메뉴 건너뛰기




Volumn 51, Issue 9, 1997, Pages 885-918

Alpha1-antitrypsin deficiency;α1-Pi-Mangel Weltgesundheitsorganisation
[No Author Info available]

Author keywords

[No Author keywords available]

Indexed keywords

ADOLESCENT; ADULT; AGED; ALLELE; ALPHA 1 ANTITRYPSIN DEFICIENCY; CHILD; CHRONIC OBSTRUCTIVE LUNG DISEASE; FEMALE; GENE EXPRESSION; GENETICS; HUMAN; MALE; MASS SCREENING; MIDDLE AGED; PHENOTYPE; PHYSIOLOGY; POPULATION GENETICS; PRESCHOOL CHILD; PROGNOSIS; REVIEW; RISK FACTOR; WORLD HEALTH ORGANIZATION;

EID: 0031226450     PISSN: 09348387     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article
Times cited : (7)

References (175)
  • 2
    • 0013828812 scopus 로고
    • 1-antitrypsin deficiency
    • 1-antitrypsin deficiency. Acta Med. Scand. 432 (1965) 1-85
    • (1965) Acta Med. Scand. , vol.432 , pp. 1-85
    • Eriksson, S.1
  • 3
    • 0020643467 scopus 로고
    • Human plasma proteinase inhibitors
    • Travis, J., G. S. Salvesen: Human plasma proteinase inhibitors. A Rev. Biochem. 52 (1983) 655-709
    • (1983) A Rev. Biochem. , vol.52 , pp. 655-709
    • Travis, J.1    Salvesen, G.S.2
  • 4
    • 0001724344 scopus 로고
    • Serpins: The superfamily of plasma serine proteinase inhibitors
    • Barrett, A., G. Salves (eds.) Eisevier Biomedical Press, Amsterdam
    • Carrell, R. W., D. R. Boswell: Serpins: the superfamily of plasma serine proteinase inhibitors. In: Barrett, A., G. Salves (eds.): Proteinase Inhibitors. Eisevier Biomedical Press, Amsterdam (1986) pp. 403-419
    • (1986) Proteinase Inhibitors. , pp. 403-419
    • Carrell, R.W.1    Boswell, D.R.2
  • 5
    • 0028206262 scopus 로고
    • The serpin family of proteinase inhibitors: Structure function and regulation
    • Potempa, J., E. Korzus, J. Travis: The serpin family of proteinase inhibitors: structure function and regulation.]. Biol. Chem. 269 (1994) 15 957-15 960
    • (1994) Biol. Chem. , vol.269 , pp. 15957-15960
    • Potempa, J.1    Korzus, E.2    Travis, J.3
  • 6
    • 0024423930 scopus 로고
    • 1-antitrypsin for structure and function of serpins
    • 1-antitrypsin for structure and function of serpins. Biochemistry 28 (1989) 8951-8966
    • (1989) Biochemistry , vol.28 , pp. 8951-8966
    • Huber, R.1    Carrell, R.W.2
  • 7
  • 9
    • 0029589824 scopus 로고
    • What do dysfunctional serpins tell us about molecular mobility and disease?
    • Stein, P. E., R. W. Carrell: What do dysfunctional serpins tell us about molecular mobility and disease? Nature Struct. Biol. 2 (1995) 96-114
    • (1995) Nature Struct. Biol. , vol.2 , pp. 96-114
    • Stein, P.E.1    Carrell, R.W.2
  • 11
    • 0017163949 scopus 로고
    • 1-antitrypsin PiZ
    • 1-antitrypsin PiZ. FEBS Lett 65 (1976) 195-197
    • (1976) FEBS Lett , vol.65 , pp. 195-197
    • Jeppsson, J.-O.1
  • 13
  • 15
    • 0025923680 scopus 로고
    • iiyama (Serine53 (TCC) to phenylalanine 53 (TCC))
    • iiyama (Serine53 (TCC) to phenylalanine 53 (TCC)). J. Biol. Chem. 266 (1991) 12 627-12 632
    • (1991) J. Biol. Chem. , vol.266 , pp. 12627-12632
    • Seyama, K.1
  • 20
    • 0017099344 scopus 로고
    • Liver disease in alpha,-antitrypsin deficiency detected by screening of 200,000 infants
    • Sveger, T.: Liver disease in alpha,-antitrypsin deficiency detected by screening of 200,000 infants. N. Engl. J. Med. 294 (1976) 1316-1321
    • (1976) N. Engl. J. Med. , vol.294 , pp. 1316-1321
    • Sveger, T.1
  • 21
    • 0017888482 scopus 로고
    • 1-antitrypsin deficiency in early childhood
    • 1-antitrypsin deficiency in early childhood. Pediatrics 62 (1978) 22-25
    • (1978) Pediatrics , vol.62 , pp. 22-25
    • Sveger, T.1
  • 22
    • 0000713561 scopus 로고
    • 1-antitrypsin deficiency
    • Scriver, C. R., A. L. Beaudet, W. S. Sly, D. Valle (eds.) McGraw-Hill, Inc., New York
    • th ed. McGraw-Hill, Inc., New York 1995
    • (1995) th Ed.
    • Cox, D.W.1
  • 23
    • 0011280531 scopus 로고
    • 1-antitrypsin deficiency and related disorders
    • 1-antitrypsin deficiency and related disorders. Princ. Prac. Med. Genet. 2 (1983) 911
    • (1983) Princ. Prac. Med. Genet. , vol.2 , pp. 911
    • Liebermann, J.1
  • 24
    • 0022400233 scopus 로고
    • 1-proteinase inhibitor protein
    • 1-proteinase inhibitor protein. Disease Markers 3 (1985) 135
    • (1985) Disease Markers , vol.3 , pp. 135
    • Kamboh, M.L.1
  • 25
    • 0021834059 scopus 로고
    • 1-antitrypsin-deficient children
    • 1-antitrypsin-deficient children. Pediatr. Res. 19 (1985) 834
    • (1985) Pediatr. Res. , vol.19 , pp. 834
    • Sveger, T.1
  • 27
    • 0024370804 scopus 로고
    • 1-antitrypsIn: Electrophoretic variation and DNA haplotypes
    • 1-antitrypsIn: Electrophoretic variation and DNA haplotypes. Am. J. Hum. Genet. 44 (1989) 844
    • (1989) Am. J. Hum. Genet. , vol.44 , pp. 844
    • Cox, D.W.1    Billingsley, G.D.2
  • 29
    • 0022257475 scopus 로고
    • 1-antitrypsin indicate a single origin for deficiency allele PIZ
    • 1-antitrypsin indicate a single origin for deficiency allele PIZ. Nature 316 (1985) 79
    • (1985) Nature , vol.316 , pp. 79
    • Cox, D.W.1    Woo, S.L.C.2    Manfield, T.3
  • 31
    • 0023108603 scopus 로고
    • 1-antitrypsin gene in normal subjects and in patients with pulmonary emphysema
    • 1-antitrypsin gene in normal subjects and in patients with pulmonary emphysema. J. Med. Genet. 24 (1987) 47
    • (1987) J. Med. Genet. , vol.24 , pp. 47
    • Hodgson, I.1    Kalsheker, N.2
  • 33
    • 0028244938 scopus 로고
    • 1-antitrypsin deficiency with special reference to non-index cases
    • 1-antitrypsin deficiency with special reference to non-index cases. Thorax 49 (1994) 695-698
    • (1994) Thorax , vol.49 , pp. 695-698
    • Seersholm, N.1    Kok-Jensen, A.2    Dirksen, A.3
  • 35
    • 0025914244 scopus 로고
    • Three cases of alpha-1-antitrypsin deficiency in the elderly
    • Jack, C. I., C. C. Evans: Three cases of alpha-1-antitrypsin deficiency in the elderly. Postgrad. Med. J. 67 (1991) 840-842
    • (1991) Postgrad. Med. J. , vol.67 , pp. 840-842
    • Jack, C.I.1    Evans, C.C.2
  • 36
    • 0018128288 scopus 로고
    • 1-antitrypsin deficiency. PiZ
    • 1-antitrypsin deficiency. PiZ. Acta Med. Scand. 204 (1978) 345-351
    • (1978) Acta Med. Scand. , vol.204 , pp. 345-351
    • Larsson, C.1
  • 38
    • 0020678269 scopus 로고
    • 1-antritrypsin deficiency: The clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi Type Z
    • 1-antritrypsin deficiency: The clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi Type Z. Br. J. Dis. Chest 77 (1983) 14-27
    • (1983) Br. J. Dis. Chest , vol.77 , pp. 14-27
    • Tobin, M.J.1    Cook, P.J.L.2    Hutchison, D.C.S.3
  • 40
    • 0027991559 scopus 로고
    • Air pollution and hospital admissions for the elderly in Detroit, Michigan
    • Schwartz, J.: Air pollution and hospital admissions for the elderly in Detroit, Michigan. Am.J. Respir. Crit. Care Med. 150 (1995) 648-655
    • (1995) Am.J. Respir. Crit. Care Med. , vol.150 , pp. 648-655
    • Schwartz, J.1
  • 41
    • 0014309289 scopus 로고
    • Chronic respiratory disease in mining communities in Marion County, West Virgina
    • Higgins, I. T. T. et al.: Chronic respiratory disease in mining communities in Marion County, West Virgina. Br. J. Ind. Med. 25 (1968) 165-175
    • (1968) Br. J. Ind. Med. , vol.25 , pp. 165-175
    • Higgins, I.T.T.1
  • 42
    • 0025998745 scopus 로고
    • Effects of urban air pollution on emergency room admissions for chronic obstructive pulmonary disease
    • Sunyer, J. et al.: Effects of urban air pollution on emergency room admissions for chronic obstructive pulmonary disease. Am. J. Epidmiol. 134 (1991) 277-286
    • (1991) Am. J. Epidmiol. , vol.134 , pp. 277-286
    • Sunyer, J.1
  • 43
    • 0029072159 scopus 로고
    • Estimated long-term ambient concentrations of PM10 and development of respiratory symptoms in a nonsmoking population
    • Abbey, D. E. et al.: Estimated long-term ambient concentrations of PM10 and development of respiratory symptoms in a nonsmoking population. Arch. Environ. Health 50 (1995) 139-152
    • (1995) Arch. Environ. Health , vol.50 , pp. 139-152
    • Abbey, D.E.1
  • 44
    • 0026321408 scopus 로고
    • Prevalence of obstructive lung disease in a general population, relation to occupational title an exposure to some airborne agents
    • Bakke, P. S. et al.: Prevalence of obstructive lung disease in a general population, relation to occupational title an exposure to some airborne agents. Thorax 46 (1991) 863-870
    • (1991) Thorax , vol.46 , pp. 863-870
    • Bakke, P.S.1
  • 45
    • 0027322797 scopus 로고
    • Occupational dust exposure and chronic obstructive pulmonary disease. a systemic overview of the evidence
    • Oxman, A. D. et al.: Occupational dust exposure and chronic obstructive pulmonary disease. A systemic overview of the evidence. Am. Rev. Respir. Dis. 148 (1993) 38-48
    • (1993) Am. Rev. Respir. Dis. , vol.148 , pp. 38-48
    • Oxman, A.D.1
  • 46
    • 0028256310 scopus 로고
    • The relationship between pneumonia in early childhood an impaired lung function in late adult life
    • Shaheen, S. O. et al.: The relationship between pneumonia in early childhood an impaired lung function in late adult life. Am. J. Respir. Care Med. 149 (1994) 616-619
    • (1994) Am. J. Respir. Care Med. , vol.149 , pp. 616-619
    • Shaheen, S.O.1
  • 47
    • 0029005697 scopus 로고
    • Do lower respiratory tract infections in early childhood cause chronic obstructive pulmonary disease?
    • Shaheen, S. O. et al.: Do lower respiratory tract infections in early childhood cause chronic obstructive pulmonary disease? Am. J. Respir. Care Med. 151 (1995) 1649-1652
    • (1995) Am. J. Respir. Care Med. , vol.151 , pp. 1649-1652
    • Shaheen, S.O.1
  • 48
    • 0029085092 scopus 로고
    • Bacterial infection in chronic obstructive pulmonary disease. a study of stable and exacerbated outpatients using the protected specimen brush
    • Monso, E. et al.: Bacterial infection in chronic obstructive pulmonary disease. A study of stable and exacerbated outpatients using the protected specimen brush. Am. J. Respir. Crit. Care Med. 152 (1995) 1316-1320
    • (1995) Am. J. Respir. Crit. Care Med. , vol.152 , pp. 1316-1320
    • Monso, E.1
  • 49
    • 0028918620 scopus 로고
    • Antibiotics in chronic obstructive pulmonary disease exacerbations. a meta-analysis
    • Saint, S. et al.: Antibiotics in chronic obstructive pulmonary disease exacerbations. A meta-analysis. JAMA 273 (1995) 857-960
    • (1995) JAMA , vol.273 , pp. 857-960
    • Saint, S.1
  • 52
    • 0024790159 scopus 로고
    • Variability of pulmonary function in alpha-1-antitrypsin deficiency: Clinical correlates
    • Silverman, E. K., J. A. Pierce, M. A. Province, D. C. Rao, E. U. Campbell: Variability of pulmonary function in alpha-1-antitrypsin deficiency: Clinical correlates. Ann. Int. Med. 111 (1989) 982
    • (1989) Ann. Int. Med. , vol.111 , pp. 982
    • Silverman, E.K.1    Pierce, J.A.2    Province, M.A.3    Rao, D.C.4    Campbell, E.U.5
  • 53
    • 0025651846 scopus 로고
    • Variability of pulmonary function in alpha-1-antitrypsin deficiency; residual family resemblance beyond the effect of the Pi locus
    • Silverman, E. K., M. A. Province, E. J. Campbell: J. A. Pierce, D. C. Rao: Variability of pulmonary function in alpha-1-antitrypsin deficiency; residual family resemblance beyond the effect of the Pi locus. Hum. Hered. 40 (1990) 34-355
    • (1990) Hum. Hered. , vol.40 , pp. 34-355
    • Silverman, E.K.1    Province, M.A.2    Campbell, E.J.3    Pierce, J.A.4    Rao, D.C.5
  • 54
    • 0025167094 scopus 로고
    • Independent mutations in the flanking sequence of the alpha-1-antitrypsin gene are associated with chronic obstructive airways disease
    • Kalsheker, N. A., G. L Watkins, S. Hill, K. Morgan, R. A. Stockley, R. B. Pick: Independent mutations in the flanking sequence of the alpha-1-antitrypsin gene are associated with chronic obstructive airways disease. Dis. Markers 8 (1990) 151-157
    • (1990) Dis. Markers , vol.8 , pp. 151-157
    • Kalsheker, N.A.1    Watkins, G.L.2    Hill, S.3    Morgan, K.4    Stockley, R.A.5    Pick, R.B.6
  • 55
    • 0026605197 scopus 로고
    • 1-antitrypsin gene commonly associated with chronic obstructive airway disease
    • 1-antitrypsin gene commonly associated with chronic obstructive airway disease. European J. Clin. Invest. 22 (1992) 134-137
    • (1992) European J. Clin. Invest. , vol.22 , pp. 134-137
    • Morgan, K.1    Scobie, G.2    Kalsheker, N.3
  • 56
    • 0022971042 scopus 로고
    • Correction of vitamin E deficiency in children with chronic cholestasis. II. Effect on gastrointestinal and hepatic function
    • Sokol, R. J., J. E. Heubi, C. Negraw, W. F. Balistreri: Correction of vitamin E deficiency in children with chronic cholestasis. II. Effect on gastrointestinal and hepatic function. Hepatology 6 (1986) 1263
    • (1986) Hepatology , vol.6 , pp. 1263
    • Sokol, R.J.1    Heubi, J.E.2    Negraw, C.3    Balistreri, W.F.4
  • 59
    • 0026447203 scopus 로고
    • Emphysema: The first two centuries - And beyond; a historical overview, with suggestions for future research: Part 2
    • Snider, G. L.: Emphysema: the first two centuries - and beyond; a historical overview, with suggestions for future research: Part 2. Am. Rev. Respir. Dis. 146(1992) 1615-1722
    • (1992) Am. Rev. Respir. Dis. , vol.146 , pp. 1615-1722
    • Snider, G.L.1
  • 60
    • 0002881286 scopus 로고
    • Chronic airflow obstruction
    • Thurlbeck, W. M. (ed.) Thieme Medical Publishers, Inc., New York
    • Thurlbeck, W. M.: Chronic airflow obstruction. In: Thurlbeck, W. M. (ed.): Pathology of the Lung, COPD. Thieme Medical Publishers, Inc., New York (1988) pp. 519-576
    • (1988) Pathology of the Lung, COPD. , pp. 519-576
    • Thurlbeck, W.M.1
  • 61
    • 0023711895 scopus 로고
    • Airway inflammation and peribronchiolar attachments in the lung of nonsmokers, current and ex-smokers
    • Wright, J. L., J. E. Hobson, B. Wiggs, P. D. Pare, J. C. Hogg: Airway inflammation and peribronchiolar attachments in the lung of nonsmokers, current and ex-smokers. Lung 166 (1988) 277-286
    • (1988) Lung , vol.166 , pp. 277-286
    • Wright, J.L.1    Hobson, J.E.2    Wiggs, B.3    Pare, P.D.4    Hogg, J.C.5
  • 62
    • 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. 132 (1985) 417-433
    • (1985) Am. Rev. Respir. Dis. , vol.132 , pp. 417-433
    • Janoff, A.1
  • 63
    • 0015847261 scopus 로고
    • Alpha-1-antitrypsin deficiency: A variant with no detectable alpha-1-antitrypsin
    • Talamo, R. C., C. E. Langley, C. E. Reed, S. Makino: Alpha-1-antitrypsin deficiency: a variant with no detectable alpha-1-antitrypsin. Science 181 (1973) 70
    • (1973) Science , vol.181 , pp. 70
    • Talamo, R.C.1    Langley, C.E.2    Reed, C.E.3    Makino, S.4
  • 64
    • 0022633717 scopus 로고
    • Alpha-1-antitrypsin deficiency and emphysema caused by homozygous inheritance of non-expressing alpha-1-antitrypsin genes
    • Carver, R. I. Jr., J.-F. Mornex, T. Nukiwa, M. Brantly, M. Courtney, J. P. LeCocq, R. G. Crystal: Alpha-1-antitrypsin deficiency and emphysema caused by homozygous inheritance of non-expressing alpha-1-antitrypsin genes. N. Engl. J. Med. 314 (1986) 762
    • (1986) N. Engl. J. Med. , vol.314 , pp. 762
    • Carver Jr., R.I.1    Mornex, J.-F.2    Nukiwa, T.3    Brantly, M.4    Courtney, M.5    Lecocq, J.P.6    Crystal, R.G.7
  • 66
    • 0028454320 scopus 로고
    • Absence of alpha-1-antitrypsin (Pi Null Bellingham) and the early onset of emphysema
    • Cook, L., E. D. Janud, S. Brenton, E. Tai, J. Burdon: Absence of alpha-1-antitrypsin (Pi Null Bellingham) and the early onset of emphysema. Aust. NZ J. Med. 24 (1994) 263-269
    • (1994) Aust. NZ J. Med. , vol.24 , pp. 263-269
    • Cook, L.1    Janud, E.D.2    Brenton, S.3    Tai, E.4    Burdon, J.5
  • 69
    • 0022586733 scopus 로고
    • Elastase inhibitors of sputum sol phase: Variability, relationship to neutrophil elastase inhibition, and effect of corticosteroid treatment
    • Stockley, R. A., H. M. Morrison, J. A. Kramps, Dijkman: Elastase inhibitors of sputum sol phase: variability, relationship to neutrophil elastase inhibition, and effect of corticosteroid treatment. Thorax 41 (1986) 442-447
    • (1986) Thorax , vol.41 , pp. 442-447
    • Stockley, R.A.1    Morrison, H.M.2    Kramps, J.A.3    Dijkman4
  • 70
    • 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, G. H. Caughey: Neutrophil elastase and cathepsin G stimulate secretion from cultured bovine airway gland serous cells. J. Clin. Invest. 85 (1990) 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
  • 71
    • 0023898432 scopus 로고
    • Molecular basis of alpha-1-antitrypsin deficiency
    • Brantly, M., T. Nukiwa, R. G. Crystal: Molecular basis of alpha-1-antitrypsin deficiency. Am. J. Med. 84 (Suppl. 6A) (1988) 13-31
    • (1988) Am. J. Med. , vol.84 , Issue.SUPPL. 6A , pp. 13-31
    • Brantly, M.1    Nukiwa, T.2    Crystal, R.G.3
  • 72
    • 0025056573 scopus 로고
    • Long term follow-up of al cohort of children with alpha-1-antitrypsin deficiency
    • Wall, M., E. Moe, J. Eisenberg, M. Powers, N. Buist, A. S. Buist: Long term follow-up of al cohort of children with alpha-1-antitrypsin deficiency. J. Pediatr. 116 (1990) 248-251
    • (1990) J. Pediatr. , vol.116 , pp. 248-251
    • Wall, M.1    Moe, E.2    Eisenberg, J.3    Powers, M.4    Buist, N.5    Buist, A.S.6
  • 73
    • 0021342356 scopus 로고
    • Prospective study of children with alpha-1-antitrypsin deficiency: Eight year follow-up
    • Sveger, T.: Prospective study of children with alpha-1-antitrypsin deficiency: eight year follow-up. J. Pediatr. 104 (1985) 91-94
    • (1985) J. Pediatr. , vol.104 , pp. 91-94
    • Sveger, T.1
  • 74
    • 0029007542 scopus 로고
    • Clinical features and lung function in 18 year old adolescents with alpha-1-antitrypsin deficiency
    • Sveger, T., E. Plitulainen, M. Arborelius: Clinical features and lung function in 18 year old adolescents with alpha-1-antitrypsin deficiency. Acta Paediatrica 84 (1995) 815-816
    • (1995) Acta Paediatrica , vol.84 , pp. 815-816
    • Sveger, T.1    Plitulainen, E.2    Arborelius, M.3
  • 75
    • 0027941322 scopus 로고
    • A registry of patients with severe deficiency of alpha-1-antitrypsIn: Design and methods
    • The Alpha-1-antitrypsin Deficiency Registry Study Group. Prepared by: Schluchter, M. D., A. R. Barker, R. G. Crystal, R. A. Robbins, J. M. Stocks, J. K. Stoller, M. C. Wu: A registry of patients with severe deficiency of alpha-1-antitrypsIn: Design and methods: Chest 106 (1994) 1223-1233
    • (1994) Chest , vol.106 , pp. 1223-1233
    • Schluchter, M.D.1    Barker, A.R.2    Crystal, R.G.3    Robbins, R.A.4    Stocks, J.M.5    Stoller, J.K.6    Wu, M.C.7
  • 76
    • 0021960082 scopus 로고
    • Smoking lung function, and alpha-1-antitrypsin deficiency
    • Janus, E. D., N. T. Phillips, R. W. Carrell: Smoking lung function, and alpha-1-antitrypsin deficiency. Lancet 1 (1985) 152-154
    • (1985) Lancet , vol.1 , pp. 152-154
    • Janus, E.D.1    Phillips, N.T.2    Carrell, R.W.3
  • 77
    • 0013828812 scopus 로고
    • Studies in alpha-1-antitrypsin deficiency
    • Eriksson, S.: Studies in alpha-1-antitrypsin deficiency. Acta Med. Scand. 1771 (1965) 1-85
    • (1965) Acta Med. Scand. , vol.1771 , pp. 1-85
    • Eriksson, S.1
  • 78
    • 0023795389 scopus 로고
    • Clinical features and history of the destructive lung disease associated with alspha-1-antitrypsin deficiency of adults with pulmonary symptoms
    • Brantly, M. L., L D. Paul, B. H. Miller, B. H. Falk, M. Wu, R. G. Crystal: Clinical features and history of the destructive lung disease associated with alspha-1-antitrypsin deficiency of adults with pulmonary symptoms. Am. Rev. Respir. Dis. 138 (1988) 327-336
    • (1988) Am. Rev. Respir. Dis. , vol.138 , pp. 327-336
    • Brantly, M.L.1    Paul, L.D.2    Miller, B.H.3    Falk, B.H.4    Wu, M.5    Crystal, R.G.6
  • 79
    • 0019963490 scopus 로고
    • Alpha-1-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., A. J. S. Saunders, M. J. Tobin, D. C. S. Hutchinson: Alpha-1-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. 33 (1982) 371-377
    • (1982) Clin. Radiol. , vol.33 , pp. 371-377
    • Gishen, P.1    Saunders, A.J.S.2    Tobin, M.J.3    Hutchinson, D.C.S.4
  • 80
    • 0022638237 scopus 로고
    • Alpha-1-antitrypsin Pi-types in 965 COPD patients
    • Lieberman, J., B. Winter, A. Sastre: Alpha-1-antitrypsin Pi-types in 965 COPD patients. Chest 89 (1986) 370-373
    • (1986) Chest , vol.89 , pp. 370-373
    • Lieberman, J.1    Winter, B.2    Sastre, A.3
  • 81
    • 33748096713 scopus 로고
    • National Health Interview Survey, 1986
    • American Lung Association
    • National Centre for Health Statistics: National Health Interview Survey, 1986. Statistical Compendium on Adult Diseases. American Lung Association (1987) p. 31
    • (1987) Statistical Compendium on Adult Diseases. , pp. 31
  • 82
    • 0017902246 scopus 로고
    • Neonatal screening for alpha-1-antitrypsin deficiency
    • O'Brien, M. L., N. R. M. Buist, W. H. Murphy: Neonatal screening for alpha-1-antitrypsin deficiency. J. Pediatr. 92 (1979) 1006-1010
    • (1979) J. Pediatr. , vol.92 , pp. 1006-1010
    • O'Brien, M.L.1    Buist, N.R.M.2    Murphy, W.H.3
  • 83
    • 0027429896 scopus 로고
    • Molecular confirmation of alpha-1-antitrypsin genotypes in newborn dried blood specimens
    • Spence, W. C., J. E. Morris, K. Pass, P. D. Murphy: Molecular confirmation of alpha-1-antitrypsin genotypes in newborn dried blood specimens. Biochem. Med. Met. Biol. 50 (1993) 233-240
    • (1993) Biochem. Med. Met. Biol. , vol.50 , pp. 233-240
    • Spence, W.C.1    Morris, J.E.2    Pass, K.3    Murphy, P.D.4
  • 84
    • 0028543862 scopus 로고
    • Physical and social impact of alpha-1-antitrypsin deficiency: Results of a mail survey of the readership of a national newsletter
    • Stoller, J. K., P. Smith, P. Yang, J. Spray: Physical and social impact of alpha-1-antitrypsin deficiency: Results of a mail survey of the readership of a national newsletter. Cleve Clin. J. Med. 61 (1995) 461-467
    • (1995) Cleve Clin. J. Med. , vol.61 , pp. 461-467
    • Stoller, J.K.1    Smith, P.2    Yang, P.3    Spray, J.4
  • 86
    • 0027400969 scopus 로고
    • Variants of alpha-1-antitrypsin in Puerto Rican children with asthma
    • Colp, C., J. Pappas, D. Moran, J. Lieberman: Variants of alpha-1-antitrypsin in Puerto Rican children with asthma. Chest 103 (1993) 812-815
    • (1993) Chest , vol.103 , pp. 812-815
    • Colp, C.1    Pappas, J.2    Moran, D.3    Lieberman, J.4
  • 87
    • 0026876092 scopus 로고
    • A model of airway narrowing in asthma and chronic obstructive pulmonary disease
    • Wiggs, B. R., C. Bosken, P. D. Pare, J. C. Hogg: A model of airway narrowing in asthma and chronic obstructive pulmonary disease. Am. Rev. Respir. Dis. 145 (1992) 1251-1258
    • (1992) Am. Rev. Respir. Dis. , vol.145 , pp. 1251-1258
    • Wiggs, B.R.1    Bosken, C.2    Pare, P.D.3    Hogg, J.C.4
  • 88
    • 0021800002 scopus 로고
    • Alpha-1-antitrypsin deficiency presenting as bronchiectasis
    • Jones, D. K., D. Godden, P. Cavanagh: Alpha-1-antitrypsin deficiency presenting as bronchiectasis. Br. J. Dis. Chest 79 (1985) 301-304
    • (1985) Br. J. Dis. Chest , vol.79 , pp. 301-304
    • Jones, D.K.1    Godden, D.2    Cavanagh, P.3
  • 89
    • 0024385640 scopus 로고
    • Alpha-1-antitrypsin serum levels in widespread bronchiectasis
    • El-Kassimi, F. A., A. S. Warsy, A. Uz-Zaman, D. K. Pillai: Alpha-1-antitrypsin serum levels in widespread bronchiectasis. Respir. Med. 83 (1989) 119-121
    • (1989) Respir. Med. , vol.83 , pp. 119-121
    • El-Kassimi, F.A.1    Warsy, A.S.2    Uz-Zaman, A.3    Pillai, D.K.4
  • 90
    • 0027519368 scopus 로고
    • Bronchiectasis in patients with alpha-1-antitrypsin deficiency: A rare occurrence?
    • Shin, M. S., K.-J. Ho: Bronchiectasis in patients with alpha-1-antitrypsin deficiency: A rare occurrence? Chest 104 (1993) 1384-1386
    • (1993) Chest , vol.104 , pp. 1384-1386
    • Shin, M.S.1    Ho, K.-J.2
  • 91
    • 0028152940 scopus 로고
    • Lung function in adolescents with alpha-1-antitrypsin deficiency
    • Sveger, T., E. Pültulainen, M. Arborelius: Lung function in adolescents with alpha-1-antitrypsin deficiency. Acta Paediatrica 83 (1994) 1170-1173
    • (1994) Acta Paediatrica , vol.83 , pp. 1170-1173
    • Sveger, T.1    Pültulainen, E.2    Arborelius, M.3
  • 92
    • 0003078086 scopus 로고
    • Longitudinal studies in alpha-1-antitrypsin deficiency: A survey by the British Thoracic Society
    • Taylor, J. C., C. Mittman (eds.) Academic Press, New York
    • Hutchison, D. C. S., M. J. Tobin, D. Cooper, D. Lowe: Longitudinal studies in alpha-1-antitrypsin deficiency: A survey by the British Thoracic Society. In: Taylor, J. C., C. Mittman (eds.): Pulmonary emphysema and proteolysis. Academic Press, New York (1987) pp. 7-15
    • (1987) Pulmonary Emphysema and Proteolysis. , pp. 7-15
    • Hutchison, D.C.S.1    Tobin, M.J.2    Cooper, D.3    Lowe, D.4
  • 93
    • 0024211686 scopus 로고
    • Lung function, smoking, and survival in severe alpha-1-antitrypsin deficiency, PiZZ
    • Wu, M. C., S. Eriksson: Lung function, smoking, and survival in severe alpha-1-antitrypsin deficiency, PiZZ. J. Clin. Epidemiol. 41 (1988) 1157-1165
    • (1988) J. Clin. Epidemiol. , vol.41 , pp. 1157-1165
    • Wu, M.C.1    Eriksson, S.2
  • 94
    • 0028812493 scopus 로고
    • Survival in relation to lung function and smoking cessation in patients with severe hereditary alpha-1-antitrypsin deficiency
    • Seersholm, N., A. Kok-Jensen: Survival in relation to lung function and smoking cessation in patients with severe hereditary alpha-1-antitrypsin deficiency. Am. J. Respir. Crit. Care Med. 151 (1995) 369-373
    • (1995) Am. J. Respir. Crit. Care Med. , vol.151 , pp. 369-373
    • Seersholm, N.1    Kok-Jensen, A.2
  • 95
    • 0024997820 scopus 로고
    • A family study of the variability of pulmonary function in alpha-1-antitrypsin deficiency. Quantitative phenotypes
    • Silverman, E. K., M. A. Province, D. C. Rao, J. A Pierce, E. J. Campbell: A family study of the variability of pulmonary function in alpha-1-antitrypsin deficiency. Quantitative phenotypes. Am. Rev. Respir. Dis. 142 (1990) 1015-1021
    • (1990) Am. Rev. Respir. Dis. , vol.142 , pp. 1015-1021
    • Silverman, E.K.1    Province, M.A.2    Rao, D.C.3    Pierce, J.A.4    Campbell, E.J.5
  • 98
    • 0028240651 scopus 로고
    • Histopathological features of liver disease in alpha-1-antitrypsin defeciency
    • Deutsch, J., H. Becker, L. Auböck: Histopathological features of liver disease in alpha-1-antitrypsin defeciency. Acta Paediatr. 393 (1994) 8-12
    • (1994) Acta Paediatr. , vol.393 , pp. 8-12
    • Deutsch, J.1    Becker, H.2    Auböck, L.3
  • 99
    • 0029083915 scopus 로고
    • The liver in adolescents with alpha-1-antitrypsin deficiency
    • Sveger, T., S. Eriksson: The liver in adolescents with alpha-1-antitrypsin deficiency. Hepatology 22 (1995) 514-517
    • (1995) Hepatology , vol.22 , pp. 514-517
    • Sveger, T.1    Eriksson, S.2
  • 101
    • 0022637178 scopus 로고
    • Risks for cirrhosis and primary liver cancer in alpha-1-antitrypsin deficiency
    • Eriksson, S., J. Carlson, R. Velez: Risks for cirrhosis and primary liver cancer in alpha-1-antitrypsin deficiency. N. Engl. J. Med. 315 (1986) 736-739
    • (1986) N. Engl. J. Med. , vol.315 , pp. 736-739
    • Eriksson, S.1    Carlson, J.2    Velez, R.3
  • 103
    • 0026737470 scopus 로고
    • High prevalence of viral infection in adults with homozygous and heterozygous alpha-1-antitrypsin deficiency and chronic liver disease
    • Propst, T., A. Propst, O. Dietze, G. Judmeier, H. Braunsteiner, H. Vogel: High prevalence of viral infection in adults with homozygous and heterozygous alpha-1-antitrypsin deficiency and chronic liver disease. Ann. Intern. Med. 117 (1992) 641-654
    • (1992) Ann. Intern. Med. , vol.117 , pp. 641-654
    • Propst, T.1    Propst, A.2    Dietze, O.3    Judmeier, G.4    Braunsteiner, H.5    Vogel, H.6
  • 104
    • 0025815961 scopus 로고
    • Alpha-1-antitrypsin deficiency and liver disease: Clinical presentation, diagnosis and treatment
    • Hussain, M., G. Mietli-Vergani, A. P. Mowat: Alpha-1-antitrypsin deficiency and liver disease: clinical presentation, diagnosis and treatment. J. Inher. Metab. Dis. 14 (1991) 497-511
    • (1991) J. Inher. Metab. Dis. , vol.14 , pp. 497-511
    • Hussain, M.1    Mietli-Vergani, G.2    Mowat, A.P.3
  • 105
    • 0025350658 scopus 로고
    • 1-antitrypsin deficiency emphysema and liver disease: Genetic basis and strategies for therapy
    • 1-antitrypsin deficiency emphysema and liver disease: genetic basis and strategies for therapy. J. Clin. Invest. 85 (1990) 1343-1352
    • (1990) J. Clin. Invest. , vol.85 , pp. 1343-1352
    • Crystal, R.G.1
  • 106
    • 0026326794 scopus 로고
    • Alpha-1-antitrypsin deficiency-associated panniculitis: Case report and review of the literature
    • Edmonds, B. K., J. A. Hodge, R. L. Rietschel: Alpha-1-antitrypsin deficiency-associated panniculitis: Case report and review of the literature. Pediatr. Dermatol. 8 (1991) 296-299
    • (1991) Pediatr. Dermatol. , vol.8 , pp. 296-299
    • Edmonds, B.K.1    Hodge, J.A.2    Rietschel, R.L.3
  • 107
    • 0025186275 scopus 로고
    • Case reports: Alpha-1-antitrypsin deficiency panniculitis
    • Irvine, C., V. Neild, C. Stephens, M. Black: Case reports: Alpha-1-antitrypsin deficiency panniculitis. J. Royal Soc. Med. 83 (1990) 743-744
    • (1990) J. Royal Soc. Med. , vol.83 , pp. 743-744
    • Irvine, C.1    Neild, V.2    Stephens, C.3    Black, M.4
  • 108
    • 0028152691 scopus 로고
    • Arterial fibromuscular dysplasia associated with severe alpha-1-antitrypsin deficiency
    • Schievink, W. I., J. Björnsson, J. E. Parisi, U. B. S. Prakash: Arterial fibromuscular dysplasia associated with severe alpha-1-antitrypsin deficiency. Mayo Clin. Proc. 69 (1994) 1040-1043
    • (1994) Mayo Clin. Proc. , vol.69 , pp. 1040-1043
    • Schievink, W.I.1    Björnsson, J.2    Parisi, J.E.3    Prakash, U.B.S.4
  • 109
    • 0027988324 scopus 로고
    • Alpha-1-antitrypsIn: A guardian of vascular tissue
    • Cox, D. W.: Alpha-1-antitrypsIn: A guardian of vascular tissue. Mayo Clin. Proc. 69 (1994) 1123-1124
    • (1994) Mayo Clin. Proc. , vol.69 , pp. 1123-1124
    • Cox, D.W.1
  • 110
    • 0020510606 scopus 로고
    • Mutation of antitrypsin to antithrombIn: Alpha-1-antitrypsin Pittsburgh (358 Met'Arg), a fatal bleeding disorder
    • Owen, M. C., S. O. Brennan, J. H. Lewis, R. W. Carrell: Mutation of antitrypsin to antithrombIn: Alpha-1-antitrypsin Pittsburgh (358 Met'Arg), a fatal bleeding disorder. N. Engl. J. Med. 309 (1983) 695-698
    • (1983) N. Engl. J. Med. , vol.309 , pp. 695-698
    • Owen, M.C.1    Brennan, S.O.2    Lewis, J.H.3    Carrell, R.W.4
  • 112
    • 0024467067 scopus 로고
    • Guidelines for the approach to the patient with severe hereditary alpha-1-antitrypsin deficiency
    • Buist, A. S., B. Burrows, A. Cohen et al.: Guidelines for the approach to the patient with severe hereditary alpha-1-antitrypsin deficiency. Am. Rev. Resp. Dis. 140 (1989) 1494
    • (1989) Am. Rev. Resp. Dis. , vol.140 , pp. 1494
    • Buist, A.S.1    Burrows, B.2    Cohen, A.3
  • 113
    • 0023784904 scopus 로고
    • Biochemical efficacy and safety of monthly augmentation therapy for alpha-1-antitrypsin deficiency
    • Hubbard, R. C., S. Sellers, D. Czerski et al.: Biochemical efficacy and safety of monthly augmentation therapy for alpha-1-antitrypsin deficiency. J. Am. Med. Ass. 260 (1988) 1259
    • (1988) J. Am. Med. Ass. , vol.260 , pp. 1259
    • Hubbard, R.C.1    Sellers, S.2    Czerski, D.3
  • 115
    • 0023588232 scopus 로고
    • Augmentation of lung antineutrophil elastase capacity with recombinant human a-1-antitrypsin
    • Casolaro, M. A., G. Fells, M. Wewers et al.: Augmentation of lung antineutrophil elastase capacity with recombinant human a-1-antitrypsin. J. Appl. Physiol. 63 (1987) 2015
    • (1987) J. Appl. Physiol. , vol.63 , pp. 2015
    • Casolaro, M.A.1    Fells, G.2    Wewers, M.3
  • 116
    • 0024211686 scopus 로고
    • Lung function, smoking and survival in servere alpha-1-antitrypsin deficiency, PiZZ
    • 115a Wu, M. C., S. Eriksson: Lung function, smoking and survival in servere alpha-1-antitrypsin deficiency, PiZZ. J. Clin. Epidemiology 41 (1988) 1157
    • (1988) J. Clin. Epidemiology , vol.41 , pp. 1157
    • Wu, M.C.1    Eriksson, S.2
  • 117
    • 25944481165 scopus 로고
    • 1-Pi-deficiency: Comparison of 161 untreated Danish and 235 German patients receiving augmentation therapy
    • 1-Pi-deficiency: comparison of 161 untreated Danish and 235 German patients receiving augmentation therapy. Am. J. Resp. Crit. Care Med. 151 (4) (1995) A835
    • (1995) Am. J. Resp. Crit. Care Med. , vol.151 , Issue.4
    • Seersholm, N.1    Wencker, M.2    Dirksen, A.3
  • 118
    • 0024414888 scopus 로고
    • Pulmonary deposition and clearance of aerosolized alpha-1- Proteinase inhibitor administered to dogs and to sheep
    • Smith, R. M., L D. Traber, D. L Traber et al.: Pulmonary deposition and clearance of aerosolized alpha-1- proteinase inhibitor administered to dogs and to sheep. J. Clin. Invest. 84 (1989) 1145
    • (1989) J. Clin. Invest. , vol.84 , pp. 1145
    • Smith, R.M.1    Traber, L.D.2    Traber, D.L.3
  • 123
    • 2642689648 scopus 로고
    • Recent advances in retrovirus technology. Curr
    • Boris-Lawrie, K. A., H. M. TemIn: Recent advances in retrovirus technology. Curr. Opin. Genet. Dev. 301 (1993) 353-362
    • (1993) Opin. Genet. Dev. , vol.301 , pp. 353-362
    • Boris-Lawrie, K.A.1    Temin, H.M.2
  • 125
    • 0027748178 scopus 로고
    • Hepatic gene therapy: Efficient retroviral-nnediated gene transfer into rat hepatocytes in vivo. Somat
    • Kolodka, T. M., M. Finegold, S. L. C. Woo: Hepatic gene therapy: efficient retroviral-nnediated gene transfer into rat hepatocytes in vivo. Somat. Cell. Mol. Genet. 19 (1993) 491-497
    • (1993) Cell. Mol. Genet. , vol.19 , pp. 491-497
    • Kolodka, T.M.1    Finegold, M.2    Woo, S.L.C.3
  • 127
    • 0003024233 scopus 로고
    • Ginsberg, H. S. (ed.) Plenum Press, New York
    • Strauss, S. E.: In: Ginsberg, H. S. (ed.): The adenoviruses. Plenum Press, New York (1984) pp. 451-496
    • (1984) The Adenoviruses. , pp. 451-496
    • Strauss, S.E.1
  • 130
    • 0028418190 scopus 로고
    • Intraperitoneal in vivo gene therapy to deliver alpha-1-antitrypsin to the systemic circulation
    • Setoguchi, Y., H. A. Jaffe, C. S. Chu, R. G. Crystal: Intraperitoneal in vivo gene therapy to deliver alpha-1-antitrypsin to the systemic circulation. Am. J. Respir. Cell Mol. Biol. 10 (1995) 369-377
    • (1995) Am. J. Respir. Cell Mol. Biol. , vol.10 , pp. 369-377
    • Setoguchi, Y.1    Jaffe, H.A.2    Chu, C.S.3    Crystal, R.G.4
  • 132
    • 0028899392 scopus 로고    scopus 로고
    • IL-6-release associated with airway administration of human CFTR cDNA adenovirus vector
    • McElvaney, N. G., R. G. Crystal: IL-6-release associated with airway administration of human CFTR cDNA adenovirus vector. Nat. Med. 1 (195) 182-184
    • Nat. Med. , vol.1 , Issue.195 , pp. 182-184
    • McElvaney, N.G.1    Crystal, R.G.2
  • 134
    • 0028464351 scopus 로고
    • Strategies to achieve targetted gene delivery via the receptor-mediated endocytosis pathway
    • Michael, S. I., D. T. Curiel: Strategies to achieve targetted gene delivery via the receptor-mediated endocytosis pathway. Gene Therapy 1 (1994) 223-232
    • (1994) Gene Therapy , vol.1 , pp. 223-232
    • Michael, S.I.1    Curiel, D.T.2
  • 135
    • 0001785325 scopus 로고
    • Elastase may play an central role in the neutrophil migration through connective tissue
    • Taylor, J. C., C. Mittman (eds.) Academic Press, Orlando
    • Sandhaus, R. A.: Elastase may play an central role in the neutrophil migration through connective tissue. In: Taylor, J. C., C. Mittman (eds.): Pulmonary Emphysema and Proteolysis. Academic Press, Orlando (1987) pp. 227-233
    • (1987) Pulmonary Emphysema and Proteolysis. , pp. 227-233
    • Sandhaus, R.A.1
  • 136
    • 0025050764 scopus 로고
    • Identification of the primary antimicrobial domains in human neutrophil cathepsin G
    • Bangolore, N., J. Travis, V. C. Onunka et al.: Identification of the primary antimicrobial domains in human neutrophil cathepsin G.J. Biol. Chem. 265 (1990) 13584
    • (1990) J. Biol. Chem. , vol.265 , pp. 13584
    • Bangolore, N.1    Travis, J.2    Onunka, V.C.3
  • 137
    • 0021255040 scopus 로고
    • The injurious effect of neutrophils on pneumocytes in vitro
    • Ayars, G. H., L. C. Altman, H. Rosen, T. Doyle: The injurious effect of neutrophils on pneumocytes in vitro. Am. J. Respir. Dis. 130 (1984) 964-973
    • (1984) Am. J. Respir. Dis. , vol.130 , pp. 964-973
    • Ayars, G.H.1    Altman, L.C.2    Rosen, H.3    Doyle, T.4
  • 138
    • 0342887471 scopus 로고
    • Consequences of proteolytic injury
    • Crystal, R. G., J. B. West (eds.) Raven Press Ltd., New York
    • Lucey, E. C., P. J. Stone, G. L. Snider: Consequences of proteolytic injury. In: Crystal, R. G., J. B. West (eds.): Lung Injury. Raven Press Ltd., New York (1992) pp. 29-41
    • (1992) Lung Injury. , pp. 29-41
    • Lucey, E.C.1    Stone, P.J.2    Snider, G.L.3
  • 140
    • 0028675419 scopus 로고
    • Regulation of secretory leukocyte proteinase inhibitor (SLPI) and elastase-specific inhibitor (ESI/Elafin) in human airway epithelial cells by cytokines and neutrophilic enzymes
    • Sallenave, J.-M., J. Shulmann, J. Crossley et al.: Regulation of secretory leukocyte proteinase inhibitor (SLPI) and elastase-specific inhibitor (ESI/Elafin) in human airway epithelial cells by cytokines and neutrophilic enzymes. Am. J. Respir. Cell Mol. Biol. 11 (1994) 733-741
    • (1994) Am. J. Respir. Cell Mol. Biol. , vol.11 , pp. 733-741
    • Sallenave, J.-M.1    Shulmann, J.2    Crossley, J.3
  • 141
    • 0028883229 scopus 로고
    • The pathogenesis of chronic obstructive lung diseases implications for therapy
    • Stockley, R. A.: The pathogenesis of chronic obstructive lung diseases: implications for therapy. Q. J. Med. 88 (1995) 141-146
    • (1995) Q. J. Med. , vol.88 , pp. 141-146
    • Stockley, R.A.1
  • 142
    • 0027251739 scopus 로고
    • Interleukin-8: An important chemoattractant in sputum of patients with chronic inflammatory airway disease
    • (Lung Cell Mol. Physiol. 8)
    • Richman-Eisenstat, J. B. et al.: Interleukin-8: an important chemoattractant in sputum of patients with chronic inflammatory airway disease. Am. J. Physiol. (Lung Cell Mol. Physiol. 8) 264 (1993) L413-L418
    • (1993) Am. J. Physiol. , vol.264
    • Richman-Eisenstat, J.B.1
  • 144
    • 0018651591 scopus 로고
    • Alpha-1-antritrypsin and leukocyte elastase in infected and noninfected sputum
    • Stockley, R. A., D. Burnett: Alpha-1-antritrypsin and leukocyte elastase in infected and noninfected sputum. Am. Rev. Resp. Dis. 120 (1979) 1081-1086
    • (1979) Am. Rev. Resp. Dis. , vol.120 , pp. 1081-1086
    • Stockley, R.A.1    Burnett, D.2
  • 145
    • 0021270155 scopus 로고
    • Elastinolytic activity in sputum and its relation to purulence and lung function in patients with bronchiectasis
    • Stockley, R. A., S. L. Hill, H. M. Morrison, C. M. Starkie: Elastinolytic activity in sputum and its relation to purulence and lung function in patients with bronchiectasis. Thorax 39 (1984) 408-413
    • (1984) Thorax , vol.39 , pp. 408-413
    • Stockley, R.A.1    Hill, S.L.2    Morrison, H.M.3    Starkie, C.M.4
  • 147
    • 0020056914 scopus 로고
    • 1-antitrypsin by electrofocusing
    • 1-antitrypsin by electrofocusing. Clin. Chem. 28 81982) 219-225
    • (1982) Clin. Chem. , vol.28 , Issue.8 , pp. 219-225
    • Jeppsson, J.O.1    Franzen, B.2
  • 148
    • 0028263205 scopus 로고
    • 1-antitrypsin followed by phenotyping of deficient speciments by isoelectric focusing
    • 1-antitrypsin followed by phenotyping of deficient speciments by isoelectric focusing. Screening 3 (1994) 23-31
    • (1994) Screening , vol.3 , pp. 23-31
    • Spence, W.C.1    Pass, K.2    Murphy, P.D.3
  • 149
    • 38249013443 scopus 로고
    • 1-antitrypsin deficiency: A need for screening?
    • 1-antitrypsin deficiency: A need for screening? Screening 1 (1992) 195-201
    • (1992) Screening , vol.1 , pp. 195-201
    • Niggemenn, B.1    Paul, K.2
  • 151
    • 0028006653 scopus 로고
    • Airways obstruction and two year survival in patients with severe alpha-1-antitrypsin deficiency
    • Seersholm, N., A. Dirksen, A. Kok-Jensen: Airways obstruction and two year survival in patients with severe alpha-1-antitrypsin deficiency. Eur. Respir. J. 7 (1994) 1985-1987
    • (1994) Eur. Respir. J. , vol.7 , pp. 1985-1987
    • Seersholm, N.1    Dirksen, A.2    Kok-Jensen, A.3
  • 153
    • 4243384038 scopus 로고
    • Pathological findings in alpha-1-antitrypsin deficiency
    • Mittmann, C. (ed.) Academic Press, New York
    • Orell, S. R., P. Mazodier: Pathological findings in alpha-1-antitrypsin deficiency. In: Mittmann, C. (ed.): Pulmonary Emphysema and Proteolysis. Academic Press, New York (1972) 69-89
    • (1972) Pulmonary Emphysema and Proteolysis. , pp. 69-89
    • Orell, S.R.1    Mazodier, P.2
  • 155
    • 0015583069 scopus 로고
    • Alpha-1-antitrypsin deficiency in association with both cirrhosis and chronic obstructive lung disease in two sibs
    • Glasgow, J. F., M. J. Lymch, A. Hercz, H. Levison, A. Sass-Kortsak: Alpha-1-antitrypsin deficiency in association with both cirrhosis and chronic obstructive lung disease in two sibs. Am. J. Med. 54 (1973) 181-194
    • (1973) Am. J. Med. , vol.54 , pp. 181-194
    • Glasgow, J.F.1    Lymch, M.J.2    Hercz, A.3    Levison, H.4    Sass-Kortsak, A.5
  • 156
    • 0016236084 scopus 로고
    • Respiratory disease profile in 22 patients with alpha-1-antitrypsin deficiencies
    • Varpela, E., Y. Salorinne: Respiratory disease profile in 22 patients with alpha-1-antitrypsin deficiencies. Scand. J. Resp. Dis. Suppl. 89 (1974) 251-260
    • (1974) Scand. J. Resp. Dis. , Issue.89 SUPPL. , pp. 251-260
    • Varpela, E.1    Salorinne, Y.2
  • 157
    • 0018927852 scopus 로고
    • Widespread panacinar emphysema with alpha-1 -antitrypsin deficiency
    • d'Almaine-Semple, P., C. B. Reid, W. S. Thompson: Widespread panacinar emphysema with alpha-1 -antitrypsin deficiency. Br. J. Dis. Chest 74 (1980) 289-295
    • (1980) Br. J. Dis. Chest , vol.74 , pp. 289-295
    • D'Almaine-Semple, P.1    Reid, C.B.2    Thompson, W.S.3
  • 158
    • 0016097113 scopus 로고
    • Alpha-1-antitrypsin and its deficiency
    • Kueppers, F., L F. Black: Alpha-1-antitrypsin and its deficiency. Am. Rev. Respir. Dis. 110 (1974) 176-194
    • (1974) Am. Rev. Respir. Dis. , vol.110 , pp. 176-194
    • Kueppers, F.1    Black, L.F.2
  • 159
    • 0017152819 scopus 로고
    • Clinical, epidemiologic and pulmonary function studies in alpha-1-antitrypsin deficient subjects of PI* Z type
    • Rawlings, W. Jr., P. Kreiss, D. Levy et al.: Clinical, epidemiologic and pulmonary function studies in alpha-1-antitrypsin deficient subjects of PI* Z type. Am. Rev. Respir. Dis. 114 (1976) 945-953
    • (1976) Am. Rev. Respir. Dis. , vol.114 , pp. 945-953
    • Rawlings Jr., W.1    Kreiss, P.2    Levy, D.3
  • 160
    • 0017904729 scopus 로고
    • Alpha-1-antitrypsin deficiency in nonsmokers
    • Black, L., F. Kueppers: Alpha-1-antitrypsin deficiency in nonsmokers. Am. Rev. Respir. Dis. 117 (1978) 421-428
    • (1978) Am. Rev. Respir. Dis. , vol.117 , pp. 421-428
    • Black, L.1    Kueppers, F.2
  • 161
    • 0015367610 scopus 로고
    • Severe pulmonary emphysema: A comparison of patients with and without alpha-1-antitrypsin deficiency
    • Hutchison, D. C. S., C. E. Barter, P. J. L. Cook, J. W. Laws, N. A. Martelli, P. Hugh-Jones: Severe pulmonary emphysema: A comparison of patients with and without alpha-1-antitrypsin deficiency. Q. J. Med. 41 (1972) 301-315
    • (1972) Q. J. Med. , vol.41 , pp. 301-315
    • Hutchison, D.C.S.1    Barter, C.E.2    Cook, P.J.L.3    Laws, J.W.4    Martelli, N.A.5    Hugh-Jones, P.6
  • 162
    • 0014506022 scopus 로고
    • Serum Pi types in patients with pulmonary diseases
    • Fagerhol, M. K., H. E. Hauge: Serum Pi types in patients with pulmonary diseases. Acta Allergologica 24 (1969) 107-114
    • (1969) Acta Allergologica , vol.24 , pp. 107-114
    • Fagerhol, M.K.1    Hauge, H.E.2
  • 164
    • 33748110742 scopus 로고
    • Alterations in lung function in children with chronic asthma and Pi variants
    • Werner, P., J. S. Hyde, R. V. Lourenco, R. C. Talamo: Alterations in lung function in children with chronic asthma and Pi variants. J. Allergy Clin. Immunol. 55 (1975) 128-129
    • (1975) J. Allergy Clin. Immunol. , vol.55 , pp. 128-129
    • Werner, P.1    Hyde, J.S.2    Lourenco, R.V.3    Talamo, R.C.4
  • 165
    • 0017284059 scopus 로고
    • Alpha-1-antitrypsin levels and prevalence of Pi-variant phentoypes in asthmatic children
    • Katz, R. M., J. Liebermann, S. C. Siegel: Alpha-1-antitrypsin levels and prevalence of Pi-variant phentoypes in asthmatic children. J. Allergy Clin. Immunol. 57 (1976) 41
    • (1976) J. Allergy Clin. Immunol. , vol.57 , pp. 41
    • Katz, R.M.1    Liebermann, J.2    Siegel, S.C.3
  • 167
    • 0018777878 scopus 로고
    • Protease inhibitor variants in children and young adults with chronic asthma
    • Hyde, J. S., P. Werner, C. M. Kumar, B. S. Morre: Protease inhibitor variants in children and young adults with chronic asthma. Ann. Allerg. 43 (1979) 8-13
    • (1979) Ann. Allerg. , vol.43 , pp. 8-13
    • Hyde, J.S.1    Werner, P.2    Kumar, C.M.3    Morre, B.S.4
  • 168
    • 0020429628 scopus 로고
    • Bronchial reactivity, smoking and alpha-1-antitrypsIn: A population-based study of middle-aged men
    • Kabiraj, M. U., G. Simonsson, S. Groth, A. Bjorklund, K. Bulow, S. E. Lindell: Bronchial reactivity, smoking and alpha-1-antitrypsIn: A population-based study of middle-aged men. Am. Rev. Respir. Dis. 126 (1982) 864-869
    • (1982) Am. Rev. Respir. Dis. , vol.126 , pp. 864-869
    • Kabiraj, M.U.1    Simonsson, G.2    Groth, S.3    Bjorklund, A.4    Bulow, K.5    Lindell, S.E.6
  • 171
    • 0016409175 scopus 로고
    • Bronchiectasis and homozygous alpha-1-antitrypsin deficiency
    • Longstreth, G. F., S. A. Wetzmann, R. J. Browning, J. Lieberman: Bronchiectasis and homozygous alpha-1-antitrypsin deficiency. Chest 67 (1975) 233-235
    • (1975) Chest , vol.67 , pp. 233-235
    • Longstreth, G.F.1    Wetzmann, S.A.2    Browning, R.J.3    Lieberman, J.4
  • 172
    • 0017624132 scopus 로고
    • Alpha-1-antitrypsin deficiency with diffuse bronchiectasis and cirrhose of the liver
    • Scott, J. H., C. L. Anderson, P. Shankar, A. Stavrides: Alpha-1-antitrypsin deficiency with diffuse bronchiectasis and cirrhose of the liver. Chest 71 (1977) 535-538
    • (1977) Chest , vol.71 , pp. 535-538
    • Scott, J.H.1    Anderson, C.L.2    Shankar, P.3    Stavrides, A.4
  • 173
    • 0018139798 scopus 로고
    • Selective IgA deficiency and PiZZ-antritrypsin deficiency. Association with recurrent sinopulmonary infections, emphysema, and bronchiectasis
    • Casterline, C. L., R. Evans III, V. C. Battista, R. C. Talamo: Selective IgA deficiency and PiZZ-antritrypsin deficiency. Association with recurrent sinopulmonary infections, emphysema, and bronchiectasis. Chest 73 (1978) 885-886
    • (1978) Chest , vol.73 , pp. 885-886
    • Casterline, C.L.1    Evans III, R.2    Battista, V.C.3    Talamo, R.C.4
  • 174
    • 0026551587 scopus 로고
    • High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency
    • Guest, P. J., D. M. Hansell: High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency. Clin. Radiol. 45 (1992) 260-266
    • (1992) Clin. Radiol. , vol.45 , pp. 260-266
    • Guest, P.J.1    Hansell, D.M.2
  • 175
    • 0028986525 scopus 로고
    • Bronchiectasis and homozygous (PiZZ) alpha-1-antitrypsin deficiency in a young man
    • Rodriguez-Cintron, W., K. Guntupalli, A. E. Fraire: Bronchiectasis and homozygous (PiZZ) alpha-1-antitrypsin deficiency in a young man. Thorax 50 (1995) 424-425
    • (1995) Thorax , vol.50 , pp. 424-425
    • Rodriguez-Cintron, W.1    Guntupalli, K.2    Fraire, A.E.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.