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Primary biliary cholangitis: a comprehensive overview

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dc.creatorLleo A.spa
dc.creatorMarzorati S.spa
dc.creatorAnaya, Juan-Manuelspa
dc.creatorGershwin M.E.spa
dc.date.accessioned2020-05-26T00:09:58Z
dc.date.available2020-05-26T00:09:58Z
dc.date.created2017spa
dc.description.abstractPrimary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets intrahepatic biliary cells. A puzzling feature of PBC is that the immune attack is predominantly organ specific, although the mitochondrial autoantigens are found in all nucleated cells. The disease results from a combination of genetic and environmental risk factors; however, the exact pathogenesis remains unclear. Serologically, PBC is characterized by presence of antimitochondrial antibodies, which are present in 90–95 % of patients and are often detectable years before clinical signs appear. Like other complex disorders, PBC is heterogeneous in its presentation, symptomatology, disease progression, and response to therapy. A significant number of patients develop end-stage liver disease and eventually require liver transplantation. Recent studies from large international cohorts have better identified prognostic factors, suggesting a change in patient management based on risk stratification. Therapeutic options are changing. In this review we discuss data on the autoimmune responses and treatment of the disease. © 2017, Asian Pacific Association for the Study of the Liver.eng
dc.format.mimetypeapplication/pdf
dc.identifier.doihttps://doi.org/10.1007/s12072-017-9830-1
dc.identifier.issn19360533
dc.identifier.issn19360541
dc.identifier.urihttps://repository.urosario.edu.co/handle/10336/24193
dc.language.isoengspa
dc.publisherSpringerspa
dc.relation.citationEndPage499
dc.relation.citationIssueNo. 6
dc.relation.citationStartPage485
dc.relation.citationTitleHepatology International
dc.relation.citationVolumeVol. 11
dc.relation.ispartofHepatology International, ISSN:19360533, 19360541, Vol.11, No.6 (2017); pp. 485-499spa
dc.relation.urihttps://www.scopus.com/inward/record.uri?eid=2-s2.0-85034650779&doi=10.1007%2fs12072-017-9830-1&partnerID=40&md5=26405a35d439dc228640aa305f024660spa
dc.rights.accesRightsinfo:eu-repo/semantics/openAccess
dc.rights.accesoAbierto (Texto Completo)spa
dc.source.instnameinstname:Universidad del Rosariospa
dc.source.reponamereponame:Repositorio Institucional EdocURspa
dc.subject.keywordAlkaline phosphatasespa
dc.subject.keywordBudesonidespa
dc.subject.keywordFibric acid derivativespa
dc.subject.keywordImmunosuppressive agentspa
dc.subject.keywordMitochondrion antibodyspa
dc.subject.keywordObeticholic acidspa
dc.subject.keywordUrsodeoxycholic acidspa
dc.subject.keywordAntiinflammatory agentspa
dc.subject.keywordBudesonidespa
dc.subject.keywordChenodeoxycholic acidspa
dc.subject.keywordCholagoguespa
dc.subject.keywordImmunosuppressive agentspa
dc.subject.keywordObeticholic acidspa
dc.subject.keywordAdaptive immunityspa
dc.subject.keywordAutoimmune diseasespa
dc.subject.keywordClinical featurespa
dc.subject.keywordDisease coursespa
dc.subject.keywordEnd stage liver diseasespa
dc.subject.keywordHistopathologyspa
dc.subject.keywordHumanspa
dc.subject.keywordImmunofluorescencespa
dc.subject.keywordInnate immunityspa
dc.subject.keywordLiver biopsyspa
dc.subject.keywordLiver transplantationspa
dc.subject.keywordNonhumanspa
dc.subject.keywordPathogenesisspa
dc.subject.keywordPathophysiologyspa
dc.subject.keywordPatient carespa
dc.subject.keywordPrimary biliary cirrhosisspa
dc.subject.keywordPriority journalspa
dc.subject.keywordPrognosisspa
dc.subject.keywordReviewspa
dc.subject.keywordRisk factorspa
dc.subject.keywordTreatment responsespa
dc.subject.keywordAnalogs and derivativesspa
dc.subject.keywordAutoimmune diseasespa
dc.subject.keywordBiopsyspa
dc.subject.keywordCholangitisspa
dc.subject.keywordImmunologyspa
dc.subject.keywordLiverspa
dc.subject.keywordPathologyspa
dc.subject.keywordAnti-inflammatory agentsspa
dc.subject.keywordAutoimmune diseasesspa
dc.subject.keywordBiopsyspa
dc.subject.keywordBudesonidespa
dc.subject.keywordChenodeoxycholic acidspa
dc.subject.keywordCholagogues and cholereticsspa
dc.subject.keywordCholangitisspa
dc.subject.keywordFibric acidsspa
dc.subject.keywordHumansspa
dc.subject.keywordImmunosuppressive agentsspa
dc.subject.keywordLiverspa
dc.subject.keywordPrognosisspa
dc.subject.keywordUrsodeoxycholic acidspa
dc.subject.keywordAntimitochondrial antibodiesspa
dc.subject.keywordBiliary epithelial cellsspa
dc.subject.keywordEpigeneticsspa
dc.subject.keywordGeneticsspa
dc.subject.keywordObeticholic acidspa
dc.subject.keywordPrimary biliary cholangitisspa
dc.subject.keywordPrognostic factorsspa
dc.subject.keywordUdcaspa
dc.titlePrimary biliary cholangitis: a comprehensive overviewspa
dc.typearticleeng
dc.type.hasVersioninfo:eu-repo/semantics/publishedVersion
dc.type.spaArtículospa
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