{"id":"https://openalex.org/W3033705644","doi":"https://doi.org/10.20344/amp.12950","title":"Alpha-1 Antitrypsin Deficiency: Principles of Care","display_name":"Alpha-1 Antitrypsin Deficiency: Principles of Care","publication_year":2020,"publication_date":"2020-06-01","ids":{"openalex":"https://openalex.org/W3033705644","doi":"https://doi.org/10.20344/amp.12950","mag":"3033705644","pmid":"https://pubmed.ncbi.nlm.nih.gov/32504520"},"language":"en","primary_location":{"id":"doi:10.20344/amp.12950","doi":"https://doi.org/10.20344/amp.12950","is_oa":true,"landing_page_url":"https://doi.org/10.20344/amp.12950","pdf_url":null,"source":{"id":"https://openalex.org/S147790641","display_name":"Acta M\u00e9dica Portuguesa","issn_l":"0870-399X","issn":["0870-399X","1646-0758"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","ddh","doaj","dongbi-c","latindex","medline","norway-1","poland-20","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310318481","host_organization_name":"Ordem dos M\u00e9dicos","host_organization_lineage":["https://openalex.org/P4310318481"],"host_organization_lineage_names":["Ordem dos M\u00e9dicos"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Acta M\u00e9dica Portuguesa","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"diamond","oa_url":"https://doi.org/10.20344/amp.12950","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5080937326","display_name":"Joana Rodrigues","orcid":"https://orcid.org/0000-0001-5366-284X","observed_orcids":["https://orcid.org/0000-0001-5366-284X"]},"institutions":[{"id":"https://openalex.org/I4405255969","display_name":"CSL Behring (Portugal)","ror":"https://ror.org/03mcd3h93","country_code":"PT","type":"company","lineage":["https://openalex.org/I4405255969"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Joana F. Rodrigues","raw_affiliation_strings":["CSL Behring. Lisboa. Portugal","CSL Behring. Lisboa"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"CSL Behring. Lisboa. Portugal","institution_ids":["https://openalex.org/I4405255969"]},{"raw_affiliation_string":"CSL Behring. Lisboa","institution_ids":["https://openalex.org/I4405255969"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5079239167","display_name":"Alexandra Mineiro","orcid":"https://orcid.org/0000-0003-4626-299X","observed_orcids":["https://orcid.org/0000-0003-4626-299X"]},"institutions":[{"id":"https://openalex.org/I4210095157","display_name":"Unidade Local de Sa\u00fade de S\u00e3o Jos\u00e9","ror":"https://ror.org/00k6r3f30","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210095157"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Alexandra Mineiro","raw_affiliation_strings":["Pulmonary Department. Centro Hospitalar Lisboa Central. Lisboa. Portugal","Pulmonary Department. Centro Hospitalar Lisboa Central. Lisboa"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Lisboa Central. Lisboa. Portugal","institution_ids":["https://openalex.org/I4210095157"]},{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Lisboa Central. Lisboa","institution_ids":["https://openalex.org/I4210095157"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Ant\u00f3nio Reis","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210087844","display_name":"Centro Hospitalar Tondela-Viseu","ror":"https://ror.org/0025r1k74","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210087844"]},{"id":"https://openalex.org/I4210095157","display_name":"Unidade Local de Sa\u00fade de S\u00e3o Jos\u00e9","ror":"https://ror.org/00k6r3f30","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210095157"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Ant\u00f3nio Reis","raw_affiliation_strings":["Pulmonary Department. Centro Hospitalar Lisboa Central. Lisboa. Portugal","Pulmonary Department. Centro Hospitalar Tondela-Viseu. Viseu"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Lisboa Central. Lisboa. Portugal","institution_ids":["https://openalex.org/I4210095157"]},{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Tondela-Viseu. Viseu","institution_ids":["https://openalex.org/I4210087844"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5046880259","display_name":"David G. Ventura","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4405255969","display_name":"CSL Behring (Portugal)","ror":"https://ror.org/03mcd3h93","country_code":"PT","type":"company","lineage":["https://openalex.org/I4405255969"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"David G. Ventura","raw_affiliation_strings":["CSL Behring. Lisboa. Portugal","CSL Behring. Lisboa"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"CSL Behring. Lisboa. Portugal","institution_ids":["https://openalex.org/I4405255969"]},{"raw_affiliation_string":"CSL Behring. Lisboa","institution_ids":["https://openalex.org/I4405255969"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5026090972","display_name":"Fernando Fern\u00e1ndez-Llim\u00f3s","orcid":"https://orcid.org/0000-0002-8529-9595","observed_orcids":["https://orcid.org/0000-0002-8529-9595"]},"institutions":[{"id":"https://openalex.org/I182534213","display_name":"Universidade do Porto","ror":"https://ror.org/043pwc612","country_code":"PT","type":"education","lineage":["https://openalex.org/I182534213"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Fernando Fernandez-Llimos","raw_affiliation_strings":["Laboratory of Pharmacology. Department of Drug Sciences. Faculty of Pharmacy. University of Porto. Porto. Portugal","Laboratory of Pharmacology. Department of Drug Sciences. Faculty of Pharmacy. University of Porto. Porto"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Laboratory of Pharmacology. Department of Drug Sciences. Faculty of Pharmacy. University of Porto. Porto. Portugal","institution_ids":["https://openalex.org/I182534213"]},{"raw_affiliation_string":"Laboratory of Pharmacology. Department of Drug Sciences. Faculty of Pharmacy. University of Porto. Porto","institution_ids":["https://openalex.org/I182534213"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5037149614","display_name":"Filipa Dias Costa","orcid":"https://orcid.org/0000-0002-9298-4005","observed_orcids":["https://orcid.org/0000-0002-9298-4005"]},"institutions":[{"id":"https://openalex.org/I2802025818","display_name":"Unidade Local de Sa\u00fade de Coimbra","ror":"https://ror.org/04032fz76","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I2802025818"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Filipa Costa","raw_affiliation_strings":["Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra. Portugal","Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra. Portugal","institution_ids":["https://openalex.org/I2802025818"]},{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra","institution_ids":["https://openalex.org/I2802025818"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5065769097","display_name":"Joana Gomes","orcid":"https://orcid.org/0000-0001-6859-2557","observed_orcids":["https://orcid.org/0000-0001-6859-2557","https://orcid.org/0009-0008-7089-5854"]},"institutions":[{"id":"https://openalex.org/I4210154840","display_name":"Centro Hospitalar do Porto","ror":"https://ror.org/04dwpyh46","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210154840","https://openalex.org/I4405257367"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Joana Gomes","raw_affiliation_strings":["Pulmonary Department. Centro Hospitalar do Porto. Porto. Portugal","Pulmonary Department. Centro Hospitalar do Porto. Porto"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar do Porto. Porto. Portugal","institution_ids":["https://openalex.org/I4210154840"]},{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar do Porto. Porto","institution_ids":["https://openalex.org/I4210154840"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5003952748","display_name":"Jos\u00e9 Manuel Silva","orcid":"https://orcid.org/0000-0001-9631-7819","observed_orcids":["https://orcid.org/0000-0001-9631-7819"]},"institutions":[{"id":"https://openalex.org/I4403386602","display_name":"Unidade Local de Sa\u00fade da Guarda EPE","ror":"https://ror.org/01bcvhf32","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4403386602"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Jos\u00e9 Manuel Silva","raw_affiliation_strings":["Pulmonary Department. Unidade Local de Sa\u00fade da Guarda. Hospital Sousa Martins. Guarda. Portugal","Pulmonary Department. Unidade Local de Sa\u00fade da Guarda. Hospital Sousa Martins. Guarda"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pulmonary Department. Unidade Local de Sa\u00fade da Guarda. Hospital Sousa Martins. Guarda. Portugal","institution_ids":["https://openalex.org/I4403386602"]},{"raw_affiliation_string":"Pulmonary Department. Unidade Local de Sa\u00fade da Guarda. Hospital Sousa Martins. Guarda","institution_ids":["https://openalex.org/I4403386602"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Paulo Lopes","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2802025818","display_name":"Unidade Local de Sa\u00fade de Coimbra","ror":"https://ror.org/04032fz76","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I2802025818"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Paulo Lopes","raw_affiliation_strings":["Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra. Portugal","Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra. Portugal","institution_ids":["https://openalex.org/I2802025818"]},{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra","institution_ids":["https://openalex.org/I2802025818"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5103105216","display_name":"Carlos Robalo Cordeiro","orcid":"https://orcid.org/0000-0002-8264-3856","observed_orcids":["https://orcid.org/0000-0002-8264-3856"]},"institutions":[{"id":"https://openalex.org/I2802025818","display_name":"Unidade Local de Sa\u00fade de Coimbra","ror":"https://ror.org/04032fz76","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I2802025818"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Carlos Robalo Cordeiro","raw_affiliation_strings":["Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra. Portugal","Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra. Portugal","institution_ids":["https://openalex.org/I2802025818"]},{"raw_affiliation_string":"Pulmonary Department. Centro Hospitalar Universit\u00e1rio de Coimbra. Coimbra","institution_ids":["https://openalex.org/I2802025818"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":7,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":0.0799,"has_fulltext":true,"cited_by_count":2,"citation_normalized_percentile":{"value":0.30420346,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":93},"biblio":{"volume":"33","issue":"6","first_page":"433","last_page":"439"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10620","display_name":"Protease and Inhibitor Mechanisms","score":0.8003000020980835,"subfield":{"id":"https://openalex.org/subfields/1306","display_name":"Cancer Research"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T10620","display_name":"Protease and Inhibitor Mechanisms","score":0.8003000020980835,"subfield":{"id":"https://openalex.org/subfields/1306","display_name":"Cancer Research"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10143","display_name":"Chronic Obstructive Pulmonary Disease (COPD) Research","score":0.0414000004529953,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11917","display_name":"Coagulation, Bradykinin, Polyphosphates, and Angioedema","score":0.021700000390410423,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/alpha-1-antitrypsin-deficiency","display_name":"alpha-1 antitrypsin deficiency","score":1.0},{"id":"https://openalex.org/keywords/lung-disease","display_name":"lung disease","score":0.8830000162124634},{"id":"https://openalex.org/keywords/liver-disease","display_name":"liver disease","score":0.9980000257492065},{"id":"https://openalex.org/keywords/early-diagnosis","display_name":"early diagnosis","score":0.8880000114440918},{"id":"https://openalex.org/keywords/disease-registries","display_name":"disease registries","score":0.824999988079071},{"id":"https://openalex.org/keywords/reference-center","display_name":"reference center","score":0.640999972820282},{"id":"https://openalex.org/keywords/access-to-treatment","display_name":"access to treatment","score":0.9190000295639038},{"id":"https://openalex.org/keywords/patient-associations","display_name":"patient associations","score":0.8759999871253967},{"id":"https://openalex.org/keywords/multidisciplinary-care","display_name":"multidisciplinary care","score":0.7519999742507935}],"concepts":[{"id":"https://openalex.org/C2780535462","wikidata":"https://www.wikidata.org/wiki/Q622437","display_name":"Alpha 1-antitrypsin deficiency","level":2,"score":0.9008463621139526},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.7109358906745911},{"id":"https://openalex.org/C177713679","wikidata":"https://www.wikidata.org/wiki/Q679690","display_name":"Intensive care medicine","level":1,"score":0.5834035873413086},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.5274629592895508},{"id":"https://openalex.org/C64943373","wikidata":"https://www.wikidata.org/wiki/Q2651003","display_name":"Alpha (finance)","level":4,"score":0.5265924334526062},{"id":"https://openalex.org/C27415008","wikidata":"https://www.wikidata.org/wiki/Q7256382","display_name":"Psychological intervention","level":2,"score":0.43674567341804504},{"id":"https://openalex.org/C3018687534","wikidata":"https://www.wikidata.org/wiki/Q7251225","display_name":"Proteinase inhibitor","level":3,"score":0.4346485435962677},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.2962013781070709},{"id":"https://openalex.org/C141071460","wikidata":"https://www.wikidata.org/wiki/Q40821","display_name":"Surgery","level":1,"score":0.15626588463783264},{"id":"https://openalex.org/C118552586","wikidata":"https://www.wikidata.org/wiki/Q7867","display_name":"Psychiatry","level":1,"score":0.10240420699119568},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.10150167346000671},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.0},{"id":"https://openalex.org/C2775944032","wikidata":"https://www.wikidata.org/wiki/Q22907659","display_name":"Patient satisfaction","level":2,"score":0.0},{"id":"https://openalex.org/C49453240","wikidata":"https://www.wikidata.org/wiki/Q1592163","display_name":"Construct validity","level":3,"score":0.0},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.0}],"mesh":[{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D017410","descriptor_name":"Practice Guidelines as Topic","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D019896","descriptor_name":"alpha 1-Antitrypsin Deficiency","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D059039","descriptor_name":"Standard of Care","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true}],"locations_count":9,"locations":[{"id":"doi:10.20344/amp.12950","doi":"https://doi.org/10.20344/amp.12950","is_oa":true,"landing_page_url":"https://doi.org/10.20344/amp.12950","pdf_url":null,"source":{"id":"https://openalex.org/S147790641","display_name":"Acta M\u00e9dica Portuguesa","issn_l":"0870-399X","issn":["0870-399X","1646-0758"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","ddh","doaj","dongbi-c","latindex","medline","norway-1","poland-20","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310318481","host_organization_name":"Ordem dos M\u00e9dicos","host_organization_lineage":["https://openalex.org/P4310318481"],"host_organization_lineage_names":["Ordem dos M\u00e9dicos"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Acta M\u00e9dica Portuguesa","raw_type":"journal-article"},{"id":"pmid:32504520","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/32504520","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Acta medica portuguesa","raw_type":"Journal Article"},{"id":"pmh:oai:estudogeral.uc.pt:10316/105540","doi":null,"is_oa":true,"landing_page_url":"https://hdl.handle.net/10316/105540","pdf_url":"https://estudogeral.uc.pt/bitstream/10316/105540/1/Alpha1-antitrypsin-deficiency-Principles-of-careActa-Medica-Portuguesa.pdf","source":{"id":"https://openalex.org/S4306402433","display_name":"Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"journal article"},{"id":"pmh:oai:ojs.www.actamedicaportuguesa.com:article/12950","doi":null,"is_oa":true,"landing_page_url":"https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/12950","pdf_url":"https://www.actamedicaportuguesa.com/revista/index.php/amp/article/download/12950/5978","source":{"id":"https://openalex.org/S4306402433","display_name":"Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"0870-399X","raw_type":"journal article"},{"id":"pmh:oai:repositorio-aberto.up.pt:10216/127379","doi":null,"is_oa":true,"landing_page_url":"https://hdl.handle.net/10216/127379","pdf_url":"https://repositorio-aberto.up.pt/bitstream/10216/127379/2/401394.pdf","source":{"id":"https://openalex.org/S4306402433","display_name":"Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"journal article"},{"id":"pmh:oai:doaj.org/article:e8ff5b112f9d43a78988733ebf43cdb4","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/e8ff5b112f9d43a78988733ebf43cdb4","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Acta M\u00e9dica Portuguesa, Vol 33, Iss 6 (2020)","raw_type":"article"},{"id":"pmh:oai:estudogeral.sib.uc.pt:10316/105540","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10316/105540","pdf_url":null,"source":{"id":"https://openalex.org/S4306402070","display_name":"Estudo Geral (Universidad de Coimbra)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I76903346","host_organization_name":"University of Coimbra","host_organization_lineage":["https://openalex.org/I76903346"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:repositorio.chlc.pt:10400.17/3478","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10400.17/3478","pdf_url":null,"source":{"id":"https://openalex.org/S4306402433","display_name":"Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"journal article"},{"id":"pmh:oai:repositorio.ulssa.pt:10400.16/2471","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10400.16/2471","pdf_url":null,"source":{"id":"https://openalex.org/S4306402433","display_name":"Portuguese National Funding Agency for Science, Research and Technology (RCAAP Project by FCT)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"journal article"}],"best_oa_location":{"id":"doi:10.20344/amp.12950","doi":"https://doi.org/10.20344/amp.12950","is_oa":true,"landing_page_url":"https://doi.org/10.20344/amp.12950","pdf_url":null,"source":{"id":"https://openalex.org/S147790641","display_name":"Acta M\u00e9dica Portuguesa","issn_l":"0870-399X","issn":["0870-399X","1646-0758"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","ddh","doaj","dongbi-c","latindex","medline","norway-1","poland-20","russia-white-list-2","vabb-shw"],"host_organization":"https://openalex.org/P4310318481","host_organization_name":"Ordem dos M\u00e9dicos","host_organization_lineage":["https://openalex.org/P4310318481"],"host_organization_lineage_names":["Ordem dos M\u00e9dicos"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Acta M\u00e9dica Portuguesa","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":true,"pdf":true},"content_urls":{"pdf":"https://content.openalex.org/works/W3033705644.pdf","grobid_xml":"https://content.openalex.org/works/W3033705644.grobid-xml"},"referenced_works_count":34,"referenced_works":["https://openalex.org/W1625460891","https://openalex.org/W1786091342","https://openalex.org/W1838370307","https://openalex.org/W1860417895","https://openalex.org/W1902980412","https://openalex.org/W1921725433","https://openalex.org/W1991020689","https://openalex.org/W1994258927","https://openalex.org/W2003339322","https://openalex.org/W2011812800","https://openalex.org/W2047674919","https://openalex.org/W2059401583","https://openalex.org/W2090378276","https://openalex.org/W2096498520","https://openalex.org/W2098750744","https://openalex.org/W2104627946","https://openalex.org/W2128934316","https://openalex.org/W2129208675","https://openalex.org/W2129795348","https://openalex.org/W2131837116","https://openalex.org/W2134377508","https://openalex.org/W2135677683","https://openalex.org/W2168216699","https://openalex.org/W2168351955","https://openalex.org/W2170756834","https://openalex.org/W2416046075","https://openalex.org/W2563542400","https://openalex.org/W2614415839","https://openalex.org/W2901784120","https://openalex.org/W4235230959","https://openalex.org/W4245576698","https://openalex.org/W4290772198","https://openalex.org/W6679204375","https://openalex.org/W7074031227"],"related_works":["https://openalex.org/W2081654271","https://openalex.org/W2147235786","https://openalex.org/W4285728427","https://openalex.org/W6963164132","https://openalex.org/W7014299091","https://openalex.org/W4394690457","https://openalex.org/W1976936881","https://openalex.org/W1641294206","https://openalex.org/W4306177257","https://openalex.org/W4309361330"],"abstract_inverted_index":{"Alpha-1":[0,147],"antitrypsin":[1,17,42,79,112,148],"deficiency":[2,113],"is":[3,36,85,96,114],"an":[4],"autosomal":[5],"co-dominant":[6],"inherited":[7],"disorder":[8],"that":[9],"results":[10],"in":[11],"decreased":[12],"circulating":[13],"levels":[14],"of":[15,51,108,144,154,173,190],"alpha-1":[16,21,41,78,81,111],"(also":[18],"known":[19],"as":[20,69],"proteinase":[22,82],"inhibitor)":[23],"and":[24,32,47,61,103,136,169,184,187],"predisposes":[25],"affected":[26,60],"individuals":[27],"to":[28,63,98,104,139,181],"early":[29],"onset":[30],"lung":[31,109],"liver":[33],"disease.":[34],"There":[35],"currently":[37],"no":[38,123],"cure":[39],"for":[40,89,146,158,163,178],"deficiency.":[43,149],"However,":[44],"appropriate":[45],"treatment":[46,88,168],"a":[48,132],"high":[49],"standard":[50],"clinical":[52,159],"care":[53,145],"can":[54],"prevent":[55],"patients":[56],"from":[57],"being":[58],"seriously":[59],"having":[62],"undergo":[64],"major":[65],"medical":[66],"interventions,":[67],"such":[68],"organ":[70],"transplantation.":[71],"Beyond":[72],"managing":[73],"the":[74,86,90,120,152,156,161,171,176,188],"symptoms":[75],"associated":[76],"with":[77],"deficiency,":[80],"inhibitor":[83],"therapy":[84],"only":[87],"condition's":[91],"underlying":[92],"cause.":[93],"Early":[94],"diagnosis":[95],"important":[97],"ensure":[99],"efficient":[100],"therapeutic":[101],"strategies":[102],"minimize":[105],"further":[106],"deterioration":[107],"function.":[110],"under":[115],"diagnosed":[116],"globally,":[117],"partly":[118],"because":[119],"disease":[121],"has":[122],"unique":[124],"presenting":[125],"symptoms.":[126],"This":[127],"document":[128],"was":[129],"prepared":[130],"by":[131],"Portuguese":[133],"multidisciplinary":[134],"group":[135],"it":[137],"aims":[138],"set":[140],"out":[141],"comprehensive":[142],"principles":[143],"These":[150],"include":[151],"importance":[153],"registries,":[155],"need":[157,162],"research,":[160],"consistent":[164],"recommendations":[165],"(regarding":[166],"diagnosis,":[167],"monitoring),":[170],"role":[172,189],"reference":[174],"centres,":[175],"requirement":[177],"sustained":[179],"access":[180],"treatment,":[182],"diagnostic":[183],"support":[185],"services,":[186],"patient":[191],"organizations.":[192]},"counts_by_year":[{"year":2024,"cited_by_count":1},{"year":2022,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2020-06-12T00:00:00"}
