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

2018-12Journal article Open access
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dc.contributor.authorLopes, A.
dc.contributor.authorMineiro, M.
dc.contributor.authorCosta, F.
dc.contributor.authorGomes, J.
dc.contributor.authorSantos, C.
dc.contributor.authorAntunes, C.
dc.contributor.authorMaia, D.
dc.contributor.authorMelo, R.
dc.contributor.authorCanotilho, M.
dc.contributor.authorMagalhães, E.
dc.contributor.authorVicente, I.
dc.contributor.authorValente, C.
dc.contributor.authorGonçalves, B.
dc.contributor.authorConde, B.
dc.contributor.authorGuimarães, C.
dc.contributor.authorSousa, C.
dc.contributor.authorAmado, J.
dc.contributor.authorBrandão, M.
dc.contributor.authorSucena, M.
dc.contributor.authorOliveira, M.
dc.contributor.authorSeixas, S.
dc.contributor.authorTeixeira, V.
dc.contributor.authorTelo, L.
dc.date.accessioned2020-03-17T13:01:31Z
dc.date.available2020-03-17T13:01:31Z
dc.date.issued2018-12
dc.description.abstractAlpha-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.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationLopes AP, Mineiro MA, Costa F, et al. Portuguese consensus document for the management of alpha-1-antitrypsin deficiency. Pulmonology. 2018;24 Suppl 1:1–21. doi:10.1016/j.pulmoe.2018.09.004pt_PT
dc.identifier.doi10.1016/j.pulmoe.2018.09.004pt_PT
dc.identifier.eissn2531-0437
dc.identifier.issn2531-0429
dc.identifier.urihttp://hdl.handle.net/10400.16/2320
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherSociedade Portuguesa de Pneumologiapt_PT
dc.relation.publisherversionhttps://www.sciencedirect.com/science/article/pii/S2531043718301351?via%3Dihub#!pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/pt_PT
dc.subjectConsensuspt_PT
dc.subjectDiagnosispt_PT
dc.subjectPulmonary Emphysemapt_PT
dc.subjectTherapeuticspt_PT
dc.subjectalpha 1-Antitrypsin Deficiencypt_PT
dc.titlePortuguese consensus document for the management of alpha-1-antitrypsin deficiencypt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceSpainpt_PT
oaire.citation.endPage21pt_PT
oaire.citation.issue24 Suppl 1pt_PT
oaire.citation.startPage1pt_PT
oaire.citation.titlePulmonologypt_PT
oaire.citation.volume24pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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