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Vol. 35. Issue 9.
Pages 446-454 (October 1999)
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Vol. 35. Issue 9.
Pages 446-454 (October 1999)
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Estado actual del tratamiento sustitutivo en el enfisema congénito por déficit de alfa-1-antitripsina. Informe del Registro Nacional
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M. Miravitllesa,
Corresponding author
marcm@separ.es

Correspondencia: Rocafort, 173-177, 3º. Iª. 08015 Barcelona.
, R. Vidala, J.C. Barros-Tizónb, A. Bustamantec, P.P. Españad, F. Casase, M.T. Martínezf, C. Escuderog, R. Jardíh
a Servei de Pneumologia. Hospital Vall d’Hebron. Barcelona
b Servicio de Neumología. Hospital Xeral-Cies. Vigo
c Unidad de Neumología. Hospital Sierrallana. Torrelavega
d Servicio de Neumología. Hospital de Galdakao. Vizcaya
e Servicio de Neumología. Hospital Clínico San Cecilio. Granada
f Servicio de Neumología. Hospital 12 de Octubre. Madrid
g Servicio de Neumología. Hospital Central de Asturias. Oviedo
h Servei de Bioquímica. Hospital Vall d’Hebron. Barcelona
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Bibliografía
[1.]
R.G. Crystal, M.L. Brantly, R.C. Hubbard, D.T. Curiel, D.J. States, M.D. Holmes.
The alpha-1-antitrypsin gene and its mutations. Clinical consequences and strategies for therapy.
Chest, 95 (1989), pp. 196-208
[2.]
M. Miravitlles, R. Vidal, J. de Gracia.
Enfisema por déficit de alfa-1-antitripsina. Situación actual y nuevas perspectivas para el tratamiento.
Arch Bronconeumol, 28 (1992), pp. 296-302
[3.]
R.W. Carell.
The molecular estructure and pathology of alpha-1-antitrypsin.
Lung, 168 (1990), pp. 530-534
[4.]
S. DeCroo, M.I. Kamboh, R.E. Ferrell.
Population genetics of alpha-1-antitrypsin polymorphism in US whites.
US blacks and African blacks. Hum Hered, 41 (1991), pp. 215-221
[5.]
I. Yuasa, K. Suenaga, Y. Gotoh, N. Yokoyama, K. Okada.
PI (alpha-1-antitrypsin) polymorphism in the Japanese: confirmation of PI*M4 and description of new PI variants.
Hum Genet, 67 (1984), pp. 209-212
[6.]
G. Beckman, L. Beckman, I. Nordenson.
alpha-1-antitrypsin phenotypes in Northern Sweden.
Hum Hered, 30 (1980), pp. 129-135
[7.]
M. Thymann.
Distribution of alpha-1-antitrypsin phenotypes in Denmark determined by separator isoelectric focusing in agarose gel.
Hum Hered, 36 (1986), pp. 19-23
[8.]
R. Vidal, M. Miravitlles, R. Jardí, M. Torrella, F. Rodríguez-Frías, P. Moral, J. Vaqué.
Estudio de la frecuencia de los diferentes fenotipos de la alfa-1-antitripsina en una población de Barcelona.
Med Clin (Barc), 107 (1996), pp. 211-214
[9.]
D.C.S. Hutchison.
The epidemiology of alpha-1-antitrypsin deficiency.
Lung, 168 (1990), pp. 535-542
[10.]
F. Ogushi, R.C. Hubbard, C.A. Fells, A. Casolaro, D.T. Curiel, M.L. Brantly, R.G. Crystal.
Evaluation of the S-type of alpha-1-antitrypsin as an in vivo and in vitro inhibitor of neutrophil elastase.
Am Rev Respir Dis, 137 (1988), pp. 364-370
[11.]
I. López-Abadía, F. Bandrés, J. Jaqueti, L. Chicharro.
Asociación de fenotipos infrecuentes con déficit parciales de alfa-1-antitripsina.
Med Clin (Barc), 102 (1994), pp. 398
[12.]
R. Jardí, F. Rodríguez-Frías, F. Casas, M. Cotrina, R. Vidal, M. Miravitlles, C. Pascual.
Caracterización molecular de dos variantes deficitarias de la alfa-1-antitripsina: PIMpalermo y PIPlovel.
Med Clin (Barc), 109 (1997), pp. 463-466
[13.]
Jardí R, Rodríguez F, Miravitlles M, Vidal R, Cotrina M, Quer J, Pascual C, Weidinger S. Identification and molecular characterization of the new alpha-1-antitrypsin deficient alíele PI Y barcelona (Asp256-Val and Pro391- His). Human Mutation, Mutation in brief# 174 (1998) Online.
[14.]
C. Larsson.
Natural history and life expectancy in severe alpha-1- antitrypsin deficiency PiZ.
Acta Med Scand, 204 (1978), pp. 343-351
[15.]
M.C. Wu, S. Eriksson.
Lung function, smoking and survival in severe alpha-1-antitrypsin deficiency, PiZZ.
J Clin Epidemiol, 41 (1988), pp. 1.157-1.165
[16.]
E.D. Janus, N.T. Phillips, R.W. Carrell.
Smoking, lung function, and alpha-1-antitrypsin deficiency.
[17.]
E.K. Silverman, J.A. Pierce, M.A. Province, D.C. Rao, E.J. Campbell.
Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates.
Ann Intern Med, 111 (1989), pp. 982-991
[18.]
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. 14-27
[19.]
E. Piitulainen, G. Tornling, S. Eriksson.
Environmental correlates of impaired lung function in non-smokers with severe alpha-1-antitrypsin deficiency (PiZZ).
Thorax, 53 (1998), pp. 939-943
[20.]
E. Piitulainen, G. Tornling, S. Eriksson.
Effect of age and occupational exposure to airway irritants on lung function in non-smoking individuals with alpha-1-antitrypsin deficiency (PiZZ).
Thorax, 52 (1997), pp. 244-248
[21.]
N. Seersholm, A. Kok-Jensen.
Clinical features and prognosis of life time non-smokers with severe alpha-1-antitrypsin deficiency.
Thorax, 53 (1998), pp. 265-268
[22.]
Miravitlles M, de Gracia J, Rodrigo MJ, Cruz MJ, Vendrell M, Vidal R, et al. Specific antibody response against the 23-valent pneumococcal vaccine in patients with alpha-1-antitrypsin deficiency with and without bronchiectasis. Chest 1999 (en prensa).
[23.]
N. Seersholm, A. Kok-Jensen, A. Dirksen.
Survival of patients with severe alpha-1-antitrypsin deficiency with special reference to non-index cases.
Thorax, 94 (1994), pp. 695-698
[24.]
N. Seersholm, A. Kok-Jensen, A. Dirksen.
Decline in FEV1 among patients with severe hereditary alpha-1-antitrypsin deficiency type PiZ.
Am J Respir Crit Care Med, 152 (1995), pp. 1.922-1.925
[25.]
N. Seersholm, 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), pp. 369-373
[26.]
N. Seersholm, A. Dirksen, A. Kok-Jensen.
Airways obstruction and two year survival in patients with severe alpha-1-antitrypsin deficiency.
Eur Respir J, 7 (1994), pp. 1.985-1.987
[27.]
E. Kerem, J. Reisman, M. Corey, G.J. Canny, H. Levison.
Prevision of mortality in patients with cystic fibrosis.
N Engl J Med, 326 (1992), pp. 1.187-1.191
[28.]
N. Seersholm.
Body mass index and mortality in patients with severe alpha-1-antitrypsin deficiency.
Respir Med, 91 (1997), pp. 77-82
[29.]
G.L. Snider.
Pulmonary disease in alpha-1-antitrypsin deficiency.
Ann Intern Med, 111 (1989), pp. 957-958
[30.]
K.M. Moser, R.M. Smith, R.J. Spragg, G.M. Tisi.
Intravenous administration of alpha-1-proteinase inhibitor in patients of PiZ and PiM phenotype.
Am J Med, 84 (1988), pp. 70-74
[31.]
M.D. Wewers, M.A. Casolaro, R.G. Crystal.
Comparison of alpha-1-antitrypsin levels and antineutrophil elastase capacity of blood and lung in a patient with the alpha-1-antitrypsin phenotype nuil-nuil before and during alpha-1-antitrypsin augmentation therapy.
Am Rev Respir Dis, 135 (1987), pp. 539-543
[32.]
M.D. Wewers, M.A. Casolaro, S.E. Sellers, S.C. Swayze, K.M. McPhaul, J.T. Wittes, et al.
Replacement therapy for alpha-1-antitrypsin deficiency associated with emphysema.
N Engl J Med, 316 (1987), pp. 1.055-1.062
[33.]
American Thoracic Society.
Guidelines for the approach to the patient with severe hereditary alpha-1-antitrypsin deficiency.
Am Rev Respir Dis, 140 (1989), pp. 1.494-1.497
[34.]
R.C. Hubbard, S. Sellers, D. Czerski, L. Stephens, R.G. Crystal.
Biochemical efficacy and safety on monthly augmentation therapy for alpha-1-antitrypsin deficiency.
JAMA, 260 (1988), pp. 1.259-1.264
[35.]
P.J. Stone, T.A. Morris III., C. Franzblau, G.L. Snider.
Preliminary evidence that augmentation therapy diminishes degradation of cross- linked elastin in alpha-1-antitrypsin-deficient humans.
Respiration, 62 (1995), pp. 76-79
[36.]
D.J. Gottlieb, P.J. Stone, D. Sparrow, M.E. Gale, S.T. Weiss, G.L. Snider, et al.
Urinary desmosine excretion in smokers with and without rapid decline of lung function: The Normative Aging Study.
Am J Respir Crit Care Med, 154 (1996), pp. 1.290-1.295
[37.]
N. Anthonisen.
Design of a clinical trial to test a treatment of the underlying cause of emphysema.
Ann NY Acad Sci, 624 (1991), pp. 31-34
[38.]
J.W. Hay, E.D. Robin.
Cost-effectiveness of alpha-1-antitrypsin replacement therapy in treatment of congenital chronic obstructive pulmonary disease.
Am J Public Health, 81 (1991), pp. 427-433
[39.]
F. Manresa.
Evolución del tratamiento sustitutivo del enfisema por déficit de alfa-1-antitripsina [carta].
Arch Bronconeumol, 31 (1995), pp. 371
[40.]
R. Bhagat, V.A. Swystun, D.W. Cockcroft.
Clinical trials needed for alpha-1-antitrypsin replacement therapy.
Chest, 108 (1995), pp. 586-587
[41.]
D.C.S. Hutchinson, M.D. Hughes.
alpha-1-antitrypsin replacement therapy: will its efficacy ever be proved?.
Eur Respir J, 10 (1997), pp. 2.191-2.193
[42.]
N. Konietzko.
alpha-1-antitrypsin substitution-treatment or prevention of emphysema.
Lung, 168 (1990), pp. 592-598
[43.]
P. Caries, J. Constans, M.C. Pujazon, J. Arnaud, D. Lauque, M. Goudemand.
Bilan à deux ans du traitement substitutif de 1’emphyséme PiZZ par 1’alpha-1-antitrypsine.
Neuf cas. Presse Méd, 19 (1990), pp. 514-518
[44.]
A. Dirksen, F. Madsen, C.S. Ulrik, A. Kok-Jensen, T. Heckscher, K. Viskum, et al.
A randomized placebo-controlled pilot study of alpha-1-protease inhibitor replacement therapy monitored by daily spirometry.
Am Rev Respir Dis, 147 (1993), pp. 325A
[45.]
A.F. Barker, F. Siemsen, D. Pasley, R. D'Silva, S. Buist.
Replacement therapy for hereditary alpha-1-antitrypsin deficiency. A program for long-term administration.
Chest, 105 (1994), pp. 1.406-1.410
[46.]
R. Vidal, M. Miravitlles, X. De Gracia, B. Gallego, F. Morell.
Tratamiento sustitutivo del enfisema por déficit de Alfa-1-antitripsina.
Med Clin (Barc), 96 (1991), pp. 180-182
[47.]
M. Miravitlles, R. Vidal, M. Torrella, J.M. Bofill, M. Cotrina, J. de Gracia.
Evaluación del tratamiento sustitutivo del enfisema por déficit de Alfa-1-antitripsina.
Arch Bronconeumol, 30 (1994), pp. 479-484
[48.]
M. Miravitlles.
¿Debemos administrar tratamiento sustitutivo a los pacientes con déficit de Alfa-1-antitripsina?.
Arch Bronconeumol, 34 (1998), pp. 109-111
[49.]
M. Schwaiblmair, C. Volgemeier, G. Fruhmann.
Long-term augmentation therapy in twenty patients with severe alpha-1-antitryp- sin deficiency.
Three-year follow-up. Respiration, 64 (1997), pp. 10-15
[50.]
B. Burrows, M.D. Lebowitz, A.E. Camilli, R.J. Knudson.
Longitudinal changes in forced expiratory volume in one second in adults.
Am Rev Respir Dis, 133 (1986), pp. 974-980
[51.]
M. Wencker, N. Banik, R. Buhl, R. Seidel, N. Konietzko, for the Wis- senschaftliche Arbeitsgemeinschaft zur Therapie von Lungener- krankungen (WATL) alpha-1-AT study group.
Long-term treatment of alpha-1-antitrypsin deficiency-related pulmonary emphysema with human alpha-1-antitrypsin.
Eur Respir J, 11 (1998), pp. 428-433
[52.]
N. Seersholm, M. Wencker, N. Banik, K. Viskum, A. Dirksen, A. KokJensen, N. Konietzko, for the Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha-1-AT study group.
Does alpha-1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha-1-antitrypsin deficiency?.
Eur Respir J, 10 (1997), pp. 2.260-2.263
[53.]
The alpha-1-Antitrypsin Deficiency Registry Study Group.
Survival and FEV1 decline in individuals with severe deficiency of alpha-1-antitrypsin.
Am J Respir Crit Care Med, 158 (1998), pp. 49-59
[54.]
J. De Gracia, M. Vendrell, L. Guarner, R. Vidal, M. Miravitlles, C. Mayordomo, et al.
Utilización de gammaglobulina humana en el tratamiento de la inmunodeficiencia común variable.
Med Clin (Barc), 104 (1995), pp. 201-206
[55.]
B. Wallaert, B. Gressier, C.H. Marquette, P. Gosset, M. Remy-Jardin, J. Mizon, et al.
Inactivation of alpha-1-proteinase inhibitor by alveolar inflammatory cells from smoking patients with or without emphysema.
Am Rev Respir Dis, 147 (1993), pp. 1.537-1.543
[56.]
A. Yoshioka, T. Betsuyaku, M. Nishimura, K. Miyamoto, T. Kondo, Y. Kawakami.
Excessive neutrophil elastase in bronchoalveolar lavage fluid in subclinical emphysema.
Am J Respir Crit Care Med, 152 (1995), pp. 2.127-2.132
[57.]
E.N. Kosmas, D. Zorpidou, V. Vassilareas, T. Roussou, S. Michaelides.
Decreased C4 complement component serum levels correlate with the degree of emphysema in patients with chronic bronchitis.
Chest, 112 (1997), pp. 341-347
[58.]
M. Wencker, J. Denker, N. Konietzko.
Serial measurements of FEV1 over 12 years in a patient with alpha-1-protease inhibitor deficiency: influence of augmentation therapy and infections.
Respiration, 61 (1994), pp. 195-198
[59.]
P.C. Cassina, H. Teschler, N. Konietzko, D. Theegarten, G. Stamatis.
Two-year results after lung volume reduction surgery in alpha-1-antitrypsin deficiency versus stnoker's emphysema.
Eur Respir J, 12 (1998), pp. 1.028-1.032
[60.]
H.F. Huizenga, S.D. Ramsey, R.K. Albert.
Estimated growth of lung volume reduction surgery among Medicare enrollees.
Chest, 114 (1998), pp. 1.583-1.587
[61.]
F.J. Meyer, M. Wencker, H. Teschler, H. Steveling, J. Sennekamp, U. Costabel, et al.
Acute allergic reaction and demonstration of specific IgE antibodies against alpha-1-protease inhibitor.
Eur Respir J, 12 (1998), pp. 996-997
[62.]
The alpha-1-Antitrypsin Deficiency Registry Study Group.
A registry of patients with severe deficiency of alpha-1-antitrypsin. Design and methods.
Chest, 106 (1994), pp. 1.223-1.232
[63.]
N.G. McElvaney, J.K. Stoller, A.S. Buist, U.B.S. Prakash, M.L. Brandy, M.D. Schluchter, et al.
Baseline characteristics of enrollees in the National Heart. Lung and Blood Institute Registry of alpha-1-antitrypsin deficiency.
Chest, 111 (1997), pp. 394-403
[64.]
M. Wencker, for the International Registry on alpha-1-antitrypsin deficiency.
New formation of the international Registry on alpha-1-antitrypsin deficiency as a joint database of multiple national registries.
Eur Respir J, 12 (1998), pp. 382
[65.]
R. Vidal, M. Miravitlles, y Grupo de Estudio del Déficit de Alfa-1-antitripsina.
Informe del Registro Español de Pacientes con Déficit de Alfa-1-antitripsina.
Arch Bronconeumol, 31 (1995), pp. 299-302
[66.]
M. Miravitlles, R. Vidal, J.C. Barros-Tizón, A. Bustamante, P.P. España, F. Casas, et al.
Usefulness of a national registry of alpha-1-antitrypsin deficiency.
The Spanish experience. Respir Med, 92 (1998), pp. 1.181-1.187
[67.]
M. Luisetti, B. Balbi, G. Massi, F.M. Menchicchi, M. Massobrio, M. Beccaria, et al.
Results of a program to detect alpha-1-antitrypsin deficiency in Italy.
Am J Respir Crit Care Med, 157 (1998), pp. 383
[68.]
M.K. Fagerhol, O.W. Tenfjord.
Serum Pi types in some European. American.
Asian and African populations. Acta Patol Microbiol Scand, 72 (1968), pp. 601-608
[69.]
M.J. Andrés Puertas, M.L. Arranz Peña.
Valoración de fenotipos de Alfa-1-antitripsina en Castilla-León: estudio en una población de recién nacidos.
Rev Clin Esp, 184 (1989), pp. 106-108
[70.]
M. Miravitlles, R. Jardí, F. Rodríguez-Frías, M. Torrella, D. Pelegrí, R. Vidal.
Utilidad de la cuantificación de la banda alfa-1 del proteinograma sérico en el cribado del déficit de Alfa-1-antitripsina.
Arch Bronconeumol, 34 (1998), pp. 536-540
[71.]
J. Constans, P. Caries, A. Boneu, J. Arnaud, A.E. Tufenkji, M.C. Pujazon, et al.
Clinical pharmacokinetics of alpha-1-antitrypsin in homozygous PiZ deficient patients.
Clin Pharmacokinet, 23 (1992), pp. 161-168
[72.]
A.F. Barker, I. Iwata-Morgan, L. Oveson, R. Roussel.
Pharmacokinetic study of alpha-1-antitrypsin infusion in alpha-1-antitrypsin deficiency.
Chest, 112 (1997), pp. 607-613
[73.]
World Health Organization.
alpha-1-antitrypsin deficiency: Memorandum from a WHO meeting.
Bulletin of the WHO, 75 (1997), pp. 397-415
[74.]
I.D. Horowitz.
alpha-1-proteinase inhibitor (human) shortage.
Chest, 110 (1996), pp. 1.624-1.625
[75.]
A.S. Buist, B. Burrows, S. Eriksson, C. Mittman, M. Wu.
The natural history of air-flow obstruction in PiZ emphysema.
Report of a NHLBI workshop. Am Rev Respir Dis, 127 (1983), pp. 43-45
[76.]
M.L. Brandy, 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 alpha-1-antitrypsin deficiency in adults with pulmonary symptoms.
Am Rev Respir Dis, 138 (1988), pp. 327-336
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