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Alpha-1 Antitrypsin Deficiency: Principles of Care

2020-06-01Journal article Open access
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dc.contributor.authorRodrigues, Joana F
dc.contributor.authorMineiro, Alexandra
dc.contributor.authorReis, António
dc.contributor.authorVentura, David G
dc.contributor.authorFernandez-Llimos, Fernando
dc.contributor.authorCosta, Filipa
dc.contributor.authorGomes, Joana
dc.contributor.authorSilva, José Manuel
dc.contributor.authorLopes, Paulo
dc.contributor.authorCordeiro, Carlos Robalo
dc.date.accessioned2021-06-11T11:25:48Z
dc.date.available2021-06-11T11:25:48Z
dc.date.issued2020-06-01
dc.description.abstractAlpha-1 antitrypsin deficiency is an autosomal co-dominant inherited disorder that results in decreased circulating levels of alpha-1 antitrypsin (also known as alpha-1 proteinase inhibitor) and predisposes affected individuals to early onset lung and liver disease. There is currently no cure for alpha-1 antitrypsin deficiency. However, appropriate treatment and a high standard of clinical care can prevent patients from being seriously affected and having to undergo major medical interventions, such as organ transplantation. Beyond managing the symptoms associated with alpha-1 antitrypsin deficiency, alpha-1 proteinase inhibitor therapy is the only treatment for the condition's underlying cause. Early diagnosis is important to ensure efficient therapeutic strategies and to minimize further deterioration of lung function. alpha-1 antitrypsin deficiency is under diagnosed globally, partly because the disease has no unique presenting symptoms. This document was prepared by a Portuguese multidisciplinary group and it aims to set out comprehensive principles of care for Alpha-1 antitrypsin deficiency. These include the importance of registries, the need for clinical research, the need for consistent recommendations (regarding diagnosis, treatment and monitoring), the role of reference centres, the requirement for sustained access to treatment, diagnostic and support services, and the role of patient organizations.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationRodrigues JF, Mineiro A, Reis A, Ventura DG, Fernandez-Llimos F, Costa F, Gomes J, Silva JM, Lopes P, Cordeiro CR. Alpha-1 Antitrypsin Deficiency: Principles of Care. Acta Med Port. 2020 Jun 1;33(6):433-439. doi: 10.20344/amp.12950. Epub 2020 May 31. PMID: 32504520.pt_PT
dc.identifier.doi10.20344/amp.12950pt_PT
dc.identifier.issn1646-0758
dc.identifier.urihttp://hdl.handle.net/10400.16/2471
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherCentro Editor Livreiro da Ordem dos Médicospt_PT
dc.relation.publisherversionhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/12950/5978pt_PT
dc.subjectHealth Care Qualitypt_PT
dc.subjectAccess, and Evaluationpt_PT
dc.subjectProfessional Practicept_PT
dc.subjectStandard of Carept_PT
dc.subjectalpha 1-Antitrypsin Deficiency/therapypt_PT
dc.titleAlpha-1 Antitrypsin Deficiency: Principles of Carept_PT
dc.title.alternativeDeficiência de Alfa-1 Antitripsina: Princípios do Tratamentopt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlacePortugalpt_PT
oaire.citation.endPage439pt_PT
oaire.citation.issue6pt_PT
oaire.citation.startPage433pt_PT
oaire.citation.titleActa medica portuguesapt_PT
oaire.citation.volume33pt_PT
person.familyNameGomes
person.givenNameJoana
person.identifier.orcid0000-0001-6859-2557
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication8909febe-56a5-40d1-9d2b-d0e9b3e3ab21
relation.isAuthorOfPublication.latestForDiscovery8909febe-56a5-40d1-9d2b-d0e9b3e3ab21

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