Article information
Full text is only aviable in PDF
Bibliografía
[1.]
C.B. Laurell, S.A. Eriksson.
The elelctrophoretic α-l-globulin pattern of serum in α-l-antitrypsin deficiency.
Scand J Clin Lab Invest, 15 (1963), pp. 132-140
[3.]
H.E. Schultze, K. Heide, H. Haupt.
Alpha-1-antitripsin aus Humanserum.
Klin Wochenschr, 40 (1962), pp. 427-429
[4.]
S. Eriksson.
Alpha 1 antitrypsin deficiency: lessons learned from the bedside to the gene and back again.
Chest, 95 (1989), pp. 181-189
[5.]
S. Eriksson.
Alpha-1-antitrypsin deficiency: Some personal experiences.
Schweiz Med Wschr, 114 (1984), pp. 893-894
[6.]
R.W. Carrell, J.O. Jeppson, C.B. Laurell, et al.
Structure and variation of human a-l-antitrypsin.
Nature, 298 (1982), pp. 329-333
[7.]
R.G. Crystal, M.L. Brantly, R.C. Hubbard, D.T. Curiel, D.J. States, M.D. Holmes.
The α-l-antitrypsin gene and its mutations. Clinical consequences and strategies for therapy.
Chest, 95 (1989), pp. 196-208
[8.]
M. Brantly, T. Nukiwa, R.G. Crystal.
Molecular basis of α-lantitrypsin deficiency.
Am J Med, 84 (1988), pp. 13-31
[9.]
N. Kalsheker, K. Morgan.
The α-l-antitrypsin gene and chronic lung disease.
Thorax, 45 (1990), pp. 759-764
[10.]
A. Rodríguez Cuartera.
Alfa-1-antitripsina: Estudio general, bioquímico, genético y clínico.
Med Clin, 64 (1975), pp. 311-317
[11.]
R.M. Senior, H. Tegner, C. Kuhn, K. Ohlsson, B.C. Starcher, J.A. Pierce.
The induction of pulmonary emphysema with leukocyte elastase.
Am Rev Respir Dis, 116 (1977), pp. 469-475
[12.]
A. Janoff, B. Sloan, G. Weinbaum, et al.
Experimental emphysema induced with purified human neutfophil elastase: Tissue localization of the instilled protease.
Am Rev Respir Dis, 115 (1977), pp. 461-478
[13.]
R.W. Carell.
The molecular estructure and pathology of α-lantitrypsin.
Lung, 168 (1990), pp. 530-534
[14.]
D.C.S. Hutchinson.
The epidemiology of α-l-antitrypsin deficiency.
Lung, 168 (1990), pp. 535-542
[15.]
H.W. Goedde, L. Hirth, H.G. Benkmann, et al.
Population genetic studies of serum protein polymorphisms in four spanish populations: Part II.
Hum Hered, 23 (1973), pp. 235-246
[16.]
F. Ogushi, R.C. Hubbard, G.A. Fells, et al.
Evaluation of the S-type of α-l-antitrypsin as an in vivo and in vitro inhibitor of neutrophil elastase.
Am Rev Respir Dis, 137 (1988), pp. 364-370
[17.]
D.W. Cox, H. Levison.
Emphysema of early onset associated with a complete deficiency of α-l-antitrypsin (null homozygotes).
Am Rev Respir Dis, 137 (1988), pp. 371-375
[18.]
R.I. Garver, J.F. Mornex, T. Nukiwa, et al.
Alpha-1-antitrypsin deficiency and emphysema caused by homozygous inheritance of non-expressing α-1-antitrypsin genes.
N Engl J Med, 314 (1986), pp. 762-766
[19.]
J.A. Pierce, emphysema. Antitrypsin.
Perspective and prospects.
JAMA, 259 (1988), pp. 2.890-2.895
[20.]
J.H. Lewis, R.M. Iammarino, J.A. Spero, et al.
Antithrombin Pittsburgh: An α-1-antitrypsin variant causing hemorrhagic disease.
Blood, 51 (1978), pp. 129-137
[21.]
J.E. Gadek, E.R. Pacht.
The protease-antiprotease balance within the human lung: Implications for the pathogenesis of emphysema.
Lung, 168 (1990), pp. 552-564
[22.]
J.E. Gadek, G.A. Fells, R.L. Zimmerman, S.I. Rennard, R.G. Crystal.
Antielastases of the human alveolar structures. Implications for the protease-antiprotease theory of emphysema.
J Clin Invest, 68 (1981), pp. 889-898
[23.]
S. Idell, U. Kucich, A. Fein, et al.
Neutrophil elastase-releasing factors in bronchoalveolar lavage from patients with adult respiratory distress syndrome.
Am Rev Respir Dis, 132 (1985), pp. 1.098-1.105
[24.]
M. Wewers.
Pathogenesis of emphysema. Asessment of basic Science concepts through clinical investigation.
Chest, 95 (1989), pp. 190-195
[25.]
C.G. Cochrane, R. Spragg, S.D. Revak.
Pathogenesis of the adult respiratory distress syndrome. Evidence of oxidant activity in bronchoalveolar lavage fluid.
J Clin Invest, 71 (1983), pp. 754-761
[26.]
W.B. Davis, G.A. Fells, M.S. Chernick, P.A. diSant’Agnese, R.G. Crystal.
A role for neutrophils in the derangements of the bronchial wall characteristic of cystic fíbrosis.
Am Rev Respir Dis, 127 (1983), pp. 207A
[27.]
R.B. Fick, H.Y. Reynolds.
Pseudomonas respiratory infection in cystic fíbrosis: A possible defect in opsonic IgG antibody.
Bull Eur Physiopathol Respir, 19 (1983), pp. 151-161
[28.]
S. Suter, I. Chevallier.
Proteolytic inactivation of α-l-proteinase inhibitor in infected bronchial secretions from patients with cystic fíbrosis.
Eur Respir J, 4 (1991), pp. 40-49
[29.]
W.R. Abrams, A.M. Fein, V. Kucich, et al.
Proteinase inhibitory function in inflammatory lung disease.
Am Rev Respir Dis, 129 (1984), pp. 735-741
[30.]
J.I. Weitz, K.A. Crowley, S.L. Landman, B.I. Lipman, J. Yu.
Increased neutrophil elastase activity in cigarette smokers.
Ann Intern Med, 107 (1987), pp. 680-682
[31.]
R.T. Abboud, T. Fera, A. Richter, M.Z. Tabona, S. Johal.
Acute effect of smoking on the functional activity of α-l-protease inhibitor in bronchoalveolar lavage fluid.
Am Rev Respir Dis, 131 (1985), pp. 79-85
[32.]
S. Mangione, F. Kueppers, C. Puglia, L.W. Greenspon.
Erythrocytes prevent inactivation of α-1-antitrypsin by cigarette smoke.
Eur Respir J, 4 (1991), pp. 26-30
[33.]
D.J. Riley, J.S. Kerr.
Oxidant injury of the extracellular matrix: Potential role in the pathogenesis of pulmonary emphysema.
Lung, 163 (1985), pp. 1-13
[34.]
S.J. Weiss, S. Regiani.
Neutrophils degrade subendothelial matrices in the presence of α-1-proteinase inhibitor: Cooperative use of lysosomal proteinases and oxygen metabolites.
J Clin Invest, 73 (1984), pp. 1.297-1.303
[35.]
P. Laurent, A. Janoff, H.M. Kagan.
Cigarette smoke blocks cross-linking of elastin in vitro.
Am Rev Respir Dis, 127 (1983), pp. 189-192
[36.]
A. Janoff.
State of the art: elastases and emphysema.
Am Rev Respir Dis, 132 (1985), pp. 417-433
[37.]
F. Pelham, M. Wewers, R. Crystal, A.S. Buist, A. Janoff.
Urinary excretion of desmosine (elastin cross-links) in subjects with PiZZ α-1-antitrypsin deficiency, a phenotype associated with hereditary predisposition to pulmonary emphysema.
Am Rev Respir Dis, 132 (1985), pp. 821-823
[38.]
K.M. Moser, R.M. Smith, R.J. Spragg, G.M. Tisi.
Intravenous administration of α-1-proteinase inhibitor in patients of PiZ and PiM phenotype.
Am J Med, 84 (1988), pp. 70-74
[39.]
D.C.S. Hutchinson.
Natural history of a-l-protease inhibitor deficiency.
Am J Med, 84 (1988), pp. 3-12
[40.]
C. Larsson.
Natural history and life expectancy in severe α-1antitrypsin deficiency PiZ.
Acta Med Scand, 204 (1978), pp. 343-351
[41.]
E.D. Janus, N.T. Phillips, R.W. Carrell.
Smoking, lung function, and α-1-antitrypsin deficiency.
Lancet, 1 (1985), pp. 152-154
[42.]
M.C. Wu, S. Eriksson.
Lung function, smoking and survival in severe α-1-antitrypsin deficiency, PiZZ.
J Clin Epidemiol, 41 (1988), pp. 1.157-1.165
[43.]
E.K. Silverman, J.A. Pierce, M.A. Province, et al.
Variability of pulmonary function in α-1-antitrypsin deficiency: clinical correlates.
Ann Intern Med, 111 (1989), pp. 982-991
[44.]
M.J. Tobin, P.J.L. Cook, C.S. Hutchinson.
Alpha-1-antitrypsin deficiency: The clinical and physiological features in subjects homozygous for Pi type Z.
Br J Dis Chest, 77 (1983), pp. 4-27
[45.]
T. Evald, A. Dirksen, S. Keittelmann, K. Viskum, A. Kok-Jensen.
Decline in pulmonary function in patients with α-1-antitrypsin deficiency.
Lung, 168 (1990), pp. 579-585
[46.]
M.L. Brantly, L.D. Paul, B.H. Miller, R.T. Falk, M. Wu, R.G. Crystal.
Clinical features and history of the destructive lung disease associated with α-1-antitrypsin deficiency of adults with pulmonary symptoms.
Am Rev Respir Dis, 138 (1988), pp. 327-336
[47.]
J. Liberman, B. Winter, A. Sastre.
Alpha-1-antitrypsin Pi-types in 965 COPD patients.
Chest, 89 (1986), pp. 370-373
[48.]
D.W. Cox, V.H. Hoeppner, H. Levison.
Protease inhibitors in patients with chronic obstructive lung disease: the α-1-antitrypsin heterozygote controversy.
Am Rev Respir Dis, 113 (1976), pp. 601-606
[49.]
J. Lieberman.
A role for intermediate, heterozygous α-1antitrypsin deficiency in obstructive lung disease.
Chest, 98 (1990), pp. 522-523
[50.]
C. Mittman.
The PiMZ phenotype: Is it a significant risk factor for the development of chronic obstructive lung disease?.
Am Rev Respir Dis, 118 (1978), pp. 649-652
[51.]
S. Eriksson, J. Carlson, R. Velez.
Risk of cirrhosis and primary liver cancer in α-1-antitrypsin deficiency.
N Engl J Med, 314 (1986), pp. 736-739
[52.]
D.M. Parham, J.R. Paterson, A. Gunn, W. Guthrie.
Cholangiocarcinoma in two siblings with emphysema and α-1-antitrypsin deficiency.
Q J Med, 264 (1989), pp. 359-367
[53.]
S.S. Buist.
Alpha-1-antitrypsin deficiency, diagnosis, treatment and control: Identification of patients.
Lung, 168 (1990), pp. 543-551
[54.]
American Thoracic Society.
Guidelines for the approach to the patient with severe hereditary α-1-antitrypsin deficiency.
Am Rev Respir Dis, 140 (1989), pp. 1.494-1.497
[55.]
R. Cordero Torres, F.J. Saenz de Santamaria, F. Lozano Gutiérrez, A. Soria Monje, M. Perez Miranda.
Déficit de α-1-antitripsina asociado a enfermedad de Paget.
Rev Clin Esp, 171 (1983), pp. 437-438
[56.]
M.K. Fagerhol, H.E. Hauge.
Serum Pi type in patients with pulmonary diseases.
Acta Allergol, 24 (1969), pp. 107-114
[57.]
J.S. Hyde, P. Werner, C.M. Kumar, B.S. Moore.
Protease inhibitor variants in children and young adults with chronic asthma.
Ann Allergy, 43 (1979), pp. 8-13
[58.]
J.P. Michalski, C.C. McCombs, E. Scopelitis, J.J. Biundo, T.A. Medsger.
Alpha-1-antitrypsin phenotypes including M subtypes in pulmonary disease associated with rheumatoid arthritis and systemic sclerosis.
Arthritis Rheum, 29 (1986), pp. 586-591
[59.]
D.W. Cox, O. Huber.
Rheumatoid arthritis and α-1-antitrypsin.
Lancet, 1 (1976), pp. 1.216-1.217
[60.]
R.C. Hubbard, S. Sellers, D. Czerski, L. Stephens, R.G. Crystal.
Biochemical efficacy and safety on monthly augmentation therapy for α-1-antitrypsin deficiency.
JAMA, 260 (1988), pp. 1.259-1.264
[61.]
G.L. Snider.
Pulmonary disease in α-1-antitrypsin deficiency.
Ann Intern Med, 111 (1989), pp. 957-958
[62.]
E.W. Schmidt, B. Rasche, W.T. Ulmer, et al.
Replacement therapy for α-l-protease inhibitor deficiency in PiZ subjects with chronic obstructive lung disease.
Am J Med, 84 (1988), pp. 63-69
[63.]
N. Konietzko.
Alpha-1-antitrypsin substitution treatment or prevention of emphysema.
Lung, 168 (1990), pp. 592-598
[64.]
K. Viskum, A. Kok-Jensen.
Criteria for α-1-antitrypsin substitution.
Lung, 168 (1990), pp. 586-591
[65.]
R. Vidal, M. Miravitlles, X. De Gracia, B. Gallego, F. Morell.
Tratamiento sustitutivo del enfisema por déficit de α-1-antitripsina.
Med Clin, 96 (1991), pp. 180-182
[66.]
M.D. Wewers, M.A. Casolaro, R.G. Crystal.
Comparison of α-1antitrypsin levels and antineutrophil elastase capacity of blood and lung in a patient with the α-1-antitrypsin phenotype nuil-nuil before and during α-1-antitrypsin augmentation therapy.
Am Rev Respir Dis, 135 (1987), pp. 539-543
[67.]
M.D. Wewers, M.A. Casolaro, S.E. Sellers, et al.
Replacement therapy for α-1-antitrypsin deficiency associated with emphysema.
N Engl J Med, 316 (1987), pp. 1.055-1.062
[68.]
M.D. Wewers, M.L. Brantly, M.A. Casolaro, R.G. Crystal.
Evaluation of tamoxifen as a therapy to augment α-1-antitrypsin concentration in Z homozygous α-1-antitrypsin deficient individuals.
Am Rev Respir Dis, 135 (1987), pp. 401-404
[69.]
P.M. George, M.C.M. Vissers, J. Travis, C.C. Winterbourn, R.W. Carell.
A genetically engineered mutant of α-l-antitrypsin protects connective tissue from neutrophil damage and may be useful in lung disease.
Lancet, 2 (1984), pp. 1.426-1.428
[70.]
M. Courtney, A. Jallat, L.H. Tessier, A. Benavente, R.G. Crystal, J.P. LeCocq.
Synthesis in E. coli of a-l-antitrypsin variants with potential in the therapy of emphysema and thrombosis.
Nature, 313 (1985), pp. 149-151
[71.]
R.C. Hubbard, M.L. Brantly, S.E. Sellers, M.E. Mitchell, R.G. Crystal.
Anti-neutrophil-elastase defenses of the lower respiratory tract in α-l-antitrypsin deficiency directly augmented with an aerosol of a-l-antitrypsin.
Ann Intern Med, 111 (1989), pp. 206-212
[72.]
M.A. Casolaro, G.A. Fells, R.C. Hubbard, et al.
Augmentation of lung antineutrophil elastase capacity with recombinant human α-lantitrypsin.
J Appl Physiol, 63 (1987), pp. 2.015-2.023
[73.]
R.C. Hubbard, N.G. McElvaney, S.E. Sellers, J.T. Healy, D.B. Czerski, R.G. Crystal.
Recombinant DNA-produced α-l-antitrypsin administered by aerosol augments lower respiratory tract antineutrophil elastase defenses in individuals with α-l-antitrypsin deficiency.
J Clin Invest, 84 (1989), pp. 1.349-1.354
[74.]
R.C. Hubbard, R.G. Crystal.
Strategies for aerosol therapy of α-l-antitrypsin deficiency by the aerosol route.
Lung, 168 (1990), pp. 565-578
[75.]
R.G. Crystal.
Reversible bronchoalveolar lavage.
Conferencia en «3rd International Conference in bronchoalveolar lavage». Viena 21-6-, (1991),
Copyright © 1992. Sociedad Española de Neumología y Cirugía Torácica
