메뉴 건너뛰기




Volumn , Issue , 2008, Pages 447-459

α1-Antitrypsin Deficiency

Author keywords

1 antitrypsin; Bronchitis; Emphysema; Neutrophils; Polymerization

Indexed keywords


EID: 84857102829     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1002/9780470755976.ch38     Document Type: Chapter
Times cited : (2)

References (129)
  • 1
    • 84907041622 scopus 로고
    • The electrophoretic α1-globulin pattern of serum in α1-antitrypsin deficiency
    • Laurell C-B, Eriksson A. The electrophoretic α1-globulin pattern of serum in α1-antitrypsin deficiency. Scand J Clin Lab Invest 1963;15:132-40.
    • (1963) Scand J Clin Lab Invest , vol.15 , pp. 132-140
    • Laurell, C.-B.1    Eriksson, A.2
  • 2
    • 0000461577 scopus 로고
    • Studies in α1-antitrypsin deficiency
    • Eriksson S. Studies in α1-antitrypsin deficiency. Acta Med Scand 1964;175:197-205.
    • (1964) Acta Med Scand , vol.175 , pp. 197-205
    • Eriksson, S.1
  • 3
    • 0013828812 scopus 로고
    • Studies in α1-antitrypsin deficiency
    • Eriksson S. Studies in α1-antitrypsin deficiency. Acta Med Scand Suppl 1965;432:1-85.
    • (1965) Acta Med Scand Suppl , vol.432 , pp. 1-85
    • Eriksson, S.1
  • 4
    • 0007442571 scopus 로고
    • Enzymatically produced pulmonary emphysema: a preliminary report
    • Gross P, Babyak MA, Tolker E, Kaschak M. Enzymatically produced pulmonary emphysema: a preliminary report. J Occup Med 1964;6:481-4.
    • (1964) J Occup Med , vol.6 , pp. 481-484
    • Gross, P.1    Babyak, M.A.2    Tolker, E.3    Kaschak, M.4
  • 6
    • 0013902105 scopus 로고
    • A possible experimental approach to the association of hereditary α1-antitrypsin deficiency and pulmonary emphysema
    • Kueppers F, Bearn AG. A possible experimental approach to the association of hereditary α1-antitrypsin deficiency and pulmonary emphysema. Proc Soc Exp Biol Med 1966;121:1207-9.
    • (1966) Proc Soc Exp Biol Med , vol.121 , pp. 1207-1209
    • Kueppers, F.1    Bearn, A.G.2
  • 7
    • 0021919760 scopus 로고
    • Elastases and emphysema: current assessment of the protease-antiprotease hypothesis
    • Janoff A. Elastases and emphysema: current assessment of the protease-antiprotease hypothesis. Am Rev Respir Dis 1985;132:417-33.
    • (1985) Am Rev Respir Dis , vol.132 , pp. 417-433
    • Janoff, A.1
  • 8
    • 0014349683 scopus 로고
    • Mediators of inflammation in leukocyte lysosomes IX. Elastinolytic activity in granules of human polymorphonuclear leukocytes
    • Janoff A, Scherer J. Mediators of inflammation in leukocyte lysosomes. IX. Elastinolytic activity in granules of human polymorphonuclear leukocytes. J Exp Med 1968;128:1137-55.
    • (1968) J Exp Med , vol.128 , pp. 1137-1155
    • Janoff, A.1    Scherer, J.2
  • 9
    • 0015151976 scopus 로고
    • Neutral leucocyte proteases and elastase inhibited by plasma α1-antitrypsin
    • Ohlsson K. Neutral leucocyte proteases and elastase inhibited by plasma α1-antitrypsin. Scand J Clin Lab Invest 1971;28:251-3.
    • (1971) Scand J Clin Lab Invest , vol.28 , pp. 251-253
    • Ohlsson, K.1
  • 10
    • 0017669252 scopus 로고
    • The induction of pulmonary emphysema with human leukocyte elastase
    • Senior RM, Tegner H, Kuhn C et al. The induction of pulmonary emphysema with human leukocyte elastase. Am Rev Respir Dis 1977;116:469-75.
    • (1977) Am Rev Respir Dis , vol.116 , pp. 469-475
    • Senior, R.M.1    Tegner, H.2    Kuhn, C.3
  • 11
    • 0016811228 scopus 로고
    • The pathology of elastaseinduced panacinar emphysema in hamsters
    • Hayes JA, Korthy A, Snider GL. The pathology of elastaseinduced panacinar emphysema in hamsters. J Pathol 1975;117:1-14.
    • (1975) J Pathol , vol.117 , pp. 1-14
    • Hayes, J.A.1    Korthy, A.2    Snider, G.L.3
  • 12
    • 0021752456 scopus 로고
    • Complete sequence of the cDNA for human α1-antitrypsin and the gene for the S variant
    • Long GL, Chandra T, Woo SL, Davie EW, Kurachi K. Complete sequence of the cDNA for human α1-antitrypsin and the gene for the S variant. Biochemistry 1984;23:4828-37.
    • (1984) Biochemistry , vol.23 , pp. 4828-4837
    • Long, G.L.1    Chandra, T.2    Woo, S.L.3    Davie, E.W.4    Kurachi, K.5
  • 13
    • 0023898432 scopus 로고
    • Molecular basis of α1-antitrypsin deficiency
    • Brantly M, Nukiwa T, Crystal RG. Molecular basis of α1-antitrypsin deficiency. Am J Med 1988;84:13-31.
    • (1988) Am J Med , vol.84 , pp. 13-31
    • Brantly, M.1    Nukiwa, T.2    Crystal, R.G.3
  • 14
    • 0014530551 scopus 로고
    • Cirrhosis associated with α1-antitrypsin deficiency: a previously unrecognized inherited disorder
    • Sharp HL, Bridges RA, Krivit W, Freier EF. Cirrhosis associated with α1-antitrypsin deficiency: a previously unrecognized inherited disorder. J Lab Clin Med 1969;73:934-9.
    • (1969) J Lab Clin Med , vol.73 , pp. 934-939
    • Sharp, H.L.1    Bridges, R.A.2    Krivit, W.3    Freier, E.F.4
  • 15
    • 0026755363 scopus 로고
    • The mechanism of Z α1-antitrypsin accumulation in the liver
    • Lomas DA, Evans DL, Finch JT, Carrell RW. The mechanism of Z α1-antitrypsin accumulation in the liver. Nature 1992;357:605-7.
    • (1992) Nature , vol.357 , pp. 605-607
    • Lomas, D.A.1    Evans, D.L.2    Finch, J.T.3    Carrell, R.W.4
  • 16
    • 0023899080 scopus 로고
    • α1-Antitrypsin augmentation therapy for α1-antitrypsin deficiency
    • Hubbard RC, Crystal RG. α1-Antitrypsin augmentation therapy for α1-antitrypsin deficiency. Am J Med 1988;84:52-62.
    • (1988) Am J Med , vol.84 , pp. 52-62
    • Hubbard, R.C.1    Crystal, R.G.2
  • 17
    • 0027941322 scopus 로고
    • A registry of patients with severe deficiency of α1-antitrypsin: design and methods
    • The Alpha-One Antitrypsin Deficiency Registry Study Group
    • The Alpha-One Antitrypsin Deficiency Registry Study Group. A registry of patients with severe deficiency of α1-antitrypsin: design and methods. Chest 1994;106:1223-32.
    • (1994) Chest , vol.106 , pp. 1223-1232
  • 18
    • 0033796561 scopus 로고    scopus 로고
    • Formation and current results of a patient-organized registry for α1-antitrypsin deficiency
    • Stoller JK, Brantly M, Fleming LE, Bean JA, Walsh J. Formation and current results of a patient-organized registry for α1-antitrypsin deficiency. Chest 2000;118:843-8.
    • (2000) Chest , vol.118 , pp. 843-848
    • Stoller, J.K.1    Brantly, M.2    Fleming, L.E.3    Bean, J.A.4    Walsh, J.5
  • 19
    • 3242687191 scopus 로고    scopus 로고
    • Summary health statistics for US adults: National Health Interview Survey, 1997
    • National Center for Health Statistics
    • Blackwell DL, Collins JG, Coles R. Summary health statistics for US adults: National Health Interview Survey, 1997. National Center for Health Statistics. Vital Health Stat 2002;10:205.
    • (2002) Vital Health Stat , vol.10 , pp. 205
    • Blackwell, D.L.1    Collins, J.G.2    Coles, R.3
  • 20
    • 0022638237 scopus 로고
    • α1-Antitrypsin Pi-types in 965 COPD patients
    • Lieberman J, Winter B, Sastre A. α1-Antitrypsin Pi-types in 965 COPD patients. Chest 1986;89:370-3.
    • (1986) Chest , vol.89 , pp. 370-373
    • Lieberman, J.1    Winter, B.2    Sastre, A.3
  • 21
    • 0036433482 scopus 로고    scopus 로고
    • Worldwide racial and ethnic distribution of α1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys
    • de Serres FJ. Worldwide racial and ethnic distribution of α1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys. Chest 2002;122:1818-29.
    • (2002) Chest , vol.122 , pp. 1818-1829
    • de Serres, F.J.1
  • 22
    • 0242525259 scopus 로고    scopus 로고
    • Genetic epidemiology of α1-antitrypsin deficiency in North America and Australia/New Zealand: Australia, Canada, New Zealand, and the United States of America
    • de Serres FJ, Blanco I, Fernández-Bustillo E. Genetic epidemiology of α1-antitrypsin deficiency in North America and Australia/New Zealand: Australia, Canada, New Zealand, and the United States of America. Clin Genet 2003;64:382-97.
    • (2003) Clin Genet , vol.64 , pp. 382-397
    • de Serres, F.J.1    Blanco, I.2    Fernández-Bustillo, E.3
  • 23
    • 0017902246 scopus 로고
    • Neonatal screening for α1-antitrypsin deficiency
    • O'Brien ML, Buist NR, Murphey WH. Neonatal screening for α1-antitrypsin deficiency. J Pediatr 1978;92:1006-10.
    • (1978) J Pediatr , vol.92 , pp. 1006-1010
    • O'Brien, M.L.1    Buist, N.R.2    Murphey, W.H.3
  • 24
    • 0024434432 scopus 로고
    • α1-Antitrypsin deficiency: high prevalence in the St Louis area determined by direct population screening
    • Silverman EK, Miletich JP, Pierce JA et al. α1-Antitrypsin deficiency: high prevalence in the St. Louis area determined by direct population screening. Am Rev Respir Dis 1989;140:961-6.
    • (1989) Am Rev Respir Dis , vol.140 , pp. 961-966
    • Silverman, E.K.1    Miletich, J.P.2    Pierce, J.A.3
  • 25
    • 0027429896 scopus 로고
    • Molecular confirmation of α1-antitrypsin genotypes in newborn dried blood specimens
    • Spence WC, Morris JE, Pass K, Murphy PD. Molecular confirmation of α1-antitrypsin genotypes in newborn dried blood specimens. Biochem Med Metab Biol 1993;50:233-40.
    • (1993) Biochem Med Metab Biol , vol.50 , pp. 233-240
    • Spence, W.C.1    Morris, J.E.2    Pass, K.3    Murphy, P.D.4
  • 26
    • 0017099344 scopus 로고
    • Liver disease in α1-antitrypsin deficiency detected by screening of 200 000 infants
    • Sveger T. Liver disease in α1-antitrypsin deficiency detected by screening of 200 000 infants. N Engl J Med 1976;294:1316-21.
    • (1976) N Engl J Med , vol.294 , pp. 1316-1321
    • Sveger, T.1
  • 27
    • 0004224063 scopus 로고    scopus 로고
    • Census 2000
    • US Census Bureau
    • US Census Bureau. Census 2000. 2002.
    • (2002)
  • 28
    • 0030465416 scopus 로고    scopus 로고
    • A 30-year perspective on α1-antitrypsin deficiency
    • Eriksson S. A 30-year perspective on α1-antitrypsin deficiency. Chest 1996;110(Suppl): 237S-42S.
    • (1996) Chest , vol.110 , Issue.SUPPL
    • Eriksson, S.1
  • 29
    • 0002729721 scopus 로고
    • α1-Antitrypsin deficiency
    • In: Stanbury JB, Wyngaarden JB, Frederickson DS et al., eds. The Metabolic Basis of Inherited Disease. New York: McGraw-Hill
    • Gadek JE, Crystal RG. α1-Antitrypsin deficiency. In: Stanbury JB, Wyngaarden JB, Frederickson DS et al., eds. The Metabolic Basis of Inherited Disease. New York: McGraw-Hill, 1983:1450-67.
    • (1983) , pp. 1450-1467
    • Gadek, J.E.1    Crystal, R.G.2
  • 30
    • 0020678269 scopus 로고
    • α1-Antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z A survey by the British Thoracic Association
    • Tobin MJ, Cook PJ, Hutchison DC. α1-Antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association. Br J Dis Chest 1983;77:14-27.
    • (1983) Br J Dis Chest , vol.77 , pp. 14-27
    • Tobin, M.J.1    Cook, P.J.2    Hutchison, D.C.3
  • 31
    • 0031567183 scopus 로고    scopus 로고
    • Genetic testing for cystic fibrosis
    • NIH Consensus Statement Online 1997 Apr 14-16
    • Genetic testing for cystic fibrosis. NIH Consensus Statement Online 1997 Apr 14-16. 1997;15:1-37.
    • (1997) , vol.15 , pp. 1-37
  • 32
    • 0028543862 scopus 로고
    • Physical and social impact of α1-antitrypsin deficiency: results of a survey
    • Stoller JK, Smith P, Yang P, Spray J. Physical and social impact of α1-antitrypsin deficiency: results of a survey. Cleve Clin J Med 1994;61:461-7.
    • (1994) Cleve Clin J Med , vol.61 , pp. 461-467
    • Stoller, J.K.1    Smith, P.2    Yang, P.3    Spray, J.4
  • 33
    • 85047684827 scopus 로고    scopus 로고
    • α1-Antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy
    • Lomas DA, Mahadeva R. α1-Antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy. J Clin Invest 2002;110:1585-90.
    • (2002) J Clin Invest , vol.110 , pp. 1585-1590
    • Lomas, D.A.1    Mahadeva, R.2
  • 34
    • 0036789017 scopus 로고    scopus 로고
    • Serpinopathies and the conformational dementias
    • Lomas DA, Carrell RW. Serpinopathies and the conformational dementias. Nat Rev Genet 2002;3:759-68.
    • (2002) Nat Rev Genet , vol.3 , pp. 759-768
    • Lomas, D.A.1    Carrell, R.W.2
  • 35
    • 0033598346 scopus 로고    scopus 로고
    • Familial dementia caused by polymerization of mutant neuroserpin
    • Davis RL, Shrimpton AE, Holohan PD et al. Familial dementia caused by polymerization of mutant neuroserpin. Nature 1999;401:376-9.
    • (1999) Nature , vol.401 , pp. 376-379
    • Davis, R.L.1    Shrimpton, A.E.2    Holohan, P.D.3
  • 39
    • 0036882160 scopus 로고    scopus 로고
    • Acid denaturation of α1-antitrypsin: characterization of a novel mechanism of serpin polymerization
    • Devlin GL, Chow MK, Howlett GJ, Bottomley SP. Acid denaturation of α1-antitrypsin: characterization of a novel mechanism of serpin polymerization. J Mol Biol 2002;324:859-70.
    • (2002) J Mol Biol , vol.324 , pp. 859-870
    • Devlin, G.L.1    Chow, M.K.2    Howlett, G.J.3    Bottomley, S.P.4
  • 40
    • 0019453831 scopus 로고
    • Antielastases of the human alveolar structures: implications for the protease-antiprotease theory of emphysema
    • Gadek JE, Fells GA, Zimmerman RL, Rennard SI, Crystal RG. Antielastases of the human alveolar structures: implications for the protease-antiprotease theory of emphysema. J Clin Invest 1981;68:889-98.
    • (1981) J Clin Invest , vol.68 , pp. 889-898
    • Gadek, J.E.1    Fells, G.A.2    Zimmerman, R.L.3    Rennard, S.I.4    Crystal, R.G.5
  • 41
    • 0019321009 scopus 로고
    • Kinetics of association of serine proteinases with native and oxidized α1-proteinase inhibitor and α1-antichymotrypsin
    • Beatty K, Bieth J, Travis J. Kinetics of association of serine proteinases with native and oxidized α1-proteinase inhibitor and α1-antichymotrypsin. J Biol Chem 1980;255:3931-4.
    • (1980) J Biol Chem , vol.255 , pp. 3931-3934
    • Beatty, K.1    Bieth, J.2    Travis, J.3
  • 42
    • 0024529042 scopus 로고
    • The α1-antitrypsin gene and its mutations: clinical consequences and strategies for therapy
    • Crystal RG, Brantly ML, Hubbard RC et al. The α1-antitrypsin gene and its mutations: clinical consequences and strategies for therapy. Chest 1989;95:196-208.
    • (1989) Chest , vol.95 , pp. 196-208
    • Crystal, R.G.1    Brantly, M.L.2    Hubbard, R.C.3
  • 43
    • 0037271797 scopus 로고    scopus 로고
    • Gene therapy progress and prospects: α1-antitrypsin
    • Stecenko AA, Brigham KL. Gene therapy progress and prospects: α1-antitrypsin. Gene Ther 2003;10:95-9.
    • (2003) Gene Ther , vol.10 , pp. 95-99
    • Stecenko, A.A.1    Brigham, K.L.2
  • 44
    • 0023546615 scopus 로고
    • Z-type α1-antitrypsin is less competent than M1-type α1-antitrypsin as an inhibitor of neutrophil elastase
    • Ogushi F, Fells GA, Hubbard RC, Straus SD, Crystal RG. Z-type α1-antitrypsin is less competent than M1-type α1-antitrypsin as an inhibitor of neutrophil elastase. J Clin Invest 1987;80:1366-74.
    • (1987) J Clin Invest , vol.80 , pp. 1366-1374
    • Ogushi, F.1    Fells, G.A.2    Hubbard, R.C.3    Straus, S.D.4    Crystal, R.G.5
  • 45
    • 0032065494 scopus 로고    scopus 로고
    • Lung polymers in Z α1-antitrypsin deficiency-related emphysema
    • Elliott PR, Bilton D, Lomas DA. Lung polymers in Z α1-antitrypsin deficiency-related emphysema. Am J Respir Cell Mol Biol 1998;18:670-4.
    • (1998) Am J Respir Cell Mol Biol , vol.18 , pp. 670-674
    • Elliott, P.R.1    Bilton, D.2    Lomas, D.A.3
  • 46
    • 0036014834 scopus 로고    scopus 로고
    • Polymers of α1-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema
    • Parmar JS, Mahadeva R, Reed BJ et al. Polymers of α1-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema. Am J Respir Cell Mol Biol 2002;26:723-30.
    • (2002) Am J Respir Cell Mol Biol , vol.26 , pp. 723-730
    • Parmar, J.S.1    Mahadeva, R.2    Reed, B.J.3
  • 47
    • 0034126045 scopus 로고    scopus 로고
    • Airways inflammation in chronic bronchitis: the effects of smoking and α1-antitrypsin deficiency
    • Hill AT, Bayley DL, Campbell EJ, Hill SL, Stockley RA. Airways inflammation in chronic bronchitis: the effects of smoking and α1-antitrypsin deficiency. Eur Respir J 2000;15:886-90.
    • (2000) Eur Respir J , vol.15 , pp. 886-890
    • Hill, A.T.1    Bayley, D.L.2    Campbell, E.J.3    Hill, S.L.4    Stockley, R.A.5
  • 48
    • 0036347640 scopus 로고    scopus 로고
    • Sputum chemotactic activity in chronic obstructive pulmonary disease: effect of α1-antitrypsin deficiency and the role of leukotriene B4 and interleukin 8
    • Woolhouse IS, Bayley DL, Stockley RA. Sputum chemotactic activity in chronic obstructive pulmonary disease: effect of α1-antitrypsin deficiency and the role of leukotriene B4 and interleukin 8. Thorax 2002;57:709-14.
    • (2002) Thorax , vol.57 , pp. 709-714
    • Woolhouse, I.S.1    Bayley, D.L.2    Stockley, R.A.3
  • 49
    • 0016832609 scopus 로고
    • Modulation of human neutrophil polymorphonuclear leucocyte migration by human plasma α-globulin inhibitors and synthetic esterase inhibitors
    • Goetzl EJ. Modulation of human neutrophil polymorphonuclear leucocyte migration by human plasma α-globulin inhibitors and synthetic esterase inhibitors. Immunology 1975;29:163-74.
    • (1975) Immunology , vol.29 , pp. 163-174
    • Goetzl, E.J.1
  • 50
    • 85047683832 scopus 로고    scopus 로고
    • Liver injury in α1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury
    • Perlmutter DH. Liver injury in α1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury. J Clin Invest 2002;110:1579-83.
    • (2002) J Clin Invest , vol.110 , pp. 1579-1583
    • Perlmutter, D.H.1
  • 51
    • 0036295592 scopus 로고    scopus 로고
    • Coexpression and accumulation of ubiquitin +1 and ZZ proteins in livers of children with α1-antitrypsin deficiency
    • Wu SS, de Chadarevian JP, McPhaul L et al. Coexpression and accumulation of ubiquitin +1 and ZZ proteins in livers of children with α1-antitrypsin deficiency. Pediatr Dev Pathol 2002;5:293-8.
    • (2002) Pediatr Dev Pathol , vol.5 , pp. 293-298
    • Wu, S.S.1    de Chadarevian, J.P.2    McPhaul, L.3
  • 52
    • 0029788023 scopus 로고    scopus 로고
    • Degradation of a mutant secretory protein, α1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity
    • Qu D, Teckman JH, Omura S, Perlmutter DH. Degradation of a mutant secretory protein, α1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity. J Biol Chem 1996;271:22791-5.
    • (1996) J Biol Chem , vol.271 , pp. 22791-22795
    • Qu, D.1    Teckman, J.H.2    Omura, S.3    Perlmutter, D.H.4
  • 53
    • 0023795389 scopus 로고
    • Clinical features and history of the destructive lung disease associated with α1-antitrypsin deficiency of adults with pulmonary symptoms
    • Brantly ML, Paul LD, Miller BH et al. Clinical features and history of the destructive lung disease associated with α1-antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis 1988;138:327-36.
    • (1988) Am Rev Respir Dis , vol.138 , pp. 327-336
    • Brantly, M.L.1    Paul, L.D.2    Miller, B.H.3
  • 54
    • 0019963490 scopus 로고
    • α1-Antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British Thoracic Association
    • Gishen P, Saunders AJ, Tobin MJ, Hutchison DC. α1-Antitrypsin deficiency: the radiological features of pulmonary emphysema in subjects of Pi type Z and Pi type SZ: a survey by the British Thoracic Association. Clin Radiol 1982;33:371-7.
    • (1982) Clin Radiol , vol.33 , pp. 371-377
    • Gishen, P.1    Saunders, A.J.2    Tobin, M.J.3    Hutchison, D.C.4
  • 55
    • 0033070995 scopus 로고    scopus 로고
    • Z and S mutations of the α1-antitrypsin gene and the risk of chronic obstructive pulmonary disease
    • Sandford AJ, Weir TD, Spinelli JJ, Pare PD. Z and S mutations of the α1-antitrypsin gene and the risk of chronic obstructive pulmonary disease. Am J Respir Cell Mol Biol 1999;20:287-91.
    • (1999) Am J Respir Cell Mol Biol , vol.20 , pp. 287-291
    • Sandford, A.J.1    Weir, T.D.2    Spinelli, J.J.3    Pare, P.D.4
  • 56
    • 0030446288 scopus 로고    scopus 로고
    • Clinical features of individuals with PI*SZ phenotype of α1-antitrypsin deficiency
    • Alpha-One Antitrypsin Deficiency Registry Study Group
    • Turino GM, Barker AF, Brantly ML et al. Clinical features of individuals with PI*SZ phenotype of α1-antitrypsin deficiency. Alpha-One Antitrypsin Deficiency Registry Study Group. Am J Respir Crit Care Med 1996;154:1718-25.
    • (1996) Am J Respir Crit Care Med , vol.154 , pp. 1718-1725
    • Turino, G.M.1    Barker, A.F.2    Brantly, M.L.3
  • 58
    • 0037133110 scopus 로고    scopus 로고
    • Change in lung function and morbidity from chronic obstructive pulmonary disease in α1-antitrypsin MZ heterozygotes: a longitudinal study of the general population
    • Dahl M, Tybjaerg-Hansen A, Lange P, Vestbo J, Nordestgaard BG. Change in lung function and morbidity from chronic obstructive pulmonary disease in α1-antitrypsin MZ heterozygotes: a longitudinal study of the general population. Ann Intern Med 2002;136:270-9.
    • (2002) Ann Intern Med , vol.136 , pp. 270-279
    • Dahl, M.1    Tybjaerg-Hansen, A.2    Lange, P.3    Vestbo, J.4    Nordestgaard, B.G.5
  • 59
    • 0037541112 scopus 로고    scopus 로고
    • A longitudinal study of α1-antitrypsin phenotypes and decline in FEV1 in a community population
    • Silva GE, Sherrill DL, Guerra S, Barbee RA. A longitudinal study of α1-antitrypsin phenotypes and decline in FEV1 in a community population. Chest 2003;123:1435-40.
    • (2003) Chest , vol.123 , pp. 1435-1440
    • Silva, G.E.1    Sherrill, D.L.2    Guerra, S.3    Barbee, R.A.4
  • 60
    • 0037338319 scopus 로고    scopus 로고
    • Asthma features in severe α1-antitrypsin deficiency: experience of the National Heart, Lung, and Blood Institute Registry
    • Eden E, Hammel J, Rouhani FN et al. Asthma features in severe α1-antitrypsin deficiency: experience of the National Heart, Lung, and Blood Institute Registry. Chest 2003;123:765-71.
    • (2003) Chest , vol.123 , pp. 765-771
    • Eden, E.1    Hammel, J.2    Rouhani, F.N.3
  • 61
    • 0036341010 scopus 로고    scopus 로고
    • Respiratory symptoms and lung function in young adults with severe α1-antitrypsin deficiency (PiZZ)
    • Piitulainen E, Sveger T. Respiratory symptoms and lung function in young adults with severe α1-antitrypsin deficiency (PiZZ). Thorax 2002;57:705-8.
    • (2002) Thorax , vol.57 , pp. 705-708
    • Piitulainen, E.1    Sveger, T.2
  • 62
    • 0037335767 scopus 로고    scopus 로고
    • Airway hyperresponsiveness in a large group of subjects with α1-antitrypsin deficiency: a cross-sectional controlled study
    • Malerba M, Radaeli A, Ceriani L, Tantucci C, Grassi V. Airway hyperresponsiveness in a large group of subjects with α1-antitrypsin deficiency: a cross-sectional controlled study. J Intern Med 2003;253:351-8.
    • (2003) J Intern Med , vol.253 , pp. 351-358
    • Malerba, M.1    Radaeli, A.2    Ceriani, L.3    Tantucci, C.4    Grassi, V.5
  • 63
    • 0026877235 scopus 로고
    • α1-Protease inhibitor in bronchial asthma: phenotypes and biochemical characteristics
    • Gaillard MC, Kilroe-Smith TA, Nogueira C et al. α1-Protease inhibitor in bronchial asthma: phenotypes and biochemical characteristics. Am Rev Respir Dis 1992;145:1311-5.
    • (1992) Am Rev Respir Dis , vol.145 , pp. 1311-1315
    • Gaillard, M.C.1    Kilroe-Smith, T.A.2    Nogueira, C.3
  • 64
    • 2642680855 scopus 로고    scopus 로고
    • α1-Antitrypsin deficiency: memorandum from a WHO meeting
    • α1-Antitrypsin deficiency: memorandum from a WHO meeting. Bull World Health Organ 1997;75:397-415.
    • (1997) Bull World Health Organ , vol.75 , pp. 397-415
  • 65
    • 0347075558 scopus 로고    scopus 로고
    • Standards for the diagnosis and management of individuals with α1-antitrypsin deficiency
    • for the α1-antitrypsin Deficiency Task Force
    • Stoller JK, Snider GL, Fallat RA, Stockley RA, Brantly M, for the α1-antitrypsin Deficiency Task Force. Standards for the diagnosis and management of individuals with α1-antitrypsin deficiency. Am J Respir Crit Care Med 2003;168:819-900.
    • (2003) Am J Respir Crit Care Med , vol.168 , pp. 819-900
    • Stoller, J.K.1    Snider, G.L.2    Fallat, R.A.3    Stockley, R.A.4    Brantly, M.5
  • 66
    • 0036430532 scopus 로고    scopus 로고
    • The relationship of chronic sputum expectoration to physiologic, radiologic, and health status characteristics in α1-antitrypsin deficiency (PiZ)
    • Dowson LJ, Guest PJ, Stockley RA. The relationship of chronic sputum expectoration to physiologic, radiologic, and health status characteristics in α1-antitrypsin deficiency (PiZ). Chest 2002;122:1247-55.
    • (2002) Chest , vol.122 , pp. 1247-1255
    • Dowson, L.J.1    Guest, P.J.2    Stockley, R.A.3
  • 67
    • 0029963017 scopus 로고    scopus 로고
    • α1-Antitrypsin deficiency: evaluation of bronchiectasis with CT
    • King MA, Stone JA, Diaz PT et al. α1-Antitrypsin deficiency: evaluation of bronchiectasis with CT. Radiology 1996;199:137-41.
    • (1996) Radiology , vol.199 , pp. 137-141
    • King, M.A.1    Stone, J.A.2    Diaz, P.T.3
  • 68
    • 0027519368 scopus 로고
    • Bronchiectasis in patients with α1-antitrypsin deficiency: a rare occurrence?
    • Shin MS, Ho KJ. Bronchiectasis in patients with α1-antitrypsin deficiency: a rare occurrence? Chest 1993;104:1384-6.
    • (1993) Chest , vol.104 , pp. 1384-1386
    • Shin, M.S.1    Ho, K.J.2
  • 69
    • 0033995116 scopus 로고    scopus 로고
    • Distribution of α1-antitrypsin alleles in patients with bronchiectasis
    • Cuvelier A, Muir JF, Hellot MF et al. Distribution of α1-antitrypsin alleles in patients with bronchiectasis. Chest 2000;117:415-9.
    • (2000) Chest , vol.117 , pp. 415-419
    • Cuvelier, A.1    Muir, J.F.2    Hellot, M.F.3
  • 70
    • 0033773862 scopus 로고    scopus 로고
    • An investigation into causative factors in patients with bronchiectasis
    • Pasteur MC, Helliwell SM, Houghton SJ et al. An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000;162:1277-84.
    • (2000) Am J Respir Crit Care Med , vol.162 , pp. 1277-1284
    • Pasteur, M.C.1    Helliwell, S.M.2    Houghton, S.J.3
  • 71
    • 0002199299 scopus 로고    scopus 로고
    • Reactive airways disease and α1-antitrypsin deficiency
    • In: Crystal RG, ed. α1-Antitrypsin Deficiency: Biology, Pathogenesis, Clinical Manifestations, Therapy. New York: Marcel Dekker
    • Fallat RJ. Reactive airways disease and α1-antitrypsin deficiency. In: Crystal RG, ed. α1-Antitrypsin Deficiency: Biology, Pathogenesis, Clinical Manifestations, Therapy. New York: Marcel Dekker, 1996:259-79.
    • (1996) , pp. 259-279
    • Fallat, R.J.1
  • 72
    • 0031729205 scopus 로고    scopus 로고
    • Environmental correlates of impaired lung function in non-smokers with severe α1-antitrypsin deficiency (PiZZ)
    • Piitulainen E, Tornling G, Eriksson S. Environmental correlates of impaired lung function in non-smokers with severe α1-antitrypsin deficiency (PiZZ). Thorax 1998;53:939-43.
    • (1998) Thorax , vol.53 , pp. 939-943
    • Piitulainen, E.1    Tornling, G.2    Eriksson, S.3
  • 73
    • 0025815961 scopus 로고
    • α1-Antitrypsin deficiency and liver disease: clinical presentation, diagnosis and treatment
    • Hussain M, Mieli-Vergani G, Mowat AP. α1-Antitrypsin deficiency and liver disease: clinical presentation, diagnosis and treatment. J Inherit Metab Dis 1991;14:497-511.
    • (1991) J Inherit Metab Dis , vol.14 , pp. 497-511
    • Hussain, M.1    Mieli-Vergani, G.2    Mowat, A.P.3
  • 74
    • 0022637178 scopus 로고
    • Risk of cirrhosis and primary liver cancer in α1-antitrypsin deficiency
    • Eriksson S, Carlson J, Velez R. Risk of cirrhosis and primary liver cancer in α1-antitrypsin deficiency. N Engl J Med 1986;314:736-9.
    • (1986) N Engl J Med , vol.314 , pp. 736-739
    • Eriksson, S.1    Carlson, J.2    Velez, R.3
  • 75
  • 76
    • 0030587946 scopus 로고    scopus 로고
    • Treatment of α1-antitrypsin deficiency, massive edema, and panniculitis with α1-protease inhibitor
    • Furey NL, Golden RS, Potts SR. Treatment of α1-antitrypsin deficiency, massive edema, and panniculitis with α1-protease inhibitor. Ann Intern Med 1996;125:699.
    • (1996) Ann Intern Med , vol.125 , pp. 699
    • Furey, N.L.1    Golden, R.S.2    Potts, S.R.3
  • 78
    • 0015407888 scopus 로고
    • Weber-Christian syndrome associated with an α1-antitrypsin deficiency: familial investigation
    • (Paris)
    • Warter J, Storck D, Grosshans E et al. Weber-Christian syndrome associated with an α1-antitrypsin deficiency: familial investigation. Ann Med Interne (Paris) 1972;123:877-82.
    • (1972) Ann Med Interne , vol.123 , pp. 877-882
    • Warter, J.1    Storck, D.2    Grosshans, E.3
  • 79
    • 0026326794 scopus 로고
    • α1-Antitrypsin deficiency-associated panniculitis: case report and review of the literature
    • Edmonds BK, Hodge JA, Rietschel RL. α1-Antitrypsin deficiency-associated panniculitis: case report and review of the literature. Pediatr Dermatol 1991;8:296-9.
    • (1991) Pediatr Dermatol , vol.8 , pp. 296-299
    • Edmonds, B.K.1    Hodge, J.A.2    Rietschel, R.L.3
  • 80
    • 0035724197 scopus 로고    scopus 로고
    • Suppurative panniculitis associated with α1-antitrypsin deficiency (PiSZ phenotype) treated with doxycycline
    • Chng WJ, Henderson CA. Suppurative panniculitis associated with α1-antitrypsin deficiency (PiSZ phenotype) treated with doxycycline. Br J Dermatol 2001;144:1282-3.
    • (2001) Br J Dermatol , vol.144 , pp. 1282-1283
    • Chng, W.J.1    Henderson, C.A.2
  • 81
    • 0027528997 scopus 로고
    • Systemic nodular panniculitis in a patient with α1-antitrypsin deficiency (PiSS phenotype)
    • Pinto AR, Maciel LS, Carneiro F et al. Systemic nodular panniculitis in a patient with α1-antitrypsin deficiency (PiSS phenotype). Clin Exp Dermatol 1993;18:154-5.
    • (1993) Clin Exp Dermatol , vol.18 , pp. 154-155
    • Pinto, A.R.1    Maciel, L.S.2    Carneiro, F.3
  • 83
    • 0035432405 scopus 로고    scopus 로고
    • Treatment of α1-antitrypsin-deficiency panniculitis with minocycline
    • Ginarte M, Roson E, Peteiro C, Toribio J. Treatment of α1-antitrypsin-deficiency panniculitis with minocycline. Cutis 2001;68:86-8.
    • (2001) Cutis , vol.68 , pp. 86-88
    • Ginarte, M.1    Roson, E.2    Peteiro, C.3    Toribio, J.4
  • 84
    • 0025780098 scopus 로고
    • Use of anticollagenase properties of doxycycline in treatment of α1-antitrypsin deficiency panniculitis
    • Humbert P, Faivre B, Gibey R, Agache P. Use of anticollagenase properties of doxycycline in treatment of α1-antitrypsin deficiency panniculitis. Acta Derm Venereol 1991;71:189-94.
    • (1991) Acta Derm Venereol , vol.71 , pp. 189-194
    • Humbert, P.1    Faivre, B.2    Gibey, R.3    Agache, P.4
  • 85
    • 0031028798 scopus 로고    scopus 로고
    • α1-Antitrypsin deficiency-associated panniculitis: resolution with intravenous α1-antitrypsin administration and liver transplantation
    • O'Riordan K, Blei A, Rao MS, Abecassis M. α1-Antitrypsin deficiency-associated panniculitis: resolution with intravenous α1-antitrypsin administration and liver transplantation. Transplantation 1997;63:480-2.
    • (1997) Transplantation , vol.63 , pp. 480-482
    • O'Riordan, K.1    Blei, A.2    Rao, M.S.3    Abecassis, M.4
  • 86
    • 0036887247 scopus 로고    scopus 로고
    • Severe panniculitis caused by homozygous ZZ α1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin)
    • Chowdhury MM, Williams EJ, Morris JS et al. Severe panniculitis caused by homozygous ZZ α1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin). Br J Dermatol 2002;147:1258-61.
    • (2002) Br J Dermatol , vol.147 , pp. 1258-1261
    • Chowdhury, M.M.1    Williams, E.J.2    Morris, J.S.3
  • 87
    • 0033708895 scopus 로고    scopus 로고
    • Autoantibodies to neutrophilic proteases in a case of panniculitis by deficit of α1-antitrypsin
    • Oxford
    • Filaci G, Contini P, Barbera P, Bernardini L, Indiveri F. Autoantibodies to neutrophilic proteases in a case of panniculitis by deficit of α1-antitrypsin. Rheumatology (Oxford) 2000;39:1289-90.
    • (2000) Rheumatology , vol.39 , pp. 1289-1290
    • Filaci, G.1    Contini, P.2    Barbera, P.3    Bernardini, L.4    Indiveri, F.5
  • 88
    • 0037355741 scopus 로고    scopus 로고
    • Spontaneous internal carotid artery dissection and α1-antitrypsin deficiency
    • Konrad C, Nabavi DG, Junker R et al. Spontaneous internal carotid artery dissection and α1-antitrypsin deficiency. Acta Neurol Scand 2003;107:233-6.
    • (2003) Acta Neurol Scand , vol.107 , pp. 233-236
    • Konrad, C.1    Nabavi, D.G.2    Junker, R.3
  • 89
    • 0032893766 scopus 로고    scopus 로고
    • Coronary artery dissection in α1-antitrypsin deficiency
    • Martin DF, Delgado PM, Garcia RM et al. Coronary artery dissection in α1-antitrypsin deficiency. Histopathology 1999;34:376-8.
    • (1999) Histopathology , vol.34 , pp. 376-378
    • Martin, D.F.1    Delgado, P.M.2    Garcia, R.M.3
  • 90
    • 0031964132 scopus 로고    scopus 로고
    • α1-Antitrypsin deficiency in spontaneous intracranial arterial dissections
    • Schievink WI, Katzmann JA, Piepgras DG. α1-Antitrypsin deficiency in spontaneous intracranial arterial dissections. Cerebrovasc Dis 1998;8:42-4.
    • (1998) Cerebrovasc Dis , vol.8 , pp. 42-44
    • Schievink, W.I.1    Katzmann, J.A.2    Piepgras, D.G.3
  • 91
    • 0034042742 scopus 로고    scopus 로고
    • α1-Antitrypsin deficiency and splenic artery aneurysm rupture: an association?
    • Gaglio PJ, Regenstein F, Slakey D et al. α1-Antitrypsin deficiency and splenic artery aneurysm rupture: an association? Am J Gastroenterol 2000;95:1531-4.
    • (2000) Am J Gastroenterol , vol.95 , pp. 1531-1534
    • Gaglio, P.J.1    Regenstein, F.2    Slakey, D.3
  • 92
    • 0025043852 scopus 로고
    • α1-Antitrypsin phenotypes in patients with abdominal aortic aneurysms
    • Cohen JR, Sarfati I, Ratner L, Tilson D. α1-Antitrypsin phenotypes in patients with abdominal aortic aneurysms. J Surg Res 1990;49:319-21.
    • (1990) J Surg Res , vol.49 , pp. 319-321
    • Cohen, J.R.1    Sarfati, I.2    Ratner, L.3    Tilson, D.4
  • 93
    • 0029796480 scopus 로고    scopus 로고
    • Giant intracranial aneurysm and fibromuscular dysplasia in an adolescent with α1-antitrypsin deficiency
    • Schievink WI, Puumala MR, Meyer FB et al. Giant intracranial aneurysm and fibromuscular dysplasia in an adolescent with α1-antitrypsin deficiency. J Neurosurg 1996;85:503-6.
    • (1996) J Neurosurg , vol.85 , pp. 503-506
    • Schievink, W.I.1    Puumala, M.R.2    Meyer, F.B.3
  • 94
    • 0032983954 scopus 로고    scopus 로고
    • Absence of plasma protease-antiprotease imbalance in the formation of saccular cerebral aneurysms
    • Sakai N, Nakayama K, Tanabe Y et al. Absence of plasma protease-antiprotease imbalance in the formation of saccular cerebral aneurysms. Neurosurgery 1999;45:34-8.
    • (1999) Neurosurgery , vol.45 , pp. 34-38
    • Sakai, N.1    Nakayama, K.2    Tanabe, Y.3
  • 95
    • 0033082840 scopus 로고    scopus 로고
    • Is there a relationship between abdominal aortic aneurysms and α1-antitrypsin deficiency (PiZ)?
    • Elzouki AN, Ryden AA, Lanne T, Sonesson B, Eriksson S. Is there a relationship between abdominal aortic aneurysms and α1-antitrypsin deficiency (PiZ)? Eur J Vasc Endovasc Surg 1999;17:149-54.
    • (1999) Eur J Vasc Endovasc Surg , vol.17 , pp. 149-154
    • Elzouki, A.N.1    Ryden, A.A.2    Lanne, T.3    Sonesson, B.4    Eriksson, S.5
  • 96
    • 0029781329 scopus 로고    scopus 로고
    • Systemic necrotizing vasculitides in severe α1-antitrypsin deficiency
    • Mazodier P, Elzouki AN, Segelmark M, Eriksson S. Systemic necrotizing vasculitides in severe α1-antitrypsin deficiency. Q J Med 1996;89:599-611.
    • (1996) Q J Med , vol.89 , pp. 599-611
    • Mazodier, P.1    Elzouki, A.N.2    Segelmark, M.3    Eriksson, S.4
  • 97
    • 0025037768 scopus 로고
    • Peripheral neuropathy associated with α1-antitrypsin deficiency
    • Frederick WG, Enriquez R, Bookbinder MJ. Peripheral neuropathy associated with α1-antitrypsin deficiency. Arch Neurol 1990;47:233-5.
    • (1990) Arch Neurol , vol.47 , pp. 233-235
    • Frederick, W.G.1    Enriquez, R.2    Bookbinder, M.J.3
  • 98
    • 0034524177 scopus 로고    scopus 로고
    • Higher risk of mismatch repair-deficient colorectal cancer in α1-antitrypsin deficiency carriers and cigarette smokers
    • Yang P, Cunningham JM, Halling KC et al. Higher risk of mismatch repair-deficient colorectal cancer in α1-antitrypsin deficiency carriers and cigarette smokers. Mol Genet Metab 2000;71:639-45.
    • (2000) Mol Genet Metab , vol.71 , pp. 639-645
    • Yang, P.1    Cunningham, J.M.2    Halling, K.C.3
  • 100
    • 0029907242 scopus 로고    scopus 로고
    • Primary prevention in a highrisk group: smoking habits in adolescents with homozygous α1-antitrypsin deficiency (ATD)
    • Thelin T, Sveger T, McNeil TF. Primary prevention in a highrisk group: smoking habits in adolescents with homozygous α1-antitrypsin deficiency (ATD). Acta Paediatr 1996;85:1207-12.
    • (1996) Acta Paediatr , vol.85 , pp. 1207-1212
    • Thelin, T.1    Sveger, T.2    McNeil, T.F.3
  • 101
    • 0024467067 scopus 로고
    • Guidelines for the approach to the patient with severe hereditary α1-antitrypsin deficiency
    • American Thoracic Society
    • Guidelines for the approach to the patient with severe hereditary α1-antitrypsin deficiency. American Thoracic Society. Am Rev Respir Dis 1989;140:1494-7.
    • (1989) Am Rev Respir Dis , vol.140 , pp. 1494-1497
  • 102
    • 0023148995 scopus 로고
    • Replacement therapy for α1-antitrypsin deficiency associated with emphysema
    • Wewers MD, Casolaro MA, Sellers SE et al. Replacement therapy for α1-antitrypsin deficiency associated with emphysema. N Engl J Med 1987;316:1055-62.
    • (1987) N Engl J Med , vol.316 , pp. 1055-1062
    • Wewers, M.D.1    Casolaro, M.A.2    Sellers, S.E.3
  • 103
    • 0036301645 scopus 로고    scopus 로고
    • Biochemical efficacy and safety of a new pooled human plasma α1-antitrypsin, Respitin
    • Stoller JK, Rouhani F, Brantly M et al. Biochemical efficacy and safety of a new pooled human plasma α1-antitrypsin, Respitin. Chest 2002;122:66-74.
    • (2002) Chest , vol.122 , pp. 66-74
    • Stoller, J.K.1    Rouhani, F.2    Brantly, M.3
  • 104
    • 0036606880 scopus 로고    scopus 로고
    • The effect of augmentation therapy on bronchial inflammation in α1-antitrypsin deficiency
    • Stockley RA, Bayley DL, Unsal I, Dowson LJ. The effect of augmentation therapy on bronchial inflammation in α1-antitrypsin deficiency. Am J Respir Crit Care Med 2002;165:1494-8.
    • (2002) Am J Respir Crit Care Med , vol.165 , pp. 1494-1498
    • Stockley, R.A.1    Bayley, D.L.2    Unsal, I.3    Dowson, L.J.4
  • 105
    • 0030610568 scopus 로고    scopus 로고
    • Pharmacokinetic study of α1-antitrypsin infusion in α1-antitrypsin deficiency
    • Barker AF, Iwata-Morgan I, Oveson L, Roussel R. Pharmacokinetic study of α1-antitrypsin infusion in α1-antitrypsin deficiency. Chest 1997;112:607-13.
    • (1997) Chest , vol.112 , pp. 607-613
    • Barker, A.F.1    Iwata-Morgan, I.2    Oveson, L.3    Roussel, R.4
  • 106
    • 0023784904 scopus 로고
    • Biochemical efficacy and safety of monthly augmentation therapy for α1-antitrypsin deficiency
    • Hubbard RC, Sellers S, Czerski D, Stephens L, Crystal RG. Biochemical efficacy and safety of monthly augmentation therapy for α1-antitrypsin deficiency. JAMA 1988;260:1259-64.
    • (1988) JAMA , vol.260 , pp. 1259-1264
    • Hubbard, R.C.1    Sellers, S.2    Czerski, D.3    Stephens, L.4    Crystal, R.G.5
  • 107
    • 0032724157 scopus 로고    scopus 로고
    • A randomized clinical trial of α1-antitrypsin augmentation therapy
    • Dirksen A, Dijkman JH, Madsen F et al. A randomized clinical trial of α1-antitrypsin augmentation therapy. Am J Respir Crit Care Med 1999;160:1468-72.
    • (1999) Am J Respir Crit Care Med , vol.160 , pp. 1468-1472
    • Dirksen, A.1    Dijkman, J.H.2    Madsen, F.3
  • 108
    • 0032110436 scopus 로고    scopus 로고
    • Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin
    • The Alpha-1-Antitrypsin Deficiency Registry Study Group
    • The Alpha-1-Antitrypsin Deficiency Registry Study Group. Survival and FEV1 decline in individuals with severe deficiency of α1-antitrypsin. Am J Respir Crit Care Med 1998;158:49-59.
    • (1998) Am J Respir Crit Care Med , vol.158 , pp. 49-59
  • 109
    • 0034523704 scopus 로고    scopus 로고
    • Short-term supplementation therapy does not affect elastin degradation in severe α1-antitrypsin deficiency
    • The American-Italian AATD Study Group
    • Gottlieb DJ, Luisetti M, Stone PJ et al. Short-term supplementation therapy does not affect elastin degradation in severe α1-antitrypsin deficiency. The American-Italian AATD Study Group. Am J Respir Crit Care Med 2000;162:2069-72.
    • (2000) Am J Respir Crit Care Med , vol.162 , pp. 2069-2072
    • Gottlieb, D.J.1    Luisetti, M.2    Stone, P.J.3
  • 110
    • 0033739182 scopus 로고    scopus 로고
    • Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data
    • Lieberman J. Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data. Chest 2000;118:1480-5.
    • (2000) Chest , vol.118 , pp. 1480-1485
    • Lieberman, J.1
  • 111
    • 0031242842 scopus 로고    scopus 로고
    • Does α1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary α1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) α1-AT study group
    • Seersholm N, Wencker M, Banik N et al. Does α1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary α1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) α1-AT study group. Eur Respir J 1997;10:2260-3.
    • (1997) Eur Respir J , vol.10 , pp. 2260-2263
    • Seersholm, N.1    Wencker, M.2    Banik, N.3
  • 112
    • 0035095548 scopus 로고    scopus 로고
    • Longitudinal follow-up of patients with α1-protease inhibitor deficiency before and during therapy with IV α1-protease inhibitor
    • Wencker M, Fuhrmann B, Banik N, Konietzko N. Longitudinal follow-up of patients with α1-protease inhibitor deficiency before and during therapy with IV α1-protease inhibitor. Chest 2001;119:737-44.
    • (2001) Chest , vol.119 , pp. 737-744
    • Wencker, M.1    Fuhrmann, B.2    Banik, N.3    Konietzko, N.4
  • 113
    • 0025360692 scopus 로고
    • Strategies for aerosol therapy of α1-antitrypsin deficiency by the aerosol route
    • Hubbard RC, Crystal RG. Strategies for aerosol therapy of α1-antitrypsin deficiency by the aerosol route. Lung 1990;168(Suppl): 565015078.
    • (1990) Lung , vol.168 , Issue.SUPPL , pp. 565015078
    • Hubbard, R.C.1    Crystal, R.G.2
  • 114
    • 0034744066 scopus 로고    scopus 로고
    • Inhalation of [123I] α1-protease inhibitor: toward a new therapeutic concept of α1-protease inhibitor deficiency?
    • Kropp J, Wencker M, Hotze A et al. Inhalation of [123I] α1-protease inhibitor: toward a new therapeutic concept of α1-protease inhibitor deficiency? J Nucl Med 2001;42:744-51.
    • (2001) J Nucl Med , vol.42 , pp. 744-751
    • Kropp, J.1    Wencker, M.2    Hotze, A.3
  • 115
    • 0037952677 scopus 로고    scopus 로고
    • Costeffectiveness analysis of augmentation therapy for severe α1-antitrypsin deficiency
    • Gildea TR, Shermock KM, Singer ME, Stoller JK. Costeffectiveness analysis of augmentation therapy for severe α1-antitrypsin deficiency. Am J Respir Crit Care Med 2003;167:1387-92.
    • (2003) Am J Respir Crit Care Med , vol.167 , pp. 1387-1392
    • Gildea, T.R.1    Shermock, K.M.2    Singer, M.E.3    Stoller, J.K.4
  • 116
    • 0031045338 scopus 로고    scopus 로고
    • The intrapulmonary half-life and safety of aerosolized α1-protease inhibitor in normal volunteers
    • Vogelmeier C, Kirlath I, Warrington S et al. The intrapulmonary half-life and safety of aerosolized α1-protease inhibitor in normal volunteers. Am J Respir Crit Care Med 1997;155:536-41.
    • (1997) Am J Respir Crit Care Med , vol.155 , pp. 536-541
    • Vogelmeier, C.1    Kirlath, I.2    Warrington, S.3
  • 117
    • 0024417082 scopus 로고
    • Recombinant DNA-produced α1-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with α1-antitrypsin deficiency
    • Hubbard RC, McElvaney NG, Sellers SE et al. Recombinant DNA-produced α1-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with α1-antitrypsin deficiency. J Clin Invest 1989;84:1349-54.
    • (1989) J Clin Invest , vol.84 , pp. 1349-1354
    • Hubbard, R.C.1    McElvaney, N.G.2    Sellers, S.E.3
  • 118
    • 0024403417 scopus 로고
    • Anti-neutrophil-elastase defenses of the lower respiratory tract in α1-antitrypsin deficiency directly augmented with an aerosol of α1-antitrypsin
    • Hubbard RC, Brantly ML, Sellers SE, Mitchell ME, Crystal RG. Anti-neutrophil-elastase defenses of the lower respiratory tract in α1-antitrypsin deficiency directly augmented with an aerosol of α1-antitrypsin. Ann Intern Med 1989;111:206-12.
    • (1989) Ann Intern Med , vol.111 , pp. 206-212
    • Hubbard, R.C.1    Brantly, M.L.2    Sellers, S.E.3    Mitchell, M.E.4    Crystal, R.G.5
  • 119
    • 0036137882 scopus 로고    scopus 로고
    • Alveolar deposition of monodisperse aerosol particles in the lung of patients with chronic obstructive pulmonary disease
    • Brand P, Meyer T, Sommerer K, Weber N, Scheuch G. Alveolar deposition of monodisperse aerosol particles in the lung of patients with chronic obstructive pulmonary disease. Exp Lung Res 2002;28:39-54.
    • (2002) Exp Lung Res , vol.28 , pp. 39-54
    • Brand, P.1    Meyer, T.2    Sommerer, K.3    Weber, N.4    Scheuch, G.5
  • 120
    • 0032821440 scopus 로고    scopus 로고
    • Influence of the technological parameters of ultrasonic nebulisation on the nebulisation quality of α1-protease inhibitor (α1-PI)
    • Flament MP, Leterme P, Gayot A. Influence of the technological parameters of ultrasonic nebulisation on the nebulisation quality of α1-protease inhibitor (α1-PI). Int J Pharm 1999;189:197-204.
    • (1999) Int J Pharm , vol.189 , pp. 197-204
    • Flament, M.P.1    Leterme, P.2    Gayot, A.3
  • 121
    • 0028873414 scopus 로고
    • Clinical implications of gene therapy for α1-antitrypsin deficiency
    • Knoell DL, Wewers MD. Clinical implications of gene therapy for α1-antitrypsin deficiency. Chest 1995;107:535-45.
    • (1995) Chest , vol.107 , pp. 535-545
    • Knoell, D.L.1    Wewers, M.D.2
  • 122
    • 0032067586 scopus 로고    scopus 로고
    • Transfer of the human α1-antitrypsin gene into pulmonary macrophages in vivo
    • Ferkol T, Mularo F, Hilliard J et al. Transfer of the human α1-antitrypsin gene into pulmonary macrophages in vivo. Am J Respir Cell Mol Biol 1998;18:591-601.
    • (1998) Am J Respir Cell Mol Biol , vol.18 , pp. 591-601
    • Ferkol, T.1    Mularo, F.2    Hilliard, J.3
  • 123
    • 0025848772 scopus 로고
    • Adenovirusmediated transfer of a recombinant α1-antitrypsin gene to the lung epithelium in vivo
    • Rosenfeld MA, Siegfried W, Yoshimura K et al. Adenovirusmediated transfer of a recombinant α1-antitrypsin gene to the lung epithelium in vivo. Science 1991;252:431-4.
    • (1991) Science , vol.252 , pp. 431-434
    • Rosenfeld, M.A.1    Siegfried, W.2    Yoshimura, K.3
  • 124
    • 0036131253 scopus 로고    scopus 로고
    • Recombinant adeno-associated virus gene therapy for cystic fibrosis and α1-antitrypsin deficiency
    • Flotte TR. Recombinant adeno-associated virus gene therapy for cystic fibrosis and α1-antitrypsin deficiency. Chest 2002;121(3 Suppl): 98S-102S.
    • (2002) Chest , vol.121 , Issue.3 SUPPL.
    • Flotte, T.R.1
  • 125
    • 0036510604 scopus 로고    scopus 로고
    • 6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization: implications for the prevention of Z α1-antitrypsin-related cirrhosis
    • Mahadeva R, Dafforn TR, Carrell RW, Lomas DA. 6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization: implications for the prevention of Z α1-antitrypsin-related cirrhosis. J Biol Chem 2002;277:6771-4.
    • (2002) J Biol Chem , vol.277 , pp. 6771-6774
    • Mahadeva, R.1    Dafforn, T.R.2    Carrell, R.W.3    Lomas, D.A.4
  • 126
    • 0034652248 scopus 로고    scopus 로고
    • Chemical chaperones mediate increased secretion of mutant α1-antitrypsin (α1-AT) Z: a potential pharmacological strategy for prevention of liver injury and emphysema in α1-AT deficiency
    • Burrows JA, Willis LK, Perlmutter DH. Chemical chaperones mediate increased secretion of mutant α1-antitrypsin (α1-AT) Z: a potential pharmacological strategy for prevention of liver injury and emphysema in α1-AT deficiency. Proc Natl Acad Sci U S A 2000;97:1796-801.
    • (2000) Proc Natl Acad Sci U S A , vol.97 , pp. 1796-1801
    • Burrows, J.A.1    Willis, L.K.2    Perlmutter, D.H.3
  • 127
    • 0037064969 scopus 로고    scopus 로고
    • Chaperones to the rescue
    • Bunk S. Chaperones to the rescue. Scientist 2002;16:21-3.
    • (2002) Scientist , vol.16 , pp. 21-23
    • Bunk, S.1
  • 128
    • 0036250774 scopus 로고    scopus 로고
    • Neutrophil elastase and acute lung injury: prospects for sivelestat and other neutrophil elastase inhibitors as therapeutics
    • Zeiher BG, Matsuoka S, Kawabata K, Repine JE. Neutrophil elastase and acute lung injury: prospects for sivelestat and other neutrophil elastase inhibitors as therapeutics. Crit Care Med 2002;30(Suppl): S281-7.
    • (2002) Crit Care Med , vol.30 , Issue.SUPPL
    • Zeiher, B.G.1    Matsuoka, S.2    Kawabata, K.3    Repine, J.E.4
  • 129
    • 0037102183 scopus 로고    scopus 로고
    • A novel oral neutrophil elastase inhibitor (ONO-6818) inhibits human neutrophil elastase-induced emphysema in rats
    • Kuraki T, Ishibashi M, Takayama M, Shiraishi M, Yoshida M. A novel oral neutrophil elastase inhibitor (ONO-6818) inhibits human neutrophil elastase-induced emphysema in rats. Am J Respir Crit Care Med 2002;166:496-500.
    • (2002) Am J Respir Crit Care Med , vol.166 , pp. 496-500
    • Kuraki, T.1    Ishibashi, M.2    Takayama, M.3    Shiraishi, M.4    Yoshida, M.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.