Goss CH, Marshall BC. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the Cystic Fibrosis Foundation patient registry. Ann Intern Med 2014;161:233-241.
-
(2014)
Ann Intern Med
, vol.161
, pp. 233-241
-
-
MacKenzie, T.1
Gifford, A.H.2
Sabadosa, K.A.3
Quinton, H.B.4
Knapp, E.A.5
Goss, C.H.6
Marshall, B.C.7
|