메뉴 건너뛰기




Volumn 148, Issue 2, 2015, Pages 533-542

The evolution of cystic fibrosis care

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLCYSTEINE; ANTIBIOTIC AGENT; ATALUREN; AZITHROMYCIN; CORTICOSTEROID; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DENUFOSOL; DOCOSAHEXAENOIC ACID; DORNASE ALFA; IBUPROFEN; IVACAFTOR; LUMACAFTOR; MUCOLYTIC AGENT; PLACEBO; SILDENAFIL; SODIUM CHLORIDE; ANTIINFECTIVE AGENT; ANTIINFLAMMATORY AGENT; CFTR PROTEIN, HUMAN; EXPECTORANT AGENT;

EID: 84939224261     PISSN: 00123692     EISSN: 19313543     Source Type: Journal    
DOI: 10.1378/chest.14-1997     Document Type: Article
Times cited : (40)

References (113)
  • 1
    • 47049115524 scopus 로고    scopus 로고
    • Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report
    • Cystic Fibrosis Foundation
    • Farrell PM, Rosenstein BJ, White TB, et al; Cystic Fibrosis Foundation. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008; 153 (2): S4-S14.
    • (2008) J Pediatr , vol.153 , Issue.2 , pp. S4-S14
    • Farrell, P.M.1    Rosenstein, B.J.2    White, T.B.3
  • 2
    • 84856857888 scopus 로고    scopus 로고
    • Genetic variation and clinical heterogeneity in cystic fibrosis
    • Drumm ML, Ziady AG, Davis PB. Genetic variation and clinical heterogeneity in cystic fibrosis. A nnu Rev Pathol. 2012; 7: 267-282.
    • (2012) Annu Rev Pathol , vol.7 , pp. 267-282
    • Drumm, M.L.1    Ziady, A.G.2    Davis, P.B.3
  • 3
    • 0020700816 scopus 로고
    • Chloride impermeability in cystic fibrosis
    • Quinton PM. Chloride impermeability in cystic fibrosis. N ature. 1983; 301 (5899): 421-422.
    • (1983) Nature , vol.301 , Issue.5899 , pp. 421-422
    • Quinton, P.M.1
  • 4
    • 0020596438 scopus 로고
    • Relative ion permeability of normal and cystic fibrosis nasal epithelium
    • Knowles M, Gatzy J, Boucher R. Relative ion permeability of normal and cystic fibrosis nasal epithelium. J Clin Invest. 1983; 71 (5): 1410-1417.
    • (1983) J Clin Invest , vol.71 , Issue.5 , pp. 1410-1417
    • Knowles, M.1    Gatzy, J.2    Boucher, R.3
  • 5
    • 0032910501 scopus 로고    scopus 로고
    • The genesis of cystic fibrosis lung disease
    • Wine JJ. The genesis of cystic fibrosis lung disease. J Clin Invest. 1999; 103 (3): 309-312.
    • (1999) J Clin Invest , vol.103 , Issue.3 , pp. 309-312
    • Wine, J.J.1
  • 6
    • 48149090717 scopus 로고    scopus 로고
    • Cystic fibrosis: Impaired bicarbonate secretion and mucoviscidosis
    • Quinton PM. Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis. Lancet. 2008; 372 (9636): 415-417.
    • (2008) Lancet , vol.372 , Issue.9636 , pp. 415-417
    • Quinton, P.M.1
  • 7
    • 0036194724 scopus 로고    scopus 로고
    • Mucus clearance as a primary innate defense mechanism for mammalian airways
    • Knowles MR, Boucher RC. Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest. 2002; 109 (5): 571-577.
    • (2002) J Clin Invest , vol.109 , Issue.5 , pp. 571-577
    • Knowles, M.R.1    Boucher, R.C.2
  • 8
    • 84863476402 scopus 로고    scopus 로고
    • Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
    • Pezzulo AA, Tang XX, Hoegger MJ, et al. Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature. 2012; 487 (7405): 109-113.
    • (2012) Nature , vol.487 , Issue.7405 , pp. 109-113
    • Pezzulo, A.A.1    Tang, X.X.2    Hoegger, M.J.3
  • 9
    • 0024453308 scopus 로고
    • Identification of the cystic fibrosis gene: Chromosome walking and jumping
    • Rommens JM, Iannuzzi MC, Kerem B, et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science. 1989; 245 (4922): 1059-1065.
    • (1989) Science , vol.245 , Issue.4922 , pp. 1059-1065
    • Rommens, J.M.1    Iannuzzi, M.C.2    Kerem, B.3
  • 10
    • 0024423668 scopus 로고
    • Identification of the cystic fibrosis gene: Genetic analysis
    • Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989; 245 (4922): 1073-1080.
    • (1989) Science , vol.245 , Issue.4922 , pp. 1073-1080
    • Kerem, B.1    Rommens, J.M.2    Buchanan, J.A.3
  • 11
    • 84877297777 scopus 로고    scopus 로고
    • The influence of genetics on cystic fibrosis phenotypes
    • Knowles MR, Drumm M. The influence of genetics on cystic fibrosis phenotypes. Cold Spring Harb Perspect Med. 2012; 2 (12): a009548.
    • (2012) Cold Spring Harb Perspect Med , vol.2 , Issue.12 , pp. a009548
    • Knowles, M.R.1    Drumm, M.2
  • 12
    • 0034089406 scopus 로고    scopus 로고
    • Genotype-phenotype relationships in cystic fibrosis
    • Mickle JE, Cutting GR. Genotype-phenotype relationships in cystic fibrosis. M ed Clin North Am. 2000; 84 (3): 597-607.
    • (2000) Med Clin North Am , vol.84 , Issue.3 , pp. 597-607
    • Mickle, J.E.1    Cutting, G.R.2
  • 13
    • 0027162649 scopus 로고
    • Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
    • Welsh MJ, Smith AE. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. C ell. 1993; 73 (7): 1251-1254.
    • (1993) Cell , vol.73 , Issue.7 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 14
    • 0029616734 scopus 로고
    • Cystic fibrosis: Genotypic and phenotypic variations
    • Zielenski J, Tsui LC. Cystic fibrosis: genotypic and phenotypic variations. Annu Rev Genet. 1995; 29: 777-807.
    • (1995) Annu Rev Genet , vol.29 , pp. 777-807
    • Zielenski, J.1    Tsui, L.C.2
  • 15
    • 0025242929 scopus 로고
    • Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
    • Cheng SH, Gregory RJ, Marshall J, et al. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. C ell. 1990; 63 (4): 827-834.
    • (1990) Cell , vol.63 , Issue.4 , pp. 827-834
    • Cheng, S.H.1    Gregory, R.J.2    Marshall, J.3
  • 16
    • 0025310336 scopus 로고
    • A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein
    • Cutting GR, Kasch LM, Rosenstein BJ, et al. A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein. Nature. 1990; 346 (6282): 366-369.
    • (1990) Nature , vol.346 , Issue.6282 , pp. 366-369
    • Cutting, G.R.1    Kasch, L.M.2    Rosenstein, B.J.3
  • 17
    • 0026337305 scopus 로고
    • Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes
    • Drumm ML, Wilkinson DJ, Smit LS, et al. Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes. Science. 1991; 254 (5039): 1797-1799.
    • (1991) Science , vol.254 , Issue.5039 , pp. 1797-1799
    • Drumm, M.L.1    Wilkinson, D.J.2    Smit, L.S.3
  • 18
    • 0027521663 scopus 로고
    • A mutation in CFTR produces different phenotypes depending on chromosomal background
    • Kiesewetter S, Macek M Jr. Davis C, et al. A mutation in CFTR produces different phenotypes depending on chromosomal background . Nat Genet. 1993; 5 (3): 274-278.
    • (1993) Nat Genet , vol.5 , Issue.3 , pp. 274-278
    • Kiesewetter, S.1    Macek, M.2    Davis, C.3
  • 20
    • 84884672649 scopus 로고    scopus 로고
    • Probability of treatment following acute decline in lung function in children with cystic fibrosis is related to baseline pulmonary function
    • Scientific Advisory Group, investigators; coordinators of the Epidemiologic Study of Cystic Fibrosis
    • Morgan WJ, Wagener JS, Yegin A, Pasta DJ, Millar SJ, Konstan MW; Scientific Advisory Group, investigators; coordinators of the Epidemiologic Study of Cystic Fibrosis. Probability of treatment following acute decline in lung function in children with cystic fibrosis is related to baseline pulmonary function. J Pediatr. 2013; 163 (4): 1152-1157.
    • (2013) J Pediatr , vol.163 , Issue.4 , pp. 1152-1157
    • Morgan, W.J.1    Wagener, J.S.2    Yegin, A.3    Pasta, D.J.4    Millar, S.J.5    Konstan, M.W.6
  • 21
    • 59349085577 scopus 로고    scopus 로고
    • Genetic and non-genetic determinants of outcomes in cystic fibrosis
    • Wolfenden LL, Schechter MS. Genetic and non-genetic determinants of outcomes in cystic fibrosis. P aediatr Respir Rev. 2009; 10 (1): 32-36.
    • (2009) Paediatr Respir Rev , vol.10 , Issue.1 , pp. 32-36
    • Wolfenden, L.L.1    Schechter, M.S.2
  • 22
    • 0347812925 scopus 로고    scopus 로고
    • Non-genetic influences on cystic fibrosis lung disease: The role of sociodemographic characteristics, environmental exposures, and healthcare interventions
    • Schechter MS. Non-genetic influences on cystic fibrosis lung disease: the role of sociodemographic characteristics, environmental exposures, and healthcare interventions. Semin Respir Crit Care Med. 2003; 24 (6): 639-652.
    • (2003) Semin Respir Crit Care Med , vol.24 , Issue.6 , pp. 639-652
    • Schechter, M.S.1
  • 23
    • 0036112237 scopus 로고    scopus 로고
    • Predictors of deterioration of lung function in cystic fibrosis
    • Schaedel C, de Monestrol I, Hjelte L, et al. Predictors of deterioration of lung function in cystic fibrosis. P ediatr Pulmonol. 2002; 33 (6): 483-491.
    • (2002) Pediatr Pulmonol , vol.33 , Issue.6 , pp. 483-491
    • Schaedel, C.1    De Monestrol, I.2    Hjelte, L.3
  • 24
    • 1642529559 scopus 로고    scopus 로고
    • Effect of ambient air pollution on pulmonary exacerbations and lung function in cystic fibrosis
    • Goss CH, Newsom SA, Schildcrout JS, Sheppard L, Kaufman JD. Effect of ambient air pollution on pulmonary exacerbations and lung function in cystic fibrosis. Am J Respir Crit Care Med. 2004; 169 (7): 816-821.
    • (2004) Am J Respir Crit Care Med , vol.169 , Issue.7 , pp. 816-821
    • Goss, C.H.1    Newsom, S.A.2    Schildcrout, J.S.3    Sheppard, L.4    Kaufman, J.D.5
  • 25
    • 77957254912 scopus 로고    scopus 로고
    • Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort
    • EPIC Study Group Participating Clinical Sites
    • Rosenfeld M, Emerson J, McNamara S, et al; EPIC Study Group Participating Clinical Sites. Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort. Pediatr Pulmonol. 2010; 45 (9): 934-944.
    • (2010) Pediatr Pulmonol , vol.45 , Issue.9 , pp. 934-944
    • Rosenfeld, M.1    Emerson, J.2    McNamara, S.3
  • 26
    • 84890445863 scopus 로고    scopus 로고
    • Air trapping and airflow obstruction in newborn cystic fibrosis piglets
    • Adam RJ, Michalski AS, Bauer C, et al. Air trapping and airflow obstruction in newborn cystic fibrosis piglets. Am J Respir Crit Care Med. 2013; 188 (12): 1434-1441.
    • (2013) Am J Respir Crit Care Med , vol.188 , Issue.12 , pp. 1434-1441
    • Adam, R.J.1    Michalski, A.S.2    Bauer, C.3
  • 27
    • 84906089250 scopus 로고    scopus 로고
    • Cystic fibrosis. Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis
    • Hoegger MJ, Fischer AJ, McMenimen JD, et al. Cystic fibrosis. Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis. S cience. 2014; 345 (6198): 818-822.
    • (2014) Science , vol.345 , Issue.6198 , pp. 818-822
    • Hoegger, M.J.1    Fischer, A.J.2    McMenimen, J.D.3
  • 28
    • 79955005042 scopus 로고    scopus 로고
    • Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis
    • Australian Respiratory Early Surveillance Team for Cystic Fibrosis
    • Ranganathan SC, Parsons F, Gangell C, Brennan S, Stick SM, Sly PD; Australian Respiratory Early Surveillance Team for Cystic Fibrosis. Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis. Thorax. 2011; 66 (5): 408-413.
    • (2011) Thorax , vol.66 , Issue.5 , pp. 408-413
    • Ranganathan, S.C.1    Parsons, F.2    Gangell, C.3    Brennan, S.4    Stick, S.M.5    Sly, P.D.6
  • 29
    • 84877114403 scopus 로고    scopus 로고
    • Nutritional care in children with cystic fibrosis: Are our patients becoming better?
    • Gaskin KJ. Nutritional care in children with cystic fibrosis: are our patients becoming better? Eur J Clin Nutr. 2013; 67 (5): 558-564.
    • (2013) Eur J Clin Nutr , vol.67 , Issue.5 , pp. 558-564
    • Gaskin, K.J.1
  • 30
    • 70349831005 scopus 로고    scopus 로고
    • Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis aft er newborn screening
    • Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF)
    • Stick SM, Brennan S, Murray C, et al; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF). Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis aft er newborn screening. J Pediatr. 2009; 155 (5): 623-628.
    • (2009) J Pediatr , vol.155 , Issue.5 , pp. 623-628
    • Stick, S.M.1    Brennan, S.2    Murray, C.3
  • 31
    • 67650712233 scopus 로고    scopus 로고
    • Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening
    • Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST-CF)
    • Sly PD, Brennan S, Gangell C, et al; 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 (2): 146-152.
    • (2009) Am J Respir Crit Care Med , vol.180 , Issue.2 , pp. 146-152
    • Sly, P.D.1    Brennan, S.2    Gangell, C.3
  • 32
    • 48949083438 scopus 로고    scopus 로고
    • Role of high-resolution computed tomography in the detection of early cystic fibrosis lung disease
    • Linnane B, Robinson P, Ranganathan S, Stick S, Murray C. Role of high-resolution computed tomography in the detection of early cystic fibrosis lung disease. Paediatr Respir Rev. 2008; 9 (3): 168-174.
    • (2008) Paediatr Respir Rev , vol.9 , Issue.3 , pp. 168-174
    • Linnane, B.1    Robinson, P.2    Ranganathan, S.3    Stick, S.4    Murray, C.5
  • 33
    • 0036320272 scopus 로고    scopus 로고
    • Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis
    • Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson RL. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. P ediatr Pulmonol. 2002; 34 (2): 91-100.
    • (2002) Pediatr Pulmonol , vol.34 , Issue.2 , pp. 91-100
    • Emerson, J.1    Rosenfeld, M.2    McNamara, S.3    Ramsey, B.4    Gibson, R.L.5
  • 34
    • 12844270608 scopus 로고    scopus 로고
    • Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis
    • Li Z, Kosorok MR, Farrell PM, et al. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. J AMA. 2005; 293 (5): 581-588.
    • (2005) JAMA , vol.293 , Issue.5 , pp. 581-588
    • Li, Z.1    Kosorok, M.R.2    Farrell, P.M.3
  • 35
    • 0035002870 scopus 로고    scopus 로고
    • Clinical outcome aft er early Pseudomonas aeruginosa infection in cystic fibrosis
    • Nixon GM, Armstrong DS, Carzino R, et al. Clinical outcome aft er early Pseudomonas aeruginosa infection in cystic fibrosis. J Pediatr. 2001; 138 (5): 699-704.
    • (2001) J Pediatr , vol.138 , Issue.5 , pp. 699-704
    • Nixon, G.M.1    Armstrong, D.S.2    Carzino, R.3
  • 36
    • 0029075157 scopus 로고
    • The emergence of a highly transmissible lineage of cbl 1 Pseudomonas (Burkholderia) cepacia causing CF centre epidemics in North America and Britain
    • Sun L, Jiang RZ, Steinbach S, et al. The emergence of a highly transmissible lineage of cbl 1 Pseudomonas (Burkholderia) cepacia causing CF centre epidemics in North America and Britain. Nat Med. 1995; 1 (7): 661-666.
    • (1995) Nat Med , vol.1 , Issue.7 , pp. 661-666
    • Sun, L.1    Jiang, R.Z.2    Steinbach, S.3
  • 37
    • 84855864416 scopus 로고    scopus 로고
    • Chronic Stenotrophomonas maltophilia infection and exacerbation outcomes in cystic fibrosis
    • Waters V, Atenafu EG, Salazar JG, et al. Chronic Stenotrophomonas maltophilia infection and exacerbation outcomes in cystic fibrosis. J Cyst Fibros. 2012; 11 (1): 8-13.
    • (2012) J Cyst Fibros , vol.11 , Issue.1 , pp. 8-13
    • Waters, V.1    Atenafu, E.G.2    Salazar, J.G.3
  • 38
    • 84882928602 scopus 로고    scopus 로고
    • Chronic Stenotrophomonas maltophilia infection and mortality or lung transplantation in cystic fibrosis patients
    • Waters V, Atenafu EG, Lu A, Yau Y, Tullis E, Ratjen F. Chronic Stenotrophomonas maltophilia infection and mortality or lung transplantation in cystic fibrosis patients. J Cyst Fibros. 2013; 12 (5): 482-486.
    • (2013) J Cyst Fibros , vol.12 , Issue.5 , pp. 482-486
    • Waters, V.1    Atenafu, E.G.2    Lu, A.3    Yau, Y.4    Tullis, E.5    Ratjen, F.6
  • 39
    • 54049139978 scopus 로고    scopus 로고
    • Persistent methicillin-resistant Staphylococcus aureus and rate of FEV1 decline in cystic fibrosis
    • Dasenbrook EC, Merlo CA, Diener-West M, Lechtzin N, Boyle MP. Persistent methicillin-resistant Staphylococcus aureus and rate of FEV1 decline in cystic fibrosis. Am J Respir Crit Care Med. 2008; 178 (8): 814-821.
    • (2008) Am J Respir Crit Care Med , vol.178 , Issue.8 , pp. 814-821
    • Dasenbrook, E.C.1    Merlo, C.A.2    Diener-West, M.3    Lechtzin, N.4    Boyle, M.P.5
  • 40
    • 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 (2): 117-123.
    • (2010) J Cyst Fibros , vol.9 , Issue.2 , pp. 117-123
    • Esther, C.R.1    Esserman, D.A.2    Gilligan, P.3    Kerr, A.4    Noone, P.G.5
  • 41
    • 84867085069 scopus 로고    scopus 로고
    • The cystic fibrosis airway microbiome
    • Rabin HR, Surette MG. The cystic fibrosis airway microbiome. Curr Opin Pulm Med. 2012; 18 (6): 622-627.
    • (2012) Curr Opin Pulm Med , vol.18 , Issue.6 , pp. 622-627
    • Rabin, H.R.1    Surette, M.G.2
  • 42
    • 84902366435 scopus 로고    scopus 로고
    • The clinical significance of the gut microbiota in cystic fibrosis and the potential for dietary therapies
    • Li L, Somerset S. The clinical significance of the gut microbiota in cystic fibrosis and the potential for dietary therapies. Clin Nutr. 2014; 33 (4): 571-580.
    • (2014) Clin Nutr , vol.33 , Issue.4 , pp. 571-580
    • Li, L.1    Somerset, S.2
  • 43
    • 84856094959 scopus 로고    scopus 로고
    • Bacterial host interactions in cystic fibrosis
    • Callaghan M, McClean S. Bacterial host interactions in cystic fibrosis. Curr Opin Microbiol. 2012; 15 (1): 71-77.
    • (2012) Curr Opin Microbiol , vol.15 , Issue.1 , pp. 71-77
    • Callaghan, M.1    McClean, S.2
  • 44
    • 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 (8): 918-951.
    • (2003) Am J Respir Crit Care Med , vol.168 , Issue.8 , pp. 918-951
    • Gibson, R.L.1    Burns, J.L.2    Ramsey, B.W.3
  • 45
    • 84887488043 scopus 로고    scopus 로고
    • Airway inflammation in cystic fibrosis: Molecular mechanisms and clinical implications
    • Cohen-Cymberknoh M, Kerem E, Ferkol T, Elizur A. Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications. Thorax. 2013; 68 (12): 1157-1162.
    • (2013) Thorax , vol.68 , Issue.12 , pp. 1157-1162
    • Cohen-Cymberknoh, M.1    Kerem, E.2    Ferkol, T.3    Elizur, A.4
  • 46
    • 78650404224 scopus 로고    scopus 로고
    • Modifier genes in Mendelian disorders: The example of cystic fibrosis
    • Cutting GR. Modifier genes in Mendelian disorders: the example of cystic fibrosis. A nn N Y Acad Sci. 2010; 1214: 57-69.
    • (2010) Ann N y Acad Sci , vol.1214 , pp. 57-69
    • Cutting, G.R.1
  • 47
    • 40549139451 scopus 로고    scopus 로고
    • Gene modifiers in cystic fibrosis
    • Accurso FJ, Sontag MK. Gene modifiers in cystic fibrosis. J Clin Invest. 2008; 118 (3): 839-841.
    • (2008) J Clin Invest , vol.118 , Issue.3 , pp. 839-841
    • Accurso, F.J.1    Sontag, M.K.2
  • 48
    • 25844491194 scopus 로고    scopus 로고
    • Genetic modifiers of lung disease in cystic fibrosis
    • Gene Modifier Study Group
    • Drumm ML, Konstan MW, Schluchter MD, et al; Gene Modifier Study Group. Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med. 2005; 353 (14): 1443-1453.
    • (2005) N Engl J Med , vol.353 , Issue.14 , pp. 1443-1453
    • Drumm, M.L.1    Konstan, M.W.2    Schluchter, M.D.3
  • 49
    • 84878000440 scopus 로고    scopus 로고
    • Risk factors for bronchiectasis in children with cystic fibrosis
    • AREST CF Investigators
    • Sly PD, Gangell CL, Chen L, et al; AREST CF Investigators. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013; 368 (21): 1963-1970.
    • (2013) N Engl J Med , vol.368 , Issue.21 , pp. 1963-1970
    • Sly, P.D.1    Gangell, C.L.2    Chen, L.3
  • 50
    • 39449133330 scopus 로고    scopus 로고
    • Airway inflammation in cystic fibrosis
    • Elizur A, Cannon CL, Ferkol TW. Airway inflammation in cystic fibrosis. C hest. 2008; 133 (2): 489-495.
    • (2008) Chest , vol.133 , Issue.2 , pp. 489-495
    • Elizur, A.1    Cannon, C.L.2    Ferkol, T.W.3
  • 52
    • 70350446684 scopus 로고    scopus 로고
    • Cystic fibrosis pulmonary guidelines: Treatment of pulmonary exacerbations
    • Clinical Practice Guidelines for Pulmonary Therapies Committee
    • Flume PA, Mogayzel PJ Jr. Robinson KA, et al; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med. 2009; 180 (9): 802-808.
    • (2009) Am J Respir Crit Care Med , vol.180 , Issue.9 , pp. 802-808
    • Flume, P.A.1    Mogayzel, P.J.2    Robinson, K.A.3
  • 53
    • 85047686252 scopus 로고    scopus 로고
    • Cystic fibrosis pulmonary guidelines: Pulmonary complications: Hemoptysis and pneumothorax
    • Clinical Practice Guidelines for Pulmonary Therapies Committee; Cystic Fibrosis Foundation Pulmonary Therapies Committee
    • Flume PA, Mogayzel PJ Jr. Robinson KA, Rosenblatt RL, Quittell L, Marshall BC; Clinical Practice Guidelines for Pulmonary Therapies Committee; Cystic Fibrosis Foundation Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med. 2010; 182 (3): 298-306.
    • (2010) Am J Respir Crit Care Med , vol.182 , Issue.3 , pp. 298-306
    • Flume, P.A.1    Mogayzel, P.J.2    Robinson, K.A.3    Rosenblatt, R.L.4    Quittell, L.5    Marshall, B.C.6
  • 55
    • 0032718969 scopus 로고    scopus 로고
    • Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis
    • Rosenfeld M, Emerson J, Accurso F, et al. Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis. P ediatr Pulmonol. 1999; 28 (5): 321-328.
    • (1999) Pediatr Pulmonol , vol.28 , Issue.5 , pp. 321-328
    • Rosenfeld, M.1    Emerson, J.2    Accurso, F.3
  • 56
    • 34247589696 scopus 로고    scopus 로고
    • Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis
    • Davis SD, Fordham LA, Brody AS, et al. Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am J Respir Crit Care Med. 2007; 175 (9): 943-950.
    • (2007) Am J Respir Crit Care Med , vol.175 , Issue.9 , pp. 943-950
    • Davis, S.D.1    Fordham, L.A.2    Brody, A.S.3
  • 57
    • 79960151730 scopus 로고    scopus 로고
    • Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: A randomized trial
    • ACFBAL Study Investigators
    • Wainwright CE, Vidmar S, Armstrong DS, et al; ACFBAL Study Investigators. Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial. JAMA. 2011; 306 (2): 163-171.
    • (2011) JAMA , vol.306 , Issue.2 , pp. 163-171
    • Wainwright, C.E.1    Vidmar, S.2    Armstrong, D.S.3
  • 58
    • 0026520359 scopus 로고
    • Prediction of mortality in patients with cystic fibrosis
    • Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992; 326 (18): 1187-1191.
    • (1992) N Engl J Med , vol.326 , Issue.18 , pp. 1187-1191
    • Kerem, E.1    Reisman, J.2    Corey, M.3    Canny, G.J.4    Levison, H.5
  • 60
    • 84862776813 scopus 로고    scopus 로고
    • Progression of early structural lung disease in young children with cystic fibrosis assessed using CT
    • AREST CF
    • Mott LS, Park J, Murray CP, et al; AREST CF. Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax. 2012; 67 (6): 509-516.
    • (2012) Thorax , vol.67 , Issue.6 , pp. 509-516
    • Mott, L.S.1    Park, J.2    Murray, C.P.3
  • 61
    • 77449133012 scopus 로고    scopus 로고
    • Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis
    • Cystic Fibrosis Foundation
    • Borowitz D, Robinson KA, Rosenfeld M, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009; 155 (suppl 6): S73-S93.
    • (2009) J Pediatr , vol.155 , pp. S73-S93
    • Borowitz, D.1    Robinson, K.A.2    Rosenfeld, M.3
  • 62
    • 84880887459 scopus 로고    scopus 로고
    • An official American Thoracic Society workshop report: Optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age
    • American Thoracic Society Assembly on Pediatrics Working Group on Infant and Preschool Lung Function Testing
    • Rosenfeld M, Allen J, Arets BH, et al; American Thoracic Society Assembly on Pediatrics Working Group on Infant and Preschool Lung Function Testing. An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age. Ann Am Thorac Soc. 2013; 10 (2): S1-S11.
    • (2013) Ann Am Thorac Soc , vol.10 , Issue.2 , pp. S1-S11
    • Rosenfeld, M.1    Allen, J.2    Arets, B.H.3
  • 63
    • 39049084491 scopus 로고    scopus 로고
    • Multiplebreath inert gas washout and spirometry versus structural lung disease in cystic fibrosis
    • Gustafsson PM, De Jong PA, Tiddens HA, Lindblad A. Multiplebreath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2008; 63 (2): 129-134.
    • (2008) Thorax , vol.63 , Issue.2 , pp. 129-134
    • Gustafsson, P.M.1    De Jong, P.A.2    Tiddens, H.A.3    Lindblad, A.4
  • 64
    • 79956360940 scopus 로고    scopus 로고
    • Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF
    • London Cystic Fibrosis Collaboration
    • Owens CM, Aurora P, Stanojevic S, et al; London Cystic Fibrosis Collaboration. Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF. Thorax. 2011; 66 (6): 481-488.
    • (2011) Thorax , vol.66 , Issue.6 , pp. 481-488
    • Owens, C.M.1    Aurora, P.2    Stanojevic, S.3
  • 65
    • 0027965872 scopus 로고
    • Critical evaluation of three chest radiograph scores in cystic fibrosis
    • Sawyer SM, Carlin JB, DeCampo M, Bowes G. Critical evaluation of three chest radiograph scores in cystic fibrosis. Thorax. 1994; 49 (9): 863-866.
    • (1994) Thorax , vol.49 , Issue.9 , pp. 863-866
    • Sawyer, S.M.1    Carlin, J.B.2    Decampo, M.3    Bowes, G.4
  • 66
    • 36248997250 scopus 로고    scopus 로고
    • Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: Comparison to thin-section CT and chest X-ray
    • Puderbach M, Eichinger M, Haeselbarth J, et al. Assessment of morphological MRI for pulmonary changes in cystic fibrosis (CF) patients: comparison to thin-section CT and chest X-ray. Invest Radiol. 2007; 42 (10): 715-725.
    • (2007) Invest Radiol , vol.42 , Issue.10 , pp. 715-725
    • Puderbach, M.1    Eichinger, M.2    Haeselbarth, J.3
  • 67
    • 0034821607 scopus 로고    scopus 로고
    • Defining a pulmonary exacerbation in cystic fibrosis
    • Rosenfeld M, Emerson J, Williams-Warren J, et al. Defining a pulmonary exacerbation in cystic fibrosis. J Pediatr. 2001; 139 (3): 359-365.
    • (2001) J Pediatr , vol.139 , Issue.3 , pp. 359-365
    • Rosenfeld, M.1    Emerson, J.2    Williams-Warren, J.3
  • 68
    • 0028129568 scopus 로고
    • Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
    • The Pulmozyme Study Group
    • Fuchs HJ, Borowitz DS, Christiansen DH, et al; The Pulmozyme Study Group. 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 (10): 637-642.
    • (1994) N Engl J Med , vol.331 , Issue.10 , pp. 637-642
    • Fuchs, H.J.1    Borowitz, D.S.2    Christiansen, D.H.3
  • 69
    • 33344468629 scopus 로고    scopus 로고
    • Cystic fibrosis pulmonary exacerbations
    • Ferkol T, Rosenfeld M, Milla CE. Cystic fibrosis pulmonary exacerbations . J Pediatr. 2006; 148 (2): 259-264.
    • (2006) J Pediatr , vol.148 , Issue.2 , pp. 259-264
    • Ferkol, T.1    Rosenfeld, M.2    Milla, C.E.3
  • 71
    • 84868539521 scopus 로고    scopus 로고
    • Extending the course of intravenous antibiotics in adult patients with cystic fibrosis with acute pulmonary exacerbations
    • Sequeiros IM, Jarad NA. Extending the course of intravenous antibiotics in adult patients with cystic fibrosis with acute pulmonary exacerbations. Chron Respir Dis. 2012; 9 (4): 213-220.
    • (2012) Chron Respir Dis , vol.9 , Issue.4 , pp. 213-220
    • Sequeiros, I.M.1    Jarad, N.A.2
  • 72
    • 77957296044 scopus 로고    scopus 로고
    • Assessing time to pulmonary function benefit following antibiotic treatment of acute cystic fibrosis exacerbations
    • VanDevanter DR, O'Riordan MA, Blumer JL, Konstan MW. Assessing time to pulmonary function benefit following antibiotic treatment of acute cystic fibrosis exacerbations. Respir Res. 2010; 11: 137-142.
    • (2010) Respir Res , vol.11 , pp. 137-142
    • Van Devanter, D.R.1    O'Riordan, M.A.2    Blumer, J.L.3    Konstan, M.W.4
  • 73
    • 84866841728 scopus 로고    scopus 로고
    • The adult cystic fibrosis airway microbiota is stable over time and infection type, and highly resilient to antibiotic treatment of exacerbations
    • Fodor AA, Klem ER, Gilpin DF, et al. The adult cystic fibrosis airway microbiota is stable over time and infection type, and highly resilient to antibiotic treatment of exacerbations. PLoS ONE. 2012; 7 (9): e45001.
    • (2012) PLoS ONE , vol.7 , Issue.9 , pp. e45001
    • Fodor, A.A.1    Klem, E.R.2    Gilpin, D.F.3
  • 74
    • 12144288633 scopus 로고    scopus 로고
    • Adult cystic fibrosis exacerbations and new strains of P seudomonas aeruginosa
    • Aaron SD, Ramotar K, Ferris W, et al. Adult cystic fibrosis exacerbations and new strains of P seudomonas aeruginosa. Am J Respir Crit Care Med. 2004; 169 (7): 811-815.
    • (2004) Am J Respir Crit Care Med , vol.169 , Issue.7 , pp. 811-815
    • Aaron, S.D.1    Ramotar, K.2    Ferris, W.3
  • 75
    • 23644439540 scopus 로고    scopus 로고
    • Eradication of early Pseudomonas aeruginosa infection
    • Høiby N, Frederiksen B, Pressler T. Eradication of early Pseudomonas aeruginosa infection. J Cyst Fibros. 2005; 4(suppl 2): 49-54.
    • (2005) J Cyst Fibros , vol.4 , pp. 49-54
    • Høiby, N.1    Frederiksen, B.2    Pressler, T.3
  • 76
    • 0030995361 scopus 로고    scopus 로고
    • Antibiotic treatment of initial colonization with Pseudomonas aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis
    • Frederiksen B, Koch C, Høiby N. Antibiotic treatment of initial colonization with Pseudomonas aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis. P ediatr Pulmonol. 1997; 23 (5): 330-335.
    • (1997) Pediatr Pulmonol , vol.23 , Issue.5 , pp. 330-335
    • Frederiksen, B.1    Koch, C.2    Høiby, N.3
  • 77
    • 0033531143 scopus 로고    scopus 로고
    • Intermittent administration of inhaled tobramycin in patients with cystic fibrosis
    • Cystic Fibrosis Inhaled Tobramycin Study Group
    • Ramsey BW, Pepe MS, Quan JM, et al; Cystic Fibrosis Inhaled Tobramycin Study Group. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. N Engl J Med. 1999; 340 (1): 23-30.
    • (1999) N Engl J Med , vol.340 , Issue.1 , pp. 23-30
    • Ramsey, B.W.1    Pepe, M.S.2    Quan, J.M.3
  • 78
    • 84877877188 scopus 로고    scopus 로고
    • Inhaled antibiotics for the treatment of chronic bronchopulmonary Pseudomonas aeruginosa infection in cystic fibrosis: Systematic review of randomised controlled trials
    • Maíz L, Girón RM, Olveira C, et al. Inhaled antibiotics for the treatment of chronic bronchopulmonary Pseudomonas aeruginosa infection in cystic fibrosis: systematic review of randomised controlled trials. Expert Opin Pharmacother. 2013; 14 (9): 1135-1149.
    • (2013) Expert Opin Pharmacother , vol.14 , Issue.9 , pp. 1135-1149
    • Maíz, L.1    Girón, R.M.2    Olveira, C.3
  • 79
    • 84898730884 scopus 로고    scopus 로고
    • Inhaled antibiotics for lower airway infections
    • Quon BS, Goss CH, Ramsey BW. Inhaled antibiotics for lower airway infections. Ann Am Thorac Soc. 2014; 11 (3): 425-434.
    • (2014) Ann Am Thorac Soc , vol.11 , Issue.3 , pp. 425-434
    • Quon, B.S.1    Goss, C.H.2    Ramsey, B.W.3
  • 80
    • 84877153770 scopus 로고    scopus 로고
    • Update in cystic fibrosis 2012
    • Goss CH, Ratjen F. Update in cystic fibrosis 2012. Am J Respir Crit Care Med. 2013; 187 (9): 915-919.
    • (2013) Am J Respir Crit Care Med , vol.187 , Issue.9 , pp. 915-919
    • Goss, C.H.1    Ratjen, F.2
  • 81
    • 18944382506 scopus 로고    scopus 로고
    • Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis
    • Main E, Prasad A, Schans C. Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. Cochrane Database Syst Rev. 2005;(1): CD002011.
    • (2005) Cochrane Database Syst Rev , Issue.1 , pp. CD002011
    • Main, E.1    Prasad, A.2    Schans, C.3
  • 82
    • 0018852847 scopus 로고
    • Short-term effects of chest physiotherapy on pulmonary function in children with cystic fibrosis
    • Weller PH, Bush E, Preece MA, Matthew DJ. Short-term effects of chest physiotherapy on pulmonary function in children with cystic fibrosis. R espiration. 1980; 40 (1): 53-56.
    • (1980) Respiration , vol.40 , Issue.1 , pp. 53-56
    • Weller, P.H.1    Bush, E.2    Preece, M.A.3    Matthew, D.J.4
  • 83
    • 0035666058 scopus 로고    scopus 로고
    • A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities
    • Pulmozyme Early Intervention Trial Study Group
    • Quan JM, Tiddens HA, Sy JP, et al; Pulmozyme Early Intervention Trial Study Group. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr. 2001; 139 (6): 813-820.
    • (2001) J Pediatr , vol.139 , Issue.6 , pp. 813-820
    • Quan, J.M.1    Tiddens, H.A.2    Sy, J.P.3
  • 84
    • 79955909454 scopus 로고    scopus 로고
    • Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis
    • Scientific Advisory Group and Investigators and Coordinators of Epidemiologic Study of Cystic Fibrosis
    • Konstan MW, Wagener JS, Pasta DJ, et al; Scientific Advisory Group and Investigators and Coordinators of Epidemiologic Study of Cystic Fibrosis. Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis. P ediatr Pulmonol. 2011; 46 (6): 545-553.
    • (2011) Pediatr Pulmonol , vol.46 , Issue.6 , pp. 545-553
    • Konstan, M.W.1    Wagener, J.S.2    Pasta, D.J.3
  • 85
    • 0032987387 scopus 로고    scopus 로고
    • Systematic review of N-acetylcysteine in cystic fibrosis
    • Duijvestijn YC, Brand PL. Systematic review of N-acetylcysteine in cystic fibrosis. A cta Paediatr. 1999; 88 (1): 38-41.
    • (1999) Acta Paediatr , vol.88 , Issue.1 , pp. 38-41
    • Duijvestijn, Y.C.1    Brand, P.L.2
  • 87
    • 30944466084 scopus 로고    scopus 로고
    • A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis
    • National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group
    • Elkins MR, Robinson M, Rose BR, et al; National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. 2006; 354 (3): 229-240.
    • (2006) N Engl J Med , vol.354 , Issue.3 , pp. 229-240
    • Elkins, M.R.1    Robinson, M.2    Rose, B.R.3
  • 88
    • 84861892411 scopus 로고    scopus 로고
    • Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: The ISIS randomized controlled trial
    • ISIS Study Group
    • Rosenfeld M, Ratjen F, Brumback L, et al; ISIS Study Group. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA. 2012; 307 (21): 2269-2277.
    • (2012) JAMA , vol.307 , Issue.21 , pp. 2269-2277
    • Rosenfeld, M.1    Ratjen, F.2    Brumback, L.3
  • 89
    • 44649166466 scopus 로고    scopus 로고
    • Inhaled mannitol improves lung function in cystic fibrosis
    • Jaques A, Daviskas E, Turton JA, et al. Inhaled mannitol improves lung function in cystic fibrosis. C hest. 2008; 133 (6): 1388-1396.
    • (2008) Chest , vol.133 , Issue.6 , pp. 1388-1396
    • Jaques, A.1    Daviskas, E.2    Turton, J.A.3
  • 90
    • 77950787429 scopus 로고    scopus 로고
    • Inhaled mannitol improves the hydration and surface properties of sputum in patients with cystic fibrosis
    • Daviskas E, Anderson SD, Jaques A, Charlton B. Inhaled mannitol improves the hydration and surface properties of sputum in patients with cystic fibrosis. C hest. 2010; 137 (4): 861-868.
    • (2010) Chest , vol.137 , Issue.4 , pp. 861-868
    • Daviskas, E.1    Anderson, S.D.2    Jaques, A.3    Charlton, B.4
  • 91
    • 34547949974 scopus 로고    scopus 로고
    • Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis
    • Cystic Fibrosis Therapeutics Development Network and the Inspire 08-103 Working Group
    • Deterding RR, Lavange LM, Engels JM, et al; Cystic Fibrosis Therapeutics Development Network and the Inspire 08-103 Working Group. Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis. Am J Respir Crit Care Med. 2007; 176 (4): 362-369.
    • (2007) Am J Respir Crit Care Med , vol.176 , Issue.4 , pp. 362-369
    • Deterding, R.R.1    Lavange, L.M.2    Engels, J.M.3
  • 92
    • 84869090967 scopus 로고    scopus 로고
    • Long term effects of denufosol tetrasodium in patients with cystic fibrosis
    • TIGER-2 Study Investigator Group
    • Ratjen F, Durham T, Navratil T, et al; TIGER-2 Study Investigator Group. Long term effects of denufosol tetrasodium in patients with cystic fibrosis. J Cyst Fibros. 2012; 11 (6): 539-549.
    • (2012) J Cyst Fibros , vol.11 , Issue.6 , pp. 539-549
    • Ratjen, F.1    Durham, T.2    Navratil, T.3
  • 93
    • 84884213627 scopus 로고    scopus 로고
    • Pitfalls of drug development: Lessons learned from trials of denufosol in cystic fibrosis
    • Moss RB. Pitfalls of drug development: lessons learned from trials of denufosol in cystic fibrosis. J Pediatr. 2013; 162 (4): 676-680.
    • (2013) J Pediatr , vol.162 , Issue.4 , pp. 676-680
    • Moss, R.B.1
  • 94
    • 84884282606 scopus 로고    scopus 로고
    • ENaC inhibitors and airway re-hydration in cystic fibrosis: State of the art
    • Althaus M. ENaC inhibitors and airway re-hydration in cystic fibrosis: state of the art. Curr Mol Pharmacol. 2013; 6 (1): 3-12.
    • (2013) Curr Mol Pharmacol , vol.6 , Issue.1 , pp. 3-12
    • Althaus, M.1
  • 95
    • 28344451370 scopus 로고    scopus 로고
    • Lower airway inflammation in infants with cystic fibrosis detected by newborn screening
    • Armstrong DS, Hook SM, Jamsen KM, et al. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr Pulmonol. 2005; 40 (6): 500-510.
    • (2005) Pediatr Pulmonol , vol.40 , Issue.6 , pp. 500-510
    • Armstrong, D.S.1    Hook, S.M.2    Jamsen, K.M.3
  • 96
    • 0037027948 scopus 로고    scopus 로고
    • Pharmacological approaches for the discovery and development of new anti-inflammatory agents for the treatment of cystic fibrosis
    • Konstan MW, Davis PB. Pharmacological approaches for the discovery and development of new anti-inflammatory agents for the treatment of cystic fibrosis. A dv Drug Deliv Rev. 2002; 54 (11): 1409-1423.
    • (2002) Adv Drug Deliv Rev , vol.54 , Issue.11 , pp. 1409-1423
    • Konstan, M.W.1    Davis, P.B.2
  • 97
    • 84891719351 scopus 로고    scopus 로고
    • Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis
    • Lands LC, Stanojevic S. Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis. C ochrane Database Syst Rev. 2013; 6: CD001505.
    • (2013) Cochrane Database Syst Rev , vol.6 , pp. CD001505
    • Lands, L.C.1    Stanojevic, S.2
  • 98
    • 0028914667 scopus 로고
    • Effect of high-dose ibuprofen in patients with cystic fibrosis
    • Konstan MW, Byard PJ, Hoppel CL, Davis PB. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med. 1995; 332 (13): 848-854.
    • (1995) N Engl J Med , vol.332 , Issue.13 , pp. 848-854
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3    Davis, P.B.4
  • 99
    • 84891689243 scopus 로고    scopus 로고
    • Oral steroids for long-term use in cystic fibrosis
    • Cheng K, Ashby D, Smyth RL. Oral steroids for long-term use in cystic fibrosis. C ochrane Database Syst Rev. 2013; 6: CD000407.
    • (2013) Cochrane Database Syst Rev , vol.6 , pp. CD000407
    • Cheng, K.1    Ashby, D.2    Smyth, R.L.3
  • 100
    • 56049085357 scopus 로고    scopus 로고
    • Relationship between inhaled corticosteroid therapy and rate of lung function decline in children with cystic fibrosis
    • Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis
    • Ren CL, Pasta DJ, Rasouliyan L, Wagener JS, Konstan MW, Morgan WJ; Scientific Advisory Group and the Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. Relationship between inhaled corticosteroid therapy and rate of lung function decline in children with cystic fibrosis. J Pediatr. 2008; 153 (6): 746-751.
    • (2008) J Pediatr , vol.153 , Issue.6 , pp. 746-751
    • Ren, C.L.1    Pasta, D.J.2    Rasouliyan, L.3    Wagener, J.S.4    Konstan, M.W.5    Morgan, W.J.6
  • 101
    • 0141816759 scopus 로고    scopus 로고
    • Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: A randomized controlled trial
    • Macrolide Study Group
    • Saiman L, Marshall BC, Mayer-Hamblett N, et al; Macrolide Study Group. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2003; 290 (13): 1749-1756.
    • (2003) JAMA , vol.290 , Issue.13 , pp. 1749-1756
    • Saiman, L.1    Marshall, B.C.2    Mayer-Hamblett, N.3
  • 102
    • 77951887288 scopus 로고    scopus 로고
    • Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: A randomized controlled trial
    • AZ0004 Azithromycin Study Group
    • Saiman L, Anstead M, Mayer-Hamblett N, et al; AZ0004 Azithromycin Study Group. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2010; 303 (17): 1707-1715.
    • (2010) JAMA , vol.303 , Issue.17 , pp. 1707-1715
    • Saiman, L.1    Anstead, M.2    Mayer-Hamblett, N.3
  • 103
    • 84871946692 scopus 로고    scopus 로고
    • Cystic fibrosis therapeutics: The road ahead
    • Hoffman LR, Ramsey BW. Cystic fibrosis therapeutics: the road ahead. Chest. 2013; 143 (1): 207-213.
    • (2013) Chest , vol.143 , Issue.1 , pp. 207-213
    • Hoffman, L.R.1    Ramsey, B.W.2
  • 104
    • 0041856071 scopus 로고    scopus 로고
    • Current status of gene therapy for cystic fibrosis pulmonary disease
    • Kennedy MJ. Current status of gene therapy for cystic fibrosis pulmonary disease. Am J Respir Med. 2002; 1 (5): 349-360.
    • (2002) Am J Respir Med , vol.1 , Issue.5 , pp. 349-360
    • Kennedy, M.J.1
  • 105
    • 59349091561 scopus 로고    scopus 로고
    • Chloride channels as drug targets
    • Verkman AS, Galietta LJ. Chloride channels as drug targets. Nat Rev Drug Discov. 2009; 8 (2): 153-171.
    • (2009) Nat Rev Drug Discov , vol.8 , Issue.2 , pp. 153-171
    • Verkman, A.S.1    Galietta, L.J.2
  • 106
    • 84939233665 scopus 로고    scopus 로고
    • Accessed June 19
    • Drug development pipeline. Cystic Fibrosis Foundation website. http://www.cff.org/research/drugdevelopmentpipeline.Accessed June 19, 2014.
    • (2014) Drug Development Pipeline
  • 107
    • 84904016376 scopus 로고    scopus 로고
    • Ataluren for the treatment of nonsensemutation cystic fibrosis: A randomised, double-blind, placebocontrolled phase 3 trial
    • Cystic Fibrosis Ataluren Study Group
    • Kerem E, Konstan MW, De Boeck K, et al; Cystic Fibrosis Ataluren Study Group. Ataluren for the treatment of nonsensemutation cystic fibrosis: a randomised, double-blind, placebocontrolled phase 3 trial. Lancet Respir Med. 2014; 2 (7): 539-547.
    • (2014) Lancet Respir Med , vol.2 , Issue.7 , pp. 539-547
    • Kerem, E.1    Konstan, M.W.2    De Boeck, K.3
  • 108
    • 80455162465 scopus 로고    scopus 로고
    • A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
    • VX08-770-102 Study Group
    • Ramsey BW, Davies J, McElvaney NG, 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(18): 1663-1672.
    • (2011) N Engl J Med , vol.365 , Issue.18 , pp. 1663-1672
    • Ramsey, B.W.1    Davies, J.2    McElvaney, N.G.3
  • 109
    • 84939228710 scopus 로고    scopus 로고
    • Accessed August 9
    • KALYDECO. US Food and Drug Administration website. http://www.accessdata.fda.gov/drugsatfda-docs/label/2014/203188s008lbl.pdf. Accessed August 9, 2014.
    • (2014) Kalydeco
  • 110
    • 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, 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 (1): 12-18.
    • (2012) Thorax , vol.67 , Issue.1 , pp. 12-18
    • Clancy, J.P.1    Rowe, S.M.2    Accurso, F.J.3
  • 111
    • 84907223425 scopus 로고    scopus 로고
    • Potentiator ivacaft or abrogates pharmacological correction of D F508 CFTR in cystic fibrosis
    • Cholon DM, Quinney NL, Fulcher ML, et al. Potentiator ivacaft or abrogates pharmacological correction of D F508 CFTR in cystic fibrosis. S ci Transl Med. 2014; 6 (246): 246ra96.
    • (2014) Sci Transl Med , vol.6 , Issue.246 , pp. 246ra96
    • Cholon, D.M.1    Quinney, N.L.2    Fulcher, M.L.3
  • 112
    • 84907211027 scopus 로고    scopus 로고
    • Some gating potentiators, including VX-770, diminish D F508-CFTR functional expression
    • Veit G, Avramescu RG, Perdomo D, et al. Some gating potentiators, including VX-770, diminish D F508-CFTR functional expression . Sci Transl Med. 2014; 6 (246): 246ra97.
    • (2014) Sci Transl Med , vol.6 , Issue.246 , pp. 246ra97
    • Veit, G.1    Avramescu, R.G.2    Perdomo, D.3
  • 113
    • 84899046260 scopus 로고    scopus 로고
    • Cystic fibrosis: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases
    • Pittman JE, Cutting G, Davis SD, Ferkol T, Boucher R. Cystic fibrosis: NHLBI Workshop on the Primary Prevention of Chronic Lung Diseases. Ann Am Thorac Soc. 2014; 11(suppl 3): S161-S168.
    • (2014) Ann Am Thorac Soc , vol.11 , pp. S161-S168
    • Pittman, J.E.1    Cutting, G.2    Davis, S.D.3    Ferkol, T.4    Boucher, R.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.