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Volumn 154, Issue 5, 1996, Pages 1229-1256

Cystic fibrosis

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBIOTIC AGENT; BRONCHODILATING AGENT; CHLORIDE; CHLORIDE CHANNEL; CORTICOSTEROID; FISH OIL; MEMBRANE PROTEIN; NONSTEROID ANTIINFLAMMATORY AGENT; PENTOXIFYLLINE; PROTEINASE INHIBITOR;

EID: 0029823833     PISSN: 1073449X     EISSN: None     Source Type: Journal    
DOI: 10.1164/ajrccm.154.5.8912731     Document Type: Review
Times cited : (844)

References (380)
  • 2
    • 0022970788 scopus 로고
    • Missing C1 conductance in cystic fibrosis
    • Quinton, P. M. 1986. Missing C1 conductance in cystic fibrosis. Am. J. Physiol. 251:C649-C652.
    • (1986) Am. J. Physiol. , vol.251
    • Quinton, P.M.1
  • 3
    • 0019809960 scopus 로고
    • Increased bioelectric potential differences across respiratory epithelial in cystic fibrosis
    • Knowles, M. R., J. Gatzy, and R. C. Boucher. 1981. Increased bioelectric potential differences across respiratory epithelial in cystic fibrosis. N. Engl. J. Med. 305:1489-1498.
    • (1981) N. Engl. J. Med. , vol.305 , pp. 1489-1498
    • Knowles, M.R.1    Gatzy, J.2    Boucher, R.C.3
  • 4
    • 0020610435 scopus 로고
    • Abnormal ion permeation through cystic fibrosis respiratory epithelium
    • Knowles, M. R., M. J. Stutts, A. Spock, N. Fischer, J. T. Gatzy, and R. C. Boucher. 1983. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 221:1067-1070.
    • (1983) Science , vol.221 , pp. 1067-1070
    • Knowles, M.R.1    Stutts, M.J.2    Spock, A.3    Fischer, N.4    Gatzy, J.T.5    Boucher, R.C.6
  • 9
    • 0004240703 scopus 로고
    • Cystic Fibrosis Foundation, Bethesda, Maryland
    • Cystic Fibrosis Foundation. 1995. Patient Registry 1994 Annual Data Report. Cystic Fibrosis Foundation, Bethesda, Maryland.
    • (1995) Patient Registry 1994 Annual Data Report
  • 10
    • 0027395872 scopus 로고
    • The changing epidemiology of cystic fibrosis
    • FitzSimmons, S. C. 1993. The changing epidemiology of cystic fibrosis. J. Pediatr. 122:1-9.
    • (1993) J. Pediatr. , vol.122 , pp. 1-9
    • FitzSimmons, S.C.1
  • 11
    • 0021269096 scopus 로고
    • Diagnosis and treatment of cystic fibrosis: An update
    • Davis, P. B., and P. A. di Sant'Agnese. 1984. Diagnosis and treatment of cystic fibrosis: an update. Chest 85:802-809.
    • (1984) Chest , vol.85 , pp. 802-809
    • Davis, P.B.1    Di Sant'Agnese, P.A.2
  • 12
    • 0018948906 scopus 로고
    • Low sweat electrolytes in patients with cystic fibrosis
    • Davis, P. B., V. S. Hubbard, and P. A. di Sant'Agnese. 1980. Low sweat electrolytes in patients with cystic fibrosis. Am. J. Med. 69:643-646.
    • (1980) Am. J. Med. , vol.69 , pp. 643-646
    • Davis, P.B.1    Hubbard, V.S.2    Di Sant'Agnese, P.A.3
  • 15
  • 16
    • 0016020543 scopus 로고
    • Cystic fibrosis in blacks in Washington DC: Incidence and characteristics
    • Kulczycki, L., and V. Schauf. 1974. Cystic fibrosis in blacks in Washington DC: incidence and characteristics. Am. J. Dis. Child. 127:64-67.
    • (1974) Am. J. Dis. Child. , vol.127 , pp. 64-67
    • Kulczycki, L.1    Schauf, V.2
  • 17
    • 0014259729 scopus 로고
    • Genetic studies on cystic fibrosis in Hawaii
    • Wright, S. W., and N. E. Morton. 1968. Genetic studies on cystic fibrosis in Hawaii. Am. J. Hum. Genet. 20:157-169.
    • (1968) Am. J. Hum. Genet. , vol.20 , pp. 157-169
    • Wright, S.W.1    Morton, N.E.2
  • 18
  • 19
    • 0024010591 scopus 로고
    • A long-range restriction map encompassing the cystic fibrosis locus and its closely linked genetic markers
    • Poustka, A. M., H. Lehrach, R. Williamson, and G. Bates. 1988. A long-range restriction map encompassing the cystic fibrosis locus and its closely linked genetic markers. Genomics 2:337-345.
    • (1988) Genomics , vol.2 , pp. 337-345
    • Poustka, A.M.1    Lehrach, H.2    Williamson, R.3    Bates, G.4
  • 20
    • 0024015509 scopus 로고
    • Physical mapping of the cystic fibrosis region by pulsed field gel electrophoresis
    • Drumm, M. L., C. L. Smith, M. Dean, J. L. Cole, M. C. Iannuzzi, and F. S. Collins. 1988. Physical mapping of the cystic fibrosis region by pulsed field gel electrophoresis. Genomics 2:346-354.
    • (1988) Genomics , vol.2 , pp. 346-354
    • Drumm, M.L.1    Smith, C.L.2    Dean, M.3    Cole, J.L.4    Iannuzzi, M.C.5    Collins, F.S.6
  • 21
    • 0024784387 scopus 로고
    • Physical localization of two DNA markers closely linked to the cystic fibrosis locus by pulsed-field gel electrophoresis
    • Rommens, J. M., S. Zengerling Lentes, B. Kerem, O. Melmer, M. Buchwald, and L. C. Tsui. 1989. Physical localization of two DNA markers closely linked to the cystic fibrosis locus by pulsed-field gel electrophoresis. Am. J. Hum. Genet. 45:932-941.
    • (1989) Am. J. Hum. Genet. , vol.45 , pp. 932-941
    • Rommens, J.M.1    Zengerling Lentes, S.2    Kerem, B.3    Melmer, O.4    Buchwald, M.5    Tsui, L.C.6
  • 22
    • 0023157065 scopus 로고
    • Construction of a general human chromosome jumping library, with application to cystic fibrosis
    • Collins, F. S., M. L. Drumm, J. L. Cole, W. K. Lockwood, G. F. Vande Woude, and M. C. Iannuzzi. 1987. Construction of a general human chromosome jumping library, with application to cystic fibrosis. Science 235:1046-1049.
    • (1987) Science , vol.235 , pp. 1046-1049
    • Collins, F.S.1    Drumm, M.L.2    Cole, J.L.3    Lockwood, W.K.4    Vande Woude, G.F.5    Iannuzzi, M.C.6
  • 23
    • 0025155528 scopus 로고
    • Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
    • Rich, D. P., M. P. Anderson, R. J. Gregory, S. H. Cheng, S. Paul, D. Jefferson, J. D. McCann, K. W. Klinger, A. E. Smith, and M. J. Welsh. 1990. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 347:358-363.
    • (1990) Nature , vol.347 , pp. 358-363
    • Rich, D.P.1    Anderson, M.P.2    Gregory, R.J.3    Cheng, S.H.4    Paul, S.5    Jefferson, D.6    McCann, J.D.7    Klinger, K.W.8    Smith, A.E.9    Welsh, M.J.10
  • 25
    • 0025776409 scopus 로고
    • The cystic fibrosis gene has a "housekeeping"-type promoter and is expressed at low levels in cells of epithelial origin
    • Yoshimura, K., H. Nakamura, B. C. Trapnell, W. Dalemans, A. Pavirani, J. P. Lecocq, and R. G. Crystal. 1991. The cystic fibrosis gene has a "housekeeping"-type promoter and is expressed at low levels in cells of epithelial origin. J. Biol. Chem. 266:9140-9144.
    • (1991) J. Biol. Chem. , vol.266 , pp. 9140-9144
    • Yoshimura, K.1    Nakamura, H.2    Trapnell, B.C.3    Dalemans, W.4    Pavirani, A.5    Lecocq, J.P.6    Crystal, R.G.7
  • 26
    • 0026641473 scopus 로고
    • Expression of the cystic fibrosis transmembrane conductance regulator gene can be regulated by protein kinase C
    • Bargon, J., B. C. Trapnell, K. Yoshimura, W. Dalemans, A. Pavirani, J. P. Lecocq, and R. G. Crystal. 1992. Expression of the cystic fibrosis transmembrane conductance regulator gene can be regulated by protein kinase C. J. Biol. Chem. 267:16056-16060.
    • (1992) J. Biol. Chem. , vol.267 , pp. 16056-16060
    • Bargon, J.1    Trapnell, B.C.2    Yoshimura, K.3    Dalemans, W.4    Pavirani, A.5    Lecocq, J.P.6    Crystal, R.G.7
  • 27
    • 0026327058 scopus 로고
    • Characterization of the promoter region of the cystic fibrosis transmembrane conductance regulator gene
    • Chou, J. L., R. Rozmahel, and L. C. Tsui. 1991. Characterization of the promoter region of the cystic fibrosis transmembrane conductance regulator gene. J. Biol. Chem. 266:24471-24476.
    • (1991) J. Biol. Chem. , vol.266 , pp. 24471-24476
    • Chou, J.L.1    Rozmahel, R.2    Tsui, L.C.3
  • 28
    • 0027185802 scopus 로고
    • Characterization of the cystic fibrosis transmembrane conductance regulator promoter region. Chromatin context and tissue-specificity
    • Koh, J., T. J. Sferra, and F. S. Collins. 1993. Characterization of the cystic fibrosis transmembrane conductance regulator promoter region. Chromatin context and tissue-specificity. J. Biol. Chem. 268: 15912-15921.
    • (1993) J. Biol. Chem. , vol.268 , pp. 15912-15921
    • Koh, J.1    Sferra, T.J.2    Collins, F.S.3
  • 29
    • 0026697598 scopus 로고
    • Induction of expression of the cystic fibrosis transmembrane conductance regulator
    • Breuer, W., N. Kartner, J. R. Riordan, and Z. I. Cabantchik. 1992. Induction of expression of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 267:10465-10469.
    • (1992) J. Biol. Chem. , vol.267 , pp. 10465-10469
    • Breuer, W.1    Kartner, N.2    Riordan, J.R.3    Cabantchik, Z.I.4
  • 31
    • 0027249601 scopus 로고
    • Cystic fibrosis transmembrane conductance regulator splice variants are not conserved and fail to produce chloride channels
    • Delaney, S. J., D. P. Rich, S. A. Thomson, M. R. Margrave, P. K. Lovelock, M. J. Welsh, and B. J. Wainwright. 1993. Cystic fibrosis transmembrane conductance regulator splice variants are not conserved and fail to produce chloride channels. Nat. Genet. 4:426-431.
    • (1993) Nat. Genet. , vol.4 , pp. 426-431
    • Delaney, S.J.1    Rich, D.P.2    Thomson, S.A.3    Margrave, M.R.4    Lovelock, P.K.5    Welsh, M.J.6    Wainwright, B.J.7
  • 32
    • 0026937999 scopus 로고
    • CFTR nonsense mutations G542X and W1282X associated with severe reduction of CFTR mRNA in nasal epithelial cells
    • Hamosh, A., B. J. Rosenstein, and G. R. Cutting. 1992. CFTR nonsense mutations G542X and W1282X associated with severe reduction of CFTR mRNA in nasal epithelial cells. Hum. Mol. Genet. 1:542-544.
    • (1992) Hum. Mol. Genet. , vol.1 , pp. 542-544
    • Hamosh, A.1    Rosenstein, B.J.2    Cutting, G.R.3
  • 34
    • 0025242929 scopus 로고
    • Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
    • Cheng, S. H., R. J. Gregory, J. Marshall, S. Paul, D. W. Souza, G. A. White, C. R. O'Riordan, and A. E. Smith. 1990. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell 63:827-834.
    • (1990) Cell , vol.63 , pp. 827-834
    • Cheng, S.H.1    Gregory, R.J.2    Marshall, J.3    Paul, S.4    Souza, D.W.5    White, G.A.6    O'Riordan, C.R.7    Smith, A.E.8
  • 35
    • 0026781952 scopus 로고
    • Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
    • Denning, G. M., M. P. Anderson, J. F. Amara, J. Marshall, A. E. Smith, and M. J. Welsh. 1992. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358:761-764.
    • (1992) Nature , vol.358 , pp. 761-764
    • Denning, G.M.1    Anderson, M.P.2    Amara, J.F.3    Marshall, J.4    Smith, A.E.5    Welsh, M.J.6
  • 36
    • 0030042386 scopus 로고    scopus 로고
    • Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
    • Sato, S., C. L. Ward, M. E. Krouse, J. J. Wine, and R. R. Kopito. 1996. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation. J. Biol. Chem. 271:635-638.
    • (1996) J. Biol. Chem. , vol.271 , pp. 635-638
    • Sato, S.1    Ward, C.L.2    Krouse, M.E.3    Wine, J.J.4    Kopito, R.R.5
  • 37
    • 0027483610 scopus 로고
    • The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR
    • Li, C., M. Ramjeesingh, E. Reyes, T. Jensen, X. Chang, J. M. Rommens, and C. E. Bear. 1993. The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR. Nat. Genet. 3:311-316.
    • (1993) Nat. Genet. , vol.3 , pp. 311-316
    • Li, C.1    Ramjeesingh, M.2    Reyes, E.3    Jensen, T.4    Chang, X.5    Rommens, J.M.6    Bear, C.E.7
  • 39
    • 0028929909 scopus 로고
    • Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency
    • Sheppard, D. N., L. S. Ostedgaard, M. C. Winter, and M. J. Welsh, 1995. Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency. E.M.B.O. J. 14: 876-883.
    • (1995) E.M.B.O. J. , vol.14 , pp. 876-883
    • Sheppard, D.N.1    Ostedgaard, L.S.2    Winter, M.C.3    Welsh, M.J.4
  • 41
    • 0028840915 scopus 로고
    • Degradation of CFTR by the ubiquitin-proteasome pathway
    • Ward, C. L., S. Omura, and R. R. Kopito. 1995. Degradation of CFTR by the ubiquitin-proteasome pathway. Cell 83:121-127.
    • (1995) Cell , vol.83 , pp. 121-127
    • Ward, C.L.1    Omura, S.2    Kopito, R.R.3
  • 42
    • 0028858161 scopus 로고
    • Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
    • Jensen, T. J., M. A. Loo, S. Pind, D. B. Williams, A. L. Goldberg, and J. R. Riordan. 1995. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 83:129-135.
    • (1995) Cell , vol.83 , pp. 129-135
    • Jensen, T.J.1    Loo, M.A.2    Pind, S.3    Williams, D.B.4    Goldberg, A.L.5    Riordan, J.R.6
  • 43
    • 0027488993 scopus 로고
    • The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
    • Yang, Y., S. Janich, J. A. Cohn, and J. M. Wilson. 1993. The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment. Proc. Natl. Acad. Sci. U.S.A. 90:9480-9484.
    • (1993) Proc. Natl. Acad. Sci. U.S.A. , vol.90 , pp. 9480-9484
    • Yang, Y.1    Janich, S.2    Cohn, J.A.3    Wilson, J.M.4
  • 44
    • 0028232167 scopus 로고
    • Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator
    • Pind, S., J. R. Riordan, and D. B. Williams. 1994. Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269:12784-12788.
    • (1994) J. Biol. Chem. , vol.269 , pp. 12784-12788
    • Pind, S.1    Riordan, J.R.2    Williams, D.B.3
  • 47
    • 0027162649 scopus 로고
    • Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
    • Welsh, M. J., and A. E. Smith. 1993. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 73:1251-1254.
    • (1993) Cell , vol.73 , pp. 1251-1254
    • Welsh, M.J.1    Smith, A.E.2
  • 49
    • 0020070795 scopus 로고
    • Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption
    • Gaskin, K., D. Gurwitz, P. Durie, M. Corey, H. Levison, and G. Forstner. 1982. Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. J. Pediatr. 100:857-862.
    • (1982) J. Pediatr. , vol.100 , pp. 857-862
    • Gaskin, K.1    Gurwitz, D.2    Durie, P.3    Corey, M.4    Levison, H.5    Forstner, G.6
  • 50
    • 0027502580 scopus 로고
    • Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA
    • Chu, C. S., B. C. Trapnell, S. Curristin, G. R. Cutting, and R. G. Crystal. 1993. Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA. Nat. Genet. 3:151-156.
    • (1993) Nat. Genet. , vol.3 , pp. 151-156
    • Chu, C.S.1    Trapnell, B.C.2    Curristin, S.3    Cutting, G.R.4    Crystal, R.G.5
  • 53
    • 0028062781 scopus 로고
    • Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model
    • Gabriel, S. E., K. N. Brigman, B. H. Koller, R. C. Boucher, and M. J. Stutts. 1994. Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model. Science 266:107-109.
    • (1994) Science , vol.266 , pp. 107-109
    • Gabriel, S.E.1    Brigman, K.N.2    Koller, B.H.3    Boucher, R.C.4    Stutts, M.J.5
  • 55
    • 0026678493 scopus 로고
    • Statement on cystic fibrosis carrier screening
    • American Society of Human Genetics. 1992. Statement on cystic fibrosis carrier screening. Am. J. Hum. Genet. 51:1443-1444.
    • (1992) Am. J. Hum. Genet. , vol.51 , pp. 1443-1444
  • 58
    • 0024694646 scopus 로고
    • Genetic analysis of DNA from single human oocytes: A model for preimplantation diagnosis of cystic fibrosis
    • Coutelle, C., C. Williams, A. Handyside, K. Hardy, R. Winston, and R. Williamson. 1989. Genetic analysis of DNA from single human oocytes: a model for preimplantation diagnosis of cystic fibrosis. B.M.J. 299:22-24.
    • (1989) B.M.J. , vol.299 , pp. 22-24
    • Coutelle, C.1    Williams, C.2    Handyside, A.3    Hardy, K.4    Winston, R.5    Williamson, R.6
  • 59
    • 0027162463 scopus 로고
    • Results of retroactive testing of human semen donors for cystic fibrosis and human immunodeficiency virus by polymerase chain reaction
    • Fugger, E. F., A. Maddalena, and J. D. Schulman. 1993. Results of retroactive testing of human semen donors for cystic fibrosis and human immunodeficiency virus by polymerase chain reaction. Hum. Reprod. 8:1435-1437.
    • (1993) Hum. Reprod. , vol.8 , pp. 1435-1437
    • Fugger, E.F.1    Maddalena, A.2    Schulman, J.D.3
  • 60
    • 0026532895 scopus 로고
    • Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)
    • Bear, C. C., H. Li, N. Kartner, R. J. Bridges, T. J. Jensen, M. Ramjeesingh, and J. R. Riordan. 1992. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR) Cell 68:809-819.
    • (1992) Cell , vol.68 , pp. 809-819
    • Bear, C.C.1    Li, H.2    Kartner, N.3    Bridges, R.J.4    Jensen, T.J.5    Ramjeesingh, M.6    Riordan, J.R.7
  • 63
    • 0026699623 scopus 로고
    • Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers
    • Tilly, B. C., M. C. Winter, L. S. Ostedgaard, C. O'Riordan, A. E. Smith, and M. J. Welsh. 1992. Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers. J. Biol. Chem. 267:9470-9473.
    • (1992) J. Biol. Chem. , vol.267 , pp. 9470-9473
    • Tilly, B.C.1    Winter, M.C.2    Ostedgaard, L.S.3    O'Riordan, C.4    Smith, A.E.5    Welsh, M.J.6
  • 64
    • 0026621245 scopus 로고
    • ABC transporters: From microorganisms to man
    • Higgins, C. F. 1992. ABC transporters: from microorganisms to man. Annu. Rev. Cell Biol. 8:67-113.
    • (1992) Annu. Rev. Cell Biol. , vol.8 , pp. 67-113
    • Higgins, C.F.1
  • 65
    • 0028241858 scopus 로고
    • Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion
    • Chang, X.-B., Y.-X. Hou, T. J. Jensen, and J. R. Riordan. 1994. Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion. J. Biol. Chem. 269: 18572-18575.
    • (1994) J. Biol. Chem. , vol.269 , pp. 18572-18575
    • Chang, X.-B.1    Hou, Y.-X.2    Jensen, T.J.3    Riordan, J.R.4
  • 67
    • 0028111941 scopus 로고
    • Novel pore-lining residues in CFTR that govern permeation and open-channel block
    • McDonough, S., N. Davidson, H. A. Lester, and N. A. McCarty. 1994. Novel pore-lining residues in CFTR that govern permeation and open-channel block. Neuron 13:623-634.
    • (1994) Neuron , vol.13 , pp. 623-634
    • McDonough, S.1    Davidson, N.2    Lester, H.A.3    McCarty, N.A.4
  • 68
    • 0028264188 scopus 로고
    • Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator
    • Akabas, M. H., C. Kaufmann, T. A. Cook, and P. Archdeacon. 1994. Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269:14865-14868.
    • (1994) J. Biol. Chem. , vol.269 , pp. 14865-14868
    • Akabas, M.H.1    Kaufmann, C.2    Cook, T.A.3    Archdeacon, P.4
  • 69
    • 0028058184 scopus 로고
    • Identification of an ion channel-forming motif in the primary structure of CFTR, the cystic fibrosis chloride channel
    • Oblatt-Montal, M., G. L. Reddy, T. Iwamoto, J. M. Tomich, and M. Montai. 1994. Identification of an ion channel-forming motif in the primary structure of CFTR, the cystic fibrosis chloride channel. Proc. Natl. Acad. Sci. U.S.A. 91:1495-1499.
    • (1994) Proc. Natl. Acad. Sci. U.S.A. , vol.91 , pp. 1495-1499
    • Oblatt-Montal, M.1    Reddy, G.L.2    Iwamoto, T.3    Tomich, J.M.4    Montai, M.5
  • 70
    • 0029063956 scopus 로고
    • Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator
    • Carroll, T. P., M. M. Morales, S. B. Fulmer, S. S. Allen, T. R. Flotte, G. R. Cutting, and W. B. Guggino. 1995. Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 270:11941-11946.
    • (1995) J. Biol. Chem. , vol.270 , pp. 11941-11946
    • Carroll, T.P.1    Morales, M.M.2    Fulmer, S.B.3    Allen, S.S.4    Flotte, T.R.5    Cutting, G.R.6    Guggino, W.B.7
  • 73
    • 0025931429 scopus 로고
    • Nucleoside triphosphates are required to open the CFTR chloride channel
    • Anderson, M. P., H. A. Berger, D. P. Rich, R. J. Gregory, A. E. Smith, and M. J. Welsh. 1991. Nucleoside triphosphates are required to open the CFTR chloride channel. Cell 67:775-784.
    • (1991) Cell , vol.67 , pp. 775-784
    • Anderson, M.P.1    Berger, H.A.2    Rich, D.P.3    Gregory, R.J.4    Smith, A.E.5    Welsh, M.J.6
  • 74
    • 0029113976 scopus 로고
    • The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase
    • Ko, Y. H., and P. L. Pedersen. 1995. The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase. J. Biol. Chem. 270:22093-22096.
    • (1995) J. Biol. Chem. , vol.270 , pp. 22093-22096
    • Ko, Y.H.1    Pedersen, P.L.2
  • 75
    • 0027364318 scopus 로고
    • Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator
    • Smit, L. S., D. J. Wilkinson, M. K. Mansoura, F. S. Collins, and D. C. Dawson. 1993. Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator. Proc. Natl. Acad. Sci. U.S.A. 90:9963-9967.
    • (1993) Proc. Natl. Acad. Sci. U.S.A. , vol.90 , pp. 9963-9967
    • Smit, L.S.1    Wilkinson, D.J.2    Mansoura, M.K.3    Collins, F.S.4    Dawson, D.C.5
  • 76
    • 0028070453 scopus 로고
    • Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating
    • Gunderson, K. L., and R. R. Kopito. 1994. Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating. J. Biol. Chem. 269:19349-19353.
    • (1994) J. Biol. Chem. , vol.269 , pp. 19349-19353
    • Gunderson, K.L.1    Kopito, R.R.2
  • 78
    • 0029871744 scopus 로고
    • Phosphorylation-dependent block of CFTR chloride channel by exogenous R domain protein
    • Ma, J., J. E. Tasch, T. Tao, J. Zhao, J. Xie, M. L. Drumm, and P. B. Davis. 1995. Phosphorylation-dependent block of CFTR chloride channel by exogenous R domain protein. J. Biol. Chem. 271: 7351-7356.
    • (1995) J. Biol. Chem. , vol.271 , pp. 7351-7356
    • Ma, J.1    Tasch, J.E.2    Tao, T.3    Zhao, J.4    Xie, J.5    Drumm, M.L.6    Davis, P.B.7
  • 80
    • 0025987020 scopus 로고
    • Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel
    • Cheng, S. H., D. P. Rich, J. Marshall, et al. 1991. Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel. Cell 66:1027-1036.
    • (1991) Cell , vol.66 , pp. 1027-1036
    • Cheng, S.H.1    Rich, D.P.2    Marshall, J.3
  • 81
    • 0027311276 scopus 로고
    • Protein kinase a (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites
    • Chang, X.-B., J. A. Tabcharani, Y.-X. Hou, T. J. Jensen, N. Kartner, N. Alon, J. W. Hanrahan, and J. R. Riordan. 1993. Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. J. Biol. Chem. 268: 11304-11311.
    • (1993) J. Biol. Chem. , vol.268 , pp. 11304-11311
    • Chang, X.-B.1    Tabcharani, J.A.2    Hou, Y.-X.3    Jensen, T.J.4    Kartner, N.5    Alon, N.6    Hanrahan, J.W.7    Riordan, J.R.8
  • 82
    • 0028906638 scopus 로고
    • cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue Ser-753 and its role in channel activation
    • Seibert, F. S., J. A. Tabcharani, X.-B. Chang, A. M. Dulhanty, C. Mathews, J. W. Hanrahan, and J. R. Riordan. 1995. cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue Ser-753 and its role in channel activation. J. Biol. Chem. 270:2158-2162.
    • (1995) J. Biol. Chem. , vol.270 , pp. 2158-2162
    • Seibert, F.S.1    Tabcharani, J.A.2    Chang, X.-B.3    Dulhanty, A.M.4    Mathews, C.5    Hanrahan, J.W.6    Riordan, J.R.7
  • 83
    • 0027319626 scopus 로고
    • Protein kinase C activates chloride conductance in C127 cells stably expressing the cystic fibrosis gene
    • Dechecchi, M. C., A. Tamanini, G. Berton, and G. Cabrini. 1993. Protein kinase C activates chloride conductance in C127 cells stably expressing the cystic fibrosis gene. J. Biol. Chem. 268:11321-11325.
    • (1993) J. Biol. Chem. , vol.268 , pp. 11321-11325
    • Dechecchi, M.C.1    Tamanini, A.2    Berton, G.3    Cabrini, G.4
  • 85
    • 0028858490 scopus 로고
    • Intracellular loop between TM4 and TM5 of CFTR is involved in regulation of chloride channel conductance
    • Xie, J., M. L. Drumm, J. Ma, and P. B. Davis. 1995. Intracellular loop between TM4 and TM5 of CFTR is involved in regulation of chloride channel conductance. J. Biol. Chem. 270:28084-28091.
    • (1995) J. Biol. Chem. , vol.270 , pp. 28084-28091
    • Xie, J.1    Drumm, M.L.2    Ma, J.3    Davis, P.B.4
  • 86
    • 0028980536 scopus 로고
    • CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP
    • Schwiebert, E. M., M. E. Egan, T.-H. Hwang, S. B. Fulmer, S. S. Allen, G. R. Cutting, and W. B. Guggino. 1995. CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 81:1063-1073.
    • (1995) Cell , vol.81 , pp. 1063-1073
    • Schwiebert, E.M.1    Egan, M.E.2    Hwang, T.-H.3    Fulmer, S.B.4    Allen, S.S.5    Cutting, G.R.6    Guggino, W.B.7
  • 89
    • 0022516747 scopus 로고
    • Altered regulation of airway epithelial cell chloride channels in cystic fibrosis
    • Frizzell, R. A., G. Rechkemmer, and R. L. Shoemaker. 1986. Altered regulation of airway epithelial cell chloride channels in cystic fibrosis. Science 233:558-560.
    • (1986) Science , vol.233 , pp. 558-560
    • Frizzell, R.A.1    Rechkemmer, G.2    Shoemaker, R.L.3
  • 90
    • 0023931773 scopus 로고
    • Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium
    • Li, M., J. D. McCann, C. M. Liedtke, A. C. Nairn, P. Greengard, and M. J. Welsh. 1988. Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium. Nature 331:358-360.
    • (1988) Nature , vol.331 , pp. 358-360
    • Li, M.1    McCann, J.D.2    Liedtke, C.M.3    Nairn, A.C.4    Greengard, P.5    Welsh, M.J.6
  • 91
    • 0027464248 scopus 로고
    • Outwardly rectifying chloride channels and CF: A divorce and remarriage
    • Guggino, W. B. 1993. Outwardly rectifying chloride channels and CF: a divorce and remarriage. J. Bioenerg. Biomembr. 25:27-35.
    • (1993) J. Bioenerg. Biomembr. , vol.25 , pp. 27-35
    • Guggino, W.B.1
  • 93
    • 0027191065 scopus 로고
    • CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship
    • Gabriel, S. E., L. L. Clarke, R. C. Boucher, and M. Jackson Stutts. 1993. CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship. Nature 363:263-266.
    • (1993) Nature , vol.363 , pp. 263-266
    • Gabriel, S.E.1    Clarke, L.L.2    Boucher, R.C.3    Jackson Stutts, M.4
  • 94
    • 0027966771 scopus 로고
    • Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents
    • Schwiebert, E. M., T. Flotte, G. R. Cutting, and W. B. Guggino. 1994. Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents. Am. J. Physiol. 266:C1464-C1477.
    • (1994) Am. J. Physiol. , vol.266
    • Schwiebert, E.M.1    Flotte, T.2    Cutting, G.R.3    Guggino, W.B.4
  • 96
    • 0028029015 scopus 로고
    • Multiple modes of regulation of airway epithelial chloride secretion by extracellular ATP
    • Stutts, M. J., J. G. Fitz, A. M. Paradiso, and R. C. Boucher. 1994. Multiple modes of regulation of airway epithelial chloride secretion by extracellular ATP. Am. J. Physiol. 267:C1442-C1451.
    • (1994) Am. J. Physiol. , vol.267
    • Stutts, M.J.1    Fitz, J.G.2    Paradiso, A.M.3    Boucher, R.C.4
  • 97
    • 0025874599 scopus 로고
    • Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
    • Knowles, M. R., L. L. Clarke, and R. C. Boucher. 1991. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N. Engl. J. Med. 325:533-538.
    • (1991) N. Engl. J. Med. , vol.325 , pp. 533-538
    • Knowles, M.R.1    Clarke, L.L.2    Boucher, R.C.3
  • 98
    • 0028047286 scopus 로고
    • Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in CFTR (-/-)mice
    • Clarke, L. L., B. R. Grubb, J. R. Yankaskas, C. U. Cotton, A. McKenzie, and R. C. Boucher. 1994. Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in CFTR (-/-)mice. Proc. Natl. Acad. Sci. U.S.A. 91:479-483.
    • (1994) Proc. Natl. Acad. Sci. U.S.A. , vol.91 , pp. 479-483
    • Clarke, L.L.1    Grubb, B.R.2    Yankaskas, J.R.3    Cotton, C.U.4    McKenzie, A.5    Boucher, R.C.6
  • 101
    • 0022973473 scopus 로고
    • + transport in cystic fibrosis respiratory epithelia: Abnormal basal rate and response to adenylate cyclase activation
    • + transport in cystic fibrosis respiratory epithelia: abnormal basal rate and response to adenylate cyclase activation. J. Clin. Invest. 78:1245-1252.
    • (1986) J. Clin. Invest. , vol.78 , pp. 1245-1252
    • Boucher, R.C.1    Stutts, M.J.2    Knowles, M.R.3    Cauthy, L.4    Gatzy, J.T.5
  • 106
    • 0026498086 scopus 로고
    • GTP-binding proteins inhibit cAMP activation of chloride channels in cystic fibrosis airway epithelial cells
    • Schwiebert, E. M., N. Kizer, D. C. Gruenert, and B. A. Stanton. 1992. GTP-binding proteins inhibit cAMP activation of chloride channels in cystic fibrosis airway epithelial cells. Proc. Natl. Acad. Sci. U.S.A. 89:10623-10627.
    • (1992) Proc. Natl. Acad. Sci. U.S.A. , vol.89 , pp. 10623-10627
    • Schwiebert, E.M.1    Kizer, N.2    Gruenert, D.C.3    Stanton, B.A.4
  • 107
    • 0029556721 scopus 로고
    • CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells
    • Kelley, T. J., L. Al-Nakkash, and M. L. Drumm. 1995. CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells. Am. J. Respir. Cell Mol. Biol. 13:657-664.
    • (1995) Am. J. Respir. Cell Mol. Biol. , vol.13 , pp. 657-664
    • Kelley, T.J.1    Al-Nakkash, L.2    Drumm, M.L.3
  • 110
    • 0026753172 scopus 로고
    • Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia
    • Denning, G. M., L. S. Ostedgaard, and M. J. Welsh. 1992. Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia. J. Cell Biol. 118:551-559.
    • (1992) J. Cell Biol. , vol.118 , pp. 551-559
    • Denning, G.M.1    Ostedgaard, L.S.2    Welsh, M.J.3
  • 112
    • 0025784535 scopus 로고
    • Defective acidification of intracellular organelles in cystic fibrosis
    • Barasch, J., B. Kiss, A. Prince, L. Saiman, D. Gruenert, and Q. AlAwqati. 1991. Defective acidification of intracellular organelles in cystic fibrosis. Nature 352:70-73.
    • (1991) Nature , vol.352 , pp. 70-73
    • Barasch, J.1    Kiss, B.2    Prince, A.3    Saiman, L.4    Gruenert, D.5    Alawqati, Q.6
  • 113
    • 0026949597 scopus 로고
    • Chloride channels of intracellular organelles and their potential role in cystic fibrosis
    • Al-Awqati, Q., J. Barasch, and D. Landry. 1992. Chloride channels of intracellular organelles and their potential role in cystic fibrosis. J. Exp. Biol. 172:245-266.
    • (1992) J. Exp. Biol. , vol.172 , pp. 245-266
    • Al-Awqati, Q.1    Barasch, J.2    Landry, D.3
  • 114
    • 0027763241 scopus 로고
    • Defective acidification of the biosynthetic pathway in cystic fibrosis
    • Barasch, J., and Q. Al-Awqati. 1993. Defective acidification of the biosynthetic pathway in cystic fibrosis. J. Cell Sci. 106 (Suppl. 17): 229-233.
    • (1993) J. Cell Sci. , vol.106 , Issue.17 SUPPL. , pp. 229-233
    • Barasch, J.1    Al-Awqati, Q.2
  • 116
    • 0008233958 scopus 로고
    • Immunolocalization of two sialyltransferases is altered in polarized LLC-PK1 epithelial cells expressing ΔF508 CFTR
    • Weyer, P., J. Barasch, Q. Al Awqati, D. A. Ausiello, and D. Brown. 1995. Immunolocalization of two sialyltransferases is altered in polarized LLC-PK1 epithelial cells expressing ΔF508 CFTR. Pediatr. Pulmonol. Suppl. 10:238.
    • (1995) Pediatr. Pulmonol. Suppl. , vol.10 , pp. 238
    • Weyer, P.1    Barasch, J.2    Al Awqati, Q.3    Ausiello, D.A.4    Brown, D.5
  • 117
    • 9244225677 scopus 로고
    • A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine iontophoresis
    • Gibson, L. E., and R. E. Cooke. 1959. A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine iontophoresis. Pediatrics 23:545-549.
    • (1959) Pediatrics , vol.23 , pp. 545-549
    • Gibson, L.E.1    Cooke, R.E.2
  • 118
    • 8944244636 scopus 로고
    • The heterozygote phenotype in cystic fibrosis
    • G. Mastellax and P. M. Quinton, editors. San Francisco Press, San Francisco
    • Wine, J. J. 1988. The heterozygote phenotype in cystic fibrosis. In G. Mastellax and P. M. Quinton, editors. Cellular and Molecular Basis of Cystic Fibrosis. San Francisco Press, San Francisco. 33-46.
    • (1988) Cellular and Molecular Basis of Cystic Fibrosis , pp. 33-46
    • Wine, J.J.1
  • 119
    • 84920211040 scopus 로고
    • Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas
    • diSant'Agnese, P. A., R. C. Darling, G. A. Perera, and E. Shea. 1953. Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 12:549-563.
    • (1953) Pediatrics , vol.12 , pp. 549-563
    • Disant'Agnese, P.A.1    Darling, R.C.2    Perera, G.A.3    Shea, E.4
  • 120
    • 0018341373 scopus 로고
    • Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis
    • Beckerman, R. C., and L. M. Taussig. 1979. Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis. Pediatrics 63: 580-583.
    • (1979) Pediatrics , vol.63 , pp. 580-583
    • Beckerman, R.C.1    Taussig, L.M.2
  • 121
    • 0015885904 scopus 로고
    • Water and electrolytes in cervical mucus from patients with CF
    • Kopito, L. E., H. J. Kosasky, and H. Shwachman. 1973. Water and electrolytes in cervical mucus from patients with CF. Fertil. Steril. 24:512-516.
    • (1973) Fertil. Steril. , vol.24 , pp. 512-516
    • Kopito, L.E.1    Kosasky, H.J.2    Shwachman, H.3
  • 123
    • 0018969932 scopus 로고
    • Cystic fibrosis and pregnancy: A national survey
    • Cohen, L. F., P. A. di Sant'Agnese, and J. Friedlander. 1980. Cystic fibrosis and pregnancy: a national survey. Lancet 2:842-844.
    • (1980) Lancet , vol.2 , pp. 842-844
    • Cohen, L.F.1    Di Sant'Agnese, P.A.2    Friedlander, J.3
  • 125
    • 0007503391 scopus 로고
    • CF and the reproductive system
    • P. B. Davis, editor. Marcel Dekker, New York
    • Stern, R. C. 1993. CF and the reproductive system. In P. B. Davis, editor. Cystic Fibrosis. Marcel Dekker, New York. 381-400.
    • (1993) Cystic Fibrosis , pp. 381-400
    • Stern, R.C.1
  • 126
    • 0023639146 scopus 로고
    • Clinical features, survival rate, and prognostic factors in young adults with cystic fibrosis
    • Yeung, W., K. Hardy, L. Quittell, and S. Fiel. 1987. Clinical features, survival rate, and prognostic factors in young adults with cystic fibrosis. Am. J. Med. 82:871-879.
    • (1987) Am. J. Med. , vol.82 , pp. 871-879
    • Yeung, W.1    Hardy, K.2    Quittell, L.3    Fiel, S.4
  • 128
    • 0025606440 scopus 로고
    • Congenital absence of the vas deferens: The fertilizing capacity of human epididymal sperm
    • Silber, S. J., T. Ord, J. Balmeceda, P. Patrizio, and R. H. Asch. 1990. Congenital absence of the vas deferens: the fertilizing capacity of human epididymal sperm. N. Engl. J. Med. 323:1788-1792.
    • (1990) N. Engl. J. Med. , vol.323 , pp. 1788-1792
    • Silber, S.J.1    Ord, T.2    Balmeceda, J.3    Patrizio, P.4    Asch, R.H.5
  • 129
    • 0042150584 scopus 로고
    • CF and the gastrointestinal tract
    • P. B. Davis, editor. Marcel Dekker, New York
    • Stern, R. C. 1993. CF and the gastrointestinal tract. In P. B. Davis, editor. Cystic Fibrosis. Marcel Dekker, New York. 401-434.
    • (1993) Cystic Fibrosis , pp. 401-434
    • Stern, R.C.1
  • 130
    • 0017389424 scopus 로고
    • Meconium ileus equivalent complicating cystic fibrosis in postneonatal children and young adults
    • Matseshe, J. W., V. L. W. Go, and E. P. DiMagno. 1977. Meconium ileus equivalent complicating cystic fibrosis in postneonatal children and young adults. Gastroenterology 72:732-736.
    • (1977) Gastroenterology , vol.72 , pp. 732-736
    • Matseshe, J.W.1    Go, V.L.W.2    Dimagno, E.P.3
  • 132
    • 0028557095 scopus 로고
    • Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR
    • Zhou, L., C. R. Dey, S. E. Wert, M. D. Duvall, R. A. Frizzell, and J. A. Whitsett. 1994. Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. Science 266:1705-1708.
    • (1994) Science , vol.266 , pp. 1705-1708
    • Zhou, L.1    Dey, C.R.2    Wert, S.E.3    Duvall, M.D.4    Frizzell, R.A.5    Whitsett, J.A.6
  • 133
    • 0002121738 scopus 로고
    • The pathology of cystic fibrosis
    • P. B. Davis, editor. Marcel Dekker, New York
    • Tomashefski, J. F., C. R. Abramowsky, and B. B. Dahms. 1993. The pathology of cystic fibrosis. In P. B. Davis, editor. Cystic Fibrosis. Marcel Dekker, New York. 435-590.
    • (1993) Cystic Fibrosis , pp. 435-590
    • Tomashefski, J.F.1    Abramowsky, C.R.2    Dahms, B.B.3
  • 134
    • 0024102578 scopus 로고
    • Epithelial mucous glycoproteins and cystic fibrosis
    • Rose, M. C. 1988. Epithelial mucous glycoproteins and cystic fibrosis. Horm. Metab. Res. 20:601-608.
    • (1988) Horm. Metab. Res. , vol.20 , pp. 601-608
    • Rose, M.C.1
  • 135
    • 0018414093 scopus 로고
    • Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature
    • diSant'Agnese, P. A., and P. B. Davis. 1979. Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature. Am. J. Med. 66:121-132.
    • (1979) Am. J. Med. , vol.66 , pp. 121-132
    • DiSant'Agnese, P.A.1    Davis, P.B.2
  • 136
    • 0023635416 scopus 로고
    • Meconium ileus: A fifteen-year experience with forty-two neonates
    • Caniano, D. A., and B. L. Beaver. 1987. Meconium ileus: a fifteen-year experience with forty-two neonates. Surgery 102:699-703.
    • (1987) Surgery , vol.102 , pp. 699-703
    • Caniano, D.A.1    Beaver, B.L.2
  • 137
    • 0022475078 scopus 로고
    • Constipation and meconium ileus equivalent in patients with cystic fibrosis
    • Rubinstein, S., R. Moss, and N. Lewiston. 1986. Constipation and meconium ileus equivalent in patients with cystic fibrosis. Pediatrics 78:473-479.
    • (1986) Pediatrics , vol.78 , pp. 473-479
    • Rubinstein, S.1    Moss, R.2    Lewiston, N.3
  • 138
    • 0022630398 scopus 로고
    • Treatment of distal intestinal obstruction in cystic fibrosis with a balanced intestinal lavage solution
    • Cleghorn, G. J., D. A. Stringer, G. G. Forstner, and P. R. Durie. 1986. Treatment of distal intestinal obstruction in cystic fibrosis with a balanced intestinal lavage solution. Lancet 1:8-11.
    • (1986) Lancet , vol.1 , pp. 8-11
    • Cleghorn, G.J.1    Stringer, D.A.2    Forstner, G.G.3    Durie, P.R.4
  • 139
    • 0000469116 scopus 로고
    • The pathological changes in the liver in cystic fibrosis of the pancreas
    • Craig, J. M., H. Haddad, and H. Shwachman. 1957. The pathological changes in the liver in cystic fibrosis of the pancreas. Ant. J. Dis. Child. 93:357-369.
    • (1957) Ant. J. Dis. Child. , vol.93 , pp. 357-369
    • Craig, J.M.1    Haddad, H.2    Shwachman, H.3
  • 140
    • 0017236920 scopus 로고
    • Symptomatic hepatic disease in cystic fibrosis: Incidence, course, and outcome of portal systemic shunting
    • Stern, R. C., D. P. Stevens, T. F. Boat, C. F. Doershuk, R. J. Izant, Jr., and L. W. Matthews. 1976. Symptomatic hepatic disease in cystic fibrosis: incidence, course, and outcome of portal systemic shunting. Gastroenterology 70:645-649.
    • (1976) Gastroenterology , vol.70 , pp. 645-649
    • Stern, R.C.1    Stevens, D.P.2    Boat, T.F.3    Doershuk, C.F.4    Izant Jr., R.J.5    Matthews, L.W.6
  • 142
    • 0022898201 scopus 로고
    • Treatment and prognosis of symptomatic gallbladder disease in patients with cystic fibrosis
    • Stern, R. C., F. C. Rothstein, and C. F. Doershuk. 1986. Treatment and prognosis of symptomatic gallbladder disease in patients with cystic fibrosis. J. Pediatr. Gastroenterol. Nutr. 5:35-40.
    • (1986) J. Pediatr. Gastroenterol. Nutr. , vol.5 , pp. 35-40
    • Stern, R.C.1    Rothstein, F.C.2    Doershuk, C.F.3
  • 144
    • 0025180712 scopus 로고
    • Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis
    • Cotting, J., M. J. Lentze, and J. Reichen. 1990. Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis. Gut 31:918-921.
    • (1990) Gut , vol.31 , pp. 918-921
    • Cotting, J.1    Lentze, M.J.2    Reichen, J.3
  • 145
    • 0026718322 scopus 로고
    • Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chronic cholestasis
    • Galabert, C., J. C. Montel, D. Lengrand, A. Lecuire, C. Sotta, C. Figarella, and J. P. Chazalette. 1992. Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chronic cholestasis. J. Pediatr. 121:138-141.
    • (1992) J. Pediatr. , vol.121 , pp. 138-141
    • Galabert, C.1    Montel, J.C.2    Lengrand, D.3    Lecuire, A.4    Sotta, C.5    Figarella, C.6    Chazalette, J.P.7
  • 146
    • 0014428204 scopus 로고
    • Pancreozymin secretin test of exocrine pancreatic function in cystic fibrosis and the significance of the result for the pathogenesis of the disease
    • Hadorn, B., P. G. Johansen, and C. M. Anderson. 1968. Pancreozymin secretin test of exocrine pancreatic function in cystic fibrosis and the significance of the result for the pathogenesis of the disease. Can. Med. Assoc. J. 98:377-385.
    • (1968) Can. Med. Assoc. J. , vol.98 , pp. 377-385
    • Hadorn, B.1    Johansen, P.G.2    Anderson, C.M.3
  • 147
    • 0021917723 scopus 로고
    • Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis
    • Kopelman, H., P. Durie, K. Gaskin, Z. Weizman, and G. Forstner. 1985. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N. Engl. J. Med. 312:329-334.
    • (1985) N. Engl. J. Med. , vol.312 , pp. 329-334
    • Kopelman, H.1    Durie, P.2    Gaskin, K.3    Weizman, Z.4    Forstner, G.5
  • 148
    • 0023719216 scopus 로고
    • Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas
    • Kopelman, H., M. Corey, K. Gaskin, P. Durie, Z. Weizman, and G. Forstner. 1988. Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas. Gastroenterology 95:349-355.
    • (1988) Gastroenterology , vol.95 , pp. 349-355
    • Kopelman, H.1    Corey, M.2    Gaskin, K.3    Durie, P.4    Weizman, Z.5    Forstner, G.6
  • 149
    • 0041857366 scopus 로고
    • The pancreas in cystic fibrosis
    • P. B. Davis, editor. Marcel Dekker, New York
    • Cotton, C. U., and P. B. Davis. 1993. The pancreas in cystic fibrosis. In P. B. Davis, editor. Cystic Fibrosis. Marcel Dekker, New York. 161-192.
    • (1993) Cystic Fibrosis , pp. 161-192
    • Cotton, C.U.1    Davis, P.B.2
  • 150
    • 0026505294 scopus 로고
    • Pancreatitis in young children with cystic fibrosis
    • Atlas, A. B., S. R. Orenstein, and D. M. Orenstein. 1992. Pancreatitis in young children with cystic fibrosis. J. Pediatr. 120:756-759.
    • (1992) J. Pediatr. , vol.120 , pp. 756-759
    • Atlas, A.B.1    Orenstein, S.R.2    Orenstein, D.M.3
  • 152
    • 0017886011 scopus 로고
    • Diagnostic tests of exocrine pancreatic function and disease
    • Arvanitakis, C., and A. R. Cooke. 1978. Diagnostic tests of exocrine pancreatic function and disease. Gastroenterology 74:932-948.
    • (1978) Gastroenterology , vol.74 , pp. 932-948
    • Arvanitakis, C.1    Cooke, A.R.2
  • 153
    • 0015959133 scopus 로고
    • Steatorrhea and azotorrhea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis
    • Lapey, A., J. Kattwinkel, P. A. diSant Agnese, and L. Lester. 1974. Steatorrhea and azotorrhea and their relation to growth and nutrition in adolescents and young adults with cystic fibrosis. J. Pediatr. 84:328-334.
    • (1974) J. Pediatr. , vol.84 , pp. 328-334
    • Lapey, A.1    Kattwinkel, J.2    Disant Agnese, P.A.3    Lester, L.4
  • 154
    • 0017810446 scopus 로고
    • Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by the synthetic peptide N-benzoyl-L-tyrosyl-p-aminobenzoic acid
    • Nousia-Arvanitakis, S., C. Arvanitakis, N. Desai, and N. J. Greenberger. 1978. Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by the synthetic peptide N-benzoyl-L-tyrosyl-p-aminobenzoic acid. J. Pediatr. 92:734-737.
    • (1978) J. Pediatr. , vol.92 , pp. 734-737
    • Nousia-Arvanitakis, S.1    Arvanitakis, C.2    Desai, N.3    Greenberger, N.J.4
  • 156
    • 0022005610 scopus 로고
    • Effect of cimetidine on enzyme inactivation, bile acid precipitation, and lipid solubilisation in pancreatic steatorrhoea due to cystic fibrosis
    • Zentler-Munro, P. L., D. R. Fine, J. C. Batten, and T. C. Northfield. 1985. Effect of cimetidine on enzyme inactivation, bile acid precipitation, and lipid solubilisation in pancreatic steatorrhoea due to cystic fibrosis. Gut 26:892-901.
    • (1985) Gut , vol.26 , pp. 892-901
    • Zentler-Munro, P.L.1    Fine, D.R.2    Batten, J.C.3    Northfield, T.C.4
  • 157
    • 0028140481 scopus 로고
    • Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes
    • Smyth, R. L., D. Van Velzen, A. R. Smyth, D. A. Lloyd, and D. P. Heaf. 1994. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes. Lancet 343:35-86.
    • (1994) Lancet , vol.343 , pp. 35-86
    • Smyth, R.L.1    Van Velzen, D.2    Smyth, A.R.3    Lloyd, D.A.4    Heaf, D.P.5
  • 158
    • 0026541404 scopus 로고
    • Nutritional assessment and management in cystic fibrosis: A consensus report
    • Ramsey, B. W., P. M. Farrell, P. Pencharz, and The Consensus Committee. 1992. Nutritional assessment and management in cystic fibrosis: a consensus report. Am. J. Clin. Nutr. 55:108-116.
    • (1992) Am. J. Clin. Nutr. , vol.55 , pp. 108-116
    • Ramsey, B.W.1    Farrell, P.M.2    Pencharz, P.3
  • 159
    • 84995267330 scopus 로고
    • Relationship between clinical parameters and linear growth in children with cystic fibrosis
    • Byard, P. J. 1989. Relationship between clinical parameters and linear growth in children with cystic fibrosis. Am. J. Hum. Biol. 1: 719-725.
    • (1989) Am. J. Hum. Biol. , vol.1 , pp. 719-725
    • Byard, P.J.1
  • 160
    • 0028120918 scopus 로고
    • Severe hemolytic anemia associated with vitamin E deficiency in infants with cystic fibrosis: Implications for neonatal screening
    • Wilfond, B. S., P. M. Farrell, A. Laxova, and E. Mischler. 1994. Severe hemolytic anemia associated with vitamin E deficiency in infants with cystic fibrosis: implications for neonatal screening. Clin. Pediatr. 33:2-7.
    • (1994) Clin. Pediatr. , vol.33 , pp. 2-7
    • Wilfond, B.S.1    Farrell, P.M.2    Laxova, A.3    Mischler, E.4
  • 161
    • 0015278141 scopus 로고
    • Blood and liver concentrations of vitamin A and E in children with cystic fibrosis of the pancreas
    • Underwood, B. A., and C. R. Denning. 1972. Blood and liver concentrations of vitamin A and E in children with cystic fibrosis of the pancreas. Pediatr. Res. 6:26.
    • (1972) Pediatr. Res. , vol.6 , pp. 26
    • Underwood, B.A.1    Denning, C.R.2
  • 163
    • 0014783317 scopus 로고
    • Cystic fibrosis presenting with severe hemorrhage due to vitamin K malabsorption
    • Tortstenson, O. L., F. B. Humphrey, J. R. Edson, and W. J. Warwick. 1970. Cystic fibrosis presenting with severe hemorrhage due to vitamin K malabsorption. Pediatrics 45:857.
    • (1970) Pediatrics , vol.45 , pp. 857
    • Tortstenson, O.L.1    Humphrey, F.B.2    Edson, J.R.3    Warwick, W.J.4
  • 164
    • 0017681688 scopus 로고
    • The occurrence and effects of human vitamin E deficiency. A study in patients with cystic fibrosis
    • Farrell, P. M., J. G. Bieri, J. F. Fratantoni, R. E. Wood, and P. A. di Sant'Agnese. 1977. The occurrence and effects of human vitamin E deficiency. A study in patients with cystic fibrosis. J. Clin. Invest. 60:233-241.
    • (1977) J. Clin. Invest. , vol.60 , pp. 233-241
    • Farrell, P.M.1    Bieri, J.G.2    Fratantoni, J.F.3    Wood, R.E.4    Di Sant'Agnese, P.A.5
  • 165
    • 0027309043 scopus 로고
    • Correction of vitamin E deficiency with fat-soluble versus water-miscible preparations of vitamin E in patients with cystic fibrosis
    • Nasr, S. Z., M. H. O'Leary, and C. Hillermeier. 1993. Correction of
    • (1993) J. Pediatr. , vol.122 , pp. 810-812
    • Nasr, S.Z.1    O'Leary, M.H.2    Hillermeier, C.3
  • 166
    • 0017595111 scopus 로고
    • Pancreatic alpha and beta cell functions in cystic fibrosis
    • Lippe, B. M., M. A. Sperling, and R. R. Dooley. 1977. Pancreatic alpha and beta cell functions in cystic fibrosis. J. Pediatr. 90:751-755.
    • (1977) J. Pediatr. , vol.90 , pp. 751-755
    • Lippe, B.M.1    Sperling, M.A.2    Dooley, R.R.3
  • 169
    • 0028221370 scopus 로고
    • Erosion of the wall of the frontal sinus caused by mucopyocele in cystic fibrosis
    • Sharma, G. D., C. F. Doershuk, and R. C. Stern. 1994. Erosion of the wall of the frontal sinus caused by mucopyocele in cystic fibrosis. J. Pediatr. 124:745-747.
    • (1994) J. Pediatr. , vol.124 , pp. 745-747
    • Sharma, G.D.1    Doershuk, C.F.2    Stern, R.C.3
  • 170
    • 0020354838 scopus 로고
    • Bacteriology of the maxillary sinuses in patients with cystic fibrosis
    • Shapiro, E. D., G. J. Milmoe, E. R. Wald, J. B. Rodnan, and A. Bowen. 1982. Bacteriology of the maxillary sinuses in patients with cystic fibrosis. J. Infect. Dis. 146:589-593.
    • (1982) J. Infect. Dis. , vol.146 , pp. 589-593
    • Shapiro, E.D.1    Milmoe, G.J.2    Wald, E.R.3    Rodnan, J.B.4    Bowen, A.5
  • 171
    • 0029024142 scopus 로고
    • Management of sinusitis in cystic fibrosis by endoscopie surgery and serial antimicrobial lavage. Reduction in recurrence requiring surgery
    • Moss, R. B., and V. V. King. 1995. Management of sinusitis in cystic fibrosis by endoscopie surgery and serial antimicrobial lavage. Reduction in recurrence requiring surgery. Arch. Otolaryngol. Head Neck Surg. 121:566-572.
    • (1995) Arch. Otolaryngol. Head Neck Surg. , vol.121 , pp. 566-572
    • Moss, R.B.1    King, V.V.2
  • 175
    • 10544244475 scopus 로고
    • Nasal polyposis and cystic fibrosis
    • di Sant'Agnese, P. A. 1965. Nasal polyposis and cystic fibrosis. J.A.M.A. 191:685.
    • (1965) J.A.M.A. , vol.191 , pp. 685
    • Di Sant'Agnese, P.A.1
  • 176
    • 0000169480 scopus 로고
    • Nasal polyps and cystic fibrosis of the pancreas
    • Rulon, J. T., H. A. Brown, and G. B. Logan. 1963. Nasal polyps and cystic fibrosis of the pancreas. Arch. Otolaryngol. 78:192-199.
    • (1963) Arch. Otolaryngol. , vol.78 , pp. 192-199
    • Rulon, J.T.1    Brown, H.A.2    Logan, G.B.3
  • 177
    • 0018359699 scopus 로고
    • Differential pathology of nasal polyps in cystic fibrosis and atopy
    • Oppenheimer, E. H., and B. J. Rosenstein. 1979. Differential pathology of nasal polyps in cystic fibrosis and atopy. Lab. Invest. 40: 445-449.
    • (1979) Lab. Invest. , vol.40 , pp. 445-449
    • Oppenheimer, E.H.1    Rosenstein, B.J.2
  • 179
    • 0020082418 scopus 로고
    • Quantitative evaluation of the development of tracheal submucosal glands in infants with cystic fibrosis and control infants
    • Sturgess, J., and J. Imrie. 1982. Quantitative evaluation of the development of tracheal submucosal glands in infants with cystic fibrosis and control infants. Am. J. Pathol. 106:303-311.
    • (1982) Am. J. Pathol. , vol.106 , pp. 303-311
    • Sturgess, J.1    Imrie, J.2
  • 180
    • 0026698816 scopus 로고
    • Localization of cystic fibrosis transmembrane conductance regulator mRNA in human fetal lung tissue by in situ hybridization
    • McCray, P. B., Jr., C. L. Wohlford-Lenane, and J. M. Snyder. 1992. Localization of cystic fibrosis transmembrane conductance regulator mRNA in human fetal lung tissue by in situ hybridization. J. Clin. Invest. 90:619-625.
    • (1992) J. Clin. Invest. , vol.90 , pp. 619-625
    • McCray Jr., P.B.1    Wohlford-Lenane, C.L.2    Snyder, J.M.3
  • 181
    • 0027546872 scopus 로고
    • Cystic fibrosis gene and protein expression during fetal lung development
    • McGrath, S. A., A. Basu, and P. L. Zeitlin. 1993. Cystic fibrosis gene and protein expression during fetal lung development. Am. J. Respir. Cell Mol. Biol. 8:201-208.
    • (1993) Am. J. Respir. Cell Mol. Biol. , vol.8 , pp. 201-208
    • McGrath, S.A.1    Basu, A.2    Zeitlin, P.L.3
  • 183
    • 0016828249 scopus 로고
    • Prevalence of mucoid strains of Pseudomonas aeruginosa in bacteriological specimens from patients with other diseases
    • Hoiby, N. 1975. Prevalence of mucoid strains of Pseudomonas aeruginosa in bacteriological specimens from patients with other diseases. Acta Pathol. Microbiol. Scand. 83:549-552.
    • (1975) Acta Pathol. Microbiol. Scand. , vol.83 , pp. 549-552
    • Hoiby, N.1
  • 184
    • 0017141756 scopus 로고
    • Mucoid Pseudomonas aeruginosa: A sign of cystic fibrosis in young adults with chronic pulmonary disease?
    • Reynolds, H. Y., P. A. di Sant'Agnese, and C. H. Zierdt. 1976. Mucoid Pseudomonas aeruginosa: a sign of cystic fibrosis in young adults with chronic pulmonary disease? J.A.M.A. 236:2190-2192.
    • (1976) J.A.M.A. , vol.236 , pp. 2190-2192
    • Reynolds, H.Y.1    Di Sant'Agnese, P.A.2    Zierdt, C.H.3
  • 185
    • 0025374923 scopus 로고
    • Neutrophil elastase cleaves C3bi on opsonized Pseudomonas as well as CR1 on neutrophils to create a functionally important opsonin receptor mismatch
    • Tosi, M. F., H. Zakem, and M. Berger. 1990. Neutrophil elastase cleaves C3bi on opsonized Pseudomonas as well as CR1 on neutrophils to create a functionally important opsonin receptor mismatch. J. Clin. Invest. 86:300-308.
    • (1990) J. Clin. Invest. , vol.86 , pp. 300-308
    • Tosi, M.F.1    Zakem, H.2    Berger, M.3
  • 186
    • 0024459145 scopus 로고
    • Complement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infecled lung in cystic fibrosis
    • Berger, M., R. U. Sorensen, M. F. Tosi, D. G. Dearborn, and G. Doring. 1989. Complement receptor expression on neutrophils at an inflammatory site, the Pseudomonas-infecled lung in cystic fibrosis. J. Clin. Invest. 84:1302-1313.
    • (1989) J. Clin. Invest. , vol.84 , pp. 1302-1313
    • Berger, M.1    Sorensen, R.U.2    Tosi, M.F.3    Dearborn, D.G.4    Doring, G.5
  • 187
    • 0021239746 scopus 로고
    • Proteins of the cystic fibrosis respiratory tract. Fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosis
    • Fick, R. B., Jr., G. P. Nagel, S. U. Squier, R. E. Wood, J. B. L. Gee, and H. Y. Reynolds. 1984. Proteins of the cystic fibrosis respiratory tract. Fragmented immunoglobulin G opsonic antibody causing defective opsonophagocytosis. J. Clin. Invest. 74:236-248.
    • (1984) J. Clin. Invest. , vol.74 , pp. 236-248
    • Fick Jr., R.B.1    Nagel, G.P.2    Squier, S.U.3    Wood, R.E.4    Gee, J.B.L.5    Reynolds, H.Y.6
  • 188
    • 0001278692 scopus 로고
    • Infection and inflammation of the lung in cystic fibrosis
    • P. B. Davis, editor. Marcel Dekker, New York
    • Konstan, M. W., and M. Berger. 1993. Infection and inflammation of the lung in cystic fibrosis. In P. B. Davis, editor. Cystic Fibrosis. Marcel Dekker, New York. 219-276.
    • (1993) Cystic Fibrosis , pp. 219-276
    • Konstan, M.W.1    Berger, M.2
  • 189
    • 0028350324 scopus 로고
    • Defective fluid transport by cystic fibrosis airway epithelia
    • Smith, J. J., P. H. Karp, and M. J. Welsh. 1994. Defective fluid transport by cystic fibrosis airway epithelia. J. Clin. Invest. 93:1307-1311.
    • (1994) J. Clin. Invest. , vol.93 , pp. 1307-1311
    • Smith, J.J.1    Karp, P.H.2    Welsh, M.J.3
  • 191
    • 0018944986 scopus 로고
    • The immotile cilia syndrome: A cause of recurrent pulmonary disease in children
    • Rooklin, A. R., S. J. McGeady, D. O. Mikaelian, R. Z. Soriano, and H. C. Mansmann. 1980. The immotile cilia syndrome: a cause of recurrent pulmonary disease in children. Pediatrics 66:526-531.
    • (1980) Pediatrics , vol.66 , pp. 526-531
    • Rooklin, A.R.1    McGeady, S.J.2    Mikaelian, D.O.3    Soriano, R.Z.4    Mansmann, H.C.5
  • 192
    • 0018305742 scopus 로고
    • Kartagener's syndrome and the syndrome of immotile cilia
    • Ron, H.-D. 1979. Kartagener's syndrome and the syndrome of immotile cilia. Hum. Genet. 46:249-261.
    • (1979) Hum. Genet. , vol.46 , pp. 249-261
    • Ron, H.-D.1
  • 193
    • 0015437286 scopus 로고
    • Kartagener's syndrome
    • Miller, R. D., and M. B. Divertie. 1972. Kartagener's syndrome. Chest 62:130-135.
    • (1972) Chest , vol.62 , pp. 130-135
    • Miller, R.D.1    Divertie, M.B.2
  • 194
    • 0016793255 scopus 로고
    • Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline
    • Wood, R. E., A. Wanner, J. Hirch, and P. M. Farrell. 1975. Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline. Am. Rev. Respir. Dis. 111:733-738.
    • (1975) Am. Rev. Respir. Dis. , vol.111 , pp. 733-738
    • Wood, R.E.1    Wanner, A.2    Hirch, J.3    Farrell, P.M.4
  • 197
    • 0026681802 scopus 로고
    • Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line
    • Nakamura, H., K. Yoshimura, N. G. McElvaney, and R. G. Crystal. 1992. Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line. J. Clin. Invest. 89:1478-1484.
    • (1992) J. Clin. Invest. , vol.89 , pp. 1478-1484
    • Nakamura, H.1    Yoshimura, K.2    McElvaney, N.G.3    Crystal, R.G.4
  • 198
    • 0021783739 scopus 로고
    • Biochemical and pathologie evidence for proteolytic destruction of lung connective tissue in cystic fibrosis
    • Bruce, M. C., L. Poncz, J. D. Klinger, R. C. Stern, J. F. Tomashefski, Jr., and D. G. Dearborn. 1985. Biochemical and pathologie evidence for proteolytic destruction of lung connective tissue in cystic fibrosis. Am. Rev. Respir. Dis. 132:529-535.
    • (1985) Am. Rev. Respir. Dis. , vol.132 , pp. 529-535
    • Bruce, M.C.1    Poncz, L.2    Klinger, J.D.3    Stern, R.C.4    Tomashefski Jr., J.F.5    Dearborn, D.G.6
  • 199
    • 0025228983 scopus 로고
    • Neutrophil elastase and cathepsin G stimulate secretion from cultured bovine airway gland serous cells
    • Sommerhoff, C. P., J. A. Nadel, C. B. Basbaum, and G. H. Caughey. 1990. Neutrophil elastase and cathepsin G stimulate secretion from cultured bovine airway gland serous cells. J. Clin. Invest. 85:682-689.
    • (1990) J. Clin. Invest. , vol.85 , pp. 682-689
    • Sommerhoff, C.P.1    Nadel, J.A.2    Basbaum, C.B.3    Caughey, G.H.4
  • 200
    • 0026753576 scopus 로고
    • Comparison of adherence of P. aeruginosa to respiratory epithelial cells from cystic fibrosis patients and healthy subjects
    • Saiman, L., G. Cacalano, D. Gruenert, and A. Prince. 1992. Comparison of adherence of P. aeruginosa to respiratory epithelial cells from cystic fibrosis patients and healthy subjects. Infect. Immun. 60:2808-2814.
    • (1992) Infect. Immun. , vol.60 , pp. 2808-2814
    • Saiman, L.1    Cacalano, G.2    Gruenert, D.3    Prince, A.4
  • 201
    • 0028847778 scopus 로고
    • Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator
    • Zar, H., L. Saiman, L. Quittell, and A. Prince. 1995. Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator. J. Pediatr. 126:230-233.
    • (1995) J. Pediatr. , vol.126 , pp. 230-233
    • Zar, H.1    Saiman, L.2    Quittell, L.3    Prince, A.4
  • 202
    • 0027428606 scopus 로고
    • Pseudomonas aeruginosa pili bind to asialoGM1, which is increased on the surface of cystic fibrosis epithelial cells
    • Saiman, L., and A. Prince. 1993. Pseudomonas aeruginosa pili bind to asialoGM1, which is increased on the surface of cystic fibrosis epithelial cells. J. Clin. Invest. 92:1875-1880.
    • (1993) J. Clin. Invest. , vol.92 , pp. 1875-1880
    • Saiman, L.1    Prince, A.2
  • 203
    • 0029016495 scopus 로고
    • Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection
    • Demko, C. A., P. J. Byard, and P. B. Davis. 1995. Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection. J. Clin. Epidemiol. 48:1041-1049.
    • (1995) J. Clin. Epidemiol. , vol.48 , pp. 1041-1049
    • Demko, C.A.1    Byard, P.J.2    Davis, P.B.3
  • 204
    • 0026831341 scopus 로고
    • Mucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis
    • Henry, R. L., C. M. Mellis, and L. Petrovic. 1992. Mucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis. Pediatr. Pulmonol. 12:158-161.
    • (1992) Pediatr. Pulmonol. , vol.12 , pp. 158-161
    • Henry, R.L.1    Mellis, C.M.2    Petrovic, L.3
  • 205
    • 0026632470 scopus 로고
    • Production of the Pseudomonas aeruginosa neuraminidase is increased under hyperosmolar conditions and is regulated by genes involved in alginate expression
    • Cacalano, G., M. Kays, L. Saiman, and A. Prince. 1992. Production of the Pseudomonas aeruginosa neuraminidase is increased under hyperosmolar conditions and is regulated by genes involved in alginate expression. J. Clin. Invest. 89:1866-1874.
    • (1992) J. Clin. Invest. , vol.89 , pp. 1866-1874
    • Cacalano, G.1    Kays, M.2    Saiman, L.3    Prince, A.4
  • 206
    • 0021352445 scopus 로고
    • Influence of culture conditions on expression of mucoid mode of growth of Pseudomonas aeruginosa
    • Chan, R., J. S. Lam, K. Lam, and J. W. Costerton. 1984. Influence of culture conditions on expression of mucoid mode of growth of Pseudomonas aeruginosa. J. Clin. Microbiol. 19:8-16.
    • (1984) J. Clin. Microbiol. , vol.19 , pp. 8-16
    • Chan, R.1    Lam, J.S.2    Lam, K.3    Costerton, J.W.4
  • 208
    • 0029010510 scopus 로고
    • Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis
    • Armstrong, D. S., K. Grimwood, R. Carzino, J. B. Carlin, A. Olinsky, and P. D. Phelan. 1995. Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis. B.M.J. 310: 1571-1572.
    • (1995) B.M.J. , vol.310 , pp. 1571-1572
    • Armstrong, D.S.1    Grimwood, K.2    Carzino, R.3    Carlin, J.B.4    Olinsky, A.5    Phelan, P.D.6
  • 210
    • 0029372481 scopus 로고
    • Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis
    • Bonfield, T. L., M. W. Konstan, P. Burfeind, J. R. Panuska, J. B. Hilliard, and M. Berger. 1995. Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis. Am. J. Respir. Cell Mol. Biol. 13:257-261.
    • (1995) Am. J. Respir. Cell Mol. Biol. , vol.13 , pp. 257-261
    • Bonfield, T.L.1    Konstan, M.W.2    Burfeind, P.3    Panuska, J.R.4    Hilliard, J.B.5    Berger, M.6
  • 212
    • 0027104335 scopus 로고
    • Wheezing in infants with cystic fibrosis: Clinical course, pulmonary function and survival analysis
    • Kerem, E., J. Reisman, M. Corey, L. Bentur, G. Canny, and H. Levison. 1992. Wheezing in infants with cystic fibrosis: clinical course, pulmonary function and survival analysis. Pediatrics 90:703-706.
    • (1992) Pediatrics , vol.90 , pp. 703-706
    • Kerem, E.1    Reisman, J.2    Corey, M.3    Bentur, L.4    Canny, G.5    Levison, H.6
  • 213
    • 0027652118 scopus 로고
    • Longitudinal evaluation of pulmonary function in infants and very young children with cystic fibrosis
    • Tepper, R. S., G. L. Montgomery, V. Ackerman, and H. Eigen. 1993. Longitudinal evaluation of pulmonary function in infants and very young children with cystic fibrosis. Pediatr. Pulmonol. 16:96-100.
    • (1993) Pediatr. Pulmonol. , vol.16 , pp. 96-100
    • Tepper, R.S.1    Montgomery, G.L.2    Ackerman, V.3    Eigen, H.4
  • 214
    • 0027410084 scopus 로고
    • Relationship of genotype to early pulmonary function in infants with cystic fibrosis identified through neonatal screening
    • Mohon, R. T., J. S. Wagener, S. H. Abman, W. K. Seltzer, and F. J. Accurso. 1993. Relationship of genotype to early pulmonary function in infants with cystic fibrosis identified through neonatal screening. J. Pediatr. 122:550-555.
    • (1993) J. Pediatr. , vol.122 , pp. 550-555
    • Mohon, R.T.1    Wagener, J.S.2    Abman, S.H.3    Seltzer, W.K.4    Accurso, F.J.5
  • 215
    • 0016192551 scopus 로고
    • The relative sensitivity of closing volume in children with asthma and cystic fibrosis
    • Cooper, D. M., E. Doron, A. L. Mansell, A. C. Bryan, and H. Levison. 1974. The relative sensitivity of closing volume in children with asthma and cystic fibrosis. Am. Rev. Respir. Dis. 109:519.
    • (1974) Am. Rev. Respir. Dis. , vol.109 , pp. 519
    • Cooper, D.M.1    Doron, E.2    Mansell, A.L.3    Bryan, A.C.4    Levison, H.5
  • 217
  • 218
  • 219
    • 0026541092 scopus 로고
    • Role of alginate in infection with mucoid Pseudomonas aeruginosa in cystic fibrosis
    • Pederson, S. S., N. Hoiby, F. Espersen, and C. Koch. 1992. Role of alginate in infection with mucoid Pseudomonas aeruginosa in cystic fibrosis. Thorax 47:6-13.
    • (1992) Thorax , vol.47 , pp. 6-13
    • Pederson, S.S.1    Hoiby, N.2    Espersen, F.3    Koch, C.4
  • 220
    • 0025013016 scopus 로고
    • Effect of Pseudomonas cepacia colonization on survival and pulmonary function of cystic fibrosis patients
    • Lewin, L. O., P. J. Byard, and P. B. Davis. 1995. Effect of Pseudomonas cepacia colonization on survival and pulmonary function of cystic fibrosis patients. J. Clin. Epidemiol. 43:125-131.
    • (1995) J. Clin. Epidemiol. , vol.43 , pp. 125-131
    • Lewin, L.O.1    Byard, P.J.2    Davis, P.B.3
  • 221
    • 0015133355 scopus 로고
    • Evaluation of postural drainage by measurement of sputum volume and consistency
    • Lorin, M. I., and C. R. Denning. 1971. Evaluation of postural drainage by measurement of sputum volume and consistency. Am. J. Phys. Med. 50:215-219.
    • (1971) Am. J. Phys. Med. , vol.50 , pp. 215-219
    • Lorin, M.I.1    Denning, C.R.2
  • 222
    • 0019965488 scopus 로고
    • Effect of chest physiotherapy on the removal of mucus in patients with cystic fibrosis
    • Rossman, C. M., R. Waldes, D. Sampson, and M. T. Newhouse. 1982. Effect of chest physiotherapy on the removal of mucus in patients with cystic fibrosis. Am. Rev. Respir. Dis. 126:131-135.
    • (1982) Am. Rev. Respir. Dis. , vol.126 , pp. 131-135
    • Rossman, C.M.1    Waldes, R.2    Sampson, D.3    Newhouse, M.T.4
  • 223
    • 0020592607 scopus 로고
    • Immediate and long-tern effects of chest physiotherapy in patients with cystic fibrosis
    • Desmond, K. J., F. W. Schwenk, E. Thomas, P. H. Beaudry, and A. L. Coates. 1983. Immediate and long-tern effects of chest physiotherapy in patients with cystic fibrosis. J. Pediatr. 103:538-542.
    • (1983) J. Pediatr. , vol.103 , pp. 538-542
    • Desmond, K.J.1    Schwenk, F.W.2    Thomas, E.3    Beaudry, P.H.4    Coates, A.L.5
  • 225
    • 0028268027 scopus 로고
    • A review of airway clearance: New techniques, indications, and recommendations
    • Hardy, K. A. 1994. A review of airway clearance: new techniques, indications, and recommendations. Respir. Care 39:440-445.
    • (1994) Respir. Care , vol.39 , pp. 440-445
    • Hardy, K.A.1
  • 226
    • 0028920138 scopus 로고
    • Chest physical therapy management of patient with cystic fibrosis: A meta-analysis
    • Thomas, J., D. J. Cook, and D. Brooks. 1995. Chest physical therapy management of patient with cystic fibrosis: a meta-analysis. Am. J. Respir. Crit. Care Med. 151:846-850.
    • (1995) Am. J. Respir. Crit. Care Med. , vol.151 , pp. 846-850
    • Thomas, J.1    Cook, D.J.2    Brooks, D.3
  • 228
    • 0024396754 scopus 로고
    • Autogenic drainage: A modern approach to physiotherapy in cystic fibrosis
    • Schoini, M. H. 1989. Autogenic drainage: a modern approach to physiotherapy in cystic fibrosis. J. R. Soc. Med. 82(Suppl. 16):32-37.
    • (1989) J. R. Soc. Med. , vol.82 , Issue.16 SUPPL. , pp. 32-37
    • Schoini, M.H.1
  • 229
    • 0018605376 scopus 로고
    • Evaluation of the forced expiration technique as an adjunct to postural drainage in treatment of cystic fibrosis
    • Pryor, J. A., B. A. Webber, M. E. Hudson, and J. C. Batten. 1979. Evaluation of the forced expiration technique as an adjunct to postural drainage in treatment of cystic fibrosis. B.M.J. 2:417-418.
    • (1979) B.M.J. , vol.2 , pp. 417-418
    • Pryor, J.A.1    Webber, B.A.2    Hudson, M.E.3    Batten, J.C.4
  • 230
    • 0002197735 scopus 로고
    • The forced expiratory technique
    • J. Pryor, editor. Churchill Livingstone, London
    • Pryor, J. 1991. The forced expiratory technique. In J. Pryor, editor. Respiratory Care. Churchill Livingstone, London. 79-100.
    • (1991) Respiratory Care , pp. 79-100
    • Pryor, J.1
  • 231
    • 0027096544 scopus 로고
    • Zum Stellenwert der physiotherapie mit dem VRP 1-Desitin ("Flutter")
    • Lindemann, H. 1992. Zum Stellenwert der physiotherapie mit dem VRP 1-Desitin ("Flutter"). Pneumologie 46:626-630.
    • (1992) Pneumologie , vol.46 , pp. 626-630
    • Lindemann, H.1
  • 232
    • 0028298378 scopus 로고
    • Efficacy of the Flutter device for airway mucus clearance in patients with cystic fibrosis
    • Konstan, M. W., R. C. Stern, and C. F. Doershuk. 1994. Efficacy of the Flutter device for airway mucus clearance in patients with cystic fibrosis. J. Pediatr. 124:689-693.
    • (1994) J. Pediatr. , vol.124 , pp. 689-693
    • Konstan, M.W.1    Stern, R.C.2    Doershuk, C.F.3
  • 233
    • 0026285807 scopus 로고
    • The long-term effect of high-frequency chest compression therapy on pulmonary complications of cystic fibrosis
    • Warwick, W. J., and L. G. Hansen. 1991. The long-term effect of high-frequency chest compression therapy on pulmonary complications of cystic fibrosis. Pediatr. Pulmonol. 11:265-271.
    • (1991) Pediatr. Pulmonol. , vol.11 , pp. 265-271
    • Warwick, W.J.1    Hansen, L.G.2
  • 234
    • 0021255508 scopus 로고
    • Positive expiratory pressure (PEP) as lung physiotherapy in cystic fibrosis: A pilot study
    • Tonnesen, P., and S. Stovring. 1984. Positive expiratory pressure (PEP) as lung physiotherapy in cystic fibrosis: a pilot study. Eur. J. Respir. Dis. 65:419-422.
    • (1984) Eur. J. Respir. Dis. , vol.65 , pp. 419-422
    • Tonnesen, P.1    Stovring, S.2
  • 235
    • 0022817973 scopus 로고
    • Forced expirations against a variable resistance: A new chest physiotherapy method in cystic fibrosis
    • Oberwaldner, B., J. C. Evans, and M. S. Zach. 1986. Forced expirations against a variable resistance: a new chest physiotherapy method in cystic fibrosis. Pediatr. Pulmonol. 2:358-367.
    • (1986) Pediatr. Pulmonol. , vol.2 , pp. 358-367
    • Oberwaldner, B.1    Evans, J.C.2    Zach, M.S.3
  • 236
    • 0023549358 scopus 로고
    • Comparison of a positive expiratory pressure (PEP) mask with postural drainage in patients with cystic fibrosis
    • Van Asperen, P. P., L. Jackson, P. Hennessy, and J. Brown. 1987. Comparison of a positive expiratory pressure (PEP) mask with postural drainage in patients with cystic fibrosis. Aust. Paediatr. J. 23:283-284.
    • (1987) Aust. Paediatr. J. , vol.23 , pp. 283-284
    • Van Asperen, P.P.1    Jackson, L.2    Hennessy, P.3    Brown, J.4
  • 238
    • 0028231849 scopus 로고
    • Comparison of intrapulmonary percussive ventilation and chest physiotherapy
    • Natale, J. E., J. Pfeifle, and D. N. Homnick. 1994. Comparison of intrapulmonary percussive ventilation and chest physiotherapy. Chest 105:1789-1793.
    • (1994) Chest , vol.105 , pp. 1789-1793
    • Natale, J.E.1    Pfeifle, J.2    Homnick, D.N.3
  • 239
    • 0015620179 scopus 로고
    • The variable effect of a bronchodilating agent on pulmonary function in cystic fibrosis
    • Landau, L. I., and P. D. Phelan. 1973. The variable effect of a bronchodilating agent on pulmonary function in cystic fibrosis. J. Pediatr. 82:863-868.
    • (1973) J. Pediatr. , vol.82 , pp. 863-868
    • Landau, L.I.1    Phelan, P.D.2
  • 240
    • 0018758250 scopus 로고
    • A comparative study of inhaled atropine sulfate and isoproterenol hydrochloride in cystic fibrosis
    • Larsen, G. L., R. J. Barron, E. K. Cotton, and J. G. Brooks. 1979. A comparative study of inhaled atropine sulfate and isoproterenol hydrochloride in cystic fibrosis. Am. Rev. Respir. Dis. 119:399-407.
    • (1979) Am. Rev. Respir. Dis. , vol.119 , pp. 399-407
    • Larsen, G.L.1    Barron, R.J.2    Cotton, E.K.3    Brooks, J.G.4
  • 241
    • 0018927212 scopus 로고
    • Response to aerosol salbutamol, SCH 1000, and placebo in cystic fibrosis
    • Kattan, M., A. Mansell, H. Levison, M. Corey, and I. R. B. Krastins. 1980. Response to aerosol salbutamol, SCH 1000, and placebo in cystic fibrosis. Thorax 35:531-535.
    • (1980) Thorax , vol.35 , pp. 531-535
    • Kattan, M.1    Mansell, A.2    Levison, H.3    Corey, M.4    Krastins, I.R.B.5
  • 245
    • 0021848184 scopus 로고
    • Bronchodilators increase airway instability in cystic fibrosis
    • Zach, M. S., B. Oberwaldner, G. Forche, and G. Polgar. 1985. Bronchodilators increase airway instability in cystic fibrosis. Am. Rev. Respir. Dis. 131:537-543.
    • (1985) Am. Rev. Respir. Dis. , vol.131 , pp. 537-543
    • Zach, M.S.1    Oberwaldner, B.2    Forche, G.3    Polgar, G.4
  • 246
    • 0023819109 scopus 로고
    • Bronchodilator responsiveness in infants and young children with cystic fibrosis
    • Hiatt, P., H. Eigen, P. Yu, and R. S. Tepper. 1988. Bronchodilator responsiveness in infants and young children with cystic fibrosis. Am. Rev. Respir. Dis. 137:119-122.
    • (1988) Am. Rev. Respir. Dis. , vol.137 , pp. 119-122
    • Hiatt, P.1    Eigen, H.2    Yu, P.3    Tepper, R.S.4
  • 247
    • 0024985472 scopus 로고
    • Longitudinal response of pulmonary function to bronchodilators in cystic fibrosis
    • Pattishall, E. N. 1990. Longitudinal response of pulmonary function to bronchodilators in cystic fibrosis. Pediatr. Pulmonol. 9:80-85.
    • (1990) Pediatr. Pulmonol. , vol.9 , pp. 80-85
    • Pattishall, E.N.1
  • 248
    • 0029383649 scopus 로고
    • Short-term and long-term effects of albuterol aerosol therapy in cystic fibrosis: A preliminary report
    • König, P., D. Gayer, G. J. Barbero, and J. Shaffer. 1995. Short-term and long-term effects of albuterol aerosol therapy in cystic fibrosis: a preliminary report. Pediatr. Pulmonol. 20:205-214.
    • (1995) Pediatr. Pulmonol. , vol.20 , pp. 205-214
    • König, P.1    Gayer, D.2    Barbero, G.J.3    Shaffer, J.4
  • 249
    • 0002542883 scopus 로고
    • Aerosols of pancreatic dornase in bronchopulmonary disease
    • Salomon, A., J. A. Herchfus, and M. S. Segal. 1954. Aerosols of pancreatic dornase in bronchopulmonary disease. Ann. Allergy 12:71-79.
    • (1954) Ann. Allergy , vol.12 , pp. 71-79
    • Salomon, A.1    Herchfus, J.A.2    Segal, M.S.3
  • 250
    • 0014340449 scopus 로고
    • Bronchospasm after inhalation of pancreatic dornase
    • Raskin, P. 1968. Bronchospasm after inhalation of pancreatic dornase. Am. Rev. Respir. Dis. 98:697-698.
    • (1968) Am. Rev. Respir. Dis. , vol.98 , pp. 697-698
    • Raskin, P.1
  • 253
    • 0026602608 scopus 로고
    • Recombinant human DNase inhalation in normal subjects and patients with cystic fibrosis: A phase 1 study
    • Aitken, M. L., W. Burke, G. McDonald, S. Shak, A. B. Montgomery, and A. Smith. 1992. Recombinant human DNase inhalation in normal subjects and patients with cystic fibrosis: a phase 1 study. J.A.M.A. 267:1947-1951.
    • (1992) J.A.M.A. , vol.267 , pp. 1947-1951
    • Aitken, M.L.1    Burke, W.2    McDonald, G.3    Shak, S.4    Montgomery, A.B.5    Smith, A.6
  • 255
    • 0028129568 scopus 로고
    • Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
    • Fuchs, H. J., D. S. Borowitz, D. H. Christiansen, E. M. Morris, M. L. Nash, B. W. Ramsey, B. J. Rosenstein, A. L. Smith, and M. E. Wohl for the Pulmozyme Study Group. 1994. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N. Engl. J. Med. 331:637-642.
    • (1994) N. Engl. J. Med. , vol.331 , pp. 637-642
    • Fuchs, H.J.1    Borowitz, D.S.2    Christiansen, D.H.3    Morris, E.M.4    Nash, M.L.5    Ramsey, B.W.6    Rosenstein, B.J.7    Smith, A.L.8    Wohl, M.E.9
  • 258
    • 0008457589 scopus 로고
    • Therapy and prognosis of fibrocystic disease of the pancreas
    • Andersen, D. H. 1949. Therapy and prognosis of fibrocystic disease of the pancreas. Pediatrics 3:406-417.
    • (1949) Pediatrics , vol.3 , pp. 406-417
    • Andersen, D.H.1
  • 259
    • 10544242482 scopus 로고
    • Mucoviscidosis: An evaluation of continuous and prolonged antibiotic therapy
    • Shwachman, H., P. Catzel, P. R. Patterson, and M. Stoppelman. 1954. Mucoviscidosis: an evaluation of continuous and prolonged antibiotic therapy. Am. J. Dis. Child. 88:380-382.
    • (1954) Am. J. Dis. Child. , vol.88 , pp. 380-382
    • Shwachman, H.1    Catzel, P.2    Patterson, P.R.3    Stoppelman, M.4
  • 263
    • 0024839281 scopus 로고
    • Antibiotic therapy for bronchopulmonary infections in cystic fibrosis. The American approach
    • Marks, M. I. 1989. Antibiotic therapy for bronchopulmonary infections in cystic fibrosis. The American approach. Antibiot. Chemother. 42:229-236.
    • (1989) Antibiot. Chemother. , vol.42 , pp. 229-236
    • Marks, M.I.1
  • 265
    • 0020573198 scopus 로고
    • Frequent antibiotic therapy improves survival of cystic fibrosis patients with chronic Pseudomonas aeruginosa infection
    • Szaff, M., N. Hoiby, and E. W. Flensborg. 1983. Frequent antibiotic therapy improves survival of cystic fibrosis patients with chronic Pseudomonas aeruginosa infection. Ada Paediatr. Scand. 72:651-657.
    • (1983) Ada Paediatr. Scand. , vol.72 , pp. 651-657
    • Szaff, M.1    Hoiby, N.2    Flensborg, E.W.3
  • 266
    • 0021878966 scopus 로고
    • Isolation medium for the recovery of Pseudomonas cepacia from respiratory secretions of patients with cystic fibrosis
    • Gilligan, P. H., P. A. Gage, L. M. Bradshaw, D. V. Schidlow, and B. T. DeCicco. 1985. Isolation medium for the recovery of Pseudomonas cepacia from respiratory secretions of patients with cystic fibrosis. J. Clin. Microbiol. 22:5-8.
    • (1985) J. Clin. Microbiol. , vol.22 , pp. 5-8
    • Gilligan, P.H.1    Gage, P.A.2    Bradshaw, L.M.3    Schidlow, D.V.4    Decicco, B.T.5
  • 267
    • 0026671894 scopus 로고
    • Optimum use of selective plated media in primary processing of respiratory tract specimens from patients with cystic fibrosis
    • Doern, G. V., and B. Brodgen-Torres. 1992. Optimum use of selective plated media in primary processing of respiratory tract specimens from patients with cystic fibrosis. J. Clin. Microbiol. 30: 2740-2742.
    • (1992) J. Clin. Microbiol. , vol.30 , pp. 2740-2742
    • Doern, G.V.1    Brodgen-Torres, B.2
  • 268
    • 0023628218 scopus 로고
    • Antibiotic pharmacokinetics in cystic fibrosis: Differences and clinical significances
    • deGroot, R., and A. L. Smith. 1987. Antibiotic pharmacokinetics in cystic fibrosis: differences and clinical significances. Clin. Pharmacokinet. 13:228-253.
    • (1987) Clin. Pharmacokinet. , vol.13 , pp. 228-253
    • DeGroot, R.1    Smith, A.L.2
  • 269
    • 0021800153 scopus 로고
    • In vitro activity of ciprofloxacin and other antibacterial agents against Pseudomonas aeruginosa and Pseudomonas cepacia from cystic fibrosis patients
    • Klinger, J. D., and S. C. Aronoff. 1985. In vitro activity of ciprofloxacin and other antibacterial agents against Pseudomonas aeruginosa and Pseudomonas cepacia from cystic fibrosis patients. J. Antimicrob. Chemother. 15:679-684.
    • (1985) J. Antimicrob. Chemother. , vol.15 , pp. 679-684
    • Klinger, J.D.1    Aronoff, S.C.2
  • 270
    • 0023270203 scopus 로고
    • Comparative pharmacokinetics of ciprofloxacin and ofloxacin in cystic fibrosis patients
    • Pedersen, S. S., T. Jensen, and E. F. Hvidberg. 1987. Comparative pharmacokinetics of ciprofloxacin and ofloxacin in cystic fibrosis patients. J. Antimicrob. Chemother. 20:575-583.
    • (1987) J. Antimicrob. Chemother. , vol.20 , pp. 575-583
    • Pedersen, S.S.1    Jensen, T.2    Hvidberg, E.F.3
  • 271
    • 0023280807 scopus 로고
    • The efficacy and safety of ciprofloxacin and ofloxacin in the treatment of chronic Pseudomonas aeruginosa infection in cystic fibrosis
    • Jensen, T., S. S. Pedersen, C. H. Nielsen, N. Hoiby, and C. Koch. 1987. The efficacy and safety of ciprofloxacin and ofloxacin in the treatment of chronic Pseudomonas aeruginosa infection in cystic fibrosis. J. Antimicrob. Chemother. 20:585-594.
    • (1987) J. Antimicrob. Chemother. , vol.20 , pp. 585-594
    • Jensen, T.1    Pedersen, S.S.2    Nielsen, C.H.3    Hoiby, N.4    Koch, C.5
  • 272
    • 0022617254 scopus 로고
    • Ciprofloxacin pharmacokinetics in patients with cystic fibrosis
    • Bender, S. W., A. Dalhoff, P. M. Shah, R. Strehl, and H. G. Posselt. 1986. Ciprofloxacin pharmacokinetics in patients with cystic fibrosis. Infection 14:17-21.
    • (1986) Infection , vol.14 , pp. 17-21
    • Bender, S.W.1    Dalhoff, A.2    Shah, P.M.3    Strehl, R.4    Posselt, H.G.5
  • 275
    • 0024267448 scopus 로고
    • Lack of unique ciprofloxacin pharmacokinetic characteristics in patients with cystic fibrosis
    • Reed, M. D., R. C. Stern, C. M. Myers, T. S. Yamashita, and J. L. Blumer. 1988. Lack of unique ciprofloxacin pharmacokinetic characteristics in patients with cystic fibrosis. J. Clin. Pharmacol. 28: 691-699.
    • (1988) J. Clin. Pharmacol. , vol.28 , pp. 691-699
    • Reed, M.D.1    Stern, R.C.2    Myers, C.M.3    Yamashita, T.S.4    Blumer, J.L.5
  • 276
    • 0022527419 scopus 로고
    • Antibiotic therapy in cystic fibrosis: Evaluation of clinical trials
    • Smith, A. L. 1986. Antibiotic therapy in cystic fibrosis: evaluation of clinical trials. J. Pediatr. 108:866-870.
    • (1986) J. Pediatr. , vol.108 , pp. 866-870
    • Smith, A.L.1
  • 278
    • 0023609577 scopus 로고
    • Randomized study of two dosing regimens of ciprofloxacin for treating chronic bronchopulmonary infection in patient with cystic fibrosis
    • Shalit, I., H. R. Stutman, M. I. Marks, S. A. Chartrand, and B. C. Human. 1987. Randomized study of two dosing regimens of ciprofloxacin for treating chronic bronchopulmonary infection in patient with cystic fibrosis. Am. J. Med. 82:189-195.
    • (1987) Am. J. Med. , vol.82 , pp. 189-195
    • Shalit, I.1    Stutman, H.R.2    Marks, M.I.3    Chartrand, S.A.4    Human, B.C.5
  • 280
    • 0023632352 scopus 로고
    • Ciprofloxacin: Comparative data in cystic fibrosis
    • Rubio, T. T. 1987. Ciprofloxacin: comparative data in cystic fibrosis. Am. J. Med. 82:185-188.
    • (1987) Am. J. Med. , vol.82 , pp. 185-188
    • Rubio, T.T.1
  • 281
    • 0023122215 scopus 로고
    • Oral ciprofloxacin compared with conventional intravenous treatment for Pseudomonas aeruginosa infection in adults with cystic fibrosis
    • Hodson, M. E., C. M. Roberts, R. J. A. Butland, M. J. Smith, and J. C. Batten. 1987. Oral ciprofloxacin compared with conventional intravenous treatment for Pseudomonas aeruginosa infection in adults with cystic fibrosis. Lancet 1:235-237.
    • (1987) Lancet , vol.1 , pp. 235-237
    • Hodson, M.E.1    Roberts, C.M.2    Butland, R.J.A.3    Smith, M.J.4    Batten, J.C.5
  • 282
    • 0024398571 scopus 로고
    • Use of ciprofloxacin in cystic fibrosis patients
    • Bosso, J. A. 1989. Use of ciprofloxacin in cystic fibrosis patients. Am. J. Med. 87:S123-S127.
    • (1989) Am. J. Med. , vol.87
    • Bosso, J.A.1
  • 283
    • 0023628903 scopus 로고
    • A controlled trial of nebulized aminoglycoside and oral flucloxacillin versus placebo in the outpatient management of children with cystic fibrosis
    • Carswell, F., C. Ward, D. A. Cook, and D. C. E. Speller. 1987. A controlled trial of nebulized aminoglycoside and oral flucloxacillin versus placebo in the outpatient management of children with cystic fibrosis. Br. J. Dis. Chest. 81:356.
    • (1987) Br. J. Dis. Chest. , vol.81 , pp. 356
    • Carswell, F.1    Ward, C.2    Cook, D.A.3    Speller, D.C.E.4
  • 284
    • 0024331219 scopus 로고
    • Long-term effects of inhaled tobramycin in patients with cystic fibrosis colonized with Pseudomonas aeruginosa
    • MacLusky, I. B., R. Gold, M. Corey, and H. Levison. 1989. Long-term effects of inhaled tobramycin in patients with cystic fibrosis colonized with Pseudomonas aeruginosa. Pediatr. Pulmonol. 7: 42-48.
    • (1989) Pediatr. Pulmonol. , vol.7 , pp. 42-48
    • MacLusky, I.B.1    Gold, R.2    Corey, M.3    Levison, H.4
  • 287
    • 0021996644 scopus 로고
    • Nebulized colomycin for early pseudomonas colonization in cystic fibrosis
    • Littlewood, J. M., M. G. Miller, A. T. Ghoneim, and C. H. Ramsden. 1985. Nebulized colomycin for early pseudomonas colonization in cystic fibrosis. Lancet 2:865-867.
    • (1985) Lancet , vol.2 , pp. 865-867
    • Littlewood, J.M.1    Miller, M.G.2    Ghoneim, A.T.3    Ramsden, C.H.4
  • 288
    • 0023265976 scopus 로고
    • Colistin inhalation therapy in cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infections
    • Jensen, T., S. S. Pedersen, S. Garne, C. Heilmann, N. Hoiby, and C. Koch. 1987. Colistin inhalation therapy in cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infections. J. Antimicrob. Chemother. 19:831-838.
    • (1987) J. Antimicrob. Chemother. , vol.19 , pp. 831-838
    • Jensen, T.1    Pedersen, S.S.2    Garne, S.3    Heilmann, C.4    Hoiby, N.5    Koch, C.6
  • 289
    • 0025868478 scopus 로고
    • Prevention of chronic Pseudomonas aeruginosa colonization in cystic fibrosis by early treatment
    • Valerius, N. H., C. Koch, and N. Hoiby. 1991. Prevention of chronic Pseudomonas aeruginosa colonization in cystic fibrosis by early treatment. Lancet 338:725-726.
    • (1991) Lancet , vol.338 , pp. 725-726
    • Valerius, N.H.1    Koch, C.2    Hoiby, N.3
  • 290
    • 0019511799 scopus 로고
    • Aerosol carbenicillin and gentamicin treatment of Pseudomonas aeruginosa infection in patients with cystic fibrosis
    • Hodson, M. E., A. R. L. Penketh, and J. C. Batten. 1981. Aerosol carbenicillin and gentamicin treatment of Pseudomonas aeruginosa infection in patients with cystic fibrosis. Lancet 2:1137-1139.
    • (1981) Lancet , vol.2 , pp. 1137-1139
    • Hodson, M.E.1    Penketh, A.R.L.2    Batten, J.C.3
  • 293
    • 0001647527 scopus 로고
    • Treatment of pulmonary disease in patients with cystic fibrosis
    • P. B. Davis, editor. Marcel Dekker, New York
    • Turpin, S. V., and M. R. Knowles. 1993. Treatment of pulmonary disease in patients with cystic fibrosis. In P. B. Davis, editor. Cystic Fibrosis. Marcel Dekker, New York. 277-344.
    • (1993) Cystic Fibrosis , pp. 277-344
    • Turpin, S.V.1    Knowles, M.R.2
  • 294
    • 0016772036 scopus 로고
    • Use of a Pseudomonas aeruginosa vaccine in patients with acute leukemia and cystic fibrosis
    • Pennington, J. E., H. Y. Reynolds, R. E. Wood, R. A. Robinson, and A. S. Levine. 1975. Use of a Pseudomonas aeruginosa vaccine in patients with acute leukemia and cystic fibrosis. Am. J. Med. 58:629-635.
    • (1975) Am. J. Med. , vol.58 , pp. 629-635
    • Pennington, J.E.1    Reynolds, H.Y.2    Wood, R.E.3    Robinson, R.A.4    Levine, A.S.5
  • 295
    • 0021028762 scopus 로고
    • Intranasal administration of a Pseudomonas lipopolysaccharide vaccine in cystic fibrosis patients
    • Wood, R. E., J. E. Pennington, and H. Y. Reynolds. 1983. Intranasal administration of a Pseudomonas lipopolysaccharide vaccine in cystic fibrosis patients. Pediatr. Infect. Dis. 2:367-369.
    • (1983) Pediatr. Infect. Dis. , vol.2 , pp. 367-369
    • Wood, R.E.1    Pennington, J.E.2    Reynolds, H.Y.3
  • 296
    • 0021711007 scopus 로고
    • Prospective, controlled study of a polyvalent Pseudomonas vaccine in cystic fibrosis: Three year results
    • Langford, D. T., and J. Hiller. 1984. Prospective, controlled study of a polyvalent Pseudomonas vaccine in cystic fibrosis: three year results. Arch. Dis. Child 59:1131-1134.
    • (1984) Arch. Dis. Child , vol.59 , pp. 1131-1134
    • Langford, D.T.1    Hiller, J.2
  • 297
    • 0025950064 scopus 로고
    • Safety and immunogenicity of Pseudomonas aeruginosa conjugate a vaccine in cystic fibrosis
    • Schaad, U. B., A. B. Lang, J. Wedgwood, A. Ruedeberg, J. U. Que, E. Furer, and S. J. J. Cryz. 1991. Safety and immunogenicity of Pseudomonas aeruginosa conjugate A vaccine in cystic fibrosis. Lancet 338:1236-1237.
    • (1991) Lancet , vol.338 , pp. 1236-1237
    • Schaad, U.B.1    Lang, A.B.2    Wedgwood, J.3    Ruedeberg, A.4    Que, J.U.5    Furer, E.6    Cryz, S.J.J.7
  • 299
    • 0024549158 scopus 로고
    • Intravenous immune globulin treatment of pulmonary exacerbations in cystic fibrosis
    • Winnie, G. B., R. G. Cowan, and N. A. Wade. 1989. Intravenous immune globulin treatment of pulmonary exacerbations in cystic fibrosis. J. Pediatr. 114:309-314.
    • (1989) J. Pediatr. , vol.114 , pp. 309-314
    • Winnie, G.B.1    Cowan, R.G.2    Wade, N.A.3
  • 301
    • 0024991665 scopus 로고
    • Protection against mucoid Pseudomonas aeruginosa in rodent models of endobronchial infections
    • Pier, G. B., G. J. Small, and H. B. Warren. 1990. Protection against mucoid Pseudomonas aeruginosa in rodent models of endobronchial infections. Science 249:537-540.
    • (1990) Science , vol.249 , pp. 537-540
    • Pier, G.B.1    Small, G.J.2    Warren, H.B.3
  • 302
    • 1842438894 scopus 로고
    • Safety and pharmacokinetics of a mucoid Pseudomonas aeruginosa immune globulin. Intravenous (human) in patients with cystic fibrosis-preliminary results of a phase I/II trial
    • Moss, R., R. Fink, S. Schroeder, B. Ramsey, L. Fries, S. Fuller, S. Harkonen, L. Muenz, and M. Thornton. 1995. Safety and pharmacokinetics of a mucoid Pseudomonas aeruginosa immune globulin. Intravenous (human) in patients with cystic fibrosis-preliminary results of a phase I/II trial. Pediatr. Pulmonol. 19:85.
    • (1995) Pediatr. Pulmonol. , vol.19 , pp. 85
    • Moss, R.1    Fink, R.2    Schroeder, S.3    Ramsey, B.4    Fries, L.5    Fuller, S.6    Harkonen, S.7    Muenz, L.8    Thornton, M.9
  • 303
    • 0021687443 scopus 로고
    • Association of respiratory viral infections with pulmonary deterioration in patients with cystic fibrosis
    • Wang, E. E. L., C. G. Prober, B. Manson, M. Corey, and H. Levison. 1984. Association of respiratory viral infections with pulmonary deterioration in patients with cystic fibrosis. N. Engl. J. Med. 311:1653-1658.
    • (1984) N. Engl. J. Med. , vol.311 , pp. 1653-1658
    • Wang, E.E.L.1    Prober, C.G.2    Manson, B.3    Corey, M.4    Levison, H.5
  • 305
    • 0028015883 scopus 로고
    • Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation
    • Konstan, M. W., K. A. Hilliard, T. M. Norvell, and M. Berger. 1994. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am. J. Respir. Crit. Care Med. 150:448-454.
    • (1994) Am. J. Respir. Crit. Care Med. , vol.150 , pp. 448-454
    • Konstan, M.W.1    Hilliard, K.A.2    Norvell, T.M.3    Berger, M.4
  • 306
    • 0029286816 scopus 로고
    • Cystic fibrosis lung inflammation: Early, sustained, and severe
    • Cantin, A. 1995. Cystic fibrosis lung inflammation: early, sustained, and severe. Am. J. Respir. Crit. Care Med. 151:939-941.
    • (1995) Am. J. Respir. Crit. Care Med. , vol.151 , pp. 939-941
    • Cantin, A.1
  • 307
    • 0021970474 scopus 로고
    • Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis
    • Auerbach, H. S., M. Williams, J. A. Kirkpatrick, and H. R. Colten. 1985. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 2:686-688.
    • (1985) Lancet , vol.2 , pp. 686-688
    • Auerbach, H.S.1    Williams, M.2    Kirkpatrick, J.A.3    Colten, H.R.4
  • 309
    • 0028969594 scopus 로고
    • A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group
    • Eigen, H., B. J. Rosenstein, S. FitzSimmons, and D. V. Schidlow. 1995. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J. Pediatr. 126:515-523.
    • (1995) J. Pediatr. , vol.126 , pp. 515-523
    • Eigen, H.1    Rosenstein, B.J.2    Fitzsimmons, S.3    Schidlow, D.V.4
  • 310
    • 0025977985 scopus 로고
    • Risks of alternate-day prednisone in patients with cystic fibrosis
    • Rosenstein, B. J., and H. Eigen. 1991. Risks of alternate-day prednisone in patients with cystic fibrosis. Pediatrics 87:245-246.
    • (1991) Pediatrics , vol.87 , pp. 245-246
    • Rosenstein, B.J.1    Eigen, H.2
  • 311
    • 0028169953 scopus 로고
    • Interleukin-1 alpha, soluble interleukin-2 receptor, and IgG concentrations in cystic fibrosis treated with prednisolone
    • Greally, P., M. J. Hussain, D. Vergani, and J. F. Price. 1994. Interleukin-1 alpha, soluble interleukin-2 receptor, and IgG concentrations in cystic fibrosis treated with prednisolone. Arch. Dis. Child. 71:35-39.
    • (1994) Arch. Dis. Child. , vol.71 , pp. 35-39
    • Greally, P.1    Hussain, M.J.2    Vergani, D.3    Price, J.F.4
  • 312
    • 0020696950 scopus 로고
    • Chronic Pseudomonas aeruginosa lung infection in cystic fibrosis. A longitudinal study of immune complex activity and inflammatory response in sputum sol-phase of cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infections: Influence of local steroid treatment
    • Schiotz, P. O., M. Jorgensen, E. W. Flensborg, O. Faero, S. Husby, N. Hoiby, S. V. Jacobsen, H. Nielsen, and S. E. Svehag. 1983. Chronic Pseudomonas aeruginosa lung infection in cystic fibrosis. A longitudinal study of immune complex activity and inflammatory response in sputum sol-phase of cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infections: influence of local steroid treatment. Acta Paediatr. Scand. 72:283-287.
    • (1983) Acta Paediatr. Scand. , vol.72 , pp. 283-287
    • Schiotz, P.O.1    Jorgensen, M.2    Flensborg, E.W.3    Faero, O.4    Husby, S.5    Hoiby, N.6    Jacobsen, S.V.7    Nielsen, H.8    Svehag, S.E.9
  • 314
    • 0030062339 scopus 로고    scopus 로고
    • Pilot study to assess the effect of inhaled corticosteroids on lung function in patients with cystic fibrosis
    • Nikolaizik, W. H., and M. H. Schöni. 1996. Pilot study to assess the effect of inhaled corticosteroids on lung function in patients with cystic fibrosis. J. Pediatr. 128:271-274.
    • (1996) J. Pediatr. , vol.128 , pp. 271-274
    • Nikolaizik, W.H.1    Schöni, M.H.2
  • 315
    • 0025019280 scopus 로고
    • Ibuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in a rat model of chronic pulmonary infection: Implications for antiinflammatory therapy in cystic fibrosis
    • Konstan, M. W., K. M. Vargo, and P. B. Davis. 1990. Ibuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in a rat model of chronic pulmonary infection: implications for antiinflammatory therapy in cystic fibrosis. Am. Rev. Respir. Dis. 141: 186-192.
    • (1990) Am. Rev. Respir. Dis. , vol.141 , pp. 186-192
    • Konstan, M.W.1    Vargo, K.M.2    Davis, P.B.3
  • 316
    • 0002208276 scopus 로고
    • Effect of ibuprofen on neutrophil delivery to mucosal surfaces
    • Konstan, M. W., K. A. Hilliard, and P. B. Davis. 1989. Effect of ibuprofen on neutrophil delivery to mucosal surfaces. Pediatr. Pulmonol. Suppl. 4:152-153.
    • (1989) Pediatr. Pulmonol. Suppl. , vol.4 , pp. 152-153
    • Konstan, M.W.1    Hilliard, K.A.2    Davis, P.B.3
  • 317
    • 0025763746 scopus 로고
    • Ibuprofen in children with cystic fibrosis: Pharmacokinetics and adverse effects
    • Konstan, M. W., C. L. Hoppel, B. Chai, and P. B. Davis. 1991. Ibuprofen in children with cystic fibrosis: pharmacokinetics and adverse effects. J. Pediatr. 118:956-964.
    • (1991) J. Pediatr. , vol.118 , pp. 956-964
    • Konstan, M.W.1    Hoppel, C.L.2    Chai, B.3    Davis, P.B.4
  • 318
    • 0028914667 scopus 로고
    • Effect of high-dose ibuprofen in patients with cystic fibrosis
    • Konstan, M. W., P. J. Byard, C. L. Hoppel, and P. B. Davis. 1995. Effect of high-dose ibuprofen in patients with cystic fibrosis. N. Engl. J. Med. 332:848-854.
    • (1995) N. Engl. J. Med. , vol.332 , pp. 848-854
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3    Davis, P.B.4
  • 319
    • 84960578076 scopus 로고
    • Piroxicam treatment protects mice from lethal pulmonary challenge with Pseudomonas aeruginosa
    • Sordelli, D. O., M. C. Cerquetti, P. A. Fontan, and R. P. Meiss. 1989. Piroxicam treatment protects mice from lethal pulmonary challenge with Pseudomonas aeruginosa. J. Infect. Dis. 159:232-238.
    • (1989) J. Infect. Dis. , vol.159 , pp. 232-238
    • Sordelli, D.O.1    Cerquetti, M.C.2    Fontan, P.A.3    Meiss, R.P.4
  • 320
    • 0028137161 scopus 로고
    • A preliminary study on the effect of anti-inflammatory treatment in cystic fibrosis patients with Pseudomonas aeruginosa lung infection
    • Sordelli, D. O., C. N. Macri, A. J. Maillie, and M. C. Cerquetti. 1994. A preliminary study on the effect of anti-inflammatory treatment in cystic fibrosis patients with Pseudomonas aeruginosa lung infection. Int. J. Immunopathol. Pharmacol. 7:109-117.
    • (1994) Int. J. Immunopathol. Pharmacol. , vol.7 , pp. 109-117
    • Sordelli, D.O.1    Macri, C.N.2    Maillie, A.J.3    Cerquetti, M.C.4
  • 321
    • 0027618412 scopus 로고
    • Pseudomonas aeruginosa endobronchitis in rhesus monkeys: II. A histopathologic analysis
    • Cheung, A. T., R. B. Moss, G. Kurland, A. B. Leong, and W. J. Novick, Jr. 1993. Pseudomonas aeruginosa endobronchitis in rhesus monkeys: II. A histopathologic analysis. J. Med. Primatol. 22: 257-262.
    • (1993) J. Med. Primatol. , vol.22 , pp. 257-262
    • Cheung, A.T.1    Moss, R.B.2    Kurland, G.3    Leong, A.B.4    Novick Jr., W.J.5
  • 322
    • 0028113854 scopus 로고
    • Effects of pentoxifylline on sputum neutophil elastase and pulmonary function in patients with cystic fibrosis: Preliminary observations
    • Aronoff, S. C., F. J. Quinn, Jr., L. S. Carpenter, and W. J. Novick, Jr. 1994. Effects of pentoxifylline on sputum neutophil elastase and pulmonary function in patients with cystic fibrosis: preliminary observations. J. Pediatr. 125:992-997.
    • (1994) J. Pediatr. , vol.125 , pp. 992-997
    • Aronoff, S.C.1    Quinn Jr., F.J.2    Carpenter, L.S.3    Novick Jr., W.J.4
  • 323
    • 0028281371 scopus 로고
    • Oral absorption of omega-3 fatty acids in patients with cystic fibrosis who have pancreatic insufficiency and in healthy control subjects
    • Henderson, W. R., Jr., S. J. Astley, M. M. McCready, P. Kushmerick, S. Casey, J. W. Becker, and B. W. Ramsey. 1994. Oral absorption of omega-3 fatty acids in patients with cystic fibrosis who have pancreatic insufficiency and in healthy control subjects. J. Pediatr. 124:400-408.
    • (1994) J. Pediatr. , vol.124 , pp. 400-408
    • Henderson Jr., W.R.1    Astley, S.J.2    McCready, M.M.3    Kushmerick, P.4    Casey, S.5    Becker, J.W.6    Ramsey, B.W.7
  • 325
    • 0026474443 scopus 로고
    • Modulation of airway inflammation in cystic fibrosis: In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant seretory leukoprotease inhibitor
    • McElvaney, N. G., H. Nakamura, P. Birrer, C. A. Hebert, W. L. Wong, M. Alphonso, J. B. Baker, M. A. Catalano, and R. G. Crystal. 1992. Modulation of airway inflammation in cystic fibrosis: In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant seretory leukoprotease inhibitor. J. Clin. Invest. 90:1296-1301.
    • (1992) J. Clin. Invest. , vol.90 , pp. 1296-1301
    • McElvaney, N.G.1    Nakamura, H.2    Birrer, P.3    Hebert, C.A.4    Wong, W.L.5    Alphonso, M.6    Baker, J.B.7    Catalano, M.A.8    Crystal, R.G.9
  • 326
    • 0027495712 scopus 로고
    • Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis
    • McElvaney, N. G., B. Doujaiji, M. J. Moan, M. R. Burnham, M. C. Wu, and R. G. Crystal. 1993. Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis. Am. Rev. Respir. Dis. 148:1056-1060.
    • (1993) Am. Rev. Respir. Dis. , vol.148 , pp. 1056-1060
    • McElvaney, N.G.1    Doujaiji, B.2    Moan, M.J.3    Burnham, M.R.4    Wu, M.C.5    Crystal, R.G.6
  • 329
    • 0027564769 scopus 로고
    • Cystic Fibrosis Foundation consensus conference report on pulmonary complications of cystic fibrosis
    • Schidlow, D. V., L. M. Taussig, and M. R. Knowles. 1993. Cystic Fibrosis Foundation consensus conference report on pulmonary complications of cystic fibrosis. Pediatr. Pulmonol. 15:187-198.
    • (1993) Pediatr. Pulmonol. , vol.15 , pp. 187-198
    • Schidlow, D.V.1    Taussig, L.M.2    Knowles, M.R.3
  • 330
    • 0018844674 scopus 로고
    • Heart failure in cystic fibrosis: Treatment and prognosis of cor pulmonale with failure of the right side of the heart
    • Stern, R. C., G. Borkat, S. S. Hirchfeld, T. F. Boat, L. W. Matthews, J. Liebman, and C. F. Doershuk. 1980. Heart failure in cystic fibrosis: treatment and prognosis of cor pulmonale with failure of the right side of the heart. Am. J. Dis. Child. 134:267-272.
    • (1980) Am. J. Dis. Child. , vol.134 , pp. 267-272
    • Stern, R.C.1    Borkat, G.2    Hirchfeld, S.S.3    Boat, T.F.4    Matthews, L.W.5    Liebman, J.6    Doershuk, C.F.7
  • 333
    • 0025601452 scopus 로고
    • Life threatening haemoptysis in cystic fibrosis: An alternative therapeutic approach
    • Bilton, D., A. K. Webb, H. Foster, P. Mulvenna, and M. Dodd. 1990. Life threatening haemoptysis in cystic fibrosis: An alternative therapeutic approach. Thorax 45:975-976.
    • (1990) Thorax , vol.45 , pp. 975-976
    • Bilton, D.1    Webb, A.K.2    Foster, H.3    Mulvenna, P.4    Dodd, M.5
  • 334
    • 0025249349 scopus 로고
    • Bronchial artery embolization to control hemoptysis in cystic fibrosis
    • Cohen, A. M., C. F. Doershuk, and R. C. Stern. 1990. Bronchial artery embolization to control hemoptysis in cystic fibrosis. Radiology 175:401-403.
    • (1990) Radiology , vol.175 , pp. 401-403
    • Cohen, A.M.1    Doershuk, C.F.2    Stern, R.C.3
  • 335
    • 0018703974 scopus 로고
    • Bronchial artery embolization in cystic fibrosis; technique and long-term results
    • Fellows, K. E., K. T. Khaw, S. Schuster, and H. Shwachman. 1995. Bronchial artery embolization in cystic fibrosis; technique and long-term results. J. Pediatr. 95:959-963.
    • (1995) J. Pediatr. , vol.95 , pp. 959-963
    • Fellows, K.E.1    Khaw, K.T.2    Schuster, S.3    Shwachman, H.4
  • 336
  • 338
    • 0014364588 scopus 로고
    • Pneumothorax as a complication of cystic fibrosis: Report of twenty cases
    • Lifschitz, M. I., F. O. Bowman, Jr., C. R. Denning, and R. H. Wylie. 1968. Pneumothorax as a complication of cystic fibrosis: report of twenty cases. Am. J. Dis. Child 116:633-640.
    • (1968) Am. J. Dis. Child , vol.116 , pp. 633-640
    • Lifschitz, M.I.1    Bowman Jr., F.O.2    Denning, C.R.3    Wylie, R.H.4
  • 341
    • 0021996338 scopus 로고
    • Pulmonary air cysts in cystic fibrosis: Relation of pathologic features to radiologic findings and history of pneumothorax
    • Tomashefski, J. F., Jr., M. Bruce, R. C. Stern, D. G. Dearborn, and B. Dahms. 1985. Pulmonary air cysts in cystic fibrosis: relation of pathologic features to radiologic findings and history of pneumothorax. Hum. Pathol. 16:253-261.
    • (1985) Hum. Pathol. , vol.16 , pp. 253-261
    • Tomashefski Jr., J.F.1    Bruce, M.2    Stern, R.C.3    Dearborn, D.G.4    Dahms, B.5
  • 342
    • 0024502869 scopus 로고
    • Pneumothorax in cystic fibrosis: A 26-year experience
    • Spector, M. L., and R. C. Stern. 1989. Pneumothorax in cystic fibrosis: a 26-year experience. Ann. Thorac. Surg. 47:204-207.
    • (1989) Ann. Thorac. Surg. , vol.47 , pp. 204-207
    • Spector, M.L.1    Stern, R.C.2
  • 343
    • 0026741723 scopus 로고
    • Treatment of pneumothorax in cystic fibrosis in the era of lung transplantation
    • Noyes, B. E., and D. M. Orenstein. 1992. Treatment of pneumothorax in cystic fibrosis in the era of lung transplantation. Chest 101: 1187-1188.
    • (1992) Chest , vol.101 , pp. 1187-1188
    • Noyes, B.E.1    Orenstein, D.M.2
  • 346
    • 0018394849 scopus 로고
    • Episodic arthritis in children with cystic fibrosis
    • Neuman, A. J., and B. M. Ansell. 1979. Episodic arthritis in children with cystic fibrosis. J. Pediatr. 94:594-596.
    • (1979) J. Pediatr. , vol.94 , pp. 594-596
    • Neuman, A.J.1    Ansell, B.M.2
  • 347
    • 0028037759 scopus 로고
    • Prevalence of circulating immune complexes in patients with cystic fibrosis and arthritis
    • Wulffraat, N. M., E. R. de Graeff Meeder, G. T. Rijkers, H. van der Laag, and W. Kuis. 1994. Prevalence of circulating immune complexes in patients with cystic fibrosis and arthritis. J. Pediatr. 125: 374-378.
    • (1994) J. Pediatr. , vol.125 , pp. 374-378
    • Wulffraat, N.M.1    De Graeff Meeder, E.R.2    Rijkers, G.T.3    Van Der Laag, H.4    Kuis, W.5
  • 348
    • 0027638571 scopus 로고
    • Arthropathies of cystic fibrosis: Case reports and review of the literature
    • Lawrence, J. M., III, T. L. Moore, K. L. Madson, A. J. Rejent, and T. G. Osborn. 1993. Arthropathies of cystic fibrosis: case reports and review of the literature. J. Rheumatol. 20(Suppl. 38):12-15.
    • (1993) J. Rheumatol. , vol.20 , Issue.38 SUPPL. , pp. 12-15
    • Lawrence III, J.M.1    Moore, T.L.2    Madson, K.L.3    Rejent, A.J.4    Osborn, T.G.5
  • 349
    • 0026551433 scopus 로고
    • Vasculitis and arthropathy in cystic fibrosis
    • Hodson, M. E. 1992. Vasculitis and arthropathy in cystic fibrosis. J. R. Soc. Med. 85(Suppl. 19):38-40.
    • (1992) J. R. Soc. Med. , vol.85 , Issue.19 SUPPL. , pp. 38-40
    • Hodson, M.E.1
  • 351
    • 0027102737 scopus 로고
    • Lung transplantation in cystic fibrosis
    • Egan, T. M. 1992. Lung transplantation in cystic fibrosis. Semin. Respir. Infect. 7:227-239.
    • (1992) Semin. Respir. Infect. , vol.7 , pp. 227-239
    • Egan, T.M.1
  • 352
    • 0026764371 scopus 로고
    • Current status of lung transplantation for cystic fibrosis
    • Shennib, H., R. Adoumie, and H. Noirclerc. 1992. Current status of lung transplantation for cystic fibrosis. Arch. Intern. Med. 152: 1585-1588.
    • (1992) Arch. Intern. Med. , vol.152 , pp. 1585-1588
    • Shennib, H.1    Adoumie, R.2    Noirclerc, H.3
  • 353
    • 0026133899 scopus 로고
    • Cystic fibrosis. 5. The current state of lung transplantation for cystic fibrosis
    • Smyth, R. L., T. Higenbottam, J. Scott, and J. Wallwork. 1991. Cystic fibrosis. 5. The current state of lung transplantation for cystic fibrosis. Thorax 46:213-216.
    • (1991) Thorax , vol.46 , pp. 213-216
    • Smyth, R.L.1    Higenbottam, T.2    Scott, J.3    Wallwork, J.4
  • 354
    • 0026495687 scopus 로고
    • Lung transplantation for cystic fibrosis
    • Tsang, V., M. E. Hodson, and M. H. Yacoub. 1992. Lung transplantation for cystic fibrosis. Br. Med. Bull. 48:949-971.
    • (1992) Br. Med. Bull. , vol.48 , pp. 949-971
    • Tsang, V.1    Hodson, M.E.2    Yacoub, M.H.3
  • 355
    • 0018089486 scopus 로고
    • Assisted ventilation for patients with cystic fibrosis
    • Davis, P. B., and P. A. di Sant'Agnese. 1978. Assisted ventilation for patients with cystic fibrosis. J.A.M.A. 239:1851-1854.
    • (1978) J.A.M.A. , vol.239 , pp. 1851-1854
    • Davis, P.B.1    Di Sant'Agnese, P.A.2
  • 356
    • 0025829901 scopus 로고
    • Noninvasive mechanical ventilation for cystic fibrosis patients: A potential bridge to transplantation
    • Hodson, M. E., B. P. Madden, M. H. Steven, V. T. Tsang, and M. H. Yacoub. 1991. Noninvasive mechanical ventilation for cystic fibrosis patients: a potential bridge to transplantation. Eur. Respir. J. 4:524-527.
    • (1991) Eur. Respir. J. , vol.4 , pp. 524-527
    • Hodson, M.E.1    Madden, B.P.2    Steven, M.H.3    Tsang, V.T.4    Yacoub, M.H.5
  • 357
    • 0026918861 scopus 로고
    • Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis
    • Johnson, L. G., J. C. Olsen, B. Sarkadi, K. L. Moore, R. Swanstrom, and R. C. Boucher. 1992. Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis. Nat. Genet. 2:21-25.
    • (1992) Nat. Genet. , vol.2 , pp. 21-25
    • Johnson, L.G.1    Olsen, J.C.2    Sarkadi, B.3    Moore, K.L.4    Swanstrom, R.5    Boucher, R.C.6
  • 360
    • 0027377523 scopus 로고
    • Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis
    • Zabner, J., L. A. Couture, R. J. Gregory, S. M. Graham, A. E. Smith, and M. J. Welsh. 1993. Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis. Cell 75:207-216.
    • (1993) Cell , vol.75 , pp. 207-216
    • Zabner, J.1    Couture, L.A.2    Gregory, R.J.3    Graham, S.M.4    Smith, A.E.5    Welsh, M.J.6
  • 361
    • 0028429545 scopus 로고
    • Adenovirus-mediated gene transfer for cystic fibrosis: Quantitative evaluation of repeated in vivo vector administration to the lung
    • Yei, S., N. Mittereder, K. Tang, C. O'Sullivan, and B. C. Trapnell. 1994. Adenovirus-mediated gene transfer for cystic fibrosis: quantitative evaluation of repeated in vivo vector administration to the lung. Gene Therap. 1:192-200.
    • (1994) Gene Therap. , vol.1 , pp. 192-200
    • Yei, S.1    Mittereder, N.2    Tang, K.3    O'Sullivan, C.4    Trapnell, B.C.5
  • 363
    • 0028229147 scopus 로고
    • Inactivation of E2a in recombinant adenoviruses improves the prospect for gene therapy in cystic fibrosis
    • Yang, Y., F. A. Nunes, K. Berencsi, E. Gönczöl, J. R. Engelhardt, and J. M. Wilson. 1994. Inactivation of E2a in recombinant adenoviruses improves the prospect for gene therapy in cystic fibrosis. Nat. Genet. 7:362-369.
    • (1994) Nat. Genet. , vol.7 , pp. 362-369
    • Yang, Y.1    Nunes, F.A.2    Berencsi, K.3    Gönczöl, E.4    Engelhardt, J.R.5    Wilson, J.M.6
  • 364
    • 0028978493 scopus 로고
    • Recombinant IL-12 prevents formation of blocking IgA antibodies to recombinant adenovirus and allows repeated gene therapy to mouse lung
    • Yang, Y., G. Trinchieri, and J. M. Wilson. 1995. Recombinant IL-12 prevents formation of blocking IgA antibodies to recombinant adenovirus and allows repeated gene therapy to mouse lung. Nat. Med. 1:890-893.
    • (1995) Nat. Med. , vol.1 , pp. 890-893
    • Yang, Y.1    Trinchieri, G.2    Wilson, J.M.3
  • 366
    • 0027412209 scopus 로고
    • Expression of the cystic fibrosis transmembrane conductance regulator from a novel adeno-associated virus promoter
    • Flotte, T. R., S. A. Afione, R. Solow, M. L. Drumm, D. Markakis, W. B. Guggino, P. L. Zeitlin, and B. J. Carter. 1993. Expression of the cystic fibrosis transmembrane conductance regulator from a novel adeno-associated virus promoter. J. Biol. Chem. 268:3781-3790.
    • (1993) J. Biol. Chem. , vol.268 , pp. 3781-3790
    • Flotte, T.R.1    Afione, S.A.2    Solow, R.3    Drumm, M.L.4    Markakis, D.5    Guggino, W.B.6    Zeitlin, P.L.7    Carter, B.J.8
  • 369
    • 0027475701 scopus 로고
    • Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy
    • Hyde, S. C., D. R. Gill, C. F. Higgins, and Ann E. O. Trezise. 1993. Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy. Nature 362:250-255.
    • (1993) Nature , vol.362 , pp. 250-255
    • Hyde, S.C.1    Gill, D.R.2    Higgins, C.F.3    Trezise, A.E.O.4
  • 372
    • 0028799484 scopus 로고
    • Gene transfer into the airway epithelium of animals by targeting the polymeric immunoglobulin receptor
    • Ferkol, T., J. C. Perales, E. Eckman, C. S. Kaetzel, R. W. Hanson, and P. B. Davis. 1995. Gene transfer into the airway epithelium of animals by targeting the polymeric immunoglobulin receptor. J. Clin. Invest. 95:493-502.
    • (1995) J. Clin. Invest. , vol.95 , pp. 493-502
    • Ferkol, T.1    Perales, J.C.2    Eckman, E.3    Kaetzel, C.S.4    Hanson, R.W.5    Davis, P.B.6
  • 374
    • 0028912888 scopus 로고
    • Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells
    • Johsnon, L. G., S. E. Boyles, J. Wilson, and R. C. Boucher. 1995. Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells. J. Clin. Invest. 95:1377-1382.
    • (1995) J. Clin. Invest. , vol.95 , pp. 1377-1382
    • Johsnon, L.G.1    Boyles, S.E.2    Wilson, J.3    Boucher, R.C.4
  • 375
    • 0028878052 scopus 로고
    • Gene therapy in xenograft model of cystic fibrosis lung corrects chloride transport more effectively than the sodium defect
    • Goldman, M. J., Y. Yang, and J. M. Wilson. 1995. Gene therapy in xenograft model of cystic fibrosis lung corrects chloride transport more effectively than the sodium defect. Nat. Genet. 9:126-131.
    • (1995) Nat. Genet. , vol.9 , pp. 126-131
    • Goldman, M.J.1    Yang, Y.2    Wilson, J.M.3


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