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Volumn 16, Issue 4, 1999, Pages 495-509

Respiratory disease in cystic fibrosis: From pathophysiology to therapy;Atteinte respiratoire au cours de la mucoviscidose: De la physiopathologie a la therapeutique

Author keywords

CFTR; Cystic fibrosis; Physiopathology; Therapeutics

Indexed keywords

ARTICLE; CYSTIC FIBROSIS; DISEASE ASSOCIATION; GENE EXPRESSION; GENE MUTATION; GENE TRANSFER; LUNG INFECTION; PATHOPHYSIOLOGY; PNEUMONIA; RESPIRATORY TRACT DISEASE; SURVIVAL;

EID: 0032879557     PISSN: 07618425     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article
Times cited : (4)

References (143)
  • 1
    • 0026316543 scopus 로고
    • Cystic fibrosis: Current survival and population estimates to the year 2000
    • ELBORN JS, SHALE DJ, BRITTON JR : Cystic fibrosis: Current survival and population estimates to the year 2000. Thorax 1991;46:881-5
    • (1991) Thorax , vol.46 , pp. 881-885
    • Elborn, J.S.1    Shale, D.J.2    Britton, J.R.3
  • 2
    • 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;245:1073-80.
    • (1989) Science , vol.245 , pp. 1073-1080
    • Kerem, B.1    Rommens, J.M.2    Buchanan, J.A.3
  • 3
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene : Cloning and characterization of complementary DNA
    • RIORDAN JR, ROMMENS JM, KEREM B et al. : Identification of the cystic fibrosis gene : cloning and characterization of complementary DNA. Science 1989;245:1066-73.
    • (1989) Science , vol.245 , pp. 1066-1073
    • Riordan, J.R.1    Rommens, J.M.2    Kerem, B.3
  • 4
    • 0025760318 scopus 로고
    • Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator (CFTR) gene
    • ZIELENSKI J, ROZMAHEL R, BOZON D et al. : Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Genomics 1991;10:214-28.
    • (1991) Genomics , vol.10 , pp. 214-228
    • Zielenski, J.1    Rozmahel, R.2    Bozon, D.3
  • 6
    • 0030956949 scopus 로고    scopus 로고
    • Nasal potential difference in cystic fibrosis patients presenting borderline sweat test
    • DELMARCO A, PRADAL U, CABRINI G, BONIZZATO A, MASTELLA G : Nasal potential difference in cystic fibrosis patients presenting borderline sweat test. Eur Respir J 1997;10:1145-9
    • (1997) Eur Respir J , vol.10 , pp. 1145-1149
    • Delmarco, A.1    Pradal, U.2    Cabrini, G.3    Bonizzato, A.4    Mastella, G.5
  • 7
    • 0030757486 scopus 로고    scopus 로고
    • A new device for in vivo measurement of nasal transepithelial potential difference in cystic fibrosis patients and normal subjects
    • DUPERREX O, BERCLAZ PY, BERTRAND D, LACROIX JS, POCHON N, BELLI D, ROCHAT : A new device for in vivo measurement of nasal transepithelial potential difference in cystic fibrosis patients and normal subjects. Eur Respir J 1997;10:1631-6.
    • (1997) Eur Respir J , vol.10 , pp. 1631-1636
    • Duperrex, O.1    Berclaz, P.Y.2    Bertrand, D.3    Lacroix, J.S.4    Pochon, N.5    Belli, D.6    Rochat7
  • 8
    • 0031006817 scopus 로고    scopus 로고
    • Conventional and modified nasal potential-difference measurement in cystic fibrosis
    • HOFMANN T, BOHMER O, HULS G et al : Conventional and modified nasal potential-difference measurement in cystic fibrosis. Am J Respir Crit Care Med 1997;155:1908-13.
    • (1997) Am J Respir Crit Care Med , vol.155 , pp. 1908-1913
    • Hofmann, T.1    Bohmer, O.2    Huls, G.3
  • 10
    • 0026954365 scopus 로고
    • Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium
    • PUCHELLE E, GAILLARD D, PLOTON D et al. : Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium. Am J Respir Cell Mol Biol 1992;7:485-91.
    • (1992) Am J Respir Cell Mol Biol , vol.7 , pp. 485-491
    • Puchelle, E.1    Gaillard, D.2    Ploton, D.3
  • 11
    • 0027402791 scopus 로고
    • Localization of the cystic fibrosis transmembrane conductance regulator in airway secretory glands
    • JACQUOT J, PUCHELLE E, HINNRASKY J : Localization of the cystic fibrosis transmembrane conductance regulator in airway secretory glands. Eur Respir J 1993;6:169-76.
    • (1993) Eur Respir J , vol.6 , pp. 169-176
    • Jacquot, J.1    Puchelle, E.2    Hinnrasky, J.3
  • 13
    • 0025349031 scopus 로고
    • Cystic fibrosis: A disease in electrolyte transport
    • QUINTON PM : Cystic fibrosis: a disease in electrolyte transport. FASEB J 1990;4:2709-17.
    • (1990) FASEB J , vol.4 , pp. 2709-2717
    • Quinton, P.M.1
  • 14
    • 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. Cell 1993;73:1251-4.
    • (1993) Cell , vol.73 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 15
    • 0028940005 scopus 로고
    • Functions of the cystic fibrosis transmembrane conductance regulator protein
    • FRIZZELL RA : Functions of the cystic fibrosis transmembrane conductance regulator protein. Am J Respir Crit Care Med 1995;151:S54-S8.
    • (1995) Am J Respir Crit Care Med , vol.151
    • Frizzell, R.A.1
  • 16
    • 0026687233 scopus 로고
    • - channels by protein kinase a corrected by insertion of CFTR
    • - channels by protein kinase A corrected by insertion of CFTR. Science 1992;358:581-4.
    • (1992) Science , vol.358 , pp. 581-584
    • Egan, M.E.1    Flotte, T.2    Afione, S.3
  • 17
    • 0028980536 scopus 로고
    • CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP
    • SCHWIEBERT EM, EGAN ME, HWANG TH et al. : CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 1995;81:1063-73.
    • (1995) Cell , vol.81 , pp. 1063-1073
    • Schwiebert, E.M.1    Egan, M.E.2    Hwang, T.H.3
  • 18
    • 0028982894 scopus 로고
    • CFTR as a c-AMP-dependent regulator of sodium channels
    • STUTTS MJ, CANESSA CM, OLSEN JC et al. : CFTR as a c-AMP-dependent regulator of sodium channels. Science 1995;269:847-50.
    • (1995) Science , vol.269 , pp. 847-850
    • Stutts, M.J.1    Canessa, C.M.2    Olsen, J.C.3
  • 20
    • 0028309683 scopus 로고
    • Human airway in ion transport (pt 1)
    • BOUCHER RC : Human airway in ion transport (pt 1). Am J Respir Crit Care Med 1994;150:271-81.
    • (1994) Am J Respir Crit Care Med , vol.150 , pp. 271-281
    • Boucher, R.C.1
  • 21
    • 0027998618 scopus 로고
    • Human airway in ion transport (pt 2)
    • BOUCHER RC : Human airway in ion transport (pt 2). Am J Respir Crit Care Med 1994;150:581-93.
    • (1994) Am J Respir Crit Care Med , vol.150 , pp. 581-593
    • Boucher, R.C.1
  • 22
    • 0031005832 scopus 로고    scopus 로고
    • CFTR: A multi-faced epithelial molecule
    • TÜMMLER B, PUCHELLE E : CFTR: a multi-faced epithelial molecule. Trends in Cell Biol 1997;7:250-1.
    • (1997) Trends in Cell Biol , vol.7 , pp. 250-251
    • Tümmler, B.1    Puchelle, E.2
  • 23
    • 0028920643 scopus 로고
    • Cystic fibrosis epithelial cells have receptor for pathogenic bacteria on their apical surface
    • IMUNDO L, BARASCH J, PRINCE A, AL-AWQATI Q : Cystic fibrosis epithelial cells have receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci USA 1995;92:3019-23.
    • (1995) Proc Natl Acad Sci USA , vol.92 , pp. 3019-3023
    • Imundo, L.1    Barasch, J.2    Prince, A.3    Al-Awqati, Q.4
  • 24
    • 0030836678 scopus 로고    scopus 로고
    • Expression of integrin cell adhesion receptors during human airway epithelial repair in vivo
    • PILEWSKI JM, LATOCHE JD, ARCASOY SM, ALBELDA SM : Expression of integrin cell adhesion receptors during human airway epithelial repair in vivo. Am J Physiol 1997;273:L256-63.
    • (1997) Am J Physiol , vol.273
    • Pilewski, J.M.1    Latoche, J.D.2    Arcasoy, S.M.3    Albelda, S.M.4
  • 25
    • 0030021470 scopus 로고    scopus 로고
    • Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections
    • PIER G, GROUD M, ZAIDI TS et al. : Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science 1996;27: 64-7.
    • (1996) Science , vol.27 , pp. 64-67
    • Pier, G.1    Groud, M.2    Zaidi, T.S.3
  • 26
    • 0029870085 scopus 로고    scopus 로고
    • Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
    • SMITH JJ, TRAVIS SM, GREEBERG EP, WELSH MJ : Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 1996;85:229-32.
    • (1996) Cell , vol.85 , pp. 229-232
    • Smith, J.J.1    Travis, S.M.2    Greeberg, E.P.3    Welsh, M.J.4
  • 27
    • 0030949875 scopus 로고    scopus 로고
    • Human β-defensine-1 is a salt-sensitive antibiotic in lung that is inactivated in cystic fibrosis
    • GOLDMAN MJ, ANDERSON GM, STOLZENBERG ED, KARI UP, ZASLOFF M, WILSON JM : Human β-defensine-1 is a salt-sensitive antibiotic in lung that is inactivated in cystic fibrosis. Cell 1997;88:553-60
    • (1997) Cell , vol.88 , pp. 553-560
    • Goldman, M.J.1    Anderson, G.M.2    Stolzenberg, E.D.3    Kari, U.P.4    Zasloff, M.5    Wilson, J.M.6
  • 28
    • 0030924036 scopus 로고    scopus 로고
    • Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis
    • HO LP, SAMWAYS JM, PORTEOUS DJ et al. : Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis. Eur Respir J 1997;10:2018-22
    • (1997) Eur Respir J , vol.10 , pp. 2018-2022
    • Ho, L.P.1    Samways, J.M.2    Porteous, D.J.3
  • 29
    • 0025241696 scopus 로고
    • The relation between genotype and phenotype in cystic fibrosis- Analysis of the most common mutation (ΔF508)
    • KEREM E, COREY M, KEREM SB et al. : The relation between genotype and phenotype in cystic fibrosis- Analysis of the most common mutation (ΔF508). N Engl J Med 1990;323:1517-22.
    • (1990) N Engl J Med , vol.323 , pp. 1517-1522
    • Kerem, E.1    Corey, M.2    Kerem, S.B.3
  • 30
    • 0029860775 scopus 로고    scopus 로고
    • Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients
    • HUBERT D, BIENVENU T, DESMAZES-DUFEU N et al. : Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients. Eur Respir J 1996;9:2207-14.
    • (1996) Eur Respir J , vol.9 , pp. 2207-2214
    • Hubert, D.1    Bienvenu, T.2    Desmazes-Dufeu, N.3
  • 31
    • 1842333889 scopus 로고    scopus 로고
    • Genotype and phenotype in cystic fibrosis
    • TSUI LC, DURIE P : Genotype and phenotype in cystic fibrosis. Hosp Pract Off Ed 1997;32:115-8, 123-9, 134.
    • (1997) Hosp Pract off Ed , vol.32 , pp. 115-118
    • Tsui, L.C.1    Durie, P.2
  • 32
    • 0028958565 scopus 로고
    • Increasd incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis
    • PIGNATTI PF, BOMBIERI C, MARIGO C, BENETAZZO M, LUISETTI M : Increasd incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis. Hum Mol Genet 1995;4:635-9.
    • (1995) Hum Mol Genet , vol.4 , pp. 635-639
    • Pignatti, P.F.1    Bombieri, C.2    Marigo, C.3    Benetazzo, M.4    Luisetti, M.5
  • 33
    • 12644300645 scopus 로고    scopus 로고
    • CFTR gene mutations in adults with disseminated bronchiectasis
    • GIRODON E, CAZENEUVE C, LEBARGY F et al. : CFTR gene mutations in adults with disseminated bronchiectasis. Eur J Hum Genet 1997;5:149-55.
    • (1997) Eur J Hum Genet , vol.5 , pp. 149-155
    • Girodon, E.1    Cazeneuve, C.2    Lebargy, F.3
  • 34
    • 0028914085 scopus 로고
    • Early pulmonary inflammation in infants with cystic fibrosis
    • KAHN TZ, WAGENER JJ, BOST T : Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995;151:1075-82.
    • (1995) Am J Respir Crit Care Med , vol.151 , pp. 1075-1082
    • Kahn, T.Z.1    Wagener, J.J.2    Bost, T.3
  • 35
    • 0029346937 scopus 로고
    • The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis
    • BALOUGH K, MCCUBBIN M, WEINBERER M : The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis. Pediatr Pulmonol 1995;20:63-70.
    • (1995) Pediatr Pulmonol , vol.20 , pp. 63-70
    • Balough, K.1    Mccubbin, M.2    Weinberer, M.3
  • 36
    • 0029286816 scopus 로고
    • Cystic fibrosis lung inflammation: Early, sustained and severe
    • CANTIN A : Cystic fibrosis lung inflammation: early, sustained and severe. Am J Respir Crit Care Med 1995;151:939-41.
    • (1995) Am J Respir Crit Care Med , vol.151 , pp. 939-941
    • Cantin, A.1
  • 38
    • 0030768159 scopus 로고    scopus 로고
    • Lower airway inflammation in infants and young children with cystic fibrosis
    • ARMSTRONG DS, GRIMWOOD K, CARLIN JB et al. : Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 1997;156:1197-1204.
    • (1997) Am J Respir Crit Care Med , vol.156 , pp. 1197-1204
    • Armstrong, D.S.1    Grimwood, K.2    Carlin, J.B.3
  • 39
    • 0001329915 scopus 로고    scopus 로고
    • IL8 in cystic fibrosis and its regulation by complexation with macromolecules. 10th Annual North American Cystic Fibrosis Conference, Orlando, Florida, USA, 24-27 October 1996
    • SHUTE JK : IL8 in cystic fibrosis and its regulation by complexation with macromolecules. 10th Annual North American Cystic Fibrosis Conference, Orlando, Florida, USA, 24-27 October 1996. Pediatr Pulmonol 1996;(supp13):187-188.
    • (1996) Pediatr Pulmonol , Issue.13 SUPPL. , pp. 187-188
    • Shute, J.K.1
  • 40
    • 0031035884 scopus 로고    scopus 로고
    • Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis
    • NOAH TL, BLACK HR, CHENG PW, WOOD RE, LEIGH MW : Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis. J Infect Dis 1997;175:638-47
    • (1997) J Infect Dis , vol.175 , pp. 638-647
    • Noah, T.L.1    Black, H.R.2    Cheng, P.W.3    Wood, R.E.4    Leigh, M.W.5
  • 44
    • 3543027125 scopus 로고    scopus 로고
    • Fonctions de la protéine CFTR dans l'épithélium respiratoire et ses anomalies dans la mucoviscidose
    • C. Caulin, C. Chastang, eds. Springer-Verlag France, Paris
    • PUCHELLE E, JACQUOT J : Fonctions de la protéine CFTR dans l'épithélium respiratoire et ses anomalies dans la mucoviscidose. In : C. Caulin, C. Chastang, eds. Recherche Clinique et Décision Thérapeutique. Les médicaments de la mucoviscidose chez l'enfant. Springer-Verlag France, Paris, 1997:33-44.
    • (1997) Recherche Clinique et Décision Thérapeutique. Les Médicaments de la Mucoviscidose Chez L'enfant , pp. 33-44
    • Puchelle, E.1    Jacquot, J.2
  • 46
    • 84866227602 scopus 로고    scopus 로고
    • Infection broncho-pulmonaire
    • FOUCAUD P : Infection broncho-pulmonaire. Référence Mucoviscidose 1997,2:22-5.
    • (1997) Référence Mucoviscidose , vol.2 , pp. 22-25
    • Foucaud, P.1
  • 47
    • 0029965836 scopus 로고    scopus 로고
    • Cystic fibrosis in adults. From researcher to practitionner
    • MARELICH GP, CROSS CE : Cystic fibrosis in adults. From researcher to practitionner. West J Med 1996;164:321-34.
    • (1996) West J Med , vol.164 , pp. 321-334
    • Marelich, G.P.1    Cross, C.E.2
  • 48
    • 0029934215 scopus 로고    scopus 로고
    • Management of pulmonary disease in patients with cystic fibrosis
    • RAMSEY BW : Management of pulmonary disease in patients with cystic fibrosis. N Engl J Med 1996;335:179-88.
    • (1996) N Engl J Med , vol.335 , pp. 179-188
    • Ramsey, B.W.1
  • 49
    • 0023936587 scopus 로고
    • The clinical effect and the effect on the ciliary motility of oral N-acetylcysteine in patients with cystic fibrosis and primary ciliary dyskinesia
    • STAFANGER G, GARNE P, HOWITZ P, MORKASSEL E, KOCH C : The clinical effect and the effect on the ciliary motility of oral N-acetylcysteine in patients with cystic fibrosis and primary ciliary dyskinesia. Eur Respir J 1988;161-7.
    • (1988) Eur Respir J , pp. 161-167
    • Stafanger, G.1    Garne, P.2    Howitz, P.3    Morkassel, E.4    Koch, C.5
  • 50
    • 0000009664 scopus 로고    scopus 로고
    • Efficacy, safety and tolerability of chronic inhalation therapy with Nacystelyn versus placebo in cystic fibrosis patients
    • APP EM, BARAN D, DAB I et al. : Efficacy, safety and tolerability of chronic inhalation therapy with Nacystelyn versus placebo in cystic fibrosis patients. Am J Respir Crit Care Med 1997;155(4):A199.
    • (1997) Am J Respir Crit Care Med , vol.155 , Issue.4
    • App, E.M.1    Baran, D.2    Dab, I.3
  • 51
    • 0029853628 scopus 로고    scopus 로고
    • Reduction in viscoelasticity in cystic fibrosis sputum in vitro using combined treatment with Nacystelyn and rhDNase
    • DASGUPTA B, KING M : Reduction in viscoelasticity in cystic fibrosis sputum in vitro using combined treatment with Nacystelyn and rhDNase. Pediatr Pulmonol 1996;22:161-6.
    • (1996) Pediatr Pulmonol , vol.22 , pp. 161-166
    • Dasgupta, B.1    King, M.2
  • 52
    • 0030034674 scopus 로고    scopus 로고
    • Effects of rhDNase on airflow obstruction and mucociliary clearance in cystic fibrosis
    • LAUBE BL, AUCI RA, SHIELDS DE et al. : Effects of rhDNase on airflow obstruction and mucociliary clearance in cystic fibrosis. Am J Respir Crit Care Med 1996;153:752-60.
    • (1996) Am J Respir Crit Care Med , vol.153 , pp. 752-760
    • Laube, B.L.1    Auci, R.A.2    Shields, D.E.3
  • 53
    • 0027278897 scopus 로고
    • Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis
    • RAMSEY BW, ASTLEY SJ, AITKEN ML et al. : Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis. Am Rev Respir Dis 1993;148:145-51.
    • (1993) Am Rev Respir Dis , vol.148 , pp. 145-151
    • Ramsey, B.W.1    Astley, S.J.2    Aitken, M.L.3
  • 54
    • 0029093124 scopus 로고
    • Two years experience with recombinant human DNase I in the treatment of pulmonary disease in cystic fibrosis
    • SHAH PL, SCOTT SF, GEDDES DM, HODSON ME : Two years experience with recombinant human DNase I in the treatment of pulmonary disease in cystic fibrosis. Respir Med 1995;89:499-502.
    • (1995) Respir Med , vol.89 , pp. 499-502
    • Shah, P.L.1    Scott, S.F.2    Geddes, D.M.3    Hodson, M.E.4
  • 55
    • 0028951077 scopus 로고
    • Aerosolized dornase alfa (rhDNase) for therapy of cystic fibrosis
    • HODSON ME : Aerosolized dornase alfa (rhDNase) for therapy of cystic fibrosis. Am J Respir Crit Care Med 1995;151:S70-S74.
    • (1995) Am J Respir Crit Care Med , vol.151
    • Hodson, M.E.1
  • 56
    • 0028129568 scopus 로고
    • Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
    • FUCHS HJ, BOROWITZ DS, CHRISTIANSEN DH et al. : 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;331:637-42.
    • (1994) N Engl J Med , vol.331 , pp. 637-642
    • Fuchs, H.J.1    Borowitz, D.S.2    Christiansen, D.H.3
  • 57
    • 0029921953 scopus 로고    scopus 로고
    • Effects of 12 week administration of dornase alfa in patients with advanced cystic fibrosis lung disease
    • MCCOY K, HAMILTON S, JOHNSON C FOR THE PULMOZYME STUDY GROUP : Effects of 12 week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Chest 1996;110:889-95.
    • (1996) Chest , vol.110 , pp. 889-895
    • Mccoy, K.1    Hamilton, S.2    Johnson, C.3
  • 59
    • 0030722116 scopus 로고    scopus 로고
    • Safety of repeated intermittent courses of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis
    • EISENBERG JD, AITKEN ML, DORKIN HL et al. : Safety of repeated intermittent courses of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis. J Pediatr 1997;131:118-24.
    • (1997) J Pediatr , vol.131 , pp. 118-124
    • Eisenberg, J.D.1    Aitken, M.L.2    Dorkin, H.L.3
  • 60
    • 0030787482 scopus 로고    scopus 로고
    • Recombinant human DNase (rhDNase) in cystic fibrosis: Is it cost effective?
    • CONWAY SP : Recombinant human DNase (rhDNase) in cystic fibrosis: is it cost effective? Arch Dis Child 1997;77:1-3.
    • (1997) Arch Dis Child , vol.77 , pp. 1-3
    • Conway, S.P.1
  • 61
    • 0029916071 scopus 로고    scopus 로고
    • Effect of hypertonic saline, amiloride and cough on mucociliary clearance in patients with cystic fibrosis
    • ROBINSON M, REGNIS JA, BAILEY DL, KING M, BAUTOVICH GJ, BYE PT : Effect of hypertonic saline, amiloride and cough on mucociliary clearance in patients with cystic fibrosis. Am J Respir Crit Care Med 1996;153:1503-9.
    • (1996) Am J Respir Crit Care Med , vol.153 , pp. 1503-1509
    • Robinson, M.1    Regnis, J.A.2    Bailey, D.L.3    King, M.4    Bautovich, G.J.5    Bye, P.T.6
  • 63
    • 0030845454 scopus 로고    scopus 로고
    • Rheology of cystic fibrosis sputum after in vitro treatment with hypertonic saline alone and in combination with recombinant human deoxyribonuclease I
    • KING M, DASGUPTA B, TOMKIEWICZ RP, BROWN NE : Rheology of cystic fibrosis sputum after in vitro treatment with hypertonic saline alone and in combination with recombinant human deoxyribonuclease I. Am J Respir Crit Care Med 1997;156:173-7.
    • (1997) Am J Respir Crit Care Med , vol.156 , pp. 173-177
    • King, M.1    Dasgupta, B.2    Tomkiewicz, R.P.3    Brown, N.E.4
  • 64
    • 0029383649 scopus 로고
    • Short-term and long-term effects of albuterol aerosol therapy in cystic fibrosis: A preliminary report
    • KONIG PD, GAYER D, BARBERO GJ, SHAFFER J : Short-term and long-term effects of albuterol aerosol therapy in cystic fibrosis: a preliminary report. Pediatr Pulmonol 1995;20:205-14.
    • (1995) Pediatr Pulmonol , vol.20 , pp. 205-214
    • Konig, P.D.1    Gayer, D.2    Barbero, G.J.3    Shaffer, J.4
  • 65
    • 0030670517 scopus 로고    scopus 로고
    • Short-term effects of regular salmeterol treatment on adult cystic fibrosis patients
    • BARGON J, VIEL K, DAULETBAEV N, WIEWRODT R, BUHL R : Short-term effects of regular salmeterol treatment on adult cystic fibrosis patients. Eur Respir J 1997;10:2307-11.
    • (1997) Eur Respir J , vol.10 , pp. 2307-2311
    • Bargon, J.1    Viel, K.2    Dauletbaev, N.3    Wiewrodt, R.4    Buhl, R.5
  • 66
    • 0027197981 scopus 로고
    • The effect of high doses of inhaled salbutamol and ipratropium bromide in patients with cystic fibrosis
    • SANCHEZ I, DE KOSTER J, HOLBROW J, CHERNICK V : The effect of high doses of inhaled salbutamol and ipratropium bromide in patients with cystic fibrosis. Chest 1993;104:842-6.
    • (1993) Chest , vol.104 , pp. 842-846
    • Sanchez, I.1    De Koster, J.2    Holbrow, J.3    Chernick, V.4
  • 67
    • 0029065886 scopus 로고
    • Role of anticholinergic agents in the treatment of cystic fibrosis
    • SANCHEZ I : Role of anticholinergic agents in the treatment of cystic fibrosis. Arch Pediatr 1995;2:154S-158S.
    • (1995) Arch Pediatr , vol.2
    • Sanchez, I.1
  • 68
    • 0027511938 scopus 로고
    • 2 adrenergic agonists on lung mucociliary clearance in patients with cystic fibrosis
    • 2 adrenergic agonists on lung mucociliary clearance in patients with cystic fibrosis. Chest 1993;103:805-11.
    • (1993) Chest , vol.103 , pp. 805-811
    • Mortensen, J.1    Hansen, A.2    Falk, M.3    Nielsen, I.K.4    Groth, S.5
  • 70
    • 0021970474 scopus 로고
    • Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis
    • AUERBACH HS, WILLIAMS M, KIRKPATRICK JA, COLTEN HR : Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 1985;2:686-8.
    • (1985) Lancet , vol.2 , pp. 686-688
    • Auerbach, H.S.1    Williams, M.2    Kirkpatrick, J.A.3    Colten, H.R.4
  • 71
    • 0028969594 scopus 로고
    • A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis
    • EIGEN HR, ROSENSTEIN BJ, FITZSIMMONS S, SHIDLOW DV : A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. J Pediatr 1995;126:515-23.
    • (1995) J Pediatr , vol.126 , pp. 515-523
    • Eigen, H.R.1    Rosenstein, B.J.2    Fitzsimmons, S.3    Shidlow, D.V.4
  • 72
    • 0025977985 scopus 로고
    • Risks of alternate-day prednisone in patients with cystic fibrosis
    • ROSENSTEIN BJ, EIGEN H : Risks of alternate-day prednisone in patients with cystic fibrosis. Pediatrics 1991;87:245-6.
    • (1991) Pediatrics , vol.87 , pp. 245-246
    • Rosenstein, B.J.1    Eigen, H.2
  • 73
    • 0030062339 scopus 로고    scopus 로고
    • Pilot study to assess the effect of inhaled corticosteroids on lung function in patients with cystic fibrosis
    • NIKOLAIZIK WH, SCHÖNI MH : Pilot study to assess the effect of inhaled corticosteroids on lung function in patients with cystic fibrosis. J Pediatr 1996;128:271-4.
    • (1996) J Pediatr , vol.128 , pp. 271-274
    • Nikolaizik, W.H.1    Schöni, M.H.2
  • 74
    • 0000666506 scopus 로고    scopus 로고
    • A double-blind, placebo-controlled study comparing the inhaled corticosteroid fluticasone propionate with placebo in adults with cystic fibrosis
    • NIEMAN R, WILLIAMS S, MADEN C, KNIGHT R, HODSON M : A double-blind, placebo-controlled study comparing the inhaled corticosteroid fluticasone propionate with placebo in adults with cystic fibrosis. Am J Respir Crit Care Med 1996;153:A72.
    • (1996) Am J Respir Crit Care Med , vol.153
    • Nieman, R.1    Williams, S.2    Maden, C.3    Knight, R.4    Hodson, M.5
  • 75
    • 0030821195 scopus 로고    scopus 로고
    • Controlled trial of inhaled budesonide in patients with cystic fibrosis and chronic bronchopulmonary pseudomonas aeruginosa infection
    • BISGAARD H, PEDERSEN SS, NIELSEN KG et al. : Controlled trial of inhaled budesonide in patients with cystic fibrosis and chronic bronchopulmonary pseudomonas aeruginosa infection. Am J Respir Crit Care Med 1997;156:1190-6.
    • (1997) Am J Respir Crit Care Med , vol.156 , pp. 1190-1196
    • Bisgaard, H.1    Pedersen, S.S.2    Nielsen, K.G.3
  • 76
    • 0028914667 scopus 로고
    • Effect of high-dose ibuprofen in patients with cystic fibrosis
    • KONSTAN MW, BYARD PJ, HOPPEL CL, DAVID PB : Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995;332:848-54.
    • (1995) N Engl J Med , vol.332 , pp. 848-854
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3    David, P.B.4
  • 77
    • 6544220719 scopus 로고
    • Clinical use of Ibuprofen for cystic fibrosis (CF) lung disease: Data from the 1996 CF Foundation National Patient Registry
    • KONSTAN MW, FITZSIMMONS SC : Clinical use of Ibuprofen for cystic fibrosis (CF) lung disease: data from the 1996 CF Foundation National Patient Registry. Pediatr Pulmonol 1991;suppl 14:A427
    • (1991) Pediatr Pulmonol , Issue.14 SUPPL.
    • Konstan, M.W.1    Fitzsimmons, S.C.2
  • 78
    • 0024549158 scopus 로고
    • Intraveinous immune globuline treatement of pulmonary exacerbations in cystic fibrosis
    • WINNIE GB, COWAN RG, WADE NA : Intraveinous immune globuline treatement of pulmonary exacerbations in cystic fibrosis. J Pediatr 1989;114:309-14.
    • (1989) J Pediatr , vol.114 , pp. 309-314
    • Winnie, G.B.1    Cowan, R.G.2    Wade, N.A.3
  • 79
    • 0024986044 scopus 로고
    • Pseudomonas hyperimmune globuline passive immunotherapy for pulmonary exacerbations in cystic fibrosis
    • VANWYE JE, COLLINS MS, BAYLOR M et al. : Pseudomonas hyperimmune globuline passive immunotherapy for pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol 1990;9:7-18.
    • (1990) Pediatr Pulmonol , vol.9 , pp. 7-18
    • Vanwye, J.E.1    Collins, M.S.2    Baylor, M.3
  • 80
    • 0028047967 scopus 로고
    • Pulmonary function in infants with cystic fibrosis: The effect of antibiotic treatment
    • BEARDSMORE CS, THOMPSON JR, WILLIAMS A et al. : Pulmonary function in infants with cystic fibrosis: the effect of antibiotic treatment. Arch Dis Child 1994;71:133-7.
    • (1994) Arch Dis Child , vol.71 , pp. 133-137
    • Beardsmore, C.S.1    Thompson, J.R.2    Williams, A.3
  • 81
    • 0023740312 scopus 로고
    • Antibacterial therapy in cystic fibrosis. A review of the literature published between 1980 and February 1987
    • MICHEL BC : Antibacterial therapy in cystic fibrosis. A review of the literature published between 1980 and February 1987. Chest 1988;94:129S-140S.
    • (1988) Chest , vol.94
    • Michel, B.C.1
  • 82
    • 0030893031 scopus 로고    scopus 로고
    • Use of meropenem in the treatment of serious infections in children: Review of the current literature
    • ARRIETA A : Use of meropenem in the treatment of serious infections in children: review of the current literature. Clin Infect Dis 1997;24(suppl 2):S207-12.
    • (1997) Clin Infect Dis , vol.24 , Issue.2 SUPPL.
    • Arrieta, A.1
  • 85
    • 0031046882 scopus 로고    scopus 로고
    • Sequential ciprofloxacin therapy in pediatric cystic fibrosis: Comparative study vs. ceftazidime/ tobramycin in the treatment of acute pulmonary exacerbations. the Cystic Fibrosis Study Group
    • CHURCH DA, KANGA JF, KUHN RJ, RUBIO TT, SPOHN WA et al. : Sequential ciprofloxacin therapy in pediatric cystic fibrosis: comparative study vs. ceftazidime/ tobramycin in the treatment of acute pulmonary exacerbations. The Cystic Fibrosis Study Group. Pediatr Infect Dis J 1997;16:97-105;123-6.
    • (1997) Pediatr Infect Dis J , vol.16 , pp. 97-105
    • Church, D.A.1    Kanga, J.F.2    Kuhn, R.J.3    Rubio, T.T.4    Spohn, W.A.5
  • 86
    • 0030956420 scopus 로고
    • Home intravenous antibiotic therapy: Practical aspects in adults
    • PHILLIPS AM : Home intravenous antibiotic therapy: practical aspects in adults. JR Soc Med 1991;90(suppl.31):34-6.
    • (1991) JR Soc Med , vol.90 , Issue.31 SUPPL. , pp. 34-36
    • Phillips, A.M.1
  • 87
    • 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, KOCK C, HOIBY N : Antibiotic treatment of initial colonization with Pseudomonas aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis. Pediatr Pulmonol 1997;23:330-5.
    • (1997) Pediatr Pulmonol , vol.23 , pp. 330-335
    • Frederiksen, B.1    Kock, C.2    Hoiby, N.3
  • 88
    • 0343303190 scopus 로고    scopus 로고
    • An american approach to the management of P. aeruginosa infection in cystic fibrosis
    • STUTMAN HR : An american approach to the management of P. aeruginosa infection in cystic fibrosis. Pediatr Pulmonol 1996;suppl 13:109-10.
    • (1996) Pediatr Pulmonol , Issue.13 SUPPL. , pp. 109-110
    • Stutman, H.R.1
  • 90
    • 0029921514 scopus 로고    scopus 로고
    • Improved survival in the Danish center-treated cystic fibrosis patients: Results of agressive treatment
    • FREDERIKSEN B, LANG S, KOCK C, HOIBY N : Improved survival in the Danish center-treated cystic fibrosis patients: results of agressive treatment. Pediatr Pulmonol 1996;21:153-8.
    • (1996) Pediatr Pulmonol , vol.21 , pp. 153-158
    • Frederiksen, B.1    Lang, S.2    Kock, C.3    Hoiby, N.4
  • 91
    • 0027492890 scopus 로고
    • Regular three monthly oral ciprofloxacin in adult cystic fibrosis patients infected with Pseudomonas aeruginosa
    • SHELDON CD, ASSOUFI BK, HODSON ME : Regular three monthly oral ciprofloxacin in adult cystic fibrosis patients infected with Pseudomonas aeruginosa. Respir Med 1993;87:587-93.
    • (1993) Respir Med , vol.87 , pp. 587-593
    • Sheldon, C.D.1    Assoufi, B.K.2    Hodson, M.E.3
  • 92
    • 0030859951 scopus 로고    scopus 로고
    • Effect of tonicity of nebulised colistin on chest tightness and pulmonary function in adults with cystic fibrosis
    • DODD ME, ABBOTT J, MADDISON J, MOORCROFT AJ, WEBB AK : Effect of tonicity of nebulised colistin on chest tightness and pulmonary function in adults with cystic fibrosis. Thorax 1997;52:656-8.
    • (1997) Thorax , vol.52 , pp. 656-658
    • Dodd, M.E.1    Abbott, J.2    Maddison, J.3    Moorcroft, A.J.4    Webb, A.K.5
  • 93
    • 0030015857 scopus 로고    scopus 로고
    • Inhaled antibiotics in cystic fibrosis: A review
    • TOSO C, WILLIAMS DM, NOONE PG : Inhaled antibiotics in cystic fibrosis: a review. Ann Pharmacother 1996;30(7-8):840-50.
    • (1996) Ann Pharmacother , vol.30 , Issue.7-8 , pp. 840-850
    • Toso, C.1    Williams, D.M.2    Noone, P.G.3
  • 94
    • 0030923948 scopus 로고    scopus 로고
    • Nebulised antipseudomonal antibiotic therapy in cystic fibrosis
    • HOEKSTRA MO, VAN-AALDEREN WM : Nebulised antipseudomonal antibiotic therapy in cystic fibrosis. Thorax 1997;52:398-9.
    • (1997) Thorax , vol.52 , pp. 398-399
    • Hoekstra, M.O.1    Van-Aalderen, W.M.2
  • 95
    • 0030922787 scopus 로고    scopus 로고
    • Nebulization and anti-Pseudomonas aeruginosa activity of colistin
    • DIOT P, GAGNADOUX F, MARTIN C et al. : Nebulization and anti-Pseudomonas aeruginosa activity of colistin. Eur Respir J 1997;10:1995-8.
    • (1997) Eur Respir J , vol.10 , pp. 1995-1998
    • Diot, P.1    Gagnadoux, F.2    Martin, C.3
  • 97
    • 0029877773 scopus 로고    scopus 로고
    • Nebulised anti-pseudomonal antibiotic therapy in cystic fibrosis: A meta-analysis of benefits and risks
    • MUKHOPADHYAY S, SINGH M, CATER JI, OGSTON S, FRANKLIN M, OLVER RE : Nebulised anti-pseudomonal antibiotic therapy in cystic fibrosis: a meta-analysis of benefits and risks. Thorax 1996;51:364-8.
    • (1996) Thorax , vol.51 , pp. 364-368
    • Mukhopadhyay, S.1    Singh, M.2    Cater, J.I.3    Ogston, S.4    Franklin, M.5    Olver, R.E.6
  • 100
    • 0027287651 scopus 로고
    • Efficacy of aerosolized tobramycin in patients with cystic fibrosis
    • RAMSEY BW, DORKIN HL, EISENBERG JD, GIBSON RL et al. : Efficacy of aerosolized tobramycin in patients with cystic fibrosis. N Eng J Med 1993;328:1740-6.
    • (1993) N Eng J Med , vol.328 , pp. 1740-1746
    • Ramsey, B.W.1    Dorkin, H.L.2    Eisenberg, J.D.3    Gibson, R.L.4
  • 102
    • 0025966879 scopus 로고
    • Aerosol alpha-1-antitrypsin treatment of cystic fibrosis
    • MC ELVANEY NG, HUBBARD RC, BIRRER P et al. : Aerosol alpha-1-antitrypsin treatment of cystic fibrosis. Lancet 1991;337:392-4.
    • (1991) Lancet , vol.337 , pp. 392-394
    • Mc Elvaney, N.G.1    Hubbard, R.C.2    Birrer, P.3
  • 103
    • 0030463020 scopus 로고    scopus 로고
    • Opportunities for the use of aerosolized alpha-1-antitrypsin for the treatment of cystic fibrosis
    • ALLEN ED : Opportunities for the use of aerosolized alpha-1-antitrypsin for the treatment of cystic fibrosis. Chest 1996;110(suppl 6):256s-60s.
    • (1996) Chest , vol.110 , Issue.6 SUPPL.
    • Allen, E.D.1
  • 104
    • 0028113854 scopus 로고
    • Effects of pentoxifylline on sputum neutrophil elastase and pulmonary function in patients with cystic fibrosis: Preliminary observations
    • ARONOFF SC, QUINN FJ, CARPENTER LS, NOVICK WJ : Effects of pentoxifylline on sputum neutrophil elastase and pulmonary function in patients with cystic fibrosis: preliminary observations. J Pediatr 1994;125:992-7.
    • (1994) J Pediatr , vol.125 , pp. 992-997
    • Aronoff, S.C.1    Quinn, F.J.2    Carpenter, L.S.3    Novick, W.J.4
  • 105
    • 6544248553 scopus 로고    scopus 로고
    • Synthetic protegrins can eliminate P. aeruginosa from the mucosal surface of cultured airway epithelia
    • SMITH JJ, MCCRAY PB, THORNE S, LOURY DJ, FIDDES JC, WELSH MJ : Synthetic protegrins can eliminate P. aeruginosa from the mucosal surface of cultured airway epithelia. Pediatr Pulmonol 1997;Suppl 14:A428.
    • (1997) Pediatr Pulmonol , Issue.14 SUPPL.
    • Smith, J.J.1    Mccray, P.B.2    Thorne, S.3    Loury, D.J.4    Fiddes, J.C.5    Welsh, M.J.6
  • 106
    • 0000920644 scopus 로고    scopus 로고
    • Safety and efficacy of Tobramycin solution for inhalation in patients with cystic fibrosis: The results of two Phase III placebo controlled clinical trial
    • RAMSEY B, BURNS J, SMITH A : Safety and efficacy of Tobramycin solution for inhalation in patients with cystic fibrosis: The results of two Phase III placebo controlled clinical trial. Pediatr Pulmonol 1997;Suppl 14:137-8.
    • (1997) Pediatr Pulmonol , vol.14 , Issue.SUPPL. , pp. 137-138
    • Ramsey, B.1    Burns, J.2    Smith, A.3
  • 107
    • 0025094612 scopus 로고
    • Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfert
    • DRUMM ML, POPE HA, CLIFF WH et al. : Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfert. Cell 1990;62:1227-33.
    • (1990) Cell , vol.62 , pp. 1227-1233
    • Drumm, M.L.1    Pope, H.A.2    Cliff, W.H.3
  • 109
    • 0029100239 scopus 로고
    • A controlled study of adenoviral-vector-mediated gene transfert in the nasal epithelium of patients with cystic fibrosis
    • KNOWLES M, HOHNEKER K, ZHOU Z, OLSEN J, NOAH T, HU P et al. : A controlled study of adenoviral-vector-mediated gene transfert in the nasal epithelium of patients with cystic fibrosis. N Engl J Med 1993;333:823-31.
    • (1993) N Engl J Med , vol.333 , pp. 823-831
    • Knowles, M.1    Hohneker, K.2    Zhou, Z.3    Olsen, J.4    Noah, T.5    Hu, P.6
  • 110
    • 0028168641 scopus 로고
    • Administration of an adenovirus containing the human CFTR cDNA to the respiratory tract of individuals with cystic fibrosis
    • CRYSTAL RG, MCELVANEY NG, ROSENFELD MA, CHU C et al. : Administration of an adenovirus containing the human CFTR cDNA to the respiratory tract of individuals with cystic fibrosis. Nature Genet 1994;8:42-51.
    • (1994) Nature Genet , vol.8 , pp. 42-51
    • Crystal, R.G.1    Mcelvaney, N.G.2    Rosenfeld, M.A.3    Chu, C.4
  • 111
    • 16944367323 scopus 로고    scopus 로고
    • Aerosol administration of a recombinant adenovirus expressing CFTR to cystic fibrosis patients: A phase I clinical trial
    • BELLON G, CALEMARD L, THOUVENOT D et al. : Aerosol administration of a recombinant adenovirus expressing CFTR to cystic fibrosis patients: a phase I clinical trial. Hum Gene Therapy 1997;8:15-25.
    • (1997) Hum Gene Therapy , vol.8 , pp. 15-25
    • Bellon, G.1    Calemard, L.2    Thouvenot, D.3
  • 115
    • 6544261542 scopus 로고    scopus 로고
    • Eleventh Annual North American Cystic Fibrosis Conference : Nashville, Tennessee, October 23-26, 1997
    • Abstracts
    • ELEVENTH ANNUAL NORTH AMERICAN CYSTIC FIBROSIS CONFERENCE : Nashville, Tennessee, October 23-26, 1997. Pediatr Pulmonol 1997;suppl 14:255-61 (Abstracts).
    • (1997) Pediatr Pulmonol , Issue.14 SUPPL. , pp. 255-261
  • 116
    • 6544259929 scopus 로고    scopus 로고
    • Tenth Annual North American Cystic Fibrosis Conference : Orlando, Florida, October 24-26, 1996
    • Abstracts
    • TENTH ANNUAL NORTH AMERICAN CYSTIC FIBROSIS CONFERENCE : Orlando, Florida, October 24-26, 1996. Pediatr Pulmonol 1996;suppl 13:91-96 (Abstracts).
    • (1996) Pediatr Pulmonol , Issue.13 SUPPL. , pp. 91-96
  • 117
    • 13344286312 scopus 로고    scopus 로고
    • Repeat administration of an adenovirus vector encoding CFTR to the nasal epithelium of patients with cystic fibrosis
    • ZABNER J, RAMSEY BW, MEEKER DP et al. : Repeat administration of an adenovirus vector encoding CFTR to the nasal epithelium of patients with cystic fibrosis. J Clin Invest 1996;97:1504-11.
    • (1996) J Clin Invest , vol.97 , pp. 1504-1511
    • Zabner, J.1    Ramsey, B.W.2    Meeker, D.P.3
  • 118
    • 0343534322 scopus 로고    scopus 로고
    • Repeat administration of AdCFTR to the airway epithelium of individuals with cystic fibrosis is well tolerated and associated with minimal systemic humoral immunity
    • HARVEY BG, SIKAND V, BRION P, KANER RJ, CRYSTAL RG : Repeat administration of AdCFTR to the airway epithelium of individuals with cystic fibrosis is well tolerated and associated with minimal systemic humoral immunity. Pediatr Pulmonol 1997;suppl 14:A182;
    • (1997) Pediatr Pulmonol , Issue.14 SUPPL.
    • Harvey, B.G.1    Sikand, V.2    Brion, P.3    Kaner, R.J.4    Crystal, R.G.5
  • 119
    • 0029898764 scopus 로고    scopus 로고
    • A phase I study of an adeno-associated virus-CFTR gene vector in adult CF patients with mild lung disease
    • FLOTTE T, CARTER B, CONRAD C et al. : A phase I study of an adeno-associated virus-CFTR gene vector in adult CF patients with mild lung disease. Hum Gene Ther 1996;7:1145-59.
    • (1996) Hum Gene Ther , vol.7 , pp. 1145-1159
    • Flotte, T.1    Carter, B.2    Conrad, C.3
  • 120
    • 8244238394 scopus 로고    scopus 로고
    • A placebo-controlled study of liposome-mediated gene transfert to the nasal epithelium of patients with cystic fibrosis
    • GILL DR, SOUTHERN KW, MOFFORD KA et al. : A placebo-controlled study of liposome-mediated gene transfert to the nasal epithelium of patients with cystic fibrosis. Gene Ther 1997;4:199-209.
    • (1997) Gene Ther , vol.4 , pp. 199-209
    • Gill, D.R.1    Southern, K.W.2    Mofford, K.A.3
  • 121
    • 8244245761 scopus 로고    scopus 로고
    • Evidence for safety and efficacy of DOTAP cationic liposome mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis
    • PORTEOUS DJ, DORIN JR, MCLACHLAN G, DAVIDSON-SMITH H, DAVIDSON H et al. : Evidence for safety and efficacy of DOTAP cationic liposome mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis. Gene Ther 1997;4:210-8
    • (1997) Gene Ther , vol.4 , pp. 210-218
    • Porteous, D.J.1    Dorin, J.R.2    Mclachlan, G.3    Davidson-Smith, H.4    Davidson, H.5
  • 122
    • 0030978868 scopus 로고
    • Glycosylated polylysines. Nonviral vectors for gene transfert into cystic fibrosis airway epithelial cells
    • KOLLEN W, ERBACHER P, MIDOUX P et al. : Glycosylated polylysines. Nonviral vectors for gene transfert into cystic fibrosis airway epithelial cells. Chest 1991;111(suppl. 6): 95S-6S.
    • (1991) Chest , vol.111 , Issue.6 SUPPL.
    • Kollen, W.1    Erbacher, P.2    Midoux, P.3
  • 124
    • 0025236867 scopus 로고
    • A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
    • KNOWLES MR, CHURCH NL, WALTNER WE et al. : A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med 1990;322:1189-94.
    • (1990) N Engl J Med , vol.322 , pp. 1189-1194
    • Knowles, M.R.1    Church, N.L.2    Waltner, W.E.3
  • 125
    • 0027486593 scopus 로고
    • No added benefit from nebulised amiloride in patients with cystic fibrosis
    • GRAHAM A, HASANI A, ALTON EW et al. : No added benefit from nebulised amiloride in patients with cystic fibrosis. Eur Respir J 1993;6:1243-8.
    • (1993) Eur Respir J , vol.6 , pp. 1243-1248
    • Graham, A.1    Hasani, A.2    Alton, E.W.3
  • 126
    • 0028787073 scopus 로고
    • Nebulized amiloride in respiratory exacerbations of cystic fibrosis : A randomized controlled trial
    • BOWLER IM, KELMAN B, WORTHINGTON D et al. : Nebulized amiloride in respiratory exacerbations of cystic fibrosis : a randomized controlled trial. Arch Dis Child 1995;73:427-30.
    • (1995) Arch Dis Child , vol.73 , pp. 427-430
    • Bowler, I.M.1    Kelman, B.2    Worthington, D.3
  • 127
    • 0037858347 scopus 로고
    • French multicenter randomized double-blind placebo controlled trial of nebulized amiloride in cystic fibrosis patients
    • Abstract
    • PONS G, MARCHAND MC, FOUCARD C et al. : French multicenter randomized double-blind placebo controlled trial of nebulized amiloride in cystic fibrosis patients. Pediatr Pulmonol 1995;suppl. 12:193 (Abstract).
    • (1995) Pediatr Pulmonol , Issue.12 SUPPL. , pp. 193
    • Pons, G.1    Marchand, M.C.2    Foucard, C.3
  • 128
    • 0030982349 scopus 로고    scopus 로고
    • Aerosolized amiloride: Dose-effect on nasal bioelectric properties, pharmacokinetics, and effects on sputum expectoration in patients with cystic fibrosis
    • HOFMANN T, SENIER I, BITTNER P, HULS G, SCHWANDT HJ, LINDEMANN H : Aerosolized amiloride: Dose-effect on nasal bioelectric properties, pharmacokinetics, and effects on sputum expectoration in patients with cystic fibrosis. J Aerosol Med 1997;10:147-58.
    • (1997) J Aerosol Med , vol.10 , pp. 147-158
    • Hofmann, T.1    Senier, I.2    Bittner, P.3    Huls, G.4    Schwandt, H.J.5    Lindemann, H.6
  • 129
    • 6544255282 scopus 로고    scopus 로고
    • Benzamil and amiloride in CF nasal epithelium: Time course of the effects on nasal potential difference in vivo
    • Abstract
    • HOFMANN T, ZIERSCH A, SENIER I et al. : Benzamil and amiloride in CF nasal epithelium: Time course of the effects on nasal potential difference in vivo. Pediatr Pulmonol 1996;suppl 13:280 (Abstract).
    • (1996) Pediatr Pulmonol , Issue.13 SUPPL. , pp. 280
    • Hofmann, T.1    Ziersch, A.2    Senier, I.3
  • 130
    • 0029584796 scopus 로고
    • Effects of benzamil in human cystic fibrosis airway epithelium
    • BLANK U, CLAUSS W, WEBER WM : Effects of benzamil in human cystic fibrosis airway epithelium. Cell Physiol Biochem 1995;5:385-90.
    • (1995) Cell Physiol Biochem , vol.5 , pp. 385-390
    • Blank, U.1    Clauss, W.2    Weber, W.M.3
  • 131
    • 0025874599 scopus 로고
    • Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
    • KNOWLES MR, CLARKE LL, BOUCHER RC: Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med 1991;325:533-8.
    • (1991) N Engl J Med , vol.325 , pp. 533-538
    • Knowles, M.R.1    Clarke, L.L.2    Boucher, R.C.3
  • 133
    • 0030056228 scopus 로고    scopus 로고
    • Acute safety and effects on mucociliary clearance of aerosolized uridine 5′-triphosphate +/- Amiloride in normal human adults
    • OLIVIER KN, BENNETT WD, HOHNEKER KW et al. : Acute safety and effects on mucociliary clearance of aerosolized uridine 5′-triphosphate +/- amiloride in normal human adults. Am J Respir Crit Care Med 1996;154:217-23.
    • (1996) Am J Respir Crit Care Med , vol.154 , pp. 217-223
    • Olivier, K.N.1    Bennett, W.D.2    Hohneker, K.W.3
  • 135
    • 0029994529 scopus 로고    scopus 로고
    • Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations
    • HOWARD M, FRIZELL RA, BEDWELL DM : Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations. Nat Med 1996;2:467-9.
    • (1996) Nat Med , vol.2 , pp. 467-469
    • Howard, M.1    Frizell, R.A.2    Bedwell, D.M.3
  • 136
    • 6544245675 scopus 로고    scopus 로고
    • Gentamicin restores endogenous CFTR expression and function by suppressing premature stop mutations
    • BEDWELL D, KAENJAK A, BEBOK Z et al. : Gentamicin restores endogenous CFTR expression and function by suppressing premature stop mutations. Pediatr Pulmonol 1997;supp 14:A 233.
    • (1997) Pediatr Pulmonol , Issue.14 SUPPL.
    • Bedwell, D.1    Kaenjak, A.2    Bebok, Z.3
  • 137
    • 0013488281 scopus 로고    scopus 로고
    • Phenylbutyrate therapy for cystic fibrosis
    • ZEITLIN PL, RUBENSTEIN RC : Phenylbutyrate therapy for cystic fibrosis. Pediatr Pulmonol 1997;(supp 14):S84:132.
    • (1997) Pediatr Pulmonol , Issue.14 SUPPL.
    • Zeitlin, P.L.1    Rubenstein, R.C.2
  • 138
    • 0031889082 scopus 로고    scopus 로고
    • A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in ΔF508-homozygous cystic fibrosis patients. Partial restoration of nasal epithelial CFTR function
    • RUBENSTEIN RC AND ZEITLIN PL : A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in ΔF508-homozygous cystic fibrosis patients. Partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med 1998;157:484-90.
    • (1998) Am J Respir Crit Care Med , vol.157 , pp. 484-490
    • Rubenstein, R.C.1    Zeitlin, P.L.2
  • 139
    • 0030943895 scopus 로고    scopus 로고
    • CPX (8-cyclopentyl-1, 3-dipropylxanthine) and other alkyl-xanthines differentially bind to the wild-type and ΔF508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator
    • COHEN BE, LEE G, JACOBSON KA et al. : CPX (8-cyclopentyl-1, 3-dipropylxanthine) and other alkyl-xanthines differentially bind to the wild-type and ΔF508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator. Biochemistry 1997;36:6455-61.
    • (1997) Biochemistry , vol.36 , pp. 6455-6461
    • Cohen, B.E.1    Lee, G.2    Jacobson, K.A.3
  • 141
    • 0030900255 scopus 로고    scopus 로고
    • In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant ΔF508 in murine nasal epithelium
    • KELLY TJ, THOMAS KR, MILGRAM LJH, DRUMM ML : In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant ΔF508 in murine nasal epithelium. Proc Natl Acad Sci USA 1997;94:2604-8.
    • (1997) Proc Natl Acad Sci USA , vol.94 , pp. 2604-2608
    • Kelly, T.J.1    Thomas, K.R.2    Milgram, L.J.H.3    Drumm, M.L.4
  • 143
    • 0030775546 scopus 로고    scopus 로고
    • Induction by antitumoral drugs of proteins that functionally complement CFTR: A novel therapy for cystic fibrosis?
    • LALLEMAND JY, STOVEN V, ANNEREAU JP et al. : Induction by antitumoral drugs of proteins that functionally complement CFTR: a novel therapy for cystic fibrosis? Lancet 1997;350(9079):711-2.
    • (1997) Lancet , vol.350 , Issue.9079 , pp. 711-712
    • Lallemand, J.Y.1    Stoven, V.2    Annereau, J.P.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.