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Volumn 192, Issue 6, 2015, Pages 669-675

Update in cystic fibrosis 2014

Author keywords

[No Author keywords available]

Indexed keywords

CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL SODIUM CHANNEL; INTERLEUKIN 1BETA; INTERLEUKIN 6; INTERLEUKIN 8; 1,3 BENZODIOXOLE DERIVATIVE; AMINOPHENOL DERIVATIVE; AMINOPYRIDINE DERIVATIVE; GENETIC MARKER; IVACAFTOR; LUMACAFTOR; QUINOLONE DERIVATIVE;

EID: 84942274759     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201504-0656UP     Document Type: Article
Times cited : (22)

References (81)
  • 4
    • 50649123290 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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
  • 11
    • 80052538896 scopus 로고    scopus 로고
    • Comparative biology of cystic fibrosis animal models
    • Fisher JT, Zhang Y, Engelhardt JF. Comparative biology of cystic fibrosis animal models. Methods Mol Biol 2011;742:311-334.
    • (2011) Methods Mol Biol , vol.742 , pp. 311-334
    • Fisher, J.T.1    Zhang, Y.2    Engelhardt, J.F.3
  • 16
    • 84907815404 scopus 로고    scopus 로고
    • 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
  • 19
    • 48149090717 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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
  • 24
    • 34447530328 scopus 로고    scopus 로고
    • 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
  • 26
    • 0142043977 scopus 로고    scopus 로고
    • 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
  • 30
    • 84907293414 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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
  • 34
    • 76749097178 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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
  • 37
    • 33847050904 scopus 로고    scopus 로고
    • 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
  • 39
    • 84908146256 scopus 로고    scopus 로고
    • 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
  • 41
    • 84877584233 scopus 로고    scopus 로고
    • 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
  • 45
    • 84928559004 scopus 로고    scopus 로고
    • 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
  • 53
    • 84906924544 scopus 로고    scopus 로고
    • 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
  • 56
    • 84918774941 scopus 로고    scopus 로고
    • 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
  • 58
    • 84906929662 scopus 로고    scopus 로고
    • 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
  • 61
    • 78649647597 scopus 로고    scopus 로고
    • 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
  • 63
    • 84890435909 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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
  • 67
    • 84855202429 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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
  • 77
    • 84928987855 scopus 로고    scopus 로고
    • 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
  • 80
    • 84903556678 scopus 로고    scopus 로고
    • 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 scopus 로고    scopus 로고
    • 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


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