The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood

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dc.creatorPriscila Menezes Ferri Liu
dc.creatorAna Cristina Simões e Silva
dc.creatorSoraya Luiza Campos Silva
dc.creatorDiego Junior Queiroga de Aquino
dc.creatorEleonora Druve Tavares Fagundes
dc.creatorDébora Marques de Miranda
dc.creatorAlexandre Rodrigues Ferreira
dc.date.accessioned2023-07-28T22:00:29Z
dc.date.accessioned2025-09-09T01:14:42Z
dc.date.available2023-07-28T22:00:29Z
dc.date.issued2016-12-16
dc.format.mimetypepdf
dc.identifier.doihttps://doi.org/10.1155/2016/3905240
dc.identifier.issn16876121
dc.identifier.urihttps://hdl.handle.net/1843/57182
dc.languageeng
dc.publisherUniversidade Federal de Minas Gerais
dc.relation.ispartofGastroenterology Research and Practice
dc.rightsAcesso Aberto
dc.subjectFatores imunológicos
dc.subjectPrimary Sclerosing Cholangitis
dc.subjectAdulto Jovem
dc.subject.otherImmune Factors
dc.subject.otherPathogenesis
dc.subject.otherPrimary Sclerosing Cholangitis
dc.subject.otherYoung Adult
dc.titleThe role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood
dc.typeArtigo de periódico
local.citation.epage8
local.citation.spage1
local.citation.volume2016
local.description.resumoPrimary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age.The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation. Differences in clinical and laboratory findings were observed in young patients, including higher incidence of overlap syndromes, mostly with autoimmune hepatitis, higher serum levels of aminotransferases and gamma-glutamyl transferase, and lower incidence of serious complications as cholangiocarcinoma. In spite of the detection of several HLA variants as associated factors in large multicenter cohorts of adult patients, the exact role and pathways of these susceptibility genes remain to be determined in pediatric population. In addition, the literature supports a role for an altered immune response to pathogens in the pathogenesis of PSC. This phenomenon contributes to abnormal immune system activation and perpetuation of the inflammatory process. In this article, we review the role of immune and genetic factors in the pathogenesis of PSC in pediatric patients.
local.publisher.countryBrasil
local.publisher.departmentMED - DEPARTAMENTO DE PEDIATRIA
local.publisher.initialsUFMG
local.url.externahttps://www.hindawi.com/journals/grp/2016/3905240/

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