Portuguese consensus document for the management of alpha-1-antitrypsin deficiency

Alpha-1-antitrypsin deficiency (AATD) is a genetic autosomal codominant disorder caused by mutations in SERPINA1 gene. It is one of the most prevalent genetic disorders, although it remains underdiagnosed. Whereas at international level there are several areas of consensus on this disorder, in Portu...

Full description

Saved in:
Bibliographic Details
Published inPulmonology Vol. 24; pp. 1 - 21
Main Authors Lopes, A.P., Mineiro, M.A., Costa, F., Gomes, J., Santos, C., Antunes, C., Maia, D., Melo, R., Canotilho, M., Magalhães, E., Vicente, I., Valente, C., Gonçalves, B.G., Conde, B., Guimarães, C., Sousa, C., Amado, J., Brandão, M.E., Sucena, M., Oliveira, M.J., Seixas, S., Teixeira, V., Telo, L.
Format Journal Article Conference Proceeding
LanguageEnglish
Published Spain Elsevier España, S.L.U 01.12.2018
Elsevier España
Subjects
Algorithms
Alpha 1-Antitrypsin Deficiency
alpha 1-Antitrypsin Deficiency - complications
alpha 1-Antitrypsin Deficiency - diagnosis
alpha 1-Antitrypsin Deficiency - therapy
Consensus
Diagnosis
Humans
Lung Diseases - diagnosis
Lung Diseases - etiology
Lung Diseases - therapy
Patient Selection
Portugal
Practice Guidelines as Topic
Pulmonary Emphysema
Therapeutics
Portugal
ATS
FDA
IgA
SERPINA1
Alpha 1-Antitrypsin Deficiency
RV
ERS
Pulmonary Emphysema
ALT
TLC
HU
MPO
AATD
BODE
DBS
DLCO
HRQoL
LTB4
Diagnosis
SEPAR
AST
IEF
EDTA
ER
Consensus
.
PR3
CT
FVC
AAT
HIV
IL8
NHLBI
GGT
URTI
FRC
PD10
Therapeutics
NE
FEV1
RCL
c-ANCA
PCR
COPD
Online AccessGet full text

Cover

Abstract Alpha-1-antitrypsin deficiency (AATD) is a genetic autosomal codominant disorder caused by mutations in SERPINA1 gene. It is one of the most prevalent genetic disorders, although it remains underdiagnosed. Whereas at international level there are several areas of consensus on this disorder, in Portugal, inter-hospital heterogeneity in clinical practice and resources available have been adding difficulties in reaching a diagnosis and in making therapeutic decisions in this group of patients. This raised a need to draft a document expressing a national consensus for AATD. To this end, a group of experts in this field was created within the Portuguese Pulmonology Society - Study group on AATD, in order to elaborate the current manuscript. The authors reviewed the existing literature and provide here general guidance and extensive recommendations for the diagnosis and management of AATD that can be adopted by Portuguese clinicians from different areas of Medicine. This article is part of a supplement entitled “Portuguese consensus document for the management of alpha-1-antitrypsin deficiency” which is sponsored by Sociedade Portuguesa de Pneumologia.
AbstractList Alpha-1-antitrypsin deficiency (AATD) is a genetic autosomal codominant disorder caused by mutations in SERPINA1 gene. It is one of the most prevalent genetic disorders, although it remains underdiagnosed. Whereas at international level there are several areas of consensus on this disorder, in Portugal, inter-hospital heterogeneity in clinical practice and resources available have been adding difficulties in reaching a diagnosis and in making therapeutic decisions in this group of patients. This raised a need to draft a document expressing a national consensus for AATD. To this end, a group of experts in this field was created within the Portuguese Pulmonology Society - Study group on AATD, in order to elaborate the current manuscript. The authors reviewed the existing literature and provide here general guidance and extensive recommendations for the diagnosis and management of AATD that can be adopted by Portuguese clinicians from different areas of Medicine. This article is part of a supplement entitled "Portuguese consensus document for the management of alpha-1-antitrypsin deficiency" which is sponsored by Sociedade Portuguesa de Pneumologia.
Alpha-1-antitrypsin deficiency (AATD) is a genetic autosomal codominant disorder caused by mutations in SERPINA1 gene. It is one of the most prevalent genetic disorders, although it remains underdiagnosed. Whereas at international level there are several areas of consensus on this disorder, in Portugal, inter-hospital heterogeneity in clinical practice and resources available have been adding difficulties in reaching a diagnosis and in making therapeutic decisions in this group of patients. This raised a need to draft a document expressing a national consensus for AATD. To this end, a group of experts in this field was created within the Portuguese Pulmonology Society - Study group on AATD, in order to elaborate the current manuscript. The authors reviewed the existing literature and provide here general guidance and extensive recommendations for the diagnosis and management of AATD that can be adopted by Portuguese clinicians from different areas of Medicine.This article is part of a supplement entitled “Portuguese consensus document for the management of alpha-1-antitrypsin deficiency” which is sponsored by Sociedade Portuguesa de Pneumologia. Keywords: Alpha 1-Antitrypsin Deficiency, Consensus, Diagnosis, Therapeutics, Pulmonary Emphysema
Alpha-1-antitrypsin deficiency (AATD) is a genetic autosomal codominant disorder caused by mutations in SERPINA1 gene. It is one of the most prevalent genetic disorders, although it remains underdiagnosed. Whereas at international level there are several areas of consensus on this disorder, in Portugal, inter-hospital heterogeneity in clinical practice and resources available have been adding difficulties in reaching a diagnosis and in making therapeutic decisions in this group of patients. This raised a need to draft a document expressing a national consensus for AATD. To this end, a group of experts in this field was created within the Portuguese Pulmonology Society - Study group on AATD, in order to elaborate the current manuscript. The authors reviewed the existing literature and provide here general guidance and extensive recommendations for the diagnosis and management of AATD that can be adopted by Portuguese clinicians from different areas of Medicine. This article is part of a supplement entitled “Portuguese consensus document for the management of alpha-1-antitrypsin deficiency” which is sponsored by Sociedade Portuguesa de Pneumologia.
Author Melo, R.
Conde, B.
Costa, F.
Maia, D.
Vicente, I.
Seixas, S.
Canotilho, M.
Amado, J.
Sucena, M.
Gomes, J.
Teixeira, V.
Sousa, C.
Santos, C.
Antunes, C.
Magalhães, E.
Gonçalves, B.G.
Valente, C.
Guimarães, C.
Lopes, A.P.
Mineiro, M.A.
Brandão, M.E.
Oliveira, M.J.
Telo, L.
Author_xml – sequence: 1
  givenname: A.P.
  surname: Lopes
  fullname: Lopes, A.P.
  email: plopeshuc@hotmail.com
  organization: Centro Hospitalar e Universitário de Coimbra (HUC)
– sequence: 2
  givenname: M.A.
  surname: Mineiro
  fullname: Mineiro, M.A.
  organization: Centro Hospital Lisboa Central
– sequence: 3
  givenname: F.
  surname: Costa
  fullname: Costa, F.
  organization: Centro Hospitalar e Universitário de Coimbra (HG)
– sequence: 4
  givenname: J.
  surname: Gomes
  fullname: Gomes, J.
  organization: Centro Hospitalar do Porto
– sequence: 5
  givenname: C.
  surname: Santos
  fullname: Santos, C.
  organization: Centro Hospitalar Lisboa Norte
– sequence: 6
  givenname: C.
  surname: Antunes
  fullname: Antunes, C.
  organization: Centro Hospitalar Lisboa Norte
– sequence: 7
  givenname: D.
  surname: Maia
  fullname: Maia, D.
  organization: Centro Hospital Lisboa Central
– sequence: 8
  givenname: R.
  surname: Melo
  fullname: Melo, R.
  organization: Hospital Prof. Doutor Fernando da Fonseca
– sequence: 9
  givenname: M.
  surname: Canotilho
  fullname: Canotilho, M.
  organization: Centro Hospitalar de Leiria
– sequence: 10
  givenname: E.
  surname: Magalhães
  fullname: Magalhães, E.
  organization: Centro Hospitalar da Cova da Beira
– sequence: 11
  givenname: I.
  surname: Vicente
  fullname: Vicente, I.
  organization: Centro Hospitalar da Cova da Beira
– sequence: 12
  givenname: C.
  surname: Valente
  fullname: Valente, C.
  organization: Centro Hospitalar do Baixo Vouga
– sequence: 13
  givenname: B.G.
  surname: Gonçalves
  fullname: Gonçalves, B.G.
  organization: Centro Hospitalar e Universitário de Coimbra (HUC)
– sequence: 14
  givenname: B.
  surname: Conde
  fullname: Conde, B.
  organization: Centro Hospitalar de Trás os Montes e Alto Douro
– sequence: 15
  givenname: C.
  surname: Guimarães
  fullname: Guimarães, C.
  organization: Hospital da Senhora da Oliveira – Guimarães
– sequence: 16
  givenname: C.
  surname: Sousa
  fullname: Sousa, C.
  organization: Centro Hospitalar de São João
– sequence: 17
  givenname: J.
  surname: Amado
  fullname: Amado, J.
  organization: Unidade Local de Saúde de Matosinhos
– sequence: 18
  givenname: M.E.
  surname: Brandão
  fullname: Brandão, M.E.
  organization: Centro Hospitalar de Trás os Montes e Alto Douro
– sequence: 19
  givenname: M.
  surname: Sucena
  fullname: Sucena, M.
  organization: Centro Hospitalar de São João
– sequence: 20
  givenname: M.J.
  surname: Oliveira
  fullname: Oliveira, M.J.
  organization: Centro Hospital de Gaia-Espinho
– sequence: 21
  givenname: S.
  surname: Seixas
  fullname: Seixas, S.
  organization: Instituto de Investigação e Inovação em Saúde, Universidade do Porto (I3S)
– sequence: 22
  givenname: V.
  surname: Teixeira
  fullname: Teixeira, V.
  organization: Serviço de Saúde da Região Autónoma da Madeira (SESARAM)
– sequence: 23
  givenname: L.
  surname: Telo
  fullname: Telo, L.
  organization: Centro Hospitalar Lisboa Norte
BackLink https://www.ncbi.nlm.nih.gov/pubmed/30473034$$D View this record in MEDLINE/PubMed
BookMark eNp9UctqHDEQFMEhfsR_EMIcc5lJ6zEPXQLB5GEwJITkLDRSa61lRppIM4H9-8i7tskp0KCmqa5qVV2SsxADEvKGQkOBdu_3zbJNc8SGAR0akA2AeEEuWMtpDYL3Z__05-Q65z0AsFJ8oK_IOQfRc-Digvz4HtO67TbMWJkYMoa85cpGs80Y1srFVK33WM066B0eR9FVelrudU1rHVa_psOSfagsOm88BnN4TV46PWW8fnyvyK_Pn37efK3vvn25vfl4VxtBO1GbXra9RscFyn6wgGAkZZIb1lNundPlXuf6QUtrunFkgiEf2oFJFHSgHfIrcnvitVHv1ZL8rNNBRe3VcRDTTum0ejOh4iMUUcap5J1wnR67FqjroLXCWTl2hevdiWtJ8XcxY1WzzwanSQeMW1aM8gHaQjAUqDhBTYo5J3TP0hTUQziqHHMMRz2Eo0CqEk5Ze_uosI0z2uelpygK4MMJgMWzPx6Tykc_0fqEZi2f8v9X-AtyI6L0
CitedBy_id crossref_primary_10_1016_j_opresp_2023_100251
crossref_primary_10_3390_jcm9082526
crossref_primary_10_1016_j_lpm_2023_104170
crossref_primary_10_1007_s40267_019_00648_2
crossref_primary_10_1016_j_pulmoe_2020_06_013
crossref_primary_10_1016_j_pulmoe_2023_08_005
crossref_primary_10_1159_000528809
crossref_primary_10_1016_j_pulmoe_2020_02_003
crossref_primary_10_1016_j_pulmoe_2020_04_016
crossref_primary_10_2147_TACG_S257511
crossref_primary_10_1136_bcr_2020_240288
crossref_primary_10_1183_16000617_0170_2023
crossref_primary_10_1186_s12887_022_03290_6
crossref_primary_10_2147_COPD_S346051
crossref_primary_10_1016_j_pulmoe_2020_05_014
crossref_primary_10_1016_j_pulmoe_2022_07_012
crossref_primary_10_3390_jcm10081546
crossref_primary_10_1016_j_pulmoe_2022_01_017
crossref_primary_10_1016_j_pulmoe_2020_01_003
crossref_primary_10_3390_biomedicines10123248
crossref_primary_10_2147_COPD_S330817
crossref_primary_10_20344_amp_18497
Cites_doi 10.1038/nsb0295-96
10.1590/S1806-37132008000700012
10.3109/00365529008999216
10.1164/ajrccm.154.6.8970361
10.1042/cs0870693
10.1146/annurev.biochem.78.082107.133320
10.1136/thx.2004.023572
10.1136/thx.53.4.265
10.1378/chest.118.5.1480
10.3109/00365520903296669
10.1136/thx.2005.057943
10.1378/chest.123.5.1435
10.1378/chest.117.2.415
10.1186/1465-9921-11-136
10.1378/chest.128.3.1179
10.1183/09031936.02.02012001
10.1378/chest.123.5.1425
10.1016/j.rmed.2005.06.009
10.1164/rccm.200406-761OC
10.1007/s00005-012-0162-5
10.3949/ccjm.61.6.461
10.1157/13095974
10.1111/j.0954-6820.1978.tb08452.x
10.1136/thx.2004.022541
10.1164/ajrccm.156.1.9606093
10.1183/13993003.00610-2017
10.1136/thorax.57.8.705
10.1016/j.rmed.2013.04.023
10.1378/chest.128.4.1989
10.1056/NEJM198603203141202
10.1016/j.rmed.2009.04.012
10.1111/j.1651-2227.1998.tb00918.x
10.1164/rccm.2109013
10.3109/00365528509088831
10.1159/000196395
10.1378/chest.10-0746
10.1097/00042737-199610000-00010
10.1038/nsb1196-910
10.1074/jbc.274.14.9548
10.1183/09031936.97.10102260
10.1002/hep.510280421
10.1016/j.healun.2014.06.014
10.1164/rccm.200703-489OC
10.1378/chest.111.2.394
10.3109/15412555.2013.771956
10.1164/ajrccm.160.5.9901055
10.1183/09031936.98.11020428
10.1016/j.cgh.2011.12.028
10.1183/09031936.00061208
10.3109/15412555.2012.719049
10.1164/rccm.200403-326OC
10.1016/0007-0971(83)90002-5
10.1074/jbc.275.3.1987
10.1183/09059180.10010714
10.1016/j.athoracsur.2006.07.080
10.7326/0003-4819-111-12-982
10.1183/09031936.05.00074704
10.1034/j.1399-3003.2000.01521.x
10.1183/09031936.99.13224799
10.1016/j.arbres.2014.05.008
10.3109/15412555.2013.771163
10.1172/JCI110360
10.1136/thx.2006.075846
10.1016/j.chest.2017.11.017
10.1016/S0954-6111(99)90091-4
10.1177/1753465810373911
10.1183/09031936.00087908
10.1136/thx.2003.006510
10.1016/S0002-9440(10)62261-4
10.1056/NEJM197606102942404
10.1164/rccm.201204-0739OC
10.1172/JCI114578
10.1016/S0140-6736(15)60860-1
10.1016/S0954-6111(99)90003-3
10.1155/2012/920918
10.1183/09031936.94.07111985
10.1183/09031936.00069709
10.1378/chest.112.3.607
10.1016/S0168-8278(00)80119-1
10.1172/JCI113214
10.1136/thoraxjnl-2011-201321
10.1378/chest.122.5.1818
10.1136/thx.2003.006502
10.3109/00365528509088844
10.1101/gr.GR-1478R
10.1136/thoraxjnl-2015-206906
10.1164/rccm.201108-1428CI
10.1378/chest.10-0187
10.1177/1753465812438387
10.1001/jama.1988.03410090091037
10.1056/NEJM198103053041001
10.1183/09031936.06.00062305
10.1513/AnnalsATS.201507-437KV
10.1164/rccm.201311-1984OC
10.1016/j.rmed.2011.02.002
10.1007/978-1-4757-9182-2_54
10.1183/09031936.05.00007305
10.1172/JCI6092
10.2741/1660
10.1164/rccm.168.7.818
10.1056/NEJM198704233161704
10.1136/oem.2009.047589
10.1007/s11748-012-0015-5
10.1186/1750-1172-8-149
10.1080/15412550902905961
10.1016/j.arbres.2016.05.024
10.1038/nrdp.2016.51
10.1007/BF01797920
10.1183/09059180.10010814
10.1157/13089541
10.1183/09031936.00104712
10.1016/S0140-6736(85)91916-6
10.3109/15412555.2015.1021913
10.2147/TCRM.S51474
10.1164/ajrccm.162.2.9907117
10.1016/S0140-6736(05)66781-5
10.1016/j.ccm.2016.04.011
10.1016/j.rmed.2016.05.002
10.1159/000196336
10.1097/01.mpg.0000226387.56612.1e
10.1016/j.trsl.2007.08.001
10.1159/000202982
10.1183/09031936.00019711
10.1164/rccm.200209-1035OC
10.3357/ASEM.2224.2008
10.1172/JCI4874
10.1007/s004390000434
10.1309/44J4KBCFQ8E9D1B8
10.1016/j.healun.2013.08.007
10.1378/chest.118.3.867
10.1038/ki.1993.186
10.1016/S0009-9260(05)80011-X
10.1016/S1579-2129(06)60583-1
10.1021/bi00053a014
10.1002/14651858.CD007851.pub2
10.3109/15412555.2015.1021909
10.1586/ers.13.20
10.1373/clinchem.2006.072991
10.1164/ajrccm.158.1.9712017
10.1164/ajrccm.156.1.9508014
10.1016/j.molmed.2013.10.007
10.1503/cmaj.111749
10.1056/NEJMoa0900928
10.1378/chest.119.3.737
10.1016/0895-4356(88)90019-4
10.1002/hep.21009
10.1111/joim.12239
10.2353/jmoldx.2007.060048
10.15326/jcopdf.3.3.2015.0182
10.1056/NEJM198309223091203
10.1172/JCI0216782
10.1007/978-94-007-4549-0_5
10.1183/09031936.00159408
10.2147/COPD.S8577
ContentType Journal Article
Conference Proceeding
Copyright 2018
Copyright © 2018. Published by Elsevier España, S.L.U.
Copyright_xml – notice: 2018
– notice: Copyright © 2018. Published by Elsevier España, S.L.U.
DBID 6I.
AAFTH
CGR
CUY
CVF
ECM
EIF
NPM
AAYXX
CITATION
7X8
DOA
DOI 10.1016/j.pulmoe.2018.09.004
DatabaseName ScienceDirect Open Access Titles
Elsevier:ScienceDirect:Open Access
Medline
MEDLINE
MEDLINE (Ovid)
MEDLINE
MEDLINE
PubMed
CrossRef
MEDLINE - Academic
Directory of Open Access Journals
DatabaseTitle MEDLINE
Medline Complete
MEDLINE with Full Text
PubMed
MEDLINE (Ovid)
CrossRef
MEDLINE - Academic
DatabaseTitleList MEDLINE
MEDLINE - Academic
Database_xml – sequence: 1
  dbid: DOA
  name: Directory of Open Access Journals
  url: https://www.doaj.org/
  sourceTypes: Open Website
– sequence: 2
  dbid: NPM
  name: PubMed
  url: https://proxy.k.utb.cz/login?url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed
  sourceTypes: Index Database
– sequence: 3
  dbid: EIF
  name: MEDLINE
  url: https://proxy.k.utb.cz/login?url=https://www.webofscience.com/wos/medline/basic-search
  sourceTypes: Index Database
DeliveryMethod fulltext_linktorsrc
Discipline Medicine
EISSN 2531-0437
EndPage 21
ExternalDocumentID oai_doaj_org_article_3b0c412319364f6ab6501f605d4fd9b6
10_1016_j_pulmoe_2018_09_004
30473034
S2531043718301351
Genre Consensus Development Conference
Journal Article
GeographicLocations Portugal
GeographicLocations_xml – name: Portugal
GroupedDBID .1-
.FO
0SF
1P~
65R
6I.
AACTN
AAEDW
AAFTH
AALRI
AAXUO
ABMAC
ACLIJ
ADBBV
AEXQZ
AFCTW
AFRHN
AFTJW
AITUG
AJUYK
ALMA_UNASSIGNED_HOLDINGS
AMRAJ
BCNDV
EBS
EJD
FDB
GROUPED_DOAJ
NCXOZ
OK1
ROL
SSZ
Z5R
ADVLN
AKRWK
CGR
CUY
CVF
ECM
EIF
NPM
TDBHL
AAYXX
CITATION
7X8
ID FETCH-LOGICAL-c4164-c7957aef34e978d0e0c91293c2713dffa020ff78a9dc6bb242e385829e41816e3
IEDL.DBID DOA
ISSN 2531-0437
IngestDate Tue Oct 22 15:14:26 EDT 2024
Fri Oct 25 09:31:31 EDT 2024
Fri Aug 23 01:40:54 EDT 2024
Wed Oct 16 00:47:27 EDT 2024
Wed May 17 01:42:08 EDT 2023
IsDoiOpenAccess true
IsOpenAccess true
IsPeerReviewed true
IsScholarly true
Keywords ATS
FDA
IgA
SERPINA1
Alpha 1-Antitrypsin Deficiency
RV
ERS
Pulmonary Emphysema
ALT
TLC
HU
MPO
AATD
BODE
DBS
DLCO
HRQoL
LTB4
Diagnosis
SEPAR
AST
IEF
EDTA
ER
Consensus
.
PR3
CT
FVC
AAT
HIV
IL8
NHLBI
GGT
URTI
FRC
PD10
Therapeutics
NE
FEV1
RCL
c-ANCA
PCR
COPD
Language English
License This is an open access article under the CC BY-NC-ND license.
Copyright © 2018. Published by Elsevier España, S.L.U.
LinkModel DirectLink
MergedId FETCHMERGED-LOGICAL-c4164-c7957aef34e978d0e0c91293c2713dffa020ff78a9dc6bb242e385829e41816e3
Notes ObjectType-News-1
content type line 25
ObjectType-Feature-4
ObjectType-Conference-2
SourceType-Conference Papers & Proceedings-1
ObjectType-Article-3
OpenAccessLink https://doaj.org/article/3b0c412319364f6ab6501f605d4fd9b6
PMID 30473034
PQID 2138052318
PQPubID 23479
PageCount 21
ParticipantIDs doaj_primary_oai_doaj_org_article_3b0c412319364f6ab6501f605d4fd9b6
proquest_miscellaneous_2138052318
crossref_primary_10_1016_j_pulmoe_2018_09_004
pubmed_primary_30473034
elsevier_sciencedirect_doi_10_1016_j_pulmoe_2018_09_004
PublicationCentury 2000
PublicationDate 2018-12-01
PublicationDateYYYYMMDD 2018-12-01
PublicationDate_xml – month: 12
  year: 2018
  text: 2018-12-01
  day: 01
PublicationDecade 2010
PublicationPlace Spain
PublicationPlace_xml – name: Spain
PublicationTitle Pulmonology
PublicationTitleAlternate Pulmonology
PublicationYear 2018
Publisher Elsevier España, S.L.U
Elsevier España
Publisher_xml – name: Elsevier España, S.L.U
– name: Elsevier España
References de la Roza, Lara, Vila, Miravitlles (bib1455) 2006; 42
American Thoracic, European Respiratory (bib0995) 2003; 168
Alpha 1-antitrypsin deficiency: memorandum from a WHO meeting. Bull World Health Organ. 1997;75(5):397-415.
Lomas, Evans, Stone, Chang, Carrell (bib1020) 1993; 32
O’Brien, Pennycooke, Carroll, Shum, Fee, O’Connor (bib1135) 2015; 12
Needham, Stockley (bib1210) 2005; 25
Dafforn, Mahadeva, Elliott, Sivasothy, Lomas (bib1260) 1999; 274
Ninane, Geltner, Bezzi, Foccoli, Gottlieb, Welte (bib1650) 2012; 39
de Serres (bib0930) 2002; 122
Miravitlles, Dirksen, Ferrarotti, Koblizek, Lange, Mahadeva (bib1430) 2017; 50
Esnault, Testa, Audrain, Roge, Hamidou, Barrier (bib1345) 1993; 43
Meira, Boaventura, Seixas, Sucena (bib0950) 2018
Silva, Sherrill, Guerra, Barbee (bib1090) 2003; 123
Gross, Grebe, Wencker, Stoller, Bjursten, Janciauskiene (bib1625) 2009; 218
Bornhorst, Procter, Meadows, Ashwood, Mao (bib1415) 2007; 128
Casas, Blanco, Martinez, Bustamante, Miravitlles, Cadenas (bib1405) 2015; 51
Sorheim, Bakke, Gulsvik, Pillai, Johannessen, Gaarder (bib1080) 2010; 138
Infarmed. [Internet]. [cited 11-02-2017]. Available from: http://www.infarmed.pt/infomed/lista.php.
Stoller, Smith, Yang, Spray (bib0990) 1994; 61
Dawkins, Dawkins, Wood, Nightingale, Stockley, Stockley (bib1185) 2009; 33
Hubbard, Sellers, Czerski, Stephens, Crystal (bib1530) 1988; 260
Campos, Diaz (bib1480) 2018; 153
Stoller, Aboussouan (bib0925) 2005; 365
Torres Redondo, Campoa, Ruano, Sucena (bib1105) 2017; 53
Black, Kueppers (bib1150) 1978; 117
Chapman, Stockley, Dawkins, Wilkes, Navickis (bib1580) 2009; 6
Elliott, Stein, Bilton, Carrell, Lomas (bib0910) 1996; 3
Luisetti, Massi, Massobrio, Guarraci, Menchicchi, Beccaria (bib1435) 1999; 93
Sveger (bib1280) 1976; 294
Stoller, Gildea, Ries, Meli, Karafa (bib1640) 2007; 83
Fischer, Ortiz-Pallardo, Ko, Esch, Zhou (bib1325) 2000; 33
Parr, Stoel, Stolk, Stockley (bib1130) 2004; 170
Wood, Harrison, Semple, Ayres, Stockley (bib1175) 2009; 34
Eriksson (bib1295) 1985; 20
Struniawski, Szpechcinski, Poplawska, Skronski, Chorostowska-Wynimko (bib1365) 2013; 756
Afdhal (bib1485) 2012; 8
Brode, Ling, Chapman (bib1620) 2012; 184
De la Roza, Soy, Lara, Vila, Esquinas, Torres (bib1670) 2005; 2
Mahadeva, Atkinson, Li, Stewart, Janciauskiene, Kelley (bib1030) 2005; 166
Marcus, Perlmutter (bib1265) 2000; 275
Marsden, Fournier (bib0890) 2005; 10
Barros-Tizon, Torres, Blanco, Martinez (bib1595) 2012; 6
Tobin, Cook, Hutchison (bib1115) 1983; 77
Vidal, Zamora, FS (bib1680) 2006; 42
Soy, de la Roza, Lara, Esquinas, Torres, Miravitlles (bib1685) 2006; 61
Miravitlles, Herr, Ferrarotti, Jardi, Rodriguez-Frias, Luisetti (bib1380) 2010; 35
Wewers, Casolaro, Sellers, Swayze, McPhaul, Wittes (bib1525) 1987; 316
Dawwas, Davies, Griffiths, Lomas, Alexander (bib1710) 2013; 187
Lhotta, Vogel, Meisl, Buxbaum, Neyer, Sandholzer (bib1355) 1994; 87
Parr, Guest, Reynolds, Dowson, Stockley (bib1240) 2007; 176
Hadzic, Quaglia, Mieli-Vergani (bib1300) 2006; 43
Piitulainen, Sveger (bib1145) 1998; 87
McElvaney, Stoller, Buist, Prakash, Brantly, Schluchter (bib1005) 1997; 111
(bib1190) 1998; 158
Gotzsche, Johansen (bib1585) 2010; 7
Hill, Campbell, Ward, Stockley (bib1200) 1999; 93
Graziadei, Joseph, Wiesner, Therneau, Batts, Porayko (bib1320) 1998; 28
Dirksen, Piitulainen, Parr, Deng, Wencker, Shaker (bib1570) 2009; 33
Bell, Schrumpf, Fagerhol (bib1315) 1990; 25
Parr, Stoel, Stolk, Nightingale, Stockley (bib1050) 2004; 170
Marciniuk, Hernandez, Balter, Bourbeau, Chapman, Ford (bib1610) 2012; 19
Campos, Wanner, Zhang, Sandhaus (bib0980) 2005; 128
Demeo, Sandhaus, Barker, Brantly, Eden, McElvaney (bib1155) 2007; 62
Campbell, Campbell, Boukedes, Owen (bib1515) 1999; 104
de la Roza, Rodriguez-Frias, Lara, Vidal, Jardi, Miravitlles (bib1450) 2005; 26
Stoller, Sandhaus, Turino, Dickson, Rodgers, Strange (bib0985) 2005; 128
Kaczor, Sanak, Szczeklik (bib1400) 2007; 9
Seersholm, Wencker, Banik, Viskum, Dirksen, Kok-Jensen (bib1550) 1997; 10
Stockley, Bayley, Unsal, Dowson (bib1535) 2002; 165
Piitulainen, Eriksson (bib1140) 1999; 13
Piitulainen, Sveger (bib1465) 2002; 57
Greulich, Ottaviani, Bals, Lepper, Vogelmeier, Luisetti (bib0975) 2013; 107
Kusu, Nakagiri, Minami, Shintani, Kadota, Inoue (bib1220) 2012; 60
Crystal (bib1510) 1990; 85
Wilson, Galvin (bib1125) 2000; 118
Cuvelier, Muir, Hellot, Benhamou, Martin, Benichou (bib1235) 2000; 117
Stockley, Miravitlles, Vogelmeier, Alpha One International (bib1705) 2013; 8
Stoller, Fallat, Schluchter, O’Brien, Connor, Gross (bib1695) 2003; 123
Hersh, Dahl, Ly, Berkey, Nordestgaard, Silverman (bib1085) 2004; 59
Snyder, Katzmann, Butz, Wiley, Yang, Dawson (bib1410) 2006; 52
(bib1245) 2016; 22
Santos Rosa (bib0945) 1988
Vidal, Blanco, Casas, Jardi, Miravitlles (bib1250) 2006; 42
Stockley, Parr, Piitulainen, Stolk, Stoel, Dirksen (bib1575) 2010; 11
Banauch, Brantly, Izbicki, Hall, Shanske, Chavko (bib1170) 2010; 138
Eden, Mitchell, Mehlman, Khouli, Nejat, Grieco (bib1460) 1997; 156
Uppaluri, Mitsa, Sonka, Hoffman, McLennan (bib1475) 1997; 156
Green, Vayalapra, Hampson, Mukherjee, Stockley, Turner (bib1055) 2015; 70
Brantly, Mishra, Zienko (bib1445) 2003; 167
Corda, Bertella, Pini, Pezzini, Medicina, Boni (bib1395) 2006; 100
Seersholm, Dirksen, Kok-Jensen (bib1120) 1994; 7
Piitulainen, Tanash (bib0920) 2015; 12
Carlson, Eriksson (bib1310) 1985; 20
Silverman, Pierce, Province, Rao, Campbell (bib1160) 1989; 111
Regev, Guaqueta, Molina, Conrad, Mishra, Brantly (bib1330) 2006; 43
Eden (bib1230) 2010; 7
Guest, Hansell (bib1470) 1992; 45
Bergin, Hurley, McElvaney, Reeves (bib0885) 2012; 60
Sandhaus, Turino, Brantly, Campos, Cross, Goodman (bib1630) 2016; 3
Owen, Brennan, Lewis, Carrell (bib0900) 1983; 309
Irving, Pike, Lesk, Whisstock (bib0875) 2000; 10
Dirksen, Dijkman, Madsen, Stoel, Hutchison, Ulrik (bib1565) 1999; 160
Chotirmall, Al-Alawi, McEnery, McElvaney (bib1700) 2015; 11
Larsson (bib1015) 1978; 204
Stoller, Aboussouan (bib0895) 2012; 185
Lin, Chiu, Chang, Cheng, Chen (bib1215) 2008; 79
DeMeo, Silverman (bib1010) 2004; 59
Turino, Barker, Brantly, Cohen, Connelly, Crystal (bib1070) 1996; 154
da Hora Lopes Meira, Boaventura, Seixas, Sucena (bib0955) 2016; 48
Laurell, Eriksson (bib0965) 2013; 10
Alpha-1 in the European Union. Expert Recommendation. [Internet]. [cited 11-02-2017]. Available from: http://www.alpha1awareness.org.uk/wp-content/uploads/2013/08/Alpha1-in-the-European-Union-Expert-Recommendations_electonic-copy.pdf.
Brebner, Stockley (bib1605) 2013; 7
Weill, Benden, Corris, Dark, Davis, Keshavjee (bib1655) 2015; 34
Hatipoglu, Stoller (bib0970) 2016; 37
Elzouki, Eriksson (bib1335) 1996; 8
Ferrarotti, Scabini, Campo, Ottaviani, Zorzetto, Gorrini (bib1385) 2007; 150
Spinola, Bruges-Armas, Pereira, Brehm, Spinola (bib1375) 2009; 103
Chapman, Burdon, Piitulainen, Sandhaus, Seersholm, Stocks (bib1590) 2015; 386
Ogushi, Fells, Hubbard, Straus, Crystal (bib1025) 1987; 80
Gadek, Klein, Holland, Crystal (bib1520) 1981; 68
Wu, Eriksson (bib1195) 1988; 41
Stone, Morris, Franzblau, Snider (bib1540) 1995; 62
Piras, Ferrarotti, Lara, Martinez, Bustamante, Ottaviani (bib1060) 2013; 42
Teschler (bib1615) 2015; 24
Wood, Harrison, Semple, Ayres, Stockley (bib1180) 2010; 67
McElvaney (bib1425) 2015; 24
Loche, Tremeau-Martinage, Laplanche, Massip, Bazex (bib1340) 1999; 9
Wencker, Banik, Buhl, Seidel, Konietzko (bib1690) 1998; 11
Nelson, Teckman, Di Bisceglie, Brenner (bib1270) 2012; 10
Tonelli, Rouhani, Li, Schreck, Brantly (bib1560) 2009; 4
Molloy, Hersh, Morris, Carroll, O’Connor, Lasky-Su (bib1075) 2014; 189
Gooptu, Dickens, Lomas (bib0865) 2014; 20
Mahadeva, Chang, Dafforn, Oakley, Foreman, Calvin (bib0915) 1999; 103
Silverman (bib1095) 2016; 13
Ferrarotti, Thun, Zorzetto, Ottaviani, Imboden, Schindler (bib1420) 2012; 67
Janciauskiene, Bals, Koczulla, Vogelmeier, Kohnlein, Welte (bib0860) 2011; 105
Seixas, Garcia, Trovoada, Santos, Amorim, Rocha (bib1370) 2001; 108
de Serres, Blanco (bib0905) 2014; 276
Hodges, Millward-Sadler, Barbatis, Wright (bib1305) 1981; 304
O’Donoghue, Guickian, Blundell, Winney (bib1350) 1993; 336
Hussain, Mieli-Vergani, Mowat (bib1285) 1991; 14
Lomas, Mahadeva (bib1040) 2002; 110
Serapinas, Obrikyte, Vaicius, Balciuviene, Valavicius, Sakalauskas (bib1225) 2014; 63
Bruce-Hickman, Greene, Gooptu (bib1490) 2015; 69
Greene, Marciniak, Teckman, Ferrarotti, Brantly, Lomas (bib1035) 2016; 2
Wencker, Denker, Konietzko (bib1205) 1994; 61
Eriksson, Carlson, Velez (bib1275) 1986; 314
Barker, Iwata-Morgan, Oveson, Roussel (bib1675) 1997; 112
Gooptu, Lomas (bib0880) 2009; 78
Holm, Borson, Sandhaus, Ford, Strange, Bowler (bib1100) 2013; 10
Stockley (bib1000) 2004; 59
Camelier, Winter, Jardim, Barboza, Cukier, Miravitlles (bib1360) 2008; 34
Wencker, Fuhrmann, Banik, Konietzko (bib1555) 2001; 119
Blanco, de Serres, Fernandez-Bustillo, Lara, Miravitlles (bib0935) 2006; 27
Silva, Oliveira, Guimaraes, Lima, Gomes, Seixas (bib0960) 2016; 116
Yusen, Christie, Edwards, Kucheryavaya, Benden, Dipchand (bib1660) 2013; 32
Eriksson (bib1045) 1996; 110
Lieberman (bib1600) 2000; 118
Dickens, Lomas (bib1495) 2011; 5
Tonelli, Brantly (bib1635) 2010; 4
Mayer, Stoller, Bucher Bartelson, James Ruttenber, Sandhaus, Newman (bib1165) 2000; 162
Sciurba, Ernst, Herth, Strange, Criner, Marquette (bib1645) 2010; 363
Bernspang, Carlson, Piitulainen (bib1290) 2009; 44
Needham, Stockley (bib1255) 2004; 59
Gildea, Shermock, Singer, Stoller (bib1500) 2003; 167
Stein, Carrell (bib0870) 1995; 2
Seersholm, Kok-Jensen (bib1065) 1998; 53
Costa, Jardi, Rodriguez, Miravitlles, Cotrina, Gonzalez (bib1390) 2000; 15
Ma, Lin, He, Rouhani, Brantly, Turino (bib1545) 2013; 10
Janus, Phillips, Carrell (bib1110) 1985; 1
Wencker, Marx, Konietzko, Schaefer, Campbell (bib1440) 2002; 20
Marcus (10.1016/j.pulmoe.2018.09.004_bib1265) 2000; 275
Gooptu (10.1016/j.pulmoe.2018.09.004_bib0865) 2014; 20
(10.1016/j.pulmoe.2018.09.004_bib1190) 1998; 158
Piras (10.1016/j.pulmoe.2018.09.004_bib1060) 2013; 42
Greene (10.1016/j.pulmoe.2018.09.004_bib1035) 2016; 2
Wood (10.1016/j.pulmoe.2018.09.004_bib1175) 2009; 34
Sandhaus (10.1016/j.pulmoe.2018.09.004_bib1630) 2016; 3
Ferrarotti (10.1016/j.pulmoe.2018.09.004_bib1420) 2012; 67
Janciauskiene (10.1016/j.pulmoe.2018.09.004_bib0860) 2011; 105
De la Roza (10.1016/j.pulmoe.2018.09.004_bib1670) 2005; 2
Barros-Tizon (10.1016/j.pulmoe.2018.09.004_bib1595) 2012; 6
Ferrarotti (10.1016/j.pulmoe.2018.09.004_bib1385) 2007; 150
Hubbard (10.1016/j.pulmoe.2018.09.004_bib1530) 1988; 260
Gooptu (10.1016/j.pulmoe.2018.09.004_bib0880) 2009; 78
Sveger (10.1016/j.pulmoe.2018.09.004_bib1280) 1976; 294
Chapman (10.1016/j.pulmoe.2018.09.004_bib1590) 2015; 386
Gross (10.1016/j.pulmoe.2018.09.004_bib1625) 2009; 218
Piitulainen (10.1016/j.pulmoe.2018.09.004_bib1465) 2002; 57
Tobin (10.1016/j.pulmoe.2018.09.004_bib1115) 1983; 77
Eden (10.1016/j.pulmoe.2018.09.004_bib1230) 2010; 7
Santos Rosa (10.1016/j.pulmoe.2018.09.004_bib0945) 1988
Ninane (10.1016/j.pulmoe.2018.09.004_bib1650) 2012; 39
Laurell (10.1016/j.pulmoe.2018.09.004_bib0965) 2013; 10
Green (10.1016/j.pulmoe.2018.09.004_bib1055) 2015; 70
Miravitlles (10.1016/j.pulmoe.2018.09.004_bib1430) 2017; 50
Wewers (10.1016/j.pulmoe.2018.09.004_bib1525) 1987; 316
Lin (10.1016/j.pulmoe.2018.09.004_bib1215) 2008; 79
Hodges (10.1016/j.pulmoe.2018.09.004_bib1305) 1981; 304
Teschler (10.1016/j.pulmoe.2018.09.004_bib1615) 2015; 24
Eriksson (10.1016/j.pulmoe.2018.09.004_bib1275) 1986; 314
Soy (10.1016/j.pulmoe.2018.09.004_bib1685) 2006; 61
10.1016/j.pulmoe.2018.09.004_bib0940
Meira (10.1016/j.pulmoe.2018.09.004_bib0950) 2018
Marsden (10.1016/j.pulmoe.2018.09.004_bib0890) 2005; 10
Dafforn (10.1016/j.pulmoe.2018.09.004_bib1260) 1999; 274
Weill (10.1016/j.pulmoe.2018.09.004_bib1655) 2015; 34
Stoller (10.1016/j.pulmoe.2018.09.004_bib0895) 2012; 185
Dirksen (10.1016/j.pulmoe.2018.09.004_bib1565) 1999; 160
Gotzsche (10.1016/j.pulmoe.2018.09.004_bib1585) 2010; 7
Hersh (10.1016/j.pulmoe.2018.09.004_bib1085) 2004; 59
Demeo (10.1016/j.pulmoe.2018.09.004_bib1155) 2007; 62
Mayer (10.1016/j.pulmoe.2018.09.004_bib1165) 2000; 162
Piitulainen (10.1016/j.pulmoe.2018.09.004_bib0920) 2015; 12
Black (10.1016/j.pulmoe.2018.09.004_bib1150) 1978; 117
Dawkins (10.1016/j.pulmoe.2018.09.004_bib1185) 2009; 33
Cuvelier (10.1016/j.pulmoe.2018.09.004_bib1235) 2000; 117
Brantly (10.1016/j.pulmoe.2018.09.004_bib1445) 2003; 167
Hadzic (10.1016/j.pulmoe.2018.09.004_bib1300) 2006; 43
Campos (10.1016/j.pulmoe.2018.09.004_bib1480) 2018; 153
Stockley (10.1016/j.pulmoe.2018.09.004_bib1575) 2010; 11
Serapinas (10.1016/j.pulmoe.2018.09.004_bib1225) 2014; 63
Corda (10.1016/j.pulmoe.2018.09.004_bib1395) 2006; 100
Dawwas (10.1016/j.pulmoe.2018.09.004_bib1710) 2013; 187
(10.1016/j.pulmoe.2018.09.004_bib1245) 2016; 22
Elzouki (10.1016/j.pulmoe.2018.09.004_bib1335) 1996; 8
Hussain (10.1016/j.pulmoe.2018.09.004_bib1285) 1991; 14
O’Donoghue (10.1016/j.pulmoe.2018.09.004_bib1350) 1993; 336
Seersholm (10.1016/j.pulmoe.2018.09.004_bib1550) 1997; 10
Lomas (10.1016/j.pulmoe.2018.09.004_bib1020) 1993; 32
Loche (10.1016/j.pulmoe.2018.09.004_bib1340) 1999; 9
Parr (10.1016/j.pulmoe.2018.09.004_bib1130) 2004; 170
Vidal (10.1016/j.pulmoe.2018.09.004_bib1250) 2006; 42
Vidal (10.1016/j.pulmoe.2018.09.004_bib1680) 2006; 42
de Serres (10.1016/j.pulmoe.2018.09.004_bib0930) 2002; 122
Graziadei (10.1016/j.pulmoe.2018.09.004_bib1320) 1998; 28
Silva (10.1016/j.pulmoe.2018.09.004_bib0960) 2016; 116
Ma (10.1016/j.pulmoe.2018.09.004_bib1545) 2013; 10
Seersholm (10.1016/j.pulmoe.2018.09.004_bib1065) 1998; 53
10.1016/j.pulmoe.2018.09.004_bib1505
Silverman (10.1016/j.pulmoe.2018.09.004_bib1095) 2016; 13
Crystal (10.1016/j.pulmoe.2018.09.004_bib1510) 1990; 85
Seersholm (10.1016/j.pulmoe.2018.09.004_bib1120) 1994; 7
Stone (10.1016/j.pulmoe.2018.09.004_bib1540) 1995; 62
Stockley (10.1016/j.pulmoe.2018.09.004_bib1705) 2013; 8
Stoller (10.1016/j.pulmoe.2018.09.004_bib0990) 1994; 61
Ogushi (10.1016/j.pulmoe.2018.09.004_bib1025) 1987; 80
Chapman (10.1016/j.pulmoe.2018.09.004_bib1580) 2009; 6
Wencker (10.1016/j.pulmoe.2018.09.004_bib1555) 2001; 119
Banauch (10.1016/j.pulmoe.2018.09.004_bib1170) 2010; 138
Stockley (10.1016/j.pulmoe.2018.09.004_bib1535) 2002; 165
Brebner (10.1016/j.pulmoe.2018.09.004_bib1605) 2013; 7
Camelier (10.1016/j.pulmoe.2018.09.004_bib1360) 2008; 34
Greulich (10.1016/j.pulmoe.2018.09.004_bib0975) 2013; 107
Uppaluri (10.1016/j.pulmoe.2018.09.004_bib1475) 1997; 156
Larsson (10.1016/j.pulmoe.2018.09.004_bib1015) 1978; 204
Blanco (10.1016/j.pulmoe.2018.09.004_bib0935) 2006; 27
Holm (10.1016/j.pulmoe.2018.09.004_bib1100) 2013; 10
Yusen (10.1016/j.pulmoe.2018.09.004_bib1660) 2013; 32
Needham (10.1016/j.pulmoe.2018.09.004_bib1255) 2004; 59
McElvaney (10.1016/j.pulmoe.2018.09.004_bib1425) 2015; 24
Struniawski (10.1016/j.pulmoe.2018.09.004_bib1365) 2013; 756
Tonelli (10.1016/j.pulmoe.2018.09.004_bib1635) 2010; 4
Parr (10.1016/j.pulmoe.2018.09.004_bib1050) 2004; 170
da Hora Lopes Meira (10.1016/j.pulmoe.2018.09.004_bib0955) 2016; 48
Lieberman (10.1016/j.pulmoe.2018.09.004_bib1600) 2000; 118
Barker (10.1016/j.pulmoe.2018.09.004_bib1675) 1997; 112
Guest (10.1016/j.pulmoe.2018.09.004_bib1470) 1992; 45
Esnault (10.1016/j.pulmoe.2018.09.004_bib1345) 1993; 43
Fischer (10.1016/j.pulmoe.2018.09.004_bib1325) 2000; 33
Nelson (10.1016/j.pulmoe.2018.09.004_bib1270) 2012; 10
Costa (10.1016/j.pulmoe.2018.09.004_bib1390) 2000; 15
Eriksson (10.1016/j.pulmoe.2018.09.004_bib1045) 1996; 110
Stoller (10.1016/j.pulmoe.2018.09.004_bib1640) 2007; 83
Stoller (10.1016/j.pulmoe.2018.09.004_bib1695) 2003; 123
Lhotta (10.1016/j.pulmoe.2018.09.004_bib1355) 1994; 87
Turino (10.1016/j.pulmoe.2018.09.004_bib1070) 1996; 154
Elliott (10.1016/j.pulmoe.2018.09.004_bib0910) 1996; 3
Stein (10.1016/j.pulmoe.2018.09.004_bib0870) 1995; 2
Eden (10.1016/j.pulmoe.2018.09.004_bib1460) 1997; 156
Brode (10.1016/j.pulmoe.2018.09.004_bib1620) 2012; 184
Wu (10.1016/j.pulmoe.2018.09.004_bib1195) 1988; 41
Campbell (10.1016/j.pulmoe.2018.09.004_bib1515) 1999; 104
Gildea (10.1016/j.pulmoe.2018.09.004_bib1500) 2003; 167
Seixas (10.1016/j.pulmoe.2018.09.004_bib1370) 2001; 108
Bernspang (10.1016/j.pulmoe.2018.09.004_bib1290) 2009; 44
Casas (10.1016/j.pulmoe.2018.09.004_bib1405) 2015; 51
Wilson (10.1016/j.pulmoe.2018.09.004_bib1125) 2000; 118
Silverman (10.1016/j.pulmoe.2018.09.004_bib1160) 1989; 111
Janus (10.1016/j.pulmoe.2018.09.004_bib1110) 1985; 1
Wood (10.1016/j.pulmoe.2018.09.004_bib1180) 2010; 67
Campos (10.1016/j.pulmoe.2018.09.004_bib0980) 2005; 128
Sciurba (10.1016/j.pulmoe.2018.09.004_bib1645) 2010; 363
McElvaney (10.1016/j.pulmoe.2018.09.004_bib1005) 1997; 111
Stoller (10.1016/j.pulmoe.2018.09.004_bib0925) 2005; 365
de la Roza (10.1016/j.pulmoe.2018.09.004_bib1450) 2005; 26
Spinola (10.1016/j.pulmoe.2018.09.004_bib1375) 2009; 103
de la Roza (10.1016/j.pulmoe.2018.09.004_bib1455) 2006; 42
Hill (10.1016/j.pulmoe.2018.09.004_bib1200) 1999; 93
Gadek (10.1016/j.pulmoe.2018.09.004_bib1520) 1981; 68
Hatipoglu (10.1016/j.pulmoe.2018.09.004_bib0970) 2016; 37
Piitulainen (10.1016/j.pulmoe.2018.09.004_bib1140) 1999; 13
Bell (10.1016/j.pulmoe.2018.09.004_bib1315) 1990; 25
Owen (10.1016/j.pulmoe.2018.09.004_bib0900) 1983; 309
Mahadeva (10.1016/j.pulmoe.2018.09.004_bib1030) 2005; 166
Chotirmall (10.1016/j.pulmoe.2018.09.004_bib1700) 2015; 11
Mahadeva (10.1016/j.pulmoe.2018.09.004_bib0915) 1999; 103
Piitulainen (10.1016/j.pulmoe.2018.09.004_bib1145) 1998; 87
Stoller (10.1016/j.pulmoe.2018.09.004_bib0985) 2005; 128
American Thoracic (10.1016/j.pulmoe.2018.09.004_bib0995) 2003; 168
Wencker (10.1016/j.pulmoe.2018.09.004_bib1440) 2002; 20
Afdhal (10.1016/j.pulmoe.2018.09.004_bib1485) 2012; 8
Marciniuk (10.1016/j.pulmoe.2018.09.004_bib1610) 2012; 19
Snyder (10.1016/j.pulmoe.2018.09.004_bib1410) 2006; 52
Dirksen (10.1016/j.pulmoe.2018.09.004_bib1570) 2009; 33
10.1016/j.pulmoe.2018.09.004_bib1665
Dickens (10.1016/j.pulmoe.2018.09.004_bib1495) 2011; 5
Kaczor (10.1016/j.pulmoe.2018.09.004_bib1400) 2007; 9
Miravitlles (10.1016/j.pulmoe.2018.09.004_bib1380) 2010; 35
Luisetti (10.1016/j.pulmoe.2018.09.004_bib1435) 1999; 93
Regev (10.1016/j.pulmoe.2018.09.004_bib1330) 2006; 43
Parr (10.1016/j.pulmoe.2018.09.004_bib1240) 2007; 176
Eriksson (10.1016/j.pulmoe.2018.09.004_bib1295) 1985; 20
Molloy (10.1016/j.pulmoe.2018.09.004_bib1075) 2014; 189
Silva (10.1016/j.pulmoe.2018.09.004_bib1090) 2003; 123
de Serres (10.1016/j.pulmoe.2018.09.004_bib0905) 2014; 276
Kusu (10.1016/j.pulmoe.2018.09.004_bib1220) 2012; 60
Irving (10.1016/j.pulmoe.2018.09.004_bib0875) 2000; 10
Wencker (10.1016/j.pulmoe.2018.09.004_bib1690) 1998; 11
Tonelli (10.1016/j.pulmoe.2018.09.004_bib1560) 2009; 4
Sorheim (10.1016/j.pulmoe.2018.09.004_bib1080) 2010; 138
Torres Redondo (10.1016/j.pulmoe.2018.09.004_bib1105) 2017; 53
Needham (10.1016/j.pulmoe.2018.09.004_bib1210) 2005; 25
Stockley (10.1016/j.pulmoe.2018.09.004_bib1000) 2004; 59
DeMeo (10.1016/j.pulmoe.2018.09.004_bib1010) 2004; 59
Lomas (10.1016/j.pulmoe.2018.09.004_bib1040) 2002; 110
Wencker (10.1016/j.pulmoe.2018.09.004_bib1205) 1994; 61
O’Brien (10.1016/j.pulmoe.2018.09.004_bib1135) 2015; 12
Bergin (10.1016/j.pulmoe.2018.09.004_bib0885) 2012; 60
Carlson (10.1016/j.pulmoe.2018.09.004_bib1310) 1985; 20
Bornhorst (10.1016/j.pulmoe.2018.09.004_bib1415) 2007; 128
Bruce-Hickman (10.1016/j.pulmoe.2018.09.004_bib1490) 2015; 69
References_xml – volume: 309
  start-page: 694
  year: 1983
  end-page: 698
  ident: bib0900
  article-title: Mutation of antitrypsin to antithrombin. alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder
  publication-title: N Engl J Med.
  contributor:
    fullname: Carrell
– volume: 117
  start-page: 415
  year: 2000
  end-page: 419
  ident: bib1235
  article-title: Distribution of alpha(1)-antitrypsin alleles in patients with bronchiectasis
  publication-title: Chest.
  contributor:
    fullname: Benichou
– year: 1988
  ident: bib0945
  article-title: Análise da intervenção do inibidor da a1 das proteases (IA1P) nas respostas inflamatória e imunitária. Estudo na população portuguesa
  publication-title: Faculdade de Medicina de Coimbra.
  contributor:
    fullname: Santos Rosa
– volume: 80
  start-page: 1366
  year: 1987
  end-page: 1374
  ident: bib1025
  article-title: Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase
  publication-title: J Clin Invest.
  contributor:
    fullname: Crystal
– volume: 24
  start-page: 52
  year: 2015
  end-page: 57
  ident: bib1425
  article-title: Diagnosing alpha1-antitrypsin deficiency: how to improve the current algorithm
  publication-title: Eur Respir Rev.
  contributor:
    fullname: McElvaney
– volume: 10
  start-page: 1768
  year: 2005
  end-page: 1778
  ident: bib0890
  article-title: Organization and expression of the human serpin gene cluster at 14q32.1
  publication-title: Front Biosci.
  contributor:
    fullname: Fournier
– volume: 45
  start-page: 260
  year: 1992
  end-page: 266
  ident: bib1470
  article-title: High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency
  publication-title: Clin Radiol.
  contributor:
    fullname: Hansell
– volume: 10
  start-page: 226
  year: 2013
  end-page: 234
  ident: bib1100
  article-title: Differences in adjustment between individuals with alpha-1 antitrypsin deficiency (AATD)-associated COPD and non-AATD COPD
  publication-title: COPD.
  contributor:
    fullname: Bowler
– volume: 33
  start-page: 883
  year: 2000
  end-page: 892
  ident: bib1325
  article-title: Chronic liver disease in heterozygous alpha1-antitrypsin deficiency PiZ
  publication-title: J Hepatol.
  contributor:
    fullname: Zhou
– volume: 25
  start-page: 992
  year: 2005
  end-page: 1000
  ident: bib1210
  article-title: Exacerbations in {alpha}1-antitrypsin deficiency
  publication-title: Eur Respir J.
  contributor:
    fullname: Stockley
– volume: 156
  start-page: 248
  year: 1997
  end-page: 254
  ident: bib1475
  article-title: Quantification of pulmonary emphysema from lung computed tomography images
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: McLennan
– volume: 107
  start-page: 1400
  year: 2013
  end-page: 1408
  ident: bib0975
  article-title: Alpha1-antitrypsin deficiency - diagnostic testing and disease awareness in Germany and Italy
  publication-title: Respir Med.
  contributor:
    fullname: Luisetti
– volume: 2
  start-page: 16051
  year: 2016
  ident: bib1035
  article-title: alpha1-Antitrypsin deficiency
  publication-title: Nat Rev Dis Primers.
  contributor:
    fullname: Lomas
– volume: 60
  start-page: 452
  year: 2012
  end-page: 455
  ident: bib1220
  article-title: Null allele alpha-1 antitrypsin deficiency: case report of the total pleural covering technique for disease-associated pneumothorax
  publication-title: Gen Thorac Cardiovasc Surg.
  contributor:
    fullname: Inoue
– volume: 68
  start-page: 1158
  year: 1981
  end-page: 1165
  ident: bib1520
  article-title: Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects
  publication-title: J Clin Invest.
  contributor:
    fullname: Crystal
– volume: 27
  start-page: 77
  year: 2006
  end-page: 84
  ident: bib0935
  article-title: Estimated numbers and prevalence of PI*S and PI*Z alleles of alpha1-antitrypsin deficiency in European countries
  publication-title: Eur Respir J.
  contributor:
    fullname: Miravitlles
– volume: 22
  start-page: 222
  year: 2016
  end-page: 235
  ident: bib1245
  article-title: Recommendations for aetiological diagnosis of bronchiectasis
  publication-title: Rev Port Pneumol.
– volume: 110
  start-page: 1585
  year: 2002
  end-page: 1590
  ident: bib1040
  article-title: Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy
  publication-title: J Clin Invest.
  contributor:
    fullname: Mahadeva
– volume: 57
  start-page: 705
  year: 2002
  end-page: 708
  ident: bib1465
  article-title: Respiratory symptoms and lung function in young adults with severe alpha(1)-antitrypsin deficiency (PiZZ)
  publication-title: Thorax.
  contributor:
    fullname: Sveger
– volume: 110
  start-page: 42S
  year: 1996
  end-page: 237S
  ident: bib1045
  article-title: A 30-year perspective on alpha 1-antitrypsin deficiency
  publication-title: Chest.
  contributor:
    fullname: Eriksson
– volume: 70
  start-page: 939
  year: 2015
  end-page: 945
  ident: bib1055
  article-title: PiSZ alpha-1 antitrypsin deficiency (AATD): pulmonary phenotype and prognosis relative to PiZZ AATD and PiMM COPD
  publication-title: Thorax
  contributor:
    fullname: Turner
– volume: 128
  start-page: 1179
  year: 2005
  end-page: 1186
  ident: bib0980
  article-title: Trends in the diagnosis of symptomatic patients with alpha1-antitrypsin deficiency between 1968 and 2003
  publication-title: Chest.
  contributor:
    fullname: Sandhaus
– volume: 103
  start-page: 1498
  year: 2009
  end-page: 1502
  ident: bib1375
  article-title: Alpha-1-antitrypsin deficiency in Madeira (Portugal): the highest prevalence in the world
  publication-title: Respir Med.
  contributor:
    fullname: Spinola
– volume: 50
  year: 2017
  ident: bib1430
  article-title: European Respiratory Society statement: diagnosis and treatment of pulmonary disease in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  contributor:
    fullname: Mahadeva
– volume: 150
  start-page: 267
  year: 2007
  end-page: 274
  ident: bib1385
  article-title: Laboratory diagnosis of alpha1-antitrypsin deficiency
  publication-title: Transl Res.
  contributor:
    fullname: Gorrini
– volume: 112
  start-page: 607
  year: 1997
  end-page: 613
  ident: bib1675
  article-title: Pharmacokinetic study of alpha1-antitrypsin infusion in alpha1-antitrypsin deficiency
  publication-title: Chest.
  contributor:
    fullname: Roussel
– volume: 79
  start-page: 704
  year: 2008
  end-page: 706
  ident: bib1215
  article-title: Spontaneous pneumothorax in flight as first manifestation of alpha-1 antitrypsin deficiency
  publication-title: Aviat Space Environ Med.
  contributor:
    fullname: Chen
– volume: 365
  start-page: 2225
  year: 2005
  end-page: 2236
  ident: bib0925
  article-title: Alpha1-antitrypsin deficiency
  publication-title: Lancet.
  contributor:
    fullname: Aboussouan
– volume: 6
  start-page: 67
  year: 2012
  end-page: 78
  ident: bib1595
  article-title: Investigators of the r EXAsg. Reduction of severe exacerbations and hospitalization-derived costs in alpha-1-antitrypsin-deficient patients treated with alpha-1-antitrypsin augmentation therapy
  publication-title: Ther Adv Respir Dis.
  contributor:
    fullname: Martinez
– volume: 165
  start-page: 1494
  year: 2002
  end-page: 1498
  ident: bib1535
  article-title: The effect of augmentation therapy on bronchial inflammation in alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Dowson
– volume: 34
  start-page: 346
  year: 2009
  end-page: 353
  ident: bib1175
  article-title: Outdoor air pollution is associated with disease severity in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  contributor:
    fullname: Stockley
– volume: 2
  start-page: 96
  year: 1995
  end-page: 113
  ident: bib0870
  article-title: What do dysfunctional serpins tell us about molecular mobility and disease?
  publication-title: Nat Struct Biol.
  contributor:
    fullname: Carrell
– volume: 53
  start-page: 265
  year: 1998
  end-page: 268
  ident: bib1065
  article-title: Clinical features and prognosis of life time non-smokers with severe alpha 1-antitrypsin deficiency
  publication-title: Thorax.
  contributor:
    fullname: Kok-Jensen
– volume: 34
  start-page: 514
  year: 2008
  end-page: 527
  ident: bib1360
  article-title: [Alpha-1 antitrypsin deficiency: diagnosis and treatment]
  publication-title: J Bras Pneumol.
  contributor:
    fullname: Miravitlles
– volume: 10
  start-page: 3
  year: 2013
  end-page: 8
  ident: bib0965
  article-title: The electrophoretic alpha1-globulin pattern of serum in alpha1-antitrypsin deficiency. 1963
  publication-title: COPD
  contributor:
    fullname: Eriksson
– volume: 41
  start-page: 1157
  year: 1988
  end-page: 1165
  ident: bib1195
  article-title: Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ
  publication-title: J Clin Epidemiol.
  contributor:
    fullname: Eriksson
– volume: 42
  start-page: 553
  year: 2006
  end-page: 556
  ident: bib1680
  article-title: Pharmacokinetics of α1-Antitrypsin Replacement Therapy in Severe Congenital Emphysema
  publication-title: Arch Bronconeumol.
  contributor:
    fullname: FS
– volume: 48
  year: 2016
  ident: bib0955
  article-title: Detection of alpha1-antitrypsin deficiency in a European country without national registry yet
  publication-title: Eur Respir J.
  contributor:
    fullname: Sucena
– volume: 128
  start-page: 482
  year: 2007
  end-page: 490
  ident: bib1415
  article-title: Evaluation of an integrative diagnostic algorithm for the identification of people at risk for alpha1-antitrypsin deficiency
  publication-title: Am J Clin Pathol.
  contributor:
    fullname: Mao
– volume: 62
  start-page: 76
  year: 1995
  end-page: 79
  ident: bib1540
  article-title: Preliminary evidence that augmentation therapy diminishes degradation of cross-linked elastin in alpha-1-antitrypsin-deficient humans
  publication-title: Respiration.
  contributor:
    fullname: Snider
– volume: 67
  start-page: 556
  year: 2010
  end-page: 561
  ident: bib1180
  article-title: Outdoor air pollution is associated with rapid decline of lung function in alpha-1-antitrypsin deficiency
  publication-title: Occup Environ Med.
  contributor:
    fullname: Stockley
– volume: 7
  year: 2010
  ident: bib1585
  article-title: Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease
  publication-title: Cochrane Database Syst Rev.
  contributor:
    fullname: Johansen
– volume: 117
  start-page: 421
  year: 1978
  end-page: 428
  ident: bib1150
  article-title: alpha1-Antitrypsin deficiency in nonsmokers
  publication-title: Am Rev Respir Dis.
  contributor:
    fullname: Kueppers
– volume: 42
  start-page: 54
  year: 2013
  end-page: 64
  ident: bib1060
  article-title: Clinical phenotypes of Italian and Spanish patients with alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  contributor:
    fullname: Ottaviani
– volume: 204
  start-page: 345
  year: 1978
  end-page: 351
  ident: bib1015
  article-title: Natural history and life expectancy in severe alpha1-antitrypsin deficiency
  publication-title: Pi Z. Acta Med Scand.
  contributor:
    fullname: Larsson
– volume: 1
  start-page: 152
  year: 1985
  end-page: 154
  ident: bib1110
  article-title: Smoking, lung function, and alpha 1-antitrypsin deficiency
  publication-title: Lancet.
  contributor:
    fullname: Carrell
– volume: 61
  start-page: 461
  year: 1994
  end-page: 467
  ident: bib0990
  article-title: Physical and social impact of alpha 1-antitrypsin deficiency: results of a survey
  publication-title: Cleve Clin J Med.
  contributor:
    fullname: Spray
– volume: 162
  start-page: 553
  year: 2000
  end-page: 558
  ident: bib1165
  article-title: Occupational exposure risks in individuals with PI*Z alpha(1)-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Newman
– volume: 62
  start-page: 806
  year: 2007
  end-page: 813
  ident: bib1155
  article-title: Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency
  publication-title: Thorax.
  contributor:
    fullname: McElvaney
– volume: 118
  start-page: 867
  year: 2000
  end-page: 871
  ident: bib1125
  article-title: Normal diffusing capacity in patients with PiZ alpha(1)-antitrypsin deficiency, severe airflow obstruction, and significant radiographic emphysema
  publication-title: Chest
  contributor:
    fullname: Galvin
– volume: 118
  start-page: 1480
  year: 2000
  end-page: 1485
  ident: bib1600
  article-title: Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data
  publication-title: Chest.
  contributor:
    fullname: Lieberman
– volume: 15
  start-page: 1111
  year: 2000
  end-page: 1115
  ident: bib1390
  article-title: Simple method for alpha1-antitrypsin deficiency screening by use of dried blood spot specimens
  publication-title: Eur Respir J.
  contributor:
    fullname: Gonzalez
– volume: 185
  start-page: 246
  year: 2012
  end-page: 259
  ident: bib0895
  article-title: A review of alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Aboussouan
– volume: 8
  start-page: 989
  year: 1996
  end-page: 994
  ident: bib1335
  article-title: Risk of hepatobiliary disease in adults with severe alpha 1-antitrypsin deficiency (PiZZ): is chronic viral hepatitis B or C an additional risk factor for cirrhosis and hepatocellular carcinoma?
  publication-title: Eur J Gastroenterol Hepatol.
  contributor:
    fullname: Eriksson
– volume: 122
  start-page: 1818
  year: 2002
  end-page: 1829
  ident: bib0930
  article-title: Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys
  publication-title: Chest.
  contributor:
    fullname: de Serres
– volume: 59
  start-page: 843
  year: 2004
  end-page: 849
  ident: bib1085
  article-title: Chronic obstructive pulmonary disease in alpha1-antitrypsin PI MZ heterozygotes: a meta-analysis
  publication-title: Thorax.
  contributor:
    fullname: Silverman
– volume: 35
  start-page: 960
  year: 2010
  end-page: 968
  ident: bib1380
  article-title: Laboratory testing of individuals with severe alpha1-antitrypsin deficiency in three European centres
  publication-title: Eur Respir J.
  contributor:
    fullname: Luisetti
– volume: 2
  start-page: A809
  year: 2005
  ident: bib1670
  article-title: Can the intervals of exogenous alpha-1-antitrypsin (AAT) administration be lengthened?
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Torres
– volume: 11
  start-page: 428
  year: 1998
  end-page: 433
  ident: bib1690
  article-title: Long-term treatment of alpha1-antitrypsin deficiency-related pulmonary emphysema with human alpha1-antitrypsin
  publication-title: Eur Respir J.
  contributor:
    fullname: Konietzko
– volume: 218
  start-page: 370
  year: 2009
  end-page: 375
  ident: bib1625
  article-title: New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy
  publication-title: Dermatology.
  contributor:
    fullname: Janciauskiene
– volume: 53
  start-page: 49
  year: 2017
  end-page: 54
  ident: bib1105
  article-title: Health-Related Quality of Life in Patients With alpha1 Antitrypsin Deficency: A Cross Sectional Study
  publication-title: Arch Bronconeumol.
  contributor:
    fullname: Sucena
– volume: 52
  start-page: 2236
  year: 2006
  end-page: 2242
  ident: bib1410
  article-title: Diagnosis of alpha-1-antitrypsin deficiency: An algorithm of quantification, genotyping, and phenotyping
  publication-title: Clin Chem.
  contributor:
    fullname: Dawson
– volume: 123
  start-page: 1435
  year: 2003
  end-page: 1440
  ident: bib1090
  article-title: A longitudinal study of alpha1-antitrypsin phenotypes and decline in FEV1 in a community population
  publication-title: Chest.
  contributor:
    fullname: Barbee
– volume: 111
  start-page: 394
  year: 1997
  end-page: 403
  ident: bib1005
  publication-title: Chest
  contributor:
    fullname: Schluchter
– volume: 336
  start-page: 331
  year: 1993
  end-page: 335
  ident: bib1350
  article-title: Alpha-1-proteinase inhibitor and pulmonary haemorrhage in systemic vasculitis
  publication-title: Adv Exp Med Biol.
  contributor:
    fullname: Winney
– volume: 3
  start-page: 910
  year: 1996
  end-page: 911
  ident: bib0910
  article-title: Structural explanation for the deficiency of S alpha 1-antitrypsin
  publication-title: Nat Struct Biol.
  contributor:
    fullname: Lomas
– volume: 108
  start-page: 20
  year: 2001
  end-page: 30
  ident: bib1370
  article-title: Patterns of haplotype diversity within the serpin gene cluster at 14q32.1: insights into the natural history of the alpha1-antitrypsin polymorphism
  publication-title: Hum Genet.
  contributor:
    fullname: Rocha
– volume: 138
  start-page: 1116
  year: 2010
  end-page: 1124
  ident: bib1170
  article-title: Accelerated spirometric decline in New York City firefighters with alpha(1)-antitrypsin deficiency
  publication-title: Chest.
  contributor:
    fullname: Chavko
– volume: 104
  start-page: 337
  year: 1999
  end-page: 344
  ident: bib1515
  article-title: Quantum proteolysis by neutrophils: implications for pulmonary emphysema in alpha 1-antitrypsin deficiency
  publication-title: J Clin Invest.
  contributor:
    fullname: Owen
– volume: 13
  start-page: S341
  year: 2016
  end-page: S345
  ident: bib1095
  article-title: Risk of Lung Disease in PI MZ Heterozygotes. Current Status and Future Research Directions
  publication-title: Ann Am Thorac Soc.
  contributor:
    fullname: Silverman
– volume: 83
  start-page: 241
  year: 2007
  end-page: 251
  ident: bib1640
  article-title: National Emphysema Treatment Trial Research G. Lung volume reduction surgery in patients with emphysema and alpha-1 antitrypsin deficiency
  publication-title: Ann Thorac Surg.
  contributor:
    fullname: Karafa
– volume: 43
  start-page: S30
  year: 2006
  end-page: S35
  ident: bib1330
  article-title: Does the heterozygous state of alpha-1 antitrypsin deficiency have a role in chronic liver diseases? Interim results of a large case-control study
  publication-title: J Pediatr Gastroenterol Nutr.
  contributor:
    fullname: Brantly
– volume: 10
  start-page: 1845
  year: 2000
  end-page: 1864
  ident: bib0875
  article-title: Phylogeny of the serpin superfamily: implications of patterns of amino acid conservation for structure and function
  publication-title: Genome Res.
  contributor:
    fullname: Whisstock
– volume: 77
  start-page: 14
  year: 1983
  end-page: 27
  ident: bib1115
  article-title: 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
  publication-title: Br J Dis Chest.
  contributor:
    fullname: Hutchison
– volume: 87
  start-page: 1120
  year: 1998
  end-page: 1124
  ident: bib1145
  article-title: Effect of environmental and clinical factors on lung function and respiratory symptoms in adolescents with alpha1-antitrypsin deficiency
  publication-title: Acta paediatr.
  contributor:
    fullname: Sveger
– volume: 274
  start-page: 9548
  year: 1999
  end-page: 9555
  ident: bib1260
  article-title: A kinetic mechanism for the polymerization of alpha1-antitrypsin
  publication-title: J Biol Chem.
  contributor:
    fullname: Lomas
– volume: 69
  start-page: 47
  year: 2015
  end-page: 84
  ident: bib1490
  article-title: Alpha1-antitrypsin deficiency
  publication-title: Eur Respir Monogr.
  contributor:
    fullname: Gooptu
– volume: 85
  start-page: 1343
  year: 1990
  end-page: 1352
  ident: bib1510
  article-title: Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy
  publication-title: J Clin Invest.
  contributor:
    fullname: Crystal
– volume: 93
  start-page: 169
  year: 1999
  end-page: 172
  ident: bib1435
  article-title: A national program for detection of alpha 1-antitrypsin deficiency in Italy
  publication-title: Respir Med.
  contributor:
    fullname: Beccaria
– volume: 61
  start-page: 1059
  year: 2006
  end-page: 1064
  ident: bib1685
  article-title: Alpha-1-antitrypsin deficiency: optimal therapeutic regimen based on population pharmacokinetics
  publication-title: Thorax.
  contributor:
    fullname: Miravitlles
– volume: 8
  start-page: 605
  year: 2012
  end-page: 607
  ident: bib1485
  article-title: Fibroscan (transient elastography) for the measurement of liver fibrosis
  publication-title: Gastroenterol Hepatol.
  contributor:
    fullname: Afdhal
– volume: 61
  start-page: 195
  year: 1994
  end-page: 198
  ident: bib1205
  article-title: Serial measurements of FEV1 over 12 years in a patient with alpha-1-protease inhibitor deficiency:influence of augmentation therapy and infections
  publication-title: Respiration.
  contributor:
    fullname: Konietzko
– volume: 43
  start-page: 194
  year: 2006
  ident: bib1300
  article-title: Hepatocellular carcinoma in a 12-year-old child with PiZZ alpha1-antitrypsin deficiency
  publication-title: Hepatology.
  contributor:
    fullname: Mieli-Vergani
– volume: 39
  start-page: 1319
  year: 2012
  end-page: 1325
  ident: bib1650
  article-title: Multicentre European study for the treatment of advanced emphysema with bronchial valves
  publication-title: Eur Respir J.
  contributor:
    fullname: Welte
– volume: 11
  start-page: 143
  year: 2015
  end-page: 151
  ident: bib1700
  article-title: Alpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes
  publication-title: Ther Clin Risk Manag.
  contributor:
    fullname: McElvaney
– volume: 7
  start-page: 213
  year: 2013
  end-page: 229
  ident: bib1605
  article-title: Recent advances in alpha-1-antitrypsin deficiency-related lung disease
  publication-title: Expert Rev Respir Med.
  contributor:
    fullname: Stockley
– volume: 24
  start-page: 46
  year: 2015
  end-page: 51
  ident: bib1615
  article-title: Long-term experience in the treatment of alpha1-antitrypsin deficiency: 25 years of augmentation therapy
  publication-title: Eur Respir Rev.
  contributor:
    fullname: Teschler
– volume: 119
  start-page: 737
  year: 2001
  end-page: 744
  ident: bib1555
  article-title: Wissenschaftliche Arbeitsgemeinschaft zur Therapie von L. Longitudinal follow-up of patients with alpha(1)-protease inhibitor deficiency before and during therapy with IV alpha(1)-protease inhibitor
  publication-title: Chest.
  contributor:
    fullname: Konietzko
– volume: 386
  start-page: 360
  year: 2015
  end-page: 368
  ident: bib1590
  article-title: Intravenous augmentation treatment and lung density in severe alpha1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial
  publication-title: Lancet.
  contributor:
    fullname: Stocks
– volume: 5
  start-page: 391
  year: 2011
  end-page: 405
  ident: bib1495
  article-title: Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis
  publication-title: Drug Des Devel Ther.
  contributor:
    fullname: Lomas
– volume: 78
  start-page: 147
  year: 2009
  end-page: 176
  ident: bib0880
  article-title: Conformational pathology of the serpins: themes, variations, and therapeutic strategies
  publication-title: Annu Rev Biochem.
  contributor:
    fullname: Lomas
– volume: 116
  start-page: 8
  year: 2016
  end-page: 18
  ident: bib0960
  article-title: Alpha-1-antitrypsin (SERPINA1) mutation spectrum: Three novel variants and haplotype characterization of rare deficiency alleles identified in Portugal
  publication-title: Respir Med.
  contributor:
    fullname: Seixas
– volume: 12
  start-page: 2
  year: 2015
  end-page: 9
  ident: bib1135
  article-title: The impact of smoke exposure on the clinical phenotype of alpha-1 antitrypsin deficiency in Ireland: exploiting a national registry to understand a rare disease
  publication-title: COPD.
  contributor:
    fullname: O’Connor
– volume: 187
  start-page: 502
  year: 2013
  end-page: 508
  ident: bib1710
  article-title: Prevalence and risk factors for liver involvement in individuals with PiZZ-related lung disease
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Alexander
– volume: 100
  start-page: 463
  year: 2006
  end-page: 470
  ident: bib1395
  article-title: Diagnostic flow chart for targeted detection of alpha1-antitrypsin deficiency
  publication-title: Respir Med.
  contributor:
    fullname: Boni
– volume: 153
  start-page: 1240
  year: 2018
  end-page: 1248
  ident: bib1480
  article-title: The Role of Computed Tomography for the Evaluation of Lung Disease in Alpha-1 Antitrypsin Deficiency
  publication-title: Chest.
  contributor:
    fullname: Diaz
– volume: 170
  start-page: 883
  year: 2004
  end-page: 890
  ident: bib1050
  article-title: Influence of calibration on densitometric studies of emphysema progression using computed tomography
  publication-title: Am J Resp Crit Care Med.
  contributor:
    fullname: Stockley
– volume: 87
  start-page: 693
  year: 1994
  end-page: 695
  ident: bib1355
  article-title: Alpha 1-antitrypsin phenotypes in patients with anti-neutrophil cytoplasmic antibody-positive vasculitis
  publication-title: Clin Sci.
  contributor:
    fullname: Sandholzer
– volume: 20
  start-page: 835
  year: 1985
  end-page: 842
  ident: bib1310
  article-title: Chronic ‘cryptogenic’ liver disease and malignant hepatoma in intermediate alpha 1-antitrypsin deficiency identified by a Pi Z-specific monoclonal antibody
  publication-title: Scand J Gastrenterol.
  contributor:
    fullname: Eriksson
– volume: 9
  start-page: 99
  year: 2007
  end-page: 104
  ident: bib1400
  article-title: Rapid and inexpensive detection of alpha1-antitrypsin deficiency-related alleles S and Z by a real-time polymerase chain reaction suitable for a large-scale population-based screening
  publication-title: J Mol Diagn.
  contributor:
    fullname: Szczeklik
– volume: 33
  start-page: 1338
  year: 2009
  end-page: 1344
  ident: bib1185
  article-title: Rate of progression of lung function impairment in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  contributor:
    fullname: Stockley
– volume: 105
  start-page: 1129
  year: 2011
  end-page: 1139
  ident: bib0860
  article-title: The discovery of alpha1-antitrypsin and its role in health and disease
  publication-title: Respir Med.
  contributor:
    fullname: Welte
– volume: 43
  start-page: 1329
  year: 1993
  end-page: 1332
  ident: bib1345
  article-title: Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis
  publication-title: Kidney Int.
  contributor:
    fullname: Barrier
– volume: 260
  start-page: 1259
  year: 1988
  end-page: 1264
  ident: bib1530
  article-title: Biochemical efficacy and safety of monthly augmentation therapy for alpha 1-antitrypsin deficiency
  publication-title: JAMA.
  contributor:
    fullname: Crystal
– volume: 7
  start-page: 1985
  year: 1994
  end-page: 1987
  ident: bib1120
  article-title: Airways obstruction and two year survival in patients with severe alpha 1-antitrypsin deficiency
  publication-title: Eur Respir J.
  contributor:
    fullname: Kok-Jensen
– volume: 10
  start-page: 2260
  year: 1997
  end-page: 2263
  ident: bib1550
  article-title: Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency?
  publication-title: Eur Respir J.
  contributor:
    fullname: Kok-Jensen
– volume: 67
  start-page: 669
  year: 2012
  end-page: 674
  ident: bib1420
  article-title: Serum levels and genotype distribution of alpha1-antitrypsin in the general population
  publication-title: Thorax.
  contributor:
    fullname: Schindler
– volume: 176
  start-page: 1215
  year: 2007
  end-page: 1221
  ident: bib1240
  article-title: Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Stockley
– volume: 111
  start-page: 982
  year: 1989
  end-page: 991
  ident: bib1160
  article-title: Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates
  publication-title: Ann Intern Med.
  contributor:
    fullname: Campbell
– volume: 167
  start-page: 1387
  year: 2003
  end-page: 1392
  ident: bib1500
  article-title: Cost-effectiveness analysis of augmentation therapy for severe alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Stoller
– volume: 167
  start-page: A222
  year: 2003
  ident: bib1445
  article-title: Statewide targeted screening and detection of AAT deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Zienko
– volume: 11
  start-page: 136
  year: 2010
  ident: bib1575
  article-title: Therapeutic efficacy of alpha-1 antitrypsin augmentation therapy on the loss of lung tissue: an integrated analysis of 2 randomised clinical trials using computed tomography densitometry
  publication-title: Respir Res.
  contributor:
    fullname: Dirksen
– volume: 37
  start-page: 487
  year: 2016
  end-page: 504
  ident: bib0970
  article-title: alpha1-Antitrypsin Deficiency
  publication-title: Clin Chest Med
  contributor:
    fullname: Stoller
– volume: 103
  start-page: 999
  year: 1999
  end-page: 1006
  ident: bib0915
  article-title: Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis
  publication-title: J Clin Invest.
  contributor:
    fullname: Calvin
– volume: 314
  start-page: 736
  year: 1986
  end-page: 739
  ident: bib1275
  article-title: Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency
  publication-title: N Engl J Med.
  contributor:
    fullname: Velez
– volume: 20
  start-page: 907
  year: 1985
  end-page: 911
  ident: bib1295
  article-title: Liver disease in alpha 1-antitrypsin deficiency. Aspects of incidence and prognosis
  publication-title: Scand J Gastroenterol.
  contributor:
    fullname: Eriksson
– volume: 10
  start-page: 473
  year: 2013
  end-page: 481
  ident: bib1545
  article-title: Alpha-1 antitrypsin augmentation therapy and biomarkers of elastin degradation
  publication-title: COPD.
  contributor:
    fullname: Turino
– volume: 32
  start-page: 965
  year: 2013
  end-page: 978
  ident: bib1660
  article-title: The Registry of the International Society for Heart and Lung Transplantation: Thirtieth Adult Lung and Heart-Lung Transplant Report--2013; focus theme: age
  publication-title: J Heart Lung Transplant.
  contributor:
    fullname: Dipchand
– volume: 13
  start-page: 247
  year: 1999
  end-page: 251
  ident: bib1140
  article-title: Decline in FEV1 related to smoking status in individuals with severe alpha1-antitrypsin deficiency (PiZZ)
  publication-title: Eur Respir J.
  contributor:
    fullname: Eriksson
– volume: 10
  start-page: 575
  year: 2012
  end-page: 580
  ident: bib1270
  article-title: Diagnosis and management of patients with alpha1-antitrypsin (A1AT) deficiency
  publication-title: Clin Gastroenterol Hepatol.
  contributor:
    fullname: Brenner
– volume: 160
  start-page: 1468
  year: 1999
  end-page: 1472
  ident: bib1565
  article-title: A randomized clinical trial of alpha(1)-antitrypsin augmentation therapy
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Ulrik
– volume: 168
  start-page: 818
  year: 2003
  end-page: 900
  ident: bib0995
  article-title: American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: European Respiratory
– volume: 20
  start-page: 319
  year: 2002
  end-page: 324
  ident: bib1440
  article-title: Screening for alpha1-Pi deficiency in patients with lung diseases
  publication-title: Eur Respir J.
  contributor:
    fullname: Campbell
– volume: 304
  start-page: 557
  year: 1981
  end-page: 560
  ident: bib1305
  article-title: Heterozygous MZ alpha 1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis
  publication-title: N Engl J Med.
  contributor:
    fullname: Wright
– volume: 34
  start-page: 1
  year: 2015
  end-page: 15
  ident: bib1655
  article-title: A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation
  publication-title: J Heart Lung Transplant.
  contributor:
    fullname: Keshavjee
– volume: 59
  start-page: 441
  year: 2004
  end-page: 445
  ident: bib1255
  article-title: Alpha 1-antitrypsin deficiency. 3: Clinical manifestations and natural history
  publication-title: Thorax
  contributor:
    fullname: Stockley
– volume: 4
  start-page: 289
  year: 2010
  end-page: 312
  ident: bib1635
  article-title: Augmentation therapy in alpha-1 antitrypsin deficiency: advances and controversies
  publication-title: Ther Adv Respir Dis.
  contributor:
    fullname: Brantly
– volume: 8
  start-page: 149
  year: 2013
  ident: bib1705
  article-title: Augmentation therapy for alpha-1 antitrypsin deficiency: towards a personalised approach
  publication-title: Orphanet J Rare Dis.
  contributor:
    fullname: Alpha One International
– volume: 9
  start-page: 565
  year: 1999
  end-page: 567
  ident: bib1340
  article-title: Panniculitis revealing qualitative alpha 1 antitrypsine deficiency (MS variant)
  publication-title: Eur J Dermatol.
  contributor:
    fullname: Bazex
– volume: 156
  start-page: 68
  year: 1997
  end-page: 74
  ident: bib1460
  article-title: Atopy, asthma, and emphysema in patients with severe alpha-1-antitrypysin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Grieco
– volume: 184
  start-page: 1365
  year: 2012
  end-page: 1371
  ident: bib1620
  article-title: Alpha-1 antitrypsin deficiency: a commonly overlooked cause of lung disease
  publication-title: Can Med Assoc J.
  contributor:
    fullname: Chapman
– volume: 6
  start-page: 177
  year: 2009
  end-page: 184
  ident: bib1580
  article-title: Augmentation therapy for alpha1 antitrypsin deficiency: a meta-analysis
  publication-title: COPD.
  contributor:
    fullname: Navickis
– volume: 32
  start-page: 500
  year: 1993
  end-page: 508
  ident: bib1020
  article-title: Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin
  publication-title: Biochemistry.
  contributor:
    fullname: Carrell
– volume: 138
  start-page: 1125
  year: 2010
  end-page: 1132
  ident: bib1080
  article-title: alpha(1)-Antitrypsin protease inhibitor MZ heterozygosity is associated with airflow obstruction in two large cohorts
  publication-title: Chest.
  contributor:
    fullname: Gaarder
– volume: 756
  start-page: 29
  year: 2013
  end-page: 37
  ident: bib1365
  article-title: Rapid DNA extraction protocol for detection of alpha-1 antitrypsin deficiency from dried blood spots by real-time PCR
  publication-title: Adv Exp Med Biol.
  contributor:
    fullname: Chorostowska-Wynimko
– volume: 3
  start-page: 668
  year: 2016
  end-page: 682
  ident: bib1630
  article-title: The diagnosis and management of alpha-1 antitrypsin deficiency in the adult
  publication-title: Chronic Obstr Pulm Dis (Miami).
  contributor:
    fullname: Goodman
– volume: 170
  start-page: 1172
  year: 2004
  end-page: 1178
  ident: bib1130
  article-title: Pattern of emphysema distribution in alpha1-antitrypsin deficiency influences lung function impairment
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Stockley
– volume: 14
  start-page: 497
  year: 1991
  end-page: 511
  ident: bib1285
  article-title: Alpha 1-antitrypsin deficiency and liver disease: clinical presentation, diagnosis and treatment
  publication-title: J Inherit Metab Dis.
  contributor:
    fullname: Mowat
– volume: 12
  start-page: 36
  year: 2015
  end-page: 41
  ident: bib0920
  article-title: The Clinical Profile of Subjects Included in the Swedish National Register on Individuals with Severe Alpha 1-Antitrypsin deficiency
  publication-title: COPD.
  contributor:
    fullname: Tanash
– volume: 7
  start-page: 366
  year: 2010
  end-page: 374
  ident: bib1230
  article-title: Asthma and COPD in alpha-1 antitrypsin deficiency
  publication-title: Evidence for the Dutch hypothesis. COPD.
  contributor:
    fullname: Eden
– volume: 28
  start-page: 1058
  year: 1998
  end-page: 1063
  ident: bib1320
  article-title: Increased risk of chronic liver failure in adults with heterozygous alpha1-antitrypsin deficiency
  publication-title: Hepatology.
  contributor:
    fullname: Porayko
– volume: 93
  start-page: 481
  year: 1999
  end-page: 490
  ident: bib1200
  article-title: Chronic obstructive pulmonary disease, with and without alpha-1-antitrypsin deficiency: management practices in the U.K
  publication-title: Respir Med.
  contributor:
    fullname: Stockley
– volume: 316
  start-page: 1055
  year: 1987
  end-page: 1062
  ident: bib1525
  article-title: Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema
  publication-title: N Engl J Med.
  contributor:
    fullname: Wittes
– volume: 44
  start-page: 1349
  year: 2009
  end-page: 1355
  ident: bib1290
  article-title: The liver in 30-year-old individuals with alpha(1)-antitrypsin deficiency
  publication-title: Scand J Gastroenterol.
  contributor:
    fullname: Piitulainen
– volume: 59
  start-page: 259
  year: 2004
  end-page: 264
  ident: bib1010
  article-title: Alpha1-antitrypsin deficiency. 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk
  publication-title: Thorax.
  contributor:
    fullname: Silverman
– volume: 363
  start-page: 1233
  year: 2010
  end-page: 1244
  ident: bib1645
  article-title: A randomized study of endobronchial valves for advanced emphysema
  publication-title: N Engl J Med.
  contributor:
    fullname: Marquette
– volume: 123
  start-page: 1425
  year: 2003
  end-page: 1434
  ident: bib1695
  article-title: Augmentation therapy with alpha1-antitrypsin: patterns of use and adverse events
  publication-title: Chest.
  contributor:
    fullname: Gross
– volume: 166
  start-page: 377
  year: 2005
  end-page: 386
  ident: bib1030
  article-title: Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo
  publication-title: Am J Pathol.
  contributor:
    fullname: Kelley
– volume: 59
  start-page: 363
  year: 2004
  end-page: 364
  ident: bib1000
  article-title: Alpha 1-antitrypsin: more than just deficiency
  publication-title: Thorax.
  contributor:
    fullname: Stockley
– volume: 275
  start-page: 1987
  year: 2000
  end-page: 1992
  ident: bib1265
  article-title: Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha1 antitrypsin Z
  publication-title: J Biol Chem.
  contributor:
    fullname: Perlmutter
– volume: 276
  start-page: 311
  year: 2014
  end-page: 335
  ident: bib0905
  article-title: Role of alpha-1 antitrypsin in human health and disease
  publication-title: J Intern Med.
  contributor:
    fullname: Blanco
– volume: 42
  start-page: 645
  year: 2006
  end-page: 659
  ident: bib1250
  article-title: Committee on the National Registry of Individuals with Alpha-1 Antitrypsin D. [Guidelines for the diagnosis and management of alpha-1 antitrypsin deficiency]
  publication-title: Arch Bronconeumol
  contributor:
    fullname: Miravitlles
– volume: 294
  start-page: 1316
  year: 1976
  end-page: 1321
  ident: bib1280
  article-title: Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants
  publication-title: N Engl J Med.
  contributor:
    fullname: Sveger
– start-page: 1
  year: 2018
  end-page: 6
  ident: bib0950
  article-title: Alpha-1 Antitrypsin Deficiency Detection in a Portuguese Population
  publication-title: COPD.
  contributor:
    fullname: Sucena
– volume: 158
  start-page: 49
  year: 1998
  end-page: 59
  ident: bib1190
  article-title: Survival and FEV
  publication-title: Am J Respir Crit Care Med.
– volume: 51
  start-page: 185
  year: 2015
  end-page: 192
  ident: bib1405
  article-title: Indications for active case searches and intravenous alpha-1 antitrypsin treatment for patients with alpha-1 antitrypsin deficiency chronic pulmonary obstructive disease: an update
  publication-title: Arch Bronconeumol.
  contributor:
    fullname: Cadenas
– volume: 42
  start-page: 290
  year: 2006
  end-page: 298
  ident: bib1455
  article-title: [Alpha1-antitrypsin deficiency: situation in Spain and development of a screening program]
  publication-title: Arch Bronconeumol.
  contributor:
    fullname: Miravitlles
– volume: 20
  start-page: 116
  year: 2014
  end-page: 127
  ident: bib0865
  article-title: The molecular and cellular pathology of alpha(1)-antitrypsin deficiency
  publication-title: Trends Mol Med.
  contributor:
    fullname: Lomas
– volume: 154
  start-page: 1718
  year: 1996
  end-page: 1725
  ident: bib1070
  article-title: Clinical features of individuals with PI*SZ phenotype of alpha 1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Crystal
– volume: 189
  start-page: 419
  year: 2014
  end-page: 427
  ident: bib1075
  article-title: Clarification of the risk of chronic obstructive pulmonary disease in alpha1-antitrypsin deficiency PiMZ heterozygotes
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Lasky-Su
– volume: 25
  start-page: 788
  year: 1990
  end-page: 792
  ident: bib1315
  article-title: Heterozygous MZ alpha-1-antitrypsin deficiency in adults with chronic liver disease
  publication-title: Scand J Gastroenterol.
  contributor:
    fullname: Fagerhol
– volume: 33
  start-page: 1345
  year: 2009
  end-page: 1353
  ident: bib1570
  article-title: Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  contributor:
    fullname: Shaker
– volume: 4
  start-page: 443
  year: 2009
  end-page: 452
  ident: bib1560
  article-title: Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank
  publication-title: Int J Chron Obstruct Pulmon Dis.
  contributor:
    fullname: Brantly
– volume: 60
  start-page: 81
  year: 2012
  end-page: 97
  ident: bib0885
  article-title: Alpha-1 antitrypsin: a potent anti-inflammatory and potential novel therapeutic agent
  publication-title: Arch Immunol Ther Exp.
  contributor:
    fullname: Reeves
– volume: 128
  start-page: 1989
  year: 2005
  end-page: 1994
  ident: bib0985
  article-title: Delay in diagnosis of alpha1-antitrypsin deficiency: a continuing problem
  publication-title: Chest.
  contributor:
    fullname: Strange
– volume: 26
  start-page: 616
  year: 2005
  end-page: 622
  ident: bib1450
  article-title: Results of a case-detection programme for alpha1-antitrypsin deficiency in COPD patients
  publication-title: Eur Respir J.
  contributor:
    fullname: Miravitlles
– volume: 63
  start-page: 32
  year: 2014
  end-page: 35
  ident: bib1225
  article-title: Alpha-1 antitrypsin deficiency and spontaneous pneumothorax: possible causal relationship
  publication-title: Pneumologia.
  contributor:
    fullname: Sakalauskas
– volume: 19
  start-page: 109
  year: 2012
  end-page: 116
  ident: bib1610
  article-title: Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline
  publication-title: Can Respir J.
  contributor:
    fullname: Ford
– volume: 2
  start-page: 96
  issue: 2
  year: 1995
  ident: 10.1016/j.pulmoe.2018.09.004_bib0870
  article-title: What do dysfunctional serpins tell us about molecular mobility and disease?
  publication-title: Nat Struct Biol.
  doi: 10.1038/nsb0295-96
  contributor:
    fullname: Stein
– volume: 8
  start-page: 605
  issue: 9
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1485
  article-title: Fibroscan (transient elastography) for the measurement of liver fibrosis
  publication-title: Gastroenterol Hepatol.
  contributor:
    fullname: Afdhal
– volume: 34
  start-page: 514
  issue: 7
  year: 2008
  ident: 10.1016/j.pulmoe.2018.09.004_bib1360
  article-title: [Alpha-1 antitrypsin deficiency: diagnosis and treatment]
  publication-title: J Bras Pneumol.
  doi: 10.1590/S1806-37132008000700012
  contributor:
    fullname: Camelier
– volume: 25
  start-page: 788
  issue: 8
  year: 1990
  ident: 10.1016/j.pulmoe.2018.09.004_bib1315
  article-title: Heterozygous MZ alpha-1-antitrypsin deficiency in adults with chronic liver disease
  publication-title: Scand J Gastroenterol.
  doi: 10.3109/00365529008999216
  contributor:
    fullname: Bell
– volume: 154
  start-page: 1718
  issue: 6 Pt 1
  year: 1996
  ident: 10.1016/j.pulmoe.2018.09.004_bib1070
  article-title: Clinical features of individuals with PI*SZ phenotype of alpha 1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/ajrccm.154.6.8970361
  contributor:
    fullname: Turino
– volume: 5
  start-page: 391
  year: 2011
  ident: 10.1016/j.pulmoe.2018.09.004_bib1495
  article-title: Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis
  publication-title: Drug Des Devel Ther.
  contributor:
    fullname: Dickens
– volume: 87
  start-page: 693
  issue: 6
  year: 1994
  ident: 10.1016/j.pulmoe.2018.09.004_bib1355
  article-title: Alpha 1-antitrypsin phenotypes in patients with anti-neutrophil cytoplasmic antibody-positive vasculitis
  publication-title: Clin Sci.
  doi: 10.1042/cs0870693
  contributor:
    fullname: Lhotta
– volume: 78
  start-page: 147
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib0880
  article-title: Conformational pathology of the serpins: themes, variations, and therapeutic strategies
  publication-title: Annu Rev Biochem.
  doi: 10.1146/annurev.biochem.78.082107.133320
  contributor:
    fullname: Gooptu
– volume: 59
  start-page: 363
  issue: 5
  year: 2004
  ident: 10.1016/j.pulmoe.2018.09.004_bib1000
  article-title: Alpha 1-antitrypsin: more than just deficiency
  publication-title: Thorax.
  doi: 10.1136/thx.2004.023572
  contributor:
    fullname: Stockley
– volume: 53
  start-page: 265
  issue: 4
  year: 1998
  ident: 10.1016/j.pulmoe.2018.09.004_bib1065
  article-title: Clinical features and prognosis of life time non-smokers with severe alpha 1-antitrypsin deficiency
  publication-title: Thorax.
  doi: 10.1136/thx.53.4.265
  contributor:
    fullname: Seersholm
– volume: 118
  start-page: 1480
  issue: 5
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib1600
  article-title: Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data
  publication-title: Chest.
  doi: 10.1378/chest.118.5.1480
  contributor:
    fullname: Lieberman
– volume: 44
  start-page: 1349
  issue: 11
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1290
  article-title: The liver in 30-year-old individuals with alpha(1)-antitrypsin deficiency
  publication-title: Scand J Gastroenterol.
  doi: 10.3109/00365520903296669
  contributor:
    fullname: Bernspang
– volume: 61
  start-page: 1059
  issue: 12
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1685
  article-title: Alpha-1-antitrypsin deficiency: optimal therapeutic regimen based on population pharmacokinetics
  publication-title: Thorax.
  doi: 10.1136/thx.2005.057943
  contributor:
    fullname: Soy
– volume: 7
  start-page: 366
  issue: 5
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1230
  article-title: Asthma and COPD in alpha-1 antitrypsin deficiency
  publication-title: Evidence for the Dutch hypothesis. COPD.
  contributor:
    fullname: Eden
– volume: 123
  start-page: 1435
  issue: 5
  year: 2003
  ident: 10.1016/j.pulmoe.2018.09.004_bib1090
  article-title: A longitudinal study of alpha1-antitrypsin phenotypes and decline in FEV1 in a community population
  publication-title: Chest.
  doi: 10.1378/chest.123.5.1435
  contributor:
    fullname: Silva
– volume: 117
  start-page: 415
  issue: 2
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib1235
  article-title: Distribution of alpha(1)-antitrypsin alleles in patients with bronchiectasis
  publication-title: Chest.
  doi: 10.1378/chest.117.2.415
  contributor:
    fullname: Cuvelier
– volume: 11
  start-page: 136
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1575
  article-title: Therapeutic efficacy of alpha-1 antitrypsin augmentation therapy on the loss of lung tissue: an integrated analysis of 2 randomised clinical trials using computed tomography densitometry
  publication-title: Respir Res.
  doi: 10.1186/1465-9921-11-136
  contributor:
    fullname: Stockley
– volume: 128
  start-page: 1179
  issue: 3
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib0980
  article-title: Trends in the diagnosis of symptomatic patients with alpha1-antitrypsin deficiency between 1968 and 2003
  publication-title: Chest.
  doi: 10.1378/chest.128.3.1179
  contributor:
    fullname: Campos
– volume: 22
  start-page: 222
  issue: 4
  year: 2016
  ident: 10.1016/j.pulmoe.2018.09.004_bib1245
  article-title: Recommendations for aetiological diagnosis of bronchiectasis
  publication-title: Rev Port Pneumol.
– volume: 20
  start-page: 319
  issue: 2
  year: 2002
  ident: 10.1016/j.pulmoe.2018.09.004_bib1440
  article-title: Screening for alpha1-Pi deficiency in patients with lung diseases
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.02.02012001
  contributor:
    fullname: Wencker
– volume: 63
  start-page: 32
  issue: 1
  year: 2014
  ident: 10.1016/j.pulmoe.2018.09.004_bib1225
  article-title: Alpha-1 antitrypsin deficiency and spontaneous pneumothorax: possible causal relationship
  publication-title: Pneumologia.
  contributor:
    fullname: Serapinas
– volume: 123
  start-page: 1425
  issue: 5
  year: 2003
  ident: 10.1016/j.pulmoe.2018.09.004_bib1695
  article-title: Augmentation therapy with alpha1-antitrypsin: patterns of use and adverse events
  publication-title: Chest.
  doi: 10.1378/chest.123.5.1425
  contributor:
    fullname: Stoller
– volume: 100
  start-page: 463
  issue: 3
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1395
  article-title: Diagnostic flow chart for targeted detection of alpha1-antitrypsin deficiency
  publication-title: Respir Med.
  doi: 10.1016/j.rmed.2005.06.009
  contributor:
    fullname: Corda
– volume: 170
  start-page: 1172
  issue: 11
  year: 2004
  ident: 10.1016/j.pulmoe.2018.09.004_bib1130
  article-title: Pattern of emphysema distribution in alpha1-antitrypsin deficiency influences lung function impairment
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.200406-761OC
  contributor:
    fullname: Parr
– volume: 2
  start-page: A809
  issue: abstracts issue
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib1670
  article-title: Can the intervals of exogenous alpha-1-antitrypsin (AAT) administration be lengthened?
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: De la Roza
– volume: 60
  start-page: 81
  issue: 2
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib0885
  article-title: Alpha-1 antitrypsin: a potent anti-inflammatory and potential novel therapeutic agent
  publication-title: Arch Immunol Ther Exp.
  doi: 10.1007/s00005-012-0162-5
  contributor:
    fullname: Bergin
– volume: 61
  start-page: 461
  issue: 6
  year: 1994
  ident: 10.1016/j.pulmoe.2018.09.004_bib0990
  article-title: Physical and social impact of alpha 1-antitrypsin deficiency: results of a survey
  publication-title: Cleve Clin J Med.
  doi: 10.3949/ccjm.61.6.461
  contributor:
    fullname: Stoller
– volume: 42
  start-page: 645
  issue: 12
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1250
  article-title: Committee on the National Registry of Individuals with Alpha-1 Antitrypsin D. [Guidelines for the diagnosis and management of alpha-1 antitrypsin deficiency]
  publication-title: Arch Bronconeumol
  doi: 10.1157/13095974
  contributor:
    fullname: Vidal
– volume: 204
  start-page: 345
  issue: 5
  year: 1978
  ident: 10.1016/j.pulmoe.2018.09.004_bib1015
  article-title: Natural history and life expectancy in severe alpha1-antitrypsin deficiency
  publication-title: Pi Z. Acta Med Scand.
  doi: 10.1111/j.0954-6820.1978.tb08452.x
  contributor:
    fullname: Larsson
– volume: 59
  start-page: 843
  issue: 10
  year: 2004
  ident: 10.1016/j.pulmoe.2018.09.004_bib1085
  article-title: Chronic obstructive pulmonary disease in alpha1-antitrypsin PI MZ heterozygotes: a meta-analysis
  publication-title: Thorax.
  doi: 10.1136/thx.2004.022541
  contributor:
    fullname: Hersh
– volume: 156
  start-page: 248
  issue: 1
  year: 1997
  ident: 10.1016/j.pulmoe.2018.09.004_bib1475
  article-title: Quantification of pulmonary emphysema from lung computed tomography images
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/ajrccm.156.1.9606093
  contributor:
    fullname: Uppaluri
– ident: 10.1016/j.pulmoe.2018.09.004_bib0940
– volume: 50
  issue: 5
  year: 2017
  ident: 10.1016/j.pulmoe.2018.09.004_bib1430
  article-title: European Respiratory Society statement: diagnosis and treatment of pulmonary disease in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  doi: 10.1183/13993003.00610-2017
  contributor:
    fullname: Miravitlles
– volume: 9
  start-page: 565
  issue: 7
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib1340
  article-title: Panniculitis revealing qualitative alpha 1 antitrypsine deficiency (MS variant)
  publication-title: Eur J Dermatol.
  contributor:
    fullname: Loche
– volume: 57
  start-page: 705
  issue: 8
  year: 2002
  ident: 10.1016/j.pulmoe.2018.09.004_bib1465
  article-title: Respiratory symptoms and lung function in young adults with severe alpha(1)-antitrypsin deficiency (PiZZ)
  publication-title: Thorax.
  doi: 10.1136/thorax.57.8.705
  contributor:
    fullname: Piitulainen
– volume: 107
  start-page: 1400
  issue: 9
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib0975
  article-title: Alpha1-antitrypsin deficiency - diagnostic testing and disease awareness in Germany and Italy
  publication-title: Respir Med.
  doi: 10.1016/j.rmed.2013.04.023
  contributor:
    fullname: Greulich
– volume: 128
  start-page: 1989
  issue: 4
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib0985
  article-title: Delay in diagnosis of alpha1-antitrypsin deficiency: a continuing problem
  publication-title: Chest.
  doi: 10.1378/chest.128.4.1989
  contributor:
    fullname: Stoller
– volume: 314
  start-page: 736
  issue: 12
  year: 1986
  ident: 10.1016/j.pulmoe.2018.09.004_bib1275
  article-title: Risk of cirrhosis and primary liver cancer in alpha 1-antitrypsin deficiency
  publication-title: N Engl J Med.
  doi: 10.1056/NEJM198603203141202
  contributor:
    fullname: Eriksson
– volume: 103
  start-page: 1498
  issue: 10
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1375
  article-title: Alpha-1-antitrypsin deficiency in Madeira (Portugal): the highest prevalence in the world
  publication-title: Respir Med.
  doi: 10.1016/j.rmed.2009.04.012
  contributor:
    fullname: Spinola
– volume: 87
  start-page: 1120
  issue: 11
  year: 1998
  ident: 10.1016/j.pulmoe.2018.09.004_bib1145
  article-title: Effect of environmental and clinical factors on lung function and respiratory symptoms in adolescents with alpha1-antitrypsin deficiency
  publication-title: Acta paediatr.
  doi: 10.1111/j.1651-2227.1998.tb00918.x
  contributor:
    fullname: Piitulainen
– volume: 165
  start-page: 1494
  issue: 11
  year: 2002
  ident: 10.1016/j.pulmoe.2018.09.004_bib1535
  article-title: The effect of augmentation therapy on bronchial inflammation in alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.2109013
  contributor:
    fullname: Stockley
– volume: 20
  start-page: 835
  issue: 7
  year: 1985
  ident: 10.1016/j.pulmoe.2018.09.004_bib1310
  article-title: Chronic ‘cryptogenic’ liver disease and malignant hepatoma in intermediate alpha 1-antitrypsin deficiency identified by a Pi Z-specific monoclonal antibody
  publication-title: Scand J Gastrenterol.
  doi: 10.3109/00365528509088831
  contributor:
    fullname: Carlson
– volume: 62
  start-page: 76
  issue: 2
  year: 1995
  ident: 10.1016/j.pulmoe.2018.09.004_bib1540
  article-title: Preliminary evidence that augmentation therapy diminishes degradation of cross-linked elastin in alpha-1-antitrypsin-deficient humans
  publication-title: Respiration.
  doi: 10.1159/000196395
  contributor:
    fullname: Stone
– volume: 110
  start-page: 42S
  issue: 6 Suppl
  year: 1996
  ident: 10.1016/j.pulmoe.2018.09.004_bib1045
  article-title: A 30-year perspective on alpha 1-antitrypsin deficiency
  publication-title: Chest.
  contributor:
    fullname: Eriksson
– volume: 138
  start-page: 1125
  issue: 5
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1080
  article-title: alpha(1)-Antitrypsin protease inhibitor MZ heterozygosity is associated with airflow obstruction in two large cohorts
  publication-title: Chest.
  doi: 10.1378/chest.10-0746
  contributor:
    fullname: Sorheim
– volume: 8
  start-page: 989
  issue: 10
  year: 1996
  ident: 10.1016/j.pulmoe.2018.09.004_bib1335
  article-title: Risk of hepatobiliary disease in adults with severe alpha 1-antitrypsin deficiency (PiZZ): is chronic viral hepatitis B or C an additional risk factor for cirrhosis and hepatocellular carcinoma?
  publication-title: Eur J Gastroenterol Hepatol.
  doi: 10.1097/00042737-199610000-00010
  contributor:
    fullname: Elzouki
– ident: 10.1016/j.pulmoe.2018.09.004_bib1665
– volume: 3
  start-page: 910
  issue: 11
  year: 1996
  ident: 10.1016/j.pulmoe.2018.09.004_bib0910
  article-title: Structural explanation for the deficiency of S alpha 1-antitrypsin
  publication-title: Nat Struct Biol.
  doi: 10.1038/nsb1196-910
  contributor:
    fullname: Elliott
– volume: 274
  start-page: 9548
  issue: 14
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib1260
  article-title: A kinetic mechanism for the polymerization of alpha1-antitrypsin
  publication-title: J Biol Chem.
  doi: 10.1074/jbc.274.14.9548
  contributor:
    fullname: Dafforn
– volume: 10
  start-page: 2260
  issue: 10
  year: 1997
  ident: 10.1016/j.pulmoe.2018.09.004_bib1550
  article-title: Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency?
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.97.10102260
  contributor:
    fullname: Seersholm
– volume: 28
  start-page: 1058
  issue: 4
  year: 1998
  ident: 10.1016/j.pulmoe.2018.09.004_bib1320
  article-title: Increased risk of chronic liver failure in adults with heterozygous alpha1-antitrypsin deficiency
  publication-title: Hepatology.
  doi: 10.1002/hep.510280421
  contributor:
    fullname: Graziadei
– volume: 34
  start-page: 1
  issue: 1
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1655
  article-title: A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation
  publication-title: J Heart Lung Transplant.
  doi: 10.1016/j.healun.2014.06.014
  contributor:
    fullname: Weill
– volume: 176
  start-page: 1215
  issue: 12
  year: 2007
  ident: 10.1016/j.pulmoe.2018.09.004_bib1240
  article-title: Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.200703-489OC
  contributor:
    fullname: Parr
– volume: 111
  start-page: 394
  issue: 2
  year: 1997
  ident: 10.1016/j.pulmoe.2018.09.004_bib1005
  publication-title: Chest
  doi: 10.1378/chest.111.2.394
  contributor:
    fullname: McElvaney
– volume: 10
  start-page: 3
  issue: Suppl 1
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib0965
  article-title: The electrophoretic alpha1-globulin pattern of serum in alpha1-antitrypsin deficiency. 1963
  publication-title: COPD
  doi: 10.3109/15412555.2013.771956
  contributor:
    fullname: Laurell
– volume: 160
  start-page: 1468
  issue: 5 Pt 1
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib1565
  article-title: A randomized clinical trial of alpha(1)-antitrypsin augmentation therapy
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/ajrccm.160.5.9901055
  contributor:
    fullname: Dirksen
– volume: 11
  start-page: 428
  issue: 2
  year: 1998
  ident: 10.1016/j.pulmoe.2018.09.004_bib1690
  article-title: Long-term treatment of alpha1-antitrypsin deficiency-related pulmonary emphysema with human alpha1-antitrypsin
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.98.11020428
  contributor:
    fullname: Wencker
– volume: 10
  start-page: 575
  issue: 6
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1270
  article-title: Diagnosis and management of patients with alpha1-antitrypsin (A1AT) deficiency
  publication-title: Clin Gastroenterol Hepatol.
  doi: 10.1016/j.cgh.2011.12.028
  contributor:
    fullname: Nelson
– volume: 33
  start-page: 1338
  issue: 6
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1185
  article-title: Rate of progression of lung function impairment in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.00061208
  contributor:
    fullname: Dawkins
– volume: 10
  start-page: 226
  issue: 2
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1100
  article-title: Differences in adjustment between individuals with alpha-1 antitrypsin deficiency (AATD)-associated COPD and non-AATD COPD
  publication-title: COPD.
  doi: 10.3109/15412555.2012.719049
  contributor:
    fullname: Holm
– volume: 170
  start-page: 883
  issue: 8
  year: 2004
  ident: 10.1016/j.pulmoe.2018.09.004_bib1050
  article-title: Influence of calibration on densitometric studies of emphysema progression using computed tomography
  publication-title: Am J Resp Crit Care Med.
  doi: 10.1164/rccm.200403-326OC
  contributor:
    fullname: Parr
– volume: 77
  start-page: 14
  issue: 1
  year: 1983
  ident: 10.1016/j.pulmoe.2018.09.004_bib1115
  article-title: 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
  publication-title: Br J Dis Chest.
  doi: 10.1016/0007-0971(83)90002-5
  contributor:
    fullname: Tobin
– volume: 275
  start-page: 1987
  issue: 3
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib1265
  article-title: Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alpha1 antitrypsin Z
  publication-title: J Biol Chem.
  doi: 10.1074/jbc.275.3.1987
  contributor:
    fullname: Marcus
– volume: 24
  start-page: 46
  issue: 135
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1615
  article-title: Long-term experience in the treatment of alpha1-antitrypsin deficiency: 25 years of augmentation therapy
  publication-title: Eur Respir Rev.
  doi: 10.1183/09059180.10010714
  contributor:
    fullname: Teschler
– volume: 83
  start-page: 241
  issue: 1
  year: 2007
  ident: 10.1016/j.pulmoe.2018.09.004_bib1640
  article-title: National Emphysema Treatment Trial Research G. Lung volume reduction surgery in patients with emphysema and alpha-1 antitrypsin deficiency
  publication-title: Ann Thorac Surg.
  doi: 10.1016/j.athoracsur.2006.07.080
  contributor:
    fullname: Stoller
– volume: 111
  start-page: 982
  issue: 12
  year: 1989
  ident: 10.1016/j.pulmoe.2018.09.004_bib1160
  article-title: Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates
  publication-title: Ann Intern Med.
  doi: 10.7326/0003-4819-111-12-982
  contributor:
    fullname: Silverman
– volume: 25
  start-page: 992
  issue: 6
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib1210
  article-title: Exacerbations in {alpha}1-antitrypsin deficiency
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.05.00074704
  contributor:
    fullname: Needham
– volume: 15
  start-page: 1111
  issue: 6
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib1390
  article-title: Simple method for alpha1-antitrypsin deficiency screening by use of dried blood spot specimens
  publication-title: Eur Respir J.
  doi: 10.1034/j.1399-3003.2000.01521.x
  contributor:
    fullname: Costa
– volume: 13
  start-page: 247
  issue: 2
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib1140
  article-title: Decline in FEV1 related to smoking status in individuals with severe alpha1-antitrypsin deficiency (PiZZ)
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.99.13224799
  contributor:
    fullname: Piitulainen
– volume: 51
  start-page: 185
  issue: 4
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1405
  article-title: Indications for active case searches and intravenous alpha-1 antitrypsin treatment for patients with alpha-1 antitrypsin deficiency chronic pulmonary obstructive disease: an update
  publication-title: Arch Bronconeumol.
  doi: 10.1016/j.arbres.2014.05.008
  contributor:
    fullname: Casas
– volume: 10
  start-page: 473
  issue: 4
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1545
  article-title: Alpha-1 antitrypsin augmentation therapy and biomarkers of elastin degradation
  publication-title: COPD.
  doi: 10.3109/15412555.2013.771163
  contributor:
    fullname: Ma
– volume: 68
  start-page: 1158
  issue: 5
  year: 1981
  ident: 10.1016/j.pulmoe.2018.09.004_bib1520
  article-title: Replacement therapy of alpha 1-antitrypsin deficiency. Reversal of protease-antiprotease imbalance within the alveolar structures of PiZ subjects
  publication-title: J Clin Invest.
  doi: 10.1172/JCI110360
  contributor:
    fullname: Gadek
– start-page: 1
  year: 2018
  ident: 10.1016/j.pulmoe.2018.09.004_bib0950
  article-title: Alpha-1 Antitrypsin Deficiency Detection in a Portuguese Population
  publication-title: COPD.
  contributor:
    fullname: Meira
– volume: 62
  start-page: 806
  issue: 9
  year: 2007
  ident: 10.1016/j.pulmoe.2018.09.004_bib1155
  article-title: Determinants of airflow obstruction in severe alpha-1-antitrypsin deficiency
  publication-title: Thorax.
  doi: 10.1136/thx.2006.075846
  contributor:
    fullname: Demeo
– volume: 153
  start-page: 1240
  issue: 5
  year: 2018
  ident: 10.1016/j.pulmoe.2018.09.004_bib1480
  article-title: The Role of Computed Tomography for the Evaluation of Lung Disease in Alpha-1 Antitrypsin Deficiency
  publication-title: Chest.
  doi: 10.1016/j.chest.2017.11.017
  contributor:
    fullname: Campos
– volume: 93
  start-page: 481
  issue: 7
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib1200
  article-title: Chronic obstructive pulmonary disease, with and without alpha-1-antitrypsin deficiency: management practices in the U.K
  publication-title: Respir Med.
  doi: 10.1016/S0954-6111(99)90091-4
  contributor:
    fullname: Hill
– volume: 4
  start-page: 289
  issue: 5
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1635
  article-title: Augmentation therapy in alpha-1 antitrypsin deficiency: advances and controversies
  publication-title: Ther Adv Respir Dis.
  doi: 10.1177/1753465810373911
  contributor:
    fullname: Tonelli
– volume: 34
  start-page: 346
  issue: 2
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1175
  article-title: Outdoor air pollution is associated with disease severity in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.00087908
  contributor:
    fullname: Wood
– volume: 59
  start-page: 441
  issue: 5
  year: 2004
  ident: 10.1016/j.pulmoe.2018.09.004_bib1255
  article-title: Alpha 1-antitrypsin deficiency. 3: Clinical manifestations and natural history
  publication-title: Thorax
  doi: 10.1136/thx.2003.006510
  contributor:
    fullname: Needham
– volume: 166
  start-page: 377
  issue: 2
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib1030
  article-title: Polymers of Z alpha1-antitrypsin co-localize with neutrophils in emphysematous alveoli and are chemotactic in vivo
  publication-title: Am J Pathol.
  doi: 10.1016/S0002-9440(10)62261-4
  contributor:
    fullname: Mahadeva
– volume: 294
  start-page: 1316
  issue: 24
  year: 1976
  ident: 10.1016/j.pulmoe.2018.09.004_bib1280
  article-title: Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants
  publication-title: N Engl J Med.
  doi: 10.1056/NEJM197606102942404
  contributor:
    fullname: Sveger
– volume: 187
  start-page: 502
  issue: 5
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1710
  article-title: Prevalence and risk factors for liver involvement in individuals with PiZZ-related lung disease
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.201204-0739OC
  contributor:
    fullname: Dawwas
– year: 1988
  ident: 10.1016/j.pulmoe.2018.09.004_bib0945
  article-title: Análise da intervenção do inibidor da a1 das proteases (IA1P) nas respostas inflamatória e imunitária. Estudo na população portuguesa
  publication-title: Faculdade de Medicina de Coimbra.
  contributor:
    fullname: Santos Rosa
– volume: 85
  start-page: 1343
  issue: 5
  year: 1990
  ident: 10.1016/j.pulmoe.2018.09.004_bib1510
  article-title: Alpha 1-antitrypsin deficiency, emphysema, and liver disease. Genetic basis and strategies for therapy
  publication-title: J Clin Invest.
  doi: 10.1172/JCI114578
  contributor:
    fullname: Crystal
– volume: 386
  start-page: 360
  issue: 9991
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1590
  article-title: Intravenous augmentation treatment and lung density in severe alpha1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial
  publication-title: Lancet.
  doi: 10.1016/S0140-6736(15)60860-1
  contributor:
    fullname: Chapman
– volume: 93
  start-page: 169
  issue: 3
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib1435
  article-title: A national program for detection of alpha 1-antitrypsin deficiency in Italy
  publication-title: Respir Med.
  doi: 10.1016/S0954-6111(99)90003-3
  contributor:
    fullname: Luisetti
– volume: 19
  start-page: 109
  issue: 2
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1610
  article-title: Alpha-1 antitrypsin deficiency targeted testing and augmentation therapy: a Canadian Thoracic Society clinical practice guideline
  publication-title: Can Respir J.
  doi: 10.1155/2012/920918
  contributor:
    fullname: Marciniuk
– volume: 7
  start-page: 1985
  issue: 11
  year: 1994
  ident: 10.1016/j.pulmoe.2018.09.004_bib1120
  article-title: Airways obstruction and two year survival in patients with severe alpha 1-antitrypsin deficiency
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.94.07111985
  contributor:
    fullname: Seersholm
– volume: 35
  start-page: 960
  issue: 5
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1380
  article-title: Laboratory testing of individuals with severe alpha1-antitrypsin deficiency in three European centres
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.00069709
  contributor:
    fullname: Miravitlles
– volume: 112
  start-page: 607
  issue: 3
  year: 1997
  ident: 10.1016/j.pulmoe.2018.09.004_bib1675
  article-title: Pharmacokinetic study of alpha1-antitrypsin infusion in alpha1-antitrypsin deficiency
  publication-title: Chest.
  doi: 10.1378/chest.112.3.607
  contributor:
    fullname: Barker
– volume: 33
  start-page: 883
  issue: 6
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib1325
  article-title: Chronic liver disease in heterozygous alpha1-antitrypsin deficiency PiZ
  publication-title: J Hepatol.
  doi: 10.1016/S0168-8278(00)80119-1
  contributor:
    fullname: Fischer
– volume: 80
  start-page: 1366
  issue: 5
  year: 1987
  ident: 10.1016/j.pulmoe.2018.09.004_bib1025
  article-title: Z-type alpha 1-antitrypsin is less competent than M1-type alpha 1-antitrypsin as an inhibitor of neutrophil elastase
  publication-title: J Clin Invest.
  doi: 10.1172/JCI113214
  contributor:
    fullname: Ogushi
– volume: 67
  start-page: 669
  issue: 8
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1420
  article-title: Serum levels and genotype distribution of alpha1-antitrypsin in the general population
  publication-title: Thorax.
  doi: 10.1136/thoraxjnl-2011-201321
  contributor:
    fullname: Ferrarotti
– volume: 122
  start-page: 1818
  issue: 5
  year: 2002
  ident: 10.1016/j.pulmoe.2018.09.004_bib0930
  article-title: Worldwide racial and ethnic distribution of alpha1-antitrypsin deficiency: summary of an analysis of published genetic epidemiologic surveys
  publication-title: Chest.
  doi: 10.1378/chest.122.5.1818
  contributor:
    fullname: de Serres
– volume: 59
  start-page: 259
  issue: 3
  year: 2004
  ident: 10.1016/j.pulmoe.2018.09.004_bib1010
  article-title: Alpha1-antitrypsin deficiency. 2: genetic aspects of alpha(1)-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk
  publication-title: Thorax.
  doi: 10.1136/thx.2003.006502
  contributor:
    fullname: DeMeo
– volume: 20
  start-page: 907
  issue: 8
  year: 1985
  ident: 10.1016/j.pulmoe.2018.09.004_bib1295
  article-title: Liver disease in alpha 1-antitrypsin deficiency. Aspects of incidence and prognosis
  publication-title: Scand J Gastroenterol.
  doi: 10.3109/00365528509088844
  contributor:
    fullname: Eriksson
– volume: 10
  start-page: 1845
  issue: 12
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib0875
  article-title: Phylogeny of the serpin superfamily: implications of patterns of amino acid conservation for structure and function
  publication-title: Genome Res.
  doi: 10.1101/gr.GR-1478R
  contributor:
    fullname: Irving
– volume: 70
  start-page: 939
  issue: 10
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1055
  article-title: PiSZ alpha-1 antitrypsin deficiency (AATD): pulmonary phenotype and prognosis relative to PiZZ AATD and PiMM COPD
  publication-title: Thorax
  doi: 10.1136/thoraxjnl-2015-206906
  contributor:
    fullname: Green
– volume: 185
  start-page: 246
  issue: 3
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib0895
  article-title: A review of alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.201108-1428CI
  contributor:
    fullname: Stoller
– volume: 138
  start-page: 1116
  issue: 5
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1170
  article-title: Accelerated spirometric decline in New York City firefighters with alpha(1)-antitrypsin deficiency
  publication-title: Chest.
  doi: 10.1378/chest.10-0187
  contributor:
    fullname: Banauch
– volume: 6
  start-page: 67
  issue: 2
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1595
  article-title: Investigators of the r EXAsg. Reduction of severe exacerbations and hospitalization-derived costs in alpha-1-antitrypsin-deficient patients treated with alpha-1-antitrypsin augmentation therapy
  publication-title: Ther Adv Respir Dis.
  doi: 10.1177/1753465812438387
  contributor:
    fullname: Barros-Tizon
– volume: 48
  issue: suppl 60
  year: 2016
  ident: 10.1016/j.pulmoe.2018.09.004_bib0955
  article-title: Detection of alpha1-antitrypsin deficiency in a European country without national registry yet
  publication-title: Eur Respir J.
  contributor:
    fullname: da Hora Lopes Meira
– volume: 260
  start-page: 1259
  issue: 9
  year: 1988
  ident: 10.1016/j.pulmoe.2018.09.004_bib1530
  article-title: Biochemical efficacy and safety of monthly augmentation therapy for alpha 1-antitrypsin deficiency
  publication-title: JAMA.
  doi: 10.1001/jama.1988.03410090091037
  contributor:
    fullname: Hubbard
– volume: 304
  start-page: 557
  issue: 10
  year: 1981
  ident: 10.1016/j.pulmoe.2018.09.004_bib1305
  article-title: Heterozygous MZ alpha 1-antitrypsin deficiency in adults with chronic active hepatitis and cryptogenic cirrhosis
  publication-title: N Engl J Med.
  doi: 10.1056/NEJM198103053041001
  contributor:
    fullname: Hodges
– volume: 27
  start-page: 77
  issue: 1
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib0935
  article-title: Estimated numbers and prevalence of PI*S and PI*Z alleles of alpha1-antitrypsin deficiency in European countries
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.06.00062305
  contributor:
    fullname: Blanco
– volume: 13
  start-page: S341
  issue: Suppl 4
  year: 2016
  ident: 10.1016/j.pulmoe.2018.09.004_bib1095
  article-title: Risk of Lung Disease in PI MZ Heterozygotes. Current Status and Future Research Directions
  publication-title: Ann Am Thorac Soc.
  doi: 10.1513/AnnalsATS.201507-437KV
  contributor:
    fullname: Silverman
– volume: 189
  start-page: 419
  issue: 4
  year: 2014
  ident: 10.1016/j.pulmoe.2018.09.004_bib1075
  article-title: Clarification of the risk of chronic obstructive pulmonary disease in alpha1-antitrypsin deficiency PiMZ heterozygotes
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.201311-1984OC
  contributor:
    fullname: Molloy
– ident: 10.1016/j.pulmoe.2018.09.004_bib1505
– volume: 105
  start-page: 1129
  issue: 8
  year: 2011
  ident: 10.1016/j.pulmoe.2018.09.004_bib0860
  article-title: The discovery of alpha1-antitrypsin and its role in health and disease
  publication-title: Respir Med.
  doi: 10.1016/j.rmed.2011.02.002
  contributor:
    fullname: Janciauskiene
– volume: 69
  start-page: 47
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1490
  article-title: Alpha1-antitrypsin deficiency
  publication-title: Eur Respir Monogr.
  contributor:
    fullname: Bruce-Hickman
– volume: 117
  start-page: 421
  issue: 3
  year: 1978
  ident: 10.1016/j.pulmoe.2018.09.004_bib1150
  article-title: alpha1-Antitrypsin deficiency in nonsmokers
  publication-title: Am Rev Respir Dis.
  contributor:
    fullname: Black
– volume: 336
  start-page: 331
  year: 1993
  ident: 10.1016/j.pulmoe.2018.09.004_bib1350
  article-title: Alpha-1-proteinase inhibitor and pulmonary haemorrhage in systemic vasculitis
  publication-title: Adv Exp Med Biol.
  doi: 10.1007/978-1-4757-9182-2_54
  contributor:
    fullname: O’Donoghue
– volume: 26
  start-page: 616
  issue: 4
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib1450
  article-title: Results of a case-detection programme for alpha1-antitrypsin deficiency in COPD patients
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.05.00007305
  contributor:
    fullname: de la Roza
– volume: 104
  start-page: 337
  issue: 3
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib1515
  article-title: Quantum proteolysis by neutrophils: implications for pulmonary emphysema in alpha 1-antitrypsin deficiency
  publication-title: J Clin Invest.
  doi: 10.1172/JCI6092
  contributor:
    fullname: Campbell
– volume: 10
  start-page: 1768
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib0890
  article-title: Organization and expression of the human serpin gene cluster at 14q32.1
  publication-title: Front Biosci.
  doi: 10.2741/1660
  contributor:
    fullname: Marsden
– volume: 168
  start-page: 818
  issue: 7
  year: 2003
  ident: 10.1016/j.pulmoe.2018.09.004_bib0995
  article-title: American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.168.7.818
  contributor:
    fullname: American Thoracic
– volume: 316
  start-page: 1055
  issue: 17
  year: 1987
  ident: 10.1016/j.pulmoe.2018.09.004_bib1525
  article-title: Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema
  publication-title: N Engl J Med.
  doi: 10.1056/NEJM198704233161704
  contributor:
    fullname: Wewers
– volume: 67
  start-page: 556
  issue: 8
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1180
  article-title: Outdoor air pollution is associated with rapid decline of lung function in alpha-1-antitrypsin deficiency
  publication-title: Occup Environ Med.
  doi: 10.1136/oem.2009.047589
  contributor:
    fullname: Wood
– volume: 60
  start-page: 452
  issue: 7
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1220
  article-title: Null allele alpha-1 antitrypsin deficiency: case report of the total pleural covering technique for disease-associated pneumothorax
  publication-title: Gen Thorac Cardiovasc Surg.
  doi: 10.1007/s11748-012-0015-5
  contributor:
    fullname: Kusu
– volume: 8
  start-page: 149
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1705
  article-title: Augmentation therapy for alpha-1 antitrypsin deficiency: towards a personalised approach
  publication-title: Orphanet J Rare Dis.
  doi: 10.1186/1750-1172-8-149
  contributor:
    fullname: Stockley
– volume: 6
  start-page: 177
  issue: 3
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1580
  article-title: Augmentation therapy for alpha1 antitrypsin deficiency: a meta-analysis
  publication-title: COPD.
  doi: 10.1080/15412550902905961
  contributor:
    fullname: Chapman
– volume: 53
  start-page: 49
  issue: 2
  year: 2017
  ident: 10.1016/j.pulmoe.2018.09.004_bib1105
  article-title: Health-Related Quality of Life in Patients With alpha1 Antitrypsin Deficency: A Cross Sectional Study
  publication-title: Arch Bronconeumol.
  doi: 10.1016/j.arbres.2016.05.024
  contributor:
    fullname: Torres Redondo
– volume: 2
  start-page: 16051
  year: 2016
  ident: 10.1016/j.pulmoe.2018.09.004_bib1035
  article-title: alpha1-Antitrypsin deficiency
  publication-title: Nat Rev Dis Primers.
  doi: 10.1038/nrdp.2016.51
  contributor:
    fullname: Greene
– volume: 14
  start-page: 497
  issue: 4
  year: 1991
  ident: 10.1016/j.pulmoe.2018.09.004_bib1285
  article-title: Alpha 1-antitrypsin deficiency and liver disease: clinical presentation, diagnosis and treatment
  publication-title: J Inherit Metab Dis.
  doi: 10.1007/BF01797920
  contributor:
    fullname: Hussain
– volume: 167
  start-page: A222
  year: 2003
  ident: 10.1016/j.pulmoe.2018.09.004_bib1445
  article-title: Statewide targeted screening and detection of AAT deficiency
  publication-title: Am J Respir Crit Care Med.
  contributor:
    fullname: Brantly
– volume: 24
  start-page: 52
  issue: 135
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1425
  article-title: Diagnosing alpha1-antitrypsin deficiency: how to improve the current algorithm
  publication-title: Eur Respir Rev.
  doi: 10.1183/09059180.10010814
  contributor:
    fullname: McElvaney
– volume: 42
  start-page: 290
  issue: 6
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1455
  article-title: [Alpha1-antitrypsin deficiency: situation in Spain and development of a screening program]
  publication-title: Arch Bronconeumol.
  doi: 10.1157/13089541
  contributor:
    fullname: de la Roza
– volume: 42
  start-page: 54
  issue: 1
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1060
  article-title: Clinical phenotypes of Italian and Spanish patients with alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.00104712
  contributor:
    fullname: Piras
– volume: 1
  start-page: 152
  issue: 8421
  year: 1985
  ident: 10.1016/j.pulmoe.2018.09.004_bib1110
  article-title: Smoking, lung function, and alpha 1-antitrypsin deficiency
  publication-title: Lancet.
  doi: 10.1016/S0140-6736(85)91916-6
  contributor:
    fullname: Janus
– volume: 12
  start-page: 2
  issue: Suppl 1
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1135
  article-title: The impact of smoke exposure on the clinical phenotype of alpha-1 antitrypsin deficiency in Ireland: exploiting a national registry to understand a rare disease
  publication-title: COPD.
  doi: 10.3109/15412555.2015.1021913
  contributor:
    fullname: O’Brien
– volume: 11
  start-page: 143
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib1700
  article-title: Alpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes
  publication-title: Ther Clin Risk Manag.
  doi: 10.2147/TCRM.S51474
  contributor:
    fullname: Chotirmall
– volume: 162
  start-page: 553
  issue: 2 Pt 1
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib1165
  article-title: Occupational exposure risks in individuals with PI*Z alpha(1)-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/ajrccm.162.2.9907117
  contributor:
    fullname: Mayer
– volume: 365
  start-page: 2225
  issue: 9478
  year: 2005
  ident: 10.1016/j.pulmoe.2018.09.004_bib0925
  article-title: Alpha1-antitrypsin deficiency
  publication-title: Lancet.
  doi: 10.1016/S0140-6736(05)66781-5
  contributor:
    fullname: Stoller
– volume: 37
  start-page: 487
  issue: 3
  year: 2016
  ident: 10.1016/j.pulmoe.2018.09.004_bib0970
  article-title: alpha1-Antitrypsin Deficiency
  publication-title: Clin Chest Med
  doi: 10.1016/j.ccm.2016.04.011
  contributor:
    fullname: Hatipoglu
– volume: 116
  start-page: 8
  year: 2016
  ident: 10.1016/j.pulmoe.2018.09.004_bib0960
  article-title: Alpha-1-antitrypsin (SERPINA1) mutation spectrum: Three novel variants and haplotype characterization of rare deficiency alleles identified in Portugal
  publication-title: Respir Med.
  doi: 10.1016/j.rmed.2016.05.002
  contributor:
    fullname: Silva
– volume: 61
  start-page: 195
  issue: 4
  year: 1994
  ident: 10.1016/j.pulmoe.2018.09.004_bib1205
  article-title: Serial measurements of FEV1 over 12 years in a patient with alpha-1-protease inhibitor deficiency:influence of augmentation therapy and infections
  publication-title: Respiration.
  doi: 10.1159/000196336
  contributor:
    fullname: Wencker
– volume: 43
  start-page: S30
  issue: Suppl 1
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1330
  article-title: Does the heterozygous state of alpha-1 antitrypsin deficiency have a role in chronic liver diseases? Interim results of a large case-control study
  publication-title: J Pediatr Gastroenterol Nutr.
  doi: 10.1097/01.mpg.0000226387.56612.1e
  contributor:
    fullname: Regev
– volume: 150
  start-page: 267
  issue: 5
  year: 2007
  ident: 10.1016/j.pulmoe.2018.09.004_bib1385
  article-title: Laboratory diagnosis of alpha1-antitrypsin deficiency
  publication-title: Transl Res.
  doi: 10.1016/j.trsl.2007.08.001
  contributor:
    fullname: Ferrarotti
– volume: 218
  start-page: 370
  issue: 4
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1625
  article-title: New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy
  publication-title: Dermatology.
  doi: 10.1159/000202982
  contributor:
    fullname: Gross
– volume: 39
  start-page: 1319
  issue: 6
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1650
  article-title: Multicentre European study for the treatment of advanced emphysema with bronchial valves
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.00019711
  contributor:
    fullname: Ninane
– volume: 167
  start-page: 1387
  issue: 10
  year: 2003
  ident: 10.1016/j.pulmoe.2018.09.004_bib1500
  article-title: Cost-effectiveness analysis of augmentation therapy for severe alpha1-antitrypsin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/rccm.200209-1035OC
  contributor:
    fullname: Gildea
– volume: 79
  start-page: 704
  issue: 7
  year: 2008
  ident: 10.1016/j.pulmoe.2018.09.004_bib1215
  article-title: Spontaneous pneumothorax in flight as first manifestation of alpha-1 antitrypsin deficiency
  publication-title: Aviat Space Environ Med.
  doi: 10.3357/ASEM.2224.2008
  contributor:
    fullname: Lin
– volume: 103
  start-page: 999
  issue: 7
  year: 1999
  ident: 10.1016/j.pulmoe.2018.09.004_bib0915
  article-title: Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis
  publication-title: J Clin Invest.
  doi: 10.1172/JCI4874
  contributor:
    fullname: Mahadeva
– volume: 108
  start-page: 20
  issue: 1
  year: 2001
  ident: 10.1016/j.pulmoe.2018.09.004_bib1370
  article-title: Patterns of haplotype diversity within the serpin gene cluster at 14q32.1: insights into the natural history of the alpha1-antitrypsin polymorphism
  publication-title: Hum Genet.
  doi: 10.1007/s004390000434
  contributor:
    fullname: Seixas
– volume: 128
  start-page: 482
  issue: 3
  year: 2007
  ident: 10.1016/j.pulmoe.2018.09.004_bib1415
  article-title: Evaluation of an integrative diagnostic algorithm for the identification of people at risk for alpha1-antitrypsin deficiency
  publication-title: Am J Clin Pathol.
  doi: 10.1309/44J4KBCFQ8E9D1B8
  contributor:
    fullname: Bornhorst
– volume: 32
  start-page: 965
  issue: 10
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1660
  article-title: The Registry of the International Society for Heart and Lung Transplantation: Thirtieth Adult Lung and Heart-Lung Transplant Report--2013; focus theme: age
  publication-title: J Heart Lung Transplant.
  doi: 10.1016/j.healun.2013.08.007
  contributor:
    fullname: Yusen
– volume: 118
  start-page: 867
  issue: 3
  year: 2000
  ident: 10.1016/j.pulmoe.2018.09.004_bib1125
  article-title: Normal diffusing capacity in patients with PiZ alpha(1)-antitrypsin deficiency, severe airflow obstruction, and significant radiographic emphysema
  publication-title: Chest
  doi: 10.1378/chest.118.3.867
  contributor:
    fullname: Wilson
– volume: 43
  start-page: 1329
  issue: 6
  year: 1993
  ident: 10.1016/j.pulmoe.2018.09.004_bib1345
  article-title: Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis
  publication-title: Kidney Int.
  doi: 10.1038/ki.1993.186
  contributor:
    fullname: Esnault
– volume: 45
  start-page: 260
  issue: 4
  year: 1992
  ident: 10.1016/j.pulmoe.2018.09.004_bib1470
  article-title: High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency
  publication-title: Clin Radiol.
  doi: 10.1016/S0009-9260(05)80011-X
  contributor:
    fullname: Guest
– volume: 42
  start-page: 553
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1680
  article-title: Pharmacokinetics of α1-Antitrypsin Replacement Therapy in Severe Congenital Emphysema
  publication-title: Arch Bronconeumol.
  doi: 10.1016/S1579-2129(06)60583-1
  contributor:
    fullname: Vidal
– volume: 32
  start-page: 500
  issue: 2
  year: 1993
  ident: 10.1016/j.pulmoe.2018.09.004_bib1020
  article-title: Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin
  publication-title: Biochemistry.
  doi: 10.1021/bi00053a014
  contributor:
    fullname: Lomas
– volume: 7
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1585
  article-title: Intravenous alpha-1 antitrypsin augmentation therapy for treating patients with alpha-1 antitrypsin deficiency and lung disease
  publication-title: Cochrane Database Syst Rev.
  doi: 10.1002/14651858.CD007851.pub2
  contributor:
    fullname: Gotzsche
– volume: 12
  start-page: 36
  issue: Suppl 1
  year: 2015
  ident: 10.1016/j.pulmoe.2018.09.004_bib0920
  article-title: The Clinical Profile of Subjects Included in the Swedish National Register on Individuals with Severe Alpha 1-Antitrypsin deficiency
  publication-title: COPD.
  doi: 10.3109/15412555.2015.1021909
  contributor:
    fullname: Piitulainen
– volume: 7
  start-page: 213
  issue: 3
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1605
  article-title: Recent advances in alpha-1-antitrypsin deficiency-related lung disease
  publication-title: Expert Rev Respir Med.
  doi: 10.1586/ers.13.20
  contributor:
    fullname: Brebner
– volume: 52
  start-page: 2236
  issue: 12
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1410
  article-title: Diagnosis of alpha-1-antitrypsin deficiency: An algorithm of quantification, genotyping, and phenotyping
  publication-title: Clin Chem.
  doi: 10.1373/clinchem.2006.072991
  contributor:
    fullname: Snyder
– volume: 158
  start-page: 49
  issue: 1
  year: 1998
  ident: 10.1016/j.pulmoe.2018.09.004_bib1190
  article-title: Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/ajrccm.158.1.9712017
– volume: 156
  start-page: 68
  issue: 1
  year: 1997
  ident: 10.1016/j.pulmoe.2018.09.004_bib1460
  article-title: Atopy, asthma, and emphysema in patients with severe alpha-1-antitrypysin deficiency
  publication-title: Am J Respir Crit Care Med.
  doi: 10.1164/ajrccm.156.1.9508014
  contributor:
    fullname: Eden
– volume: 20
  start-page: 116
  issue: 2
  year: 2014
  ident: 10.1016/j.pulmoe.2018.09.004_bib0865
  article-title: The molecular and cellular pathology of alpha(1)-antitrypsin deficiency
  publication-title: Trends Mol Med.
  doi: 10.1016/j.molmed.2013.10.007
  contributor:
    fullname: Gooptu
– volume: 184
  start-page: 1365
  issue: 12
  year: 2012
  ident: 10.1016/j.pulmoe.2018.09.004_bib1620
  article-title: Alpha-1 antitrypsin deficiency: a commonly overlooked cause of lung disease
  publication-title: Can Med Assoc J.
  doi: 10.1503/cmaj.111749
  contributor:
    fullname: Brode
– volume: 363
  start-page: 1233
  issue: 13
  year: 2010
  ident: 10.1016/j.pulmoe.2018.09.004_bib1645
  article-title: A randomized study of endobronchial valves for advanced emphysema
  publication-title: N Engl J Med.
  doi: 10.1056/NEJMoa0900928
  contributor:
    fullname: Sciurba
– volume: 119
  start-page: 737
  issue: 3
  year: 2001
  ident: 10.1016/j.pulmoe.2018.09.004_bib1555
  article-title: Wissenschaftliche Arbeitsgemeinschaft zur Therapie von L. Longitudinal follow-up of patients with alpha(1)-protease inhibitor deficiency before and during therapy with IV alpha(1)-protease inhibitor
  publication-title: Chest.
  doi: 10.1378/chest.119.3.737
  contributor:
    fullname: Wencker
– volume: 41
  start-page: 1157
  issue: 12
  year: 1988
  ident: 10.1016/j.pulmoe.2018.09.004_bib1195
  article-title: Lung function, smoking and survival in severe alpha 1-antitrypsin deficiency, PiZZ
  publication-title: J Clin Epidemiol.
  doi: 10.1016/0895-4356(88)90019-4
  contributor:
    fullname: Wu
– volume: 43
  start-page: 194
  issue: 1
  year: 2006
  ident: 10.1016/j.pulmoe.2018.09.004_bib1300
  article-title: Hepatocellular carcinoma in a 12-year-old child with PiZZ alpha1-antitrypsin deficiency
  publication-title: Hepatology.
  doi: 10.1002/hep.21009
  contributor:
    fullname: Hadzic
– volume: 276
  start-page: 311
  issue: 4
  year: 2014
  ident: 10.1016/j.pulmoe.2018.09.004_bib0905
  article-title: Role of alpha-1 antitrypsin in human health and disease
  publication-title: J Intern Med.
  doi: 10.1111/joim.12239
  contributor:
    fullname: de Serres
– volume: 9
  start-page: 99
  issue: 1
  year: 2007
  ident: 10.1016/j.pulmoe.2018.09.004_bib1400
  article-title: Rapid and inexpensive detection of alpha1-antitrypsin deficiency-related alleles S and Z by a real-time polymerase chain reaction suitable for a large-scale population-based screening
  publication-title: J Mol Diagn.
  doi: 10.2353/jmoldx.2007.060048
  contributor:
    fullname: Kaczor
– volume: 3
  start-page: 668
  issue: 3
  year: 2016
  ident: 10.1016/j.pulmoe.2018.09.004_bib1630
  article-title: The diagnosis and management of alpha-1 antitrypsin deficiency in the adult
  publication-title: Chronic Obstr Pulm Dis (Miami).
  doi: 10.15326/jcopdf.3.3.2015.0182
  contributor:
    fullname: Sandhaus
– volume: 309
  start-page: 694
  issue: 12
  year: 1983
  ident: 10.1016/j.pulmoe.2018.09.004_bib0900
  article-title: Mutation of antitrypsin to antithrombin. alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder
  publication-title: N Engl J Med.
  doi: 10.1056/NEJM198309223091203
  contributor:
    fullname: Owen
– volume: 110
  start-page: 1585
  issue: 11
  year: 2002
  ident: 10.1016/j.pulmoe.2018.09.004_bib1040
  article-title: Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy
  publication-title: J Clin Invest.
  doi: 10.1172/JCI0216782
  contributor:
    fullname: Lomas
– volume: 756
  start-page: 29
  year: 2013
  ident: 10.1016/j.pulmoe.2018.09.004_bib1365
  article-title: Rapid DNA extraction protocol for detection of alpha-1 antitrypsin deficiency from dried blood spots by real-time PCR
  publication-title: Adv Exp Med Biol.
  doi: 10.1007/978-94-007-4549-0_5
  contributor:
    fullname: Struniawski
– volume: 33
  start-page: 1345
  issue: 6
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1570
  article-title: Exploring the role of CT densitometry: a randomised study of augmentation therapy in alpha1-antitrypsin deficiency
  publication-title: Eur Respir J.
  doi: 10.1183/09031936.00159408
  contributor:
    fullname: Dirksen
– volume: 4
  start-page: 443
  year: 2009
  ident: 10.1016/j.pulmoe.2018.09.004_bib1560
  article-title: Alpha-1-antitrypsin augmentation therapy in deficient individuals enrolled in the Alpha-1 Foundation DNA and Tissue Bank
  publication-title: Int J Chron Obstruct Pulmon Dis.
  doi: 10.2147/COPD.S8577
  contributor:
    fullname: Tonelli
SSID ssj0002002381
Score 2.2684312
Snippet Alpha-1-antitrypsin deficiency (AATD) is a genetic autosomal codominant disorder caused by mutations in SERPINA1 gene. It is one of the most prevalent genetic...
SourceID doaj
proquest
crossref
pubmed
elsevier
SourceType Open Website
Aggregation Database
Index Database
Publisher
StartPage 1
SubjectTerms Algorithms
Alpha 1-Antitrypsin Deficiency
alpha 1-Antitrypsin Deficiency - complications
alpha 1-Antitrypsin Deficiency - diagnosis
alpha 1-Antitrypsin Deficiency - therapy
Consensus
Diagnosis
Humans
Lung Diseases - diagnosis
Lung Diseases - etiology
Lung Diseases - therapy
Patient Selection
Portugal
Practice Guidelines as Topic
Pulmonary Emphysema
Therapeutics
Title Portuguese consensus document for the management of alpha-1-antitrypsin deficiency
URI https://dx.doi.org/10.1016/j.pulmoe.2018.09.004
https://www.ncbi.nlm.nih.gov/pubmed/30473034
https://search.proquest.com/docview/2138052318
https://doaj.org/article/3b0c412319364f6ab6501f605d4fd9b6
Volume 24
hasFullText 1
inHoldings 1
isFullTextHit
isPrint
link http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwrV1LS8QwEA6iIF7Et-uLCF6L7SZttkcVZRFUEIXFS8hTdnHbxbUH_70zSbvoQbx4LSUNM22-bzoz3xBylgnW98KWSWa0SvhA2WTA4HvMde5N7i3TJhTI3hfDZ347ykffRn1hTViUB46GO2c6NRyOVyAaBfeF0kApMg8k3HJvSx3FttPyWzA1Cem1gEU4WQ5esgQFfLq-uVDcNWvepjWqZGaDIHPazmnrcCnI9_-Ap9_oZ4Chmw2y3vJHehH3vUmWXLVFVu_aDPk2ecTS0OYV1nDUYKV0NW_m1Namwd-AFCgqBcpHp4uqF1p7GhpuIcpT2LP7_jmbjytqHWpLYGPmDnm-uX66Gibt3IQELFXwxIgyF8p5xh3EiDZ1qSkR1k0fIlLrvQLzeC8GqrSm0BpA2mF6sF86DnhfOLZLlqu6cvuE5ilTrDCZMExxob3ChhKIMYRPbapc0SNJZzU5i_IYsqsbm8hoZYlWlmkpwco9commXdyL4tbhArhcti6Xf7m8R0TnGNnyhIj_sNT4j8efdn6U8BlhbkRVrm7msp8xHO4AJ1yP7EUHLzaJmUlAen7wH5s_JGu4oVgRc0SWP94bdwy85kOfhFf4hKzcX40eXr4AX5DzyA
link.rule.ids 310,311,315,783,787,792,793,867,2109,23944,23945,25154,27938,27939
linkProvider Directory of Open Access Journals
openUrl ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=proceeding&rft.title=Pulmonology&rft.atitle=Portuguese+consensus+document+for+the+management+of+alpha-1-antitrypsin+deficiency&rft.au=Lopes%2C+A+P&rft.au=Mineiro%2C+M+A&rft.au=Costa%2C+F&rft.au=Gomes%2C+J&rft.date=2018-12-01&rft.eissn=2531-0437&rft.volume=24+Suppl+1&rft.spage=1&rft.epage=21&rft_id=info:doi/10.1016%2Fj.pulmoe.2018.09.004&rft.externalDBID=NO_FULL_TEXT
thumbnail_l http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=2531-0437&client=summon
thumbnail_m http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=2531-0437&client=summon
thumbnail_s http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=2531-0437&client=summon