-
2
-
-
20744433150
-
1 antitrypsin and to Carl-Bertil Laurell
-
1 antitrypsin and to Carl-Bertil Laurell COPD 1 2004 71 84
-
(2004)
COPD
, vol.1
, pp. 71-84
-
-
Carrell, R.W.1
-
3
-
-
84907041622
-
The electrophoretic alpha 1-globulin pattern of serum in alpha 1-antitrypsin deficiency
-
C-B Laurell, A Eriksson The electrophoretic alpha 1-globulin pattern of serum in alpha 1-antitrypsin deficiency Scand J Clin Lab Invest 15 1963 132 40
-
(1963)
Scand J Clin Lab Invest
, vol.15
, pp. 132-140
-
-
Laurell, C.B.1
Eriksson, A.2
-
4
-
-
4544284761
-
-
National Center for Health Statistics Hyattsville
-
M Lethbridge-Çejku, JS Schiller, L Bernadel Vital and Health Statistics, series 10, number222 summary health statistics for US adults - National Health Interview Survey, 2002 2004 National Center for Health Statistics Hyattsville http://www.cdc.gov/nchs/data/series/sr_10/sr10_222.pdf (accessed April 18, 2005)
-
(2004)
Vital and Health Statistics, Series 10, number222 Summary Health Statistics for US Adults - National Health Interview Survey, 2002
-
-
Lethbridge-ÇEjku, M.1
Schiller, J.S.2
Bernadel, L.3
-
5
-
-
0022638237
-
Alpha 1-antitrypsin Pi-types in 965 COPD patients
-
J Lieberman, B Winter, A Sastre Alpha 1-antitrypsin Pi-types in 965 COPD patients Chest 89 1986 370 73
-
(1986)
Chest
, vol.89
, pp. 370-373
-
-
Lieberman, J.1
Winter, B.2
Sastre, A.3
-
6
-
-
0242525259
-
Genetic epidemiology of alpha-1 antitrypsin deficiency in North America and Australia/New Zealand: Australia, Canada, New Zealand and the United States of America
-
FJ De Serres, I Blanco, E Fernandez-Bustillo Genetic epidemiology of alpha-1 antitrypsin deficiency in North America and Australia/New Zealand: Australia, Canada, New Zealand and the United States of America Clin Genet 64 2003 382 97
-
(2003)
Clin Genet
, vol.64
, pp. 382-397
-
-
De Serres, F.J.1
Blanco, I.2
Fernandez-Bustillo, E.3
-
7
-
-
0036433482
-
Worldwide racial and ethnic distribution of alpha(1)-antitrypsin deficiency: Summary of an analysis of published genetic epidemiologic surveys
-
FJ de Serres Worldwide racial and ethnic distribution of alpha(1)-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys Chest 122 2002 1818 29
-
(2002)
Chest
, vol.122
, pp. 1818-1829
-
-
De Serres, F.J.1
-
8
-
-
1242343871
-
Alpha1-antitrypsin deficiency, 1: Epidemiology of alpha 1-antitrypsin deficiency
-
M Luisetti, N Seersholm Alpha1-antitrypsin deficiency, 1: epidemiology of alpha 1-antitrypsin deficiency Thorax 59 2004 164 69
-
(2004)
Thorax
, vol.59
, pp. 164-169
-
-
Luisetti, M.1
Seersholm, N.2
-
9
-
-
0017902246
-
Neonatal screening for alpha 1-antitrypsin deficiency
-
ML O'Brien, NR Buist, WH Murphey Neonatal screening for alpha 1-antitrypsin deficiency J Pediatr 92 1978 1006 10
-
(1978)
J Pediatr
, vol.92
, pp. 1006-1010
-
-
O'Brien, M.L.1
Buist, N.R.2
Murphey, W.H.3
-
10
-
-
0024434432
-
Alpha-1-antitrypsin deficiency: High prevalence in the St Louis area determined by direct population screening
-
EK Silverman, JP Miletich, JA Pierce Alpha-1-antitrypsin deficiency: high prevalence in the St Louis area determined by direct population screening Am Rev Respir Dis 140 1989 961 66
-
(1989)
Am Rev Respir Dis
, vol.140
, pp. 961-966
-
-
Silverman, E.K.1
Miletich, J.P.2
Pierce, J.A.3
-
11
-
-
0027429896
-
Molecular confirmation of alpha 1-antitrypsin genotypes in newborn dried blood specimens
-
WC Spence, JE Morris, K Pass, PD Murphy Molecular confirmation of alpha 1-antitrypsin genotypes in newborn dried blood specimens Biochem Med Metab Biol 50 1993 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
-
12
-
-
0017099344
-
Liver disease in alpha 1-antitrypsin deficiency detected by screening of 200,000 infants
-
T Sveger Liver disease in alpha 1-antitrypsin deficiency detected by screening of 200,000 infants N Engl J Med 294 1976 1316 21
-
(1976)
N Engl J Med
, vol.294
, pp. 1316-1321
-
-
Sveger, T.1
-
14
-
-
0030465416
-
A 30-year perspective on alpha 1-antitrypsin deficiency
-
S Eriksson A 30-year perspective on alpha 1-antitrypsin deficiency Chest 110 6 suppl 1996 237S 42S
-
(1996)
Chest
, vol.110
, Issue.6 SUPPL.
-
-
Eriksson, S.1
-
15
-
-
0002729721
-
Alpha 1-antitrypsin deficiency
-
J.B. Stanbury J.B. Wyngaarden D.S. Frederickson McGraw-Hill New York *et al.
-
JE Gadek, RG Crystal Alpha 1-antitrypsin deficiency JB Stanbury JB Wyngaarden DS Frederickson The metabolic basis of inherited disease 1983 McGraw-Hill New York 1450 67
-
(1983)
The Metabolic Basis of Inherited Disease
, pp. 1450-1467
-
-
Gadek, J.E.1
Crystal, R.G.2
-
16
-
-
0020678269
-
Alpha 1 antitrypsin deficiency: The clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z - A survey by the British Thoracic Association
-
MJ Tobin, PJ Cook, DC Hutchison Alpha 1 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 77 1983 14 27
-
(1983)
Br J Dis Chest
, vol.77
, pp. 14-27
-
-
Tobin, M.J.1
Cook, P.J.2
Hutchison, D.C.3
-
17
-
-
0028543862
-
Physical and social impact of alpha 1-antitrypsin deficiency: Results of a survey
-
JK Stoller, P Smith, P Yang, J Spray Physical and social impact of alpha 1-antitrypsin deficiency: results of a survey Cleve Clin J Med 61 1994 461 67
-
(1994)
Cleve Clin J Med
, vol.61
, pp. 461-467
-
-
Stoller, J.K.1
Smith, P.2
Yang, P.3
Spray, J.4
-
18
-
-
20744439773
-
Quality of life among patients with alpha-1 antitrypsin deficiency on augmentation therapy
-
MA Campos, R Sandhaus Quality of life among patients with alpha-1 antitrypsin deficiency on augmentation therapy Am J Respir Crit Care Med 169 2004 A517
-
(2004)
Am J Respir Crit Care Med
, vol.169
, pp. 517
-
-
Campos, M.A.1
Sandhaus, R.2
-
19
-
-
27144471656
-
Delay in diagnosis of alpha-1 antitrypsin deficiency: A continuing problem
-
(in press)
-
Stoller JK, Sandhaus RA, Turino GM, Dickson R, Rodger K, Strange C. Delay in diagnosis of alpha-1 antitrypsin deficiency: a continuing problem. Chest (in press)
-
Chest
-
-
Stoller, J.K.1
Sandhaus, R.A.2
Turino, G.M.3
Dickson, R.4
Rodger, K.5
Strange, C.6
-
20
-
-
1542713396
-
Alpha 1-antitrypsin deficiency, 2: Genetic aspects of alpha(1)-antitrypsin deficiency: Phenotypes and genetic modifiers of emphysema risk
-
DL DeMeo, EK Silverman Alpha 1-antitrypsin deficiency, 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk Thorax 59 2004 259 64
-
(2004)
Thorax
, vol.59
, pp. 259-264
-
-
Demeo, D.L.1
Silverman, E.K.2
-
21
-
-
0020510606
-
Mutation of antitrypsin to antithrombin: Alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder
-
MC Owen, SO Brennan, JH Lewis, RW Carrell Mutation of antitrypsin to antithrombin: alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder N Engl J Med 309 1983 694 98
-
(1983)
N Engl J Med
, vol.309
, pp. 694-698
-
-
Owen, M.C.1
Brennan, S.O.2
Lewis, J.H.3
Carrell, R.W.4
-
22
-
-
85047684827
-
Alpha 1-antitrypsin polymerization and the serpinopathies: Pathobiology and prospects for therapy
-
DA Lomas, R Mahadeva Alpha 1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy J Clin Invest 110 2002 1585 90
-
(2002)
J Clin Invest
, vol.110
, pp. 1585-1590
-
-
Lomas, D.A.1
Mahadeva, R.2
-
24
-
-
0037271797
-
Gene therapy progress and prospects: Alpha-1 antitrypsin
-
AA Stecenko, KL Brigham Gene therapy progress and prospects: alpha-1 antitrypsin Gene Ther 10 2003 95 99
-
(2003)
Gene Ther
, vol.10
, pp. 95-99
-
-
Stecenko, A.A.1
Brigham, K.L.2
-
25
-
-
0019321009
-
Kinetics of association of serine proteinases with native and oxidized alpha-1-proteinase inhibitor and alpha-1-antichymotrypsin
-
K Beatty, J Bieth, J Travis Kinetics of association of serine proteinases with native and oxidized alpha-1-proteinase inhibitor and alpha-1- antichymotrypsin J Biol Chem 255 1980 3931 34
-
(1980)
J Biol Chem
, vol.255
, pp. 3931-3934
-
-
Beatty, K.1
Bieth, J.2
Travis, J.3
-
26
-
-
0036793752
-
Chronic obstructive pulmonary disease 3: Experimental animal models of pulmonary emphysema
-
R Mahadeva, SD Shapiro Chronic obstructive pulmonary disease 3: experimental animal models of pulmonary emphysema Thorax 57 2002 908 14
-
(2002)
Thorax
, vol.57
, pp. 908-914
-
-
Mahadeva, R.1
Shapiro, S.D.2
-
27
-
-
0036545472
-
Proteinases in chronic obstructive pulmonary disease
-
SD Shapiro Proteinases in chronic obstructive pulmonary disease Biochem Soc Trans 30 2002 98 102
-
(2002)
Biochem Soc Trans
, vol.30
, pp. 98-102
-
-
Shapiro, S.D.1
-
28
-
-
0034687422
-
Structure of a serpin-protease complex shows inhibition by deformation
-
JA Huntington, RJ Read, RW Carrell Structure of a serpin-protease complex shows inhibition by deformation Nature 407 2000 923 26
-
(2000)
Nature
, vol.407
, pp. 923-926
-
-
Huntington, J.A.1
Read, R.J.2
Carrell, R.W.3
-
29
-
-
0033515559
-
A kinetic mechanism for the polymerization of alpha 1-antitrypsin
-
TR Dafforn, R Mahadeva, PR Elliott, P Sivasothy, DA Lomas A kinetic mechanism for the polymerization of alpha 1-antitrypsin J Biol Chem 274 1999 9548 55
-
(1999)
J Biol Chem
, vol.274
, pp. 9548-9555
-
-
Dafforn, T.R.1
Mahadeva, R.2
Elliott, P.R.3
Sivasothy, P.4
Lomas, D.A.5
-
30
-
-
0036882160
-
Acid denaturation of alpha1-antitrypsin: Characterization of a novel mechanism of serpin polymerization
-
GL Devlin, MK Chow, GJ Howlett, SP Bottomley Acid denaturation of alpha1-antitrypsin: characterization of a novel mechanism of serpin polymerization J Mol Biol 324 2002 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
-
31
-
-
0034721790
-
Pathogenic alpha 1-antitrypsin polymers are formed by reactive loop-beta-sheet a linkage
-
P Sivasothy, TR Dafforn, PG Gettins, DA Lomas Pathogenic alpha 1-antitrypsin polymers are formed by reactive loop-beta-sheet A linkage J Biol Chem 275 2000 33663 68
-
(2000)
J Biol Chem
, vol.275
, pp. 33663-33668
-
-
Sivasothy, P.1
Dafforn, T.R.2
Gettins, P.G.3
Lomas, D.A.4
-
32
-
-
0026755363
-
The mechanism of Z alpha 1-antitrypsin accumulation in the liver
-
DA Lomas, DL Evans, JT Finch, RW Carrell The mechanism of Z alpha 1-antitrypsin accumulation in the liver Nature 357 1992 605 07
-
(1992)
Nature
, vol.357
, pp. 605-607
-
-
Lomas, D.A.1
Evans, D.L.2
Finch, J.T.3
Carrell, R.W.4
-
33
-
-
0021919760
-
Elastases and emphysema: Current assessment of the protease-antiprotease hypothesis
-
A Janoff Elastases and emphysema: current assessment of the protease-antiprotease hypothesis Am Rev Respir Dis 132 1985 417 33
-
(1985)
Am Rev Respir Dis
, vol.132
, pp. 417-433
-
-
Janoff, A.1
-
34
-
-
0023546615
-
Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase
-
F Ogushi, GA Fells, RC Hubbard, SD Straus, RG Crystal Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase J Clin Invest 80 1987 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
-
35
-
-
0032065494
-
Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema
-
PR Elliott, D Bilton, DA Lomas Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema Am J Respir Cell Mol Biol 18 1998 670 74
-
(1998)
Am J Respir Cell Mol Biol
, vol.18
, pp. 670-674
-
-
Elliott, P.R.1
Bilton, D.2
Lomas, D.A.3
-
36
-
-
2442488539
-
Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant
-
AT Mulgrew, CC Taggart, MW Lawless Z alpha1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant Chest 125 2004 1952 57
-
(2004)
Chest
, vol.125
, pp. 1952-1957
-
-
Mulgrew, A.T.1
Taggart, C.C.2
Lawless, M.W.3
-
37
-
-
0036014834
-
Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: A new paradigm for the pathogenesis of emphysema
-
JS Parmar, R Mahadeva, BJ Reed Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema Am J Respir Cell Mol Biol 26 2002 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
-
38
-
-
0036606880
-
The effect of augmentation therapy on bronchial inflammation in alpha 1 antitrypsin deficiency
-
RA Stockley, DL Bayley, I Unsal, LJ Dowson The effect of augmentation therapy on bronchial inflammation in alpha 1 antitrypsin deficiency Am J Respir Crit Care Med 165 2002 1494 98
-
(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
-
39
-
-
1242306798
-
Role of human neutrophil peptides in lung inflammation associated with alpha 1-antitrypsin deficiency
-
LT Spencer, G Paone, PM Krein, FN Rouhani, J Rivera-Nieves, ML Brantly Role of human neutrophil peptides in lung inflammation associated with alpha 1-antitrypsin deficiency Am J Physiol Lung Cell Mol Physiol 286 2004 L514 20
-
(2004)
Am J Physiol Lung Cell Mol Physiol
, vol.286
, pp. 514-520
-
-
Spencer, L.T.1
Paone, G.2
Krein, P.M.3
Rouhani, F.N.4
Rivera-Nieves, J.5
Brantly, M.L.6
-
40
-
-
2342667393
-
Concentration-dependent effects of native and polymerised alpha1-antitrypsin on primary human monocytes, in vitro
-
R Aldonyte, L Jansson, S Janciauskiene Concentration-dependent effects of native and polymerised alpha1-antitrypsin on primary human monocytes, in vitro BMC Cell Biol 5 2004 11
-
(2004)
BMC Cell Biol
, vol.5
, pp. 11
-
-
Aldonyte, R.1
Jansson, L.2
Janciauskiene, S.3
-
41
-
-
4344673827
-
Inhibition of lipopolysaccharide-mediated human monocyte activation, in vitro, by alpha1-antitrypsin
-
S Janciauskiene, S Larsson, P Larsson, R Virtala, L Jansson, T Stevens Inhibition of lipopolysaccharide-mediated human monocyte activation, in vitro, by alpha1-antitrypsin Biochem Biophys Res Commun 321 2004 592 600
-
(2004)
Biochem Biophys Res Commun
, vol.321
, pp. 592-600
-
-
Janciauskiene, S.1
Larsson, S.2
Larsson, P.3
Virtala, R.4
Jansson, L.5
Stevens, T.6
-
42
-
-
0027295822
-
Alpha 1-antitrypsin Siiyama (Ser53?Phe): Further evidence for intracellular loop-sheet polymerization
-
DA Lomas, JT Finch, K Seyama, T Nukiwa, RW Carrell Alpha 1-antitrypsin Siiyama (Ser53?Phe): further evidence for intracellular loop-sheet polymerization J Biol Chem 268 1993 15333 35
-
(1993)
J Biol Chem
, vol.268
, pp. 15333-15335
-
-
Lomas, D.A.1
Finch, J.T.2
Seyama, K.3
Nukiwa, T.4
Carrell, R.W.5
-
43
-
-
0029012309
-
52-deleted) forms loop-sheet polymers in vivo: Evidence for the C sheet mechanism of polymerization
-
52-deleted) forms loop-sheet polymers in vivo: evidence for the C sheet mechanism of polymerization J Biol Chem 270 1995 16864 70
-
(1995)
J Biol Chem
, vol.270
, pp. 16864-16870
-
-
Lomas, D.A.1
Elliott, P.R.2
Sidhar, S.K.3
-
44
-
-
0029788023
-
Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity
-
D Qu, JH Teckman, S Omura, DH Perlmutter Degradation of a mutant secretory protein, alpha1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity J Biol Chem 271 1996 22791 95
-
(1996)
J Biol Chem
, vol.271
, pp. 22791-22795
-
-
Qu, D.1
Teckman, J.H.2
Omura, S.3
Perlmutter, D.H.4
-
45
-
-
0034695450
-
Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha 1 antitrypsin Z
-
NY Marcus, DH Perlmutter Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha 1 antitrypsin Z J Biol Chem 275 2000 1987 92
-
(2000)
J Biol Chem
, vol.275
, pp. 1987-1992
-
-
Marcus, N.Y.1
Perlmutter, D.H.2
-
46
-
-
0037816258
-
Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation
-
Y Wu, MT Swulius, KW Moremen, RN Sifers Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation Proc Natl Acad Sci USA 100 2003 8229 34
-
(2003)
Proc Natl Acad Sci USA
, vol.100
, pp. 8229-8234
-
-
Wu, Y.1
Swulius, M.T.2
Moremen, K.W.3
Sifers, R.N.4
-
47
-
-
0037470326
-
Cell biology: Protein degradation unlocked
-
RN Sifers Cell biology: protein degradation unlocked Science 299 2003 1330 31
-
(2003)
Science
, vol.299
, pp. 1330-1331
-
-
Sifers, R.N.1
-
48
-
-
0036295592
-
Coexpression and accumulation of ubiquitin +1 and ZZ proteins in livers of children with alpha(1)-antitrypsin deficiency
-
SS Wu, JP de Chadarevian, L McPhaul, NE Riley, FW van Leeuwen, SW French Coexpression and accumulation of ubiquitin +1 and ZZ proteins in livers of children with alpha(1)-antitrypsin deficiency Pediatr Dev Pathol 5 2002 293 98
-
(2002)
Pediatr Dev Pathol
, vol.5
, pp. 293-298
-
-
Wu, S.S.1
De Chadarevian, J.P.2
McPhaul, L.3
Riley, N.E.4
Van Leeuwen, F.W.5
French, S.W.6
-
49
-
-
10944232402
-
The bronchopulmonary pathology of alpha-1 antitrypsin (AAT) deficiency: Findings of the death review committee of the national registry for individuals with severe deficiency of alpha-1 antitrypsin
-
JF Tomashefski Jr, RG Crystal, HP Wiedemann, E Mascha, JK Stoller The bronchopulmonary pathology of alpha-1 antitrypsin (AAT) deficiency: findings of the death review committee of the national registry for individuals with severe deficiency of alpha-1 antitrypsin Hum Pathol 35 2004 1452 61
-
(2004)
Hum Pathol
, vol.35
, pp. 1452-1461
-
-
Tomashefski Jr., J.F.1
Crystal, R.G.2
Wiedemann, H.P.3
Mascha, E.4
Stoller, J.K.5
-
50
-
-
0022637178
-
Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency
-
S Eriksson, J Carlson, R Velez Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency N Engl J Med 314 1986 736 39
-
(1986)
N Engl J Med
, vol.314
, pp. 736-739
-
-
Eriksson, S.1
Carlson, J.2
Velez, R.3
-
51
-
-
0025815961
-
Alpha 1-antitrypsin deficiency and liver disease: Clinical presentation, diagnosis and treatment
-
M Hussain, G Mieli-Vergani, AP Mowat Alpha 1-antitrypsin deficiency and liver disease: clinical presentation, diagnosis and treatment J Inherit Metab Dis 14 1991 497 511
-
(1991)
J Inherit Metab Dis
, vol.14
, pp. 497-511
-
-
Hussain, M.1
Mieli-Vergani, G.2
Mowat, A.P.3
-
53
-
-
0029801844
-
Wegener's granulomatosis in a patient with severe PiZZ alpha 1-antitrypsin deficiency
-
AN Elzouki, M Segalmark, P Mazodier, S Eriksson Wegener's granulomatosis in a patient with severe PiZZ alpha 1-antitrypsin deficiency QJM 89 1996 877
-
(1996)
QJM
, vol.89
, pp. 877
-
-
Elzouki, A.N.1
Segalmark, M.2
Mazodier, P.3
Eriksson, S.4
-
54
-
-
0033228751
-
The prevalence and clinical significance of alpha 1-antitrypsin deficiency (PiZ) and ANCA specificities (proteinase 3, BPI) in patients with ulcerative colitis
-
AN Elzouki, S Eriksson, R Lofberg, L Nassberger, J Wieslander, S Lindgren The prevalence and clinical significance of alpha 1-antitrypsin deficiency (PiZ) and ANCA specificities (proteinase 3, BPI) in patients with ulcerative colitis Inflamm Bowel Dis 5 1999 246 52
-
(1999)
Inflamm Bowel Dis
, vol.5
, pp. 246-252
-
-
Elzouki, A.N.1
Eriksson, S.2
Lofberg, R.3
Nassberger, L.4
Wieslander, J.5
Lindgren, S.6
-
55
-
-
0036139399
-
Alpha-1-antitrypsin deficiency and nephropathy
-
A Montanelli, E Mainardi, L Pini, L Corda, V Grassi Alpha-1-antitrypsin deficiency and nephropathy Nephron 90 2002 114 15
-
(2002)
Nephron
, vol.90
, pp. 114-115
-
-
Montanelli, A.1
Mainardi, E.2
Pini, L.3
Corda, L.4
Grassi, V.5
-
56
-
-
0023007646
-
Could alpha 1 antitrypsin deficiency have any role in the development of celiac sprue after gastric operations?
-
RF Pons, F Casafont, dL Rodriguez, G San Miguel, E Artinano, J Cagigas Could alpha 1 antitrypsin deficiency have any role in the development of celiac sprue after gastric operations? J Clin Gastroenterol 8 1986 559 61
-
(1986)
J Clin Gastroenterol
, vol.8
, pp. 559-561
-
-
Pons, R.F.1
Casafont, F.2
Rodriguez, D.L.3
San Miguel, G.4
Artinano, E.5
Cagigas, J.6
-
57
-
-
1342287837
-
Role of imbalance between neutrophil elastase and α1-antitrypsin in cancer development and progression
-
Z Sun, P Yang Role of imbalance between neutrophil elastase and α1-antitrypsin in cancer development and progression Lancet Oncol 5 2004 182 90
-
(2004)
Lancet Oncol
, vol.5
, pp. 182-190
-
-
Sun, Z.1
Yang, P.2
-
58
-
-
0033082840
-
Is there a relationship between abdominal aortic aneurysms and alpha1-antitrypsin deficiency (PiZ)?
-
AN Elzouki, AA Ryden, T Lanne, B Sonesson, S Eriksson Is there a relationship between abdominal aortic aneurysms and alpha1-antitrypsin deficiency (PiZ)? Eur J Vasc Endovasc Surg 17 1999 149 54
-
(1999)
Eur J Vasc Endovasc Surg
, vol.17
, pp. 149-154
-
-
Elzouki, A.N.1
Ryden, A.N.2
Lanne, T.3
Sonesson, B.4
Eriksson, S.5
-
59
-
-
0033951435
-
Alpha-1-antitrypsin phenotypes in patients with renal arterial fibromuscular dysplasia
-
A Bofinger, C Hawley, P Fisher, N Daunt, M Stowasser, R Gordon Alpha-1-antitrypsin phenotypes in patients with renal arterial fibromuscular dysplasia J Hum Hypertens 14 2000 91 94
-
(2000)
J Hum Hypertens
, vol.14
, pp. 91-94
-
-
Bofinger, A.1
Hawley, C.2
Fisher, P.3
Daunt, N.4
Stowasser, M.5
Gordon, R.6
-
60
-
-
0028891940
-
Alpha 1-antitrypsin deficiency and chronic pancreatitis
-
AA Rabassa, MR Schwartz, A Ertan Alpha 1-antitrypsin deficiency and chronic pancreatitis Dig Dis Sci 40 1995 1997 2001
-
(1995)
Dig Dis Sci
, vol.40
, pp. 1997-2001
-
-
Rabassa, A.A.1
Schwartz, M.R.2
Ertan, A.3
-
61
-
-
0023795389
-
Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms
-
ML Brantly, LD Paul, BH Miller, RT Falk, M Wu, RG Crystal Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms Am Rev Respir Dis 138 1988 327 36
-
(1988)
Am Rev Respir Dis
, vol.138
, pp. 327-336
-
-
Brantly, M.L.1
Paul, L.D.2
Miller, B.H.3
Falk, R.T.4
Wu, M.5
Crystal, R.G.6
-
62
-
-
0019963490
-
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
-
P Gishen, AJ Saunders, MJ Tobin, DC Hutchison 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 77
-
(1982)
Clin Radiol
, vol.33
, pp. 371-377
-
-
Gishen, P.1
Saunders, A.J.2
Tobin, M.J.3
Hutchison, D.C.4
-
63
-
-
0031027927
-
Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency
-
NG McElvaney, JK Stoller, AS Buist Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency Chest 111 1997 394 403
-
(1997)
Chest
, vol.111
, pp. 394-403
-
-
McElvaney, N.G.1
Stoller, J.K.2
Buist, A.S.3
-
64
-
-
9644295576
-
Pattern of emphysema distribution in alpha 1-antitrypsin deficiency influences lung function impairment
-
DG Parr, BC Stoel, J Stolk, RA Stockley Pattern of emphysema distribution in alpha 1-antitrypsin deficiency influences lung function impairment Am J Respir Crit Care Med 170 2004 1172 78
-
(2004)
Am J Respir Crit Care Med
, vol.170
, pp. 1172-1178
-
-
Parr, D.G.1
Stoel, B.C.2
Stolk, J.3
Stockley, R.A.4
-
65
-
-
0022625717
-
Bronchodilator response in chronic obstructive pulmonary disease
-
NR Anthonisen, EC Wright Bronchodilator response in chronic obstructive pulmonary disease Am Rev Respir Dis 133 1986 814 19
-
(1986)
Am Rev Respir Dis
, vol.133
, pp. 814-819
-
-
Anthonisen, N.R.1
Wright, E.C.2
-
66
-
-
0018128288
-
Natural history and life expectancy in severe alpha 1-antitrypsin deficiency, Pi Z
-
C Larsson Natural history and life expectancy in severe alpha 1-antitrypsin deficiency, Pi Z Acta Med Scand 204 1978 345 51
-
(1978)
Acta Med Scand
, vol.204
, pp. 345-351
-
-
Larsson, C.1
-
68
-
-
0033995116
-
Distribution of alpha(1)-antitrypsin alleles in patients with bronchiectasis
-
A Cuvelier, JF Muir, MF Hellot Distribution of alpha(1)-antitrypsin alleles in patients with bronchiectasis Chest 117 2000 415 19
-
(2000)
Chest
, vol.117
, pp. 415-419
-
-
Cuvelier, A.1
Muir, J.F.2
Hellot, M.F.3
-
69
-
-
0141706635
-
American Thoracic Society/European Respiratory Society statement: Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency
-
American Thoracic Society/European Respiratory Society
-
American Thoracic Society/European Respiratory Society American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency Am J Respir Crit Care Med 168 2003 818 900
-
(2003)
Am J Respir Crit Care Med
, vol.168
, pp. 818-900
-
-
-
71
-
-
0030446288
-
Clinical features of individuals with PI*SZ phenotype of alpha 1-antitrypsin deficiency
-
GM Turino, AF Barker, ML Brantly Clinical features of individuals with PI*SZ phenotype of alpha 1-antitrypsin deficiency Am J Respir Crit Care Med 154 1996 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
-
72
-
-
0023218940
-
Alpha 1-antitrypsin deficiency and liver cirrhosis in adults: An analysis of 35 Swedish autopsied cases
-
S Eriksson Alpha 1-antitrypsin deficiency and liver cirrhosis in adults: an analysis of 35 Swedish autopsied cases Acta Med Scand 221 1987 461 67
-
(1987)
Acta Med Scand
, vol.221
, pp. 461-467
-
-
Eriksson, S.1
-
73
-
-
0015407888
-
[Weber-Christian syndrome associated with an alpha-1 antitrypsin deficiency. Familial investigation]
-
J Warter, D Storck, E Grosshans, P Metais, JL Kuntz, T Klumpp [Weber-Christian syndrome associated with an alpha-1 antitrypsin deficiency. Familial investigation] Ann Med Interne (Paris) 123 1972 877 82
-
(1972)
Ann Med Interne (Paris)
, vol.123
, pp. 877-882
-
-
Warter, J.1
Storck, D.2
Grosshans, E.3
Metais, P.4
Kuntz, J.L.5
Klumpp, T.6
-
74
-
-
0026326794
-
Alpha 1-antitrypsin deficiency-associated panniculitis: Case report and review of the literature
-
BK Edmonds, JA Hodge, RL Rietschel Alpha 1-antitrypsin deficiency-associated panniculitis: case report and review of the literature Pediatr Dermatol 8 1991 296 99
-
(1991)
Pediatr Dermatol
, vol.8
, pp. 296-299
-
-
Edmonds, B.K.1
Hodge, J.A.2
Rietschel, R.L.3
-
75
-
-
0035724197
-
Suppurative panniculitis associated with alpha 1-antitrypsin deficiency (PiSZ phenotype) treated with doxycycline
-
WJ Chng, CA Henderson Suppurative panniculitis associated with alpha 1-antitrypsin deficiency (PiSZ phenotype) treated with doxycycline Br J Dermatol 144 2001 1282 83
-
(2001)
Br J Dermatol
, vol.144
, pp. 1282-1283
-
-
Chng, W.J.1
Henderson, C.A.2
-
76
-
-
0027528997
-
Systemic nodular panniculitis in a patient with alpha-1 antitrypsin deficiency (PiSS phenotype)
-
AR Pinto, LS Maciel, F Carneiro, C Resende, FC Chaves, AF Freitas Systemic nodular panniculitis in a patient with alpha-1 antitrypsin deficiency (PiSS phenotype) Clin Exp Dermatol 18 1993 154 55
-
(1993)
Clin Exp Dermatol
, vol.18
, pp. 154-155
-
-
Pinto, A.R.1
MacIel, L.S.2
Carneiro, F.3
Resende, C.4
Chaves, F.C.5
Freitas, A.F.6
-
77
-
-
0032697554
-
Panniculitis revealing qualitative alpha 1 antitrypsin deficiency (MS variant)
-
F Loche, C Tremeau-Martinage, G Laplanche, P Massip, J Bazex Panniculitis revealing qualitative alpha 1 antitrypsin deficiency (MS variant) Eur J Dermatol 9 1999 565 67
-
(1999)
Eur J Dermatol
, vol.9
, pp. 565-567
-
-
Loche, F.1
Tremeau-Martinage, C.2
Laplanche, G.3
Massip, P.4
Bazex, J.5
-
78
-
-
0031028798
-
Alpha 1-antitrypsin deficiency-associated panniculitis: Resolution with intravenous alpha 1-antitrypsin administration and liver transplantation
-
K O'Riordan, A Blei, MS Rao, M Abecassis alpha 1-antitrypsin deficiency-associated panniculitis: resolution with intravenous alpha 1-antitrypsin administration and liver transplantation Transplantation 63 1997 480 82
-
(1997)
Transplantation
, vol.63
, pp. 480-482
-
-
O'Riordan, K.1
Blei, A.2
Rao, M.S.3
Abecassis, M.4
-
79
-
-
0036887247
-
Severe panniculitis caused by homozygous ZZ alpha 1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin)
-
MM Chowdhury, EJ Williams, JS Morris Severe panniculitis caused by homozygous ZZ alpha 1-antitrypsin deficiency treated successfully with human purified enzyme (Prolastin) Br J Dermatol 147 2002 1258 61
-
(2002)
Br J Dermatol
, vol.147
, pp. 1258-1261
-
-
Chowdhury, M.M.1
Williams, E.J.2
Morris, J.S.3
-
80
-
-
0027336667
-
Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis
-
VL Esnault, A Testa, M Audrain Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis Kidney Int 43 1993 1329 32
-
(1993)
Kidney Int
, vol.43
, pp. 1329-1332
-
-
Esnault, V.L.1
Testa, A.2
Audrain, M.3
-
81
-
-
0032110436
-
Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin
-
Alpha-1-Antitrypsin Deficiency Registry Study Group
-
Alpha-1-Antitrypsin Deficiency Registry Study Group Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin Am J Respir Crit Care Med 158 1998 49 59
-
(1998)
Am J Respir Crit Care Med
, vol.158
, pp. 49-59
-
-
-
82
-
-
0003078086
-
Longitudinal studies in alpha-1 antitrypsin deficiency: A survey by the British Thoracic Society
-
J.C. Taylor C. Mittman Academic Press Orlando
-
DC Hutchison, MJ Tobin, D Cooper Longitudinal studies in alpha-1 antitrypsin deficiency: a survey by the British Thoracic Society JC Taylor C Mittman Pulmonary emphysema and proteolysis 1987 Academic Press Orlando
-
(1987)
Pulmonary Emphysema and Proteolysis
-
-
Hutchison, D.C.1
Tobin, M.J.2
Cooper, D.3
-
84
-
-
0032586927
-
Decline in FEV1 related to smoking status in individuals with severe alpha1-antitrypsin deficiency (PiZZ)
-
E Piitulainen, S Eriksson Decline in FEV1 related to smoking status in individuals with severe alpha1-antitrypsin deficiency (PiZZ) Eur Respir J 13 1999 247 51
-
(1999)
Eur Respir J
, vol.13
, pp. 247-251
-
-
Piitulainen, E.1
Eriksson, S.2
-
85
-
-
0031242842
-
Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency?
-
N Seersholm, M Wencker, N Banik Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Eur Respir J 10 1997 2260 63
-
(1997)
Eur Respir J
, vol.10
, pp. 2260-2263
-
-
Seersholm, N.1
Wencker, M.2
Banik, N.3
-
86
-
-
0024211686
-
Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ
-
MC Wu, S Eriksson Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ J Clin Epidemiol 41 1988 1157 65
-
(1988)
J Clin Epidemiol
, vol.41
, pp. 1157-1165
-
-
Wu, M.C.1
Eriksson, S.2
-
87
-
-
0028811087
-
Decline in FEV1 among patients with severe hereditary alpha 1-antitrypsin deficiency type PiZ
-
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 1922 25
-
(1995)
Am J Respir Crit Care Med
, vol.152
, pp. 1922-1925
-
-
Seersholm, N.1
Kok-Jensen, A.2
Dirksen, A.3
-
89
-
-
0028006653
-
Airways obstruction and two year survival in patients with severe alpha 1-antitrypsin deficiency
-
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 1985 87
-
(1994)
Eur Respir J
, vol.7
, pp. 1985-1987
-
-
Seersholm, N.1
Dirksen, A.2
Kok-Jensen, A.3
-
90
-
-
0037187951
-
Clinical practice: Acute exacerbations of chronic obstructive pulmonary disease
-
JK Stoller Clinical practice: acute exacerbations of chronic obstructive pulmonary disease N Engl J Med 346 2002 988 94
-
(2002)
N Engl J Med
, vol.346
, pp. 988-994
-
-
Stoller, J.K.1
-
91
-
-
2942729608
-
Management of chronic obstructive pulmonary disease
-
ER Sutherland, RM Cherniack Management of chronic obstructive pulmonary disease N Engl J Med 350 2004 2689 97
-
(2004)
N Engl J Med
, vol.350
, pp. 2689-2697
-
-
Sutherland, E.R.1
Cherniack, R.M.2
-
92
-
-
0038075468
-
A randomized trial comparing lung-volume-reduction surgery with medical therapy for severe emphysema
-
A Fishman, F Martinez, K Naunheim A randomized trial comparing lung-volume-reduction surgery with medical therapy for severe emphysema N Engl J Med 348 2003 2059 73
-
(2003)
N Engl J Med
, vol.348
, pp. 2059-2073
-
-
Fishman, A.1
Martinez, F.2
Naunheim, K.3
-
93
-
-
4444268460
-
Long-term results after lung volume reduction surgery in patients with alpha 1-antitrypsin deficiency
-
M Tutic, KE Bloch, D Lardinois, T Brack, EW Russi, W Weder Long-term results after lung volume reduction surgery in patients with alpha 1-antitrypsin deficiency J Thorac Cardiovasc Surg 128 2004 408 13
-
(2004)
J Thorac Cardiovasc Surg
, vol.128
, pp. 408-413
-
-
Tutic, M.1
Bloch, K.E.2
Lardinois, D.3
Brack, T.4
Russi, E.W.5
Weder, W.6
-
94
-
-
0023148995
-
Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema
-
MD Wewers, MA Casolaro, SE Sellers Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema N Engl J Med 316 1987 1055 62
-
(1987)
N Engl J Med
, vol.316
, pp. 1055-1062
-
-
Wewers, M.D.1
Casolaro, M.A.2
Sellers, S.E.3
-
95
-
-
0030610568
-
Pharmacokinetic study of alpha 1-antitrypsin infusion in alpha1-antitrypsin deficiency
-
AF Barker, I Iwata-Morgan, L Oveson, R Roussel Pharmacokinetic study of alpha 1-antitrypsin infusion in alpha1-antitrypsin deficiency Chest 112 1997 607 13
-
(1997)
Chest
, vol.112
, pp. 607-613
-
-
Barker, A.F.1
Iwata-Morgan, I.2
Oveson, L.3
Roussel, R.4
-
96
-
-
0023784904
-
Biochemical efficacy and safety of monthly augmentation therapy for alpha 1-antitrypsin deficiency
-
RC Hubbard, S Sellers, D Czerski, L Stephens, RG Crystal Biochemical efficacy and safety of monthly augmentation therapy for alpha 1-antitrypsin deficiency JAMA 260 1988 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
-
97
-
-
0032724157
-
A randomized clinical trial of alpha(1)-antitrypsin augmentation therapy
-
A Dirksen, JH Dijkman, F Madsen A randomized clinical trial of alpha(1)-antitrypsin augmentation therapy Am J Respir Crit Care Med 160 1999 1468 72
-
(1999)
Am J Respir Crit Care Med
, vol.160
, pp. 1468-1472
-
-
Dirksen, A.1
Dijkman, J.H.2
Madsen, F.3
-
98
-
-
0034523704
-
Short-term supplementation therapy does not affect elastin degradation in severe alpha(1)-antitrypsin deficiency
-
DJ Gottlieb, M Luisetti, PJ Stone Short-term supplementation therapy does not affect elastin degradation in severe alpha(1)-antitrypsin deficiency Am J Respir Crit Care Med 162 2000 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
-
99
-
-
0035095548
-
Longitudinal follow-up of patients with alpha(1)-protease inhibitor deficiency before and during therapy with IV alpha(1)-protease inhibitor
-
M Wencker, B Fuhrmann, N Banik, N Konietzko Longitudinal follow-up of patients with alpha(1)-protease inhibitor deficiency before and during therapy with IV alpha(1)-protease inhibitor Chest 119 2001 737 44
-
(2001)
Chest
, vol.119
, pp. 737-744
-
-
Wencker, M.1
Fuhrmann, B.2
Banik, N.3
Konietzko, N.4
-
100
-
-
0033739182
-
Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: A new hypothesis with supporting data
-
J Lieberman Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data Chest 118 2000 1480 85
-
(2000)
Chest
, vol.118
, pp. 1480-1485
-
-
Lieberman, J.1
-
101
-
-
0028916761
-
Preliminary evidence that augmentation therapy diminishes degradation of cross-linked elastin in alpha-1-antitrypsin-deficient humans
-
PJ Stone, TA Morris III, C Franzblau, GL Snider Preliminary evidence that augmentation therapy diminishes degradation of cross-linked elastin in alpha-1-antitrypsin-deficient humans Respiration 62 1995 76 79
-
(1995)
Respiration
, vol.62
, pp. 76-79
-
-
Stone, P.J.1
Morris III, T.A.2
Franzblau, C.3
Snider, G.L.4
-
102
-
-
0034743745
-
Alpha 1-antitrypsin deficiency: A position statement of the Canadian Thoracic Society
-
RT Abboud, GT Ford, KR Chapman Alpha 1-antitrypsin deficiency: a position statement of the Canadian Thoracic Society Can Respir J 8 2001 81 88
-
(2001)
Can Respir J
, vol.8
, pp. 81-88
-
-
Abboud, R.T.1
Ford, G.T.2
Chapman, K.R.3
-
103
-
-
0038216603
-
Augmentation therapy with alpha-1 antitrypsin: Patterns of use and adverse events
-
JK Stoller, R Fallat, MD Schluchter Augmentation therapy with alpha-1 antitrypsin: patterns of use and adverse events Chest 123 2003 1425 34
-
(2003)
Chest
, vol.123
, pp. 1425-1434
-
-
Stoller, J.K.1
Fallat, R.2
Schluchter, M.D.3
-
104
-
-
0031884479
-
Long-term treatment of alpha 1-antitrypsin deficiency-related pulmonary emphysema with human alpha 1-antitrypsin
-
M Wencker, N Banik, R Buhl, R Seidel, N Konietzko Long-term treatment of alpha 1-antitrypsin deficiency-related pulmonary emphysema with human alpha 1-antitrypsin Eur Respir J 11 1998 428 33
-
(1998)
Eur Respir J
, vol.11
, pp. 428-433
-
-
Wencker, M.1
Banik, N.2
Buhl, R.3
Seidel, R.4
Konietzko, N.5
-
105
-
-
0036301645
-
Biochemical efficacy and safety of a new pooled human plasma alpha(1)-antitrypsin, Respitin
-
JK Stoller, F Rouhani, M Brantly Biochemical efficacy and safety of a new pooled human plasma alpha(1)-antitrypsin, Respitin Chest 122 2002 66 74
-
(2002)
Chest
, vol.122
, pp. 66-74
-
-
Stoller, J.K.1
Rouhani, F.2
Brantly, M.3
-
106
-
-
0034017941
-
Should health-care systems pay for replacement therapy in patients with alpha(1)-antitrypsin deficiency? a critical review and cost-effectiveness analysis
-
SA Alkins, P O'Malley Should health-care systems pay for replacement therapy in patients with alpha(1)-antitrypsin deficiency? A critical review and cost-effectiveness analysis Chest 117 2000 875 80
-
(2000)
Chest
, vol.117
, pp. 875-880
-
-
Alkins, S.A.1
O'Malley, P.2
-
107
-
-
0037952677
-
Cost-effectiveness analysis of augmentation therapy for severe alpha-1 antitrypsin deficiency
-
TR Gildea, KM Shermock, ME Singer, JK Stoller Cost-effectiveness analysis of augmentation therapy for severe alpha-1 antitrypsin deficiency Am J Respir Crit Care Med 167 2003 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
-
108
-
-
0025890293
-
Cost-effectiveness of alpha-1 antitrypsin replacement therapy in treatment of congenital chronic obstructive pulmonary disease
-
JW Hay, ED Robin Cost-effectiveness of alpha-1 antitrypsin replacement therapy in treatment of congenital chronic obstructive pulmonary disease Am J Public Health 81 1991 427 33
-
(1991)
Am J Public Health
, vol.81
, pp. 427-433
-
-
Hay, J.W.1
Robin, E.D.2
-
109
-
-
0027941322
-
A registry of patients with severe deficiency of alpha 1-antitrypsin: Design and methods
-
The Alpha-1 Antitrypsin Deficiency Registry Study Group
-
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 1223 32
-
(1994)
Chest
, vol.106
, pp. 1223-1232
-
-
-
110
-
-
0742307402
-
Phase I trial of intramuscular injection of a recombinant adeno-associated virus alpha 1-antitrypsin (rAAV2-CB-hAAT) gene vector to AAT-deficient adults
-
TR Flotte, ML Brantly, LT Spencer Phase I trial of intramuscular injection of a recombinant adeno-associated virus alpha 1-antitrypsin (rAAV2-CB-hAAT) gene vector to AAT-deficient adults Hum Gene Ther 15 2004 93 128
-
(2004)
Hum Gene Ther
, vol.15
, pp. 93-128
-
-
Flotte, T.R.1
Brantly, M.L.2
Spencer, L.T.3
-
111
-
-
4143133190
-
Inhibiting polymerization: New therapeutic strategies for Z alpha 1 antitrypsin-related emphysema
-
H Parfrey, TR Dafforn, D Belorgey, DA Lomas, R Mahadeva Inhibiting polymerization: new therapeutic strategies for Z alpha 1 antitrypsin-related emphysema Am J Respir Cell Mol Biol 31 2004 133 39
-
(2004)
Am J Respir Cell Mol Biol
, vol.31
, pp. 133-139
-
-
Parfrey, H.1
Dafforn, T.R.2
Belorgey, D.3
Lomas, D.A.4
Mahadeva, R.5
-
112
-
-
4444332086
-
How small peptides block and reverse serpin polymerisation
-
A Zhou, PE Stein, JA Huntington, P Sivasothy, DA Lomas, RW Carrell How small peptides block and reverse serpin polymerisation J Mol Biol 342 2004 931 41
-
(2004)
J Mol Biol
, vol.342
, pp. 931-941
-
-
Zhou, A.1
Stein, P.E.2
Huntington, J.A.3
Sivasothy, P.4
Lomas, D.A.5
Carrell, R.W.6
-
113
-
-
0034652248
-
Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency
-
JA Burrows, LK Willis, DH Perlmutter Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: a potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency Proc Natl Acad Sci USA 97 2000 1796 801
-
(2000)
Proc Natl Acad Sci USA
, vol.97
, pp. 1796-1801
-
-
Burrows, J.A.1
Willis L.K.Perlmutter, D.H.2
-
114
-
-
0036891151
-
Polyethylene glycol conjugation at Cys232 prolongs the half-life of alpha 1 proteinase inhibitor
-
AM Cantin, DE Woods, D Cloutier, EK Dufour, R Leduc Polyethylene glycol conjugation at Cys232 prolongs the half-life of alpha 1 proteinase inhibitor Am J Respir Cell Mol Biol 27 2002 659 65
-
(2002)
Am J Respir Cell Mol Biol
, vol.27
, pp. 659-665
-
-
Cantin, A.M.1
Woods, D.E.2
Cloutier, D.3
Dufour, E.K.4
Leduc, R.5
|