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Short-term complications after renal transplantation in AFibE526V (p.Glu545Val) amyloidosis

2019-07-25Journal article Restricted access
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dc.contributor.authorTavares, Isabel
dc.contributor.authorSilvano, José
dc.contributor.authorMoreira, Luciana
dc.contributor.authorOliveira, Márcia E.
dc.contributor.authorSilva, Roberto
dc.contributor.authorSampaio, Susana
dc.contributor.authorCosta, Paulo Pinho
dc.contributor.authorLobato, Luísa
dc.date.accessioned2020-05-07T17:57:42Z
dc.date.available2020-05-07T17:57:42Z
dc.date.issued2019-07-25
dc.description.abstractIntroduction: Fibrinogen Aa chain (AFib) amyloidosis is an autosomal dominant disease that typically presents with predominant renal involvement and has a predictable progressive clinical course [1]. The p.Glu545Val AFib gene (FGA) mutation is at the origin of all AFib amyloidosis cases (AFibE526V [p.Glu545Val]) that have been identified in Portugal so far, and accounts for the disease in 17 families. Current treatment of AFib amyloidosis comprises both supportive measures and disease-modifying approaches, such as liver transplantation [2,3]. Renal transplantation (RTx) is a supportive therapy with risk of premature graft loss due to amyloid recurrence or other unexpected complications related to progression of extrarenal amyloid deposition. The aim of this study was to evaluate the outcome of Portuguese patients with AFibE526V (p.Glu545Val) amyloidosis after RTx.
dc.description.sponsorshipThis work was supported by Sociedade Portuguesa de Nefrologiapt_PT
dc.description.sponsorshipThis work was supported by Sociedade Portuguesa de Nefrologia.
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationAmyloid. 2019;26(Supl1):162-163. doi: 10.1080/13506129.2019.1583196. Epub 2019 Jul 25pt_PT
dc.identifier.doi10.1080/13506129.2019.1583196pt_PT
dc.identifier.issn1350-6129
dc.identifier.urihttp://hdl.handle.net/10400.18/6608
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherTaylor & Francispt_PT
dc.relation.publisherversionhttps://www.tandfonline.com/doi/full/10.1080/13506129.2019.1583196?scroll=top&needAccess=truept_PT
dc.rights.urihttp://creativecommons.org/licenses/by-nc/4.0/pt_PT
dc.subjectAdultpt_PT
dc.subjectAgedpt_PT
dc.subjectAmino Acid Substitutionpt_PT
dc.subjectFemalept_PT
dc.subjectHumanspt_PT
dc.subjectMalept_PT
dc.subjectMiddle Agedpt_PT
dc.subjectAmyloidosispt_PT
dc.subjectFibrinogenpt_PT
dc.subjectGraft Rejectionpt_PT
dc.subjectKidney Transplantationpt_PT
dc.subjectMutation, Missensept_PT
dc.subjectDoenças Genéticaspt_PT
dc.titleShort-term complications after renal transplantation in AFibE526V (p.Glu545Val) amyloidosispt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage163pt_PT
oaire.citation.issueSupl1pt_PT
oaire.citation.startPage162pt_PT
oaire.citation.titleAmyloidpt_PT
oaire.citation.volume26pt_PT
rcaap.embargofctDe acordo com política editorial da revista.pt_PT
rcaap.rightsembargoedAccesspt_PT
rcaap.typearticlept_PT

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