The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood
Autor(a) principal: | |
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Data de Publicação: | 2016 |
Outros Autores: | , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFMG |
Texto Completo: | https://doi.org/10.1155/2016/3905240 http://hdl.handle.net/1843/57182 |
Resumo: | Primary 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. |
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2023-07-28T22:00:29Z2023-07-28T22:00:29Z2016-12-16201618https://doi.org/10.1155/2016/390524016876121http://hdl.handle.net/1843/57182Primary 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.engUniversidade Federal de Minas GeraisUFMGBrasilMED - DEPARTAMENTO DE PEDIATRIAGastroenterology Research and PracticeFatores imunológicosPrimary Sclerosing CholangitisAdulto JovemImmune FactorsPathogenesisPrimary Sclerosing CholangitisYoung AdultThe role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhoodinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://www.hindawi.com/journals/grp/2016/3905240/Priscila Menezes Ferri LiuAna Cristina Simões e SilvaSoraya Luiza Campos SilvaDiego Junior Queiroga de AquinoEleonora Druve Tavares FagundesDébora Marques de MirandaAlexandre Rodrigues Ferreiraapplication/pdfinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFMGinstname:Universidade Federal de Minas Gerais (UFMG)instacron:UFMGLICENSELicense.txtLicense.txttext/plain; charset=utf-82042https://repositorio.ufmg.br/bitstream/1843/57182/1/License.txtfa505098d172de0bc8864fc1287ffe22MD51ORIGINALThe Role of Genetic and Immune Factors for the Pathogenesis of Primary Sclerosing Cholangitis in Childhood pdfa.pdfThe Role of Genetic and Immune Factors for the Pathogenesis of Primary Sclerosing Cholangitis in Childhood pdfa.pdfapplication/pdf192871https://repositorio.ufmg.br/bitstream/1843/57182/2/The%20Role%20of%20Genetic%20and%20Immune%20Factors%20for%20the%20Pathogenesis%20of%20Primary%20Sclerosing%20Cholangitis%20in%20Childhood%20pdfa.pdf1d32ebeb28444bb7754c3357dce17773MD521843/571822023-07-31 13:37:51.943oai:repositorio.ufmg.br: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Repositório de PublicaçõesPUBhttps://repositorio.ufmg.br/oaiopendoar:2023-07-31T16:37:51Repositório Institucional da UFMG - Universidade Federal de Minas Gerais (UFMG)false |
dc.title.pt_BR.fl_str_mv |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood |
title |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood |
spellingShingle |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood Priscila Menezes Ferri Liu Immune Factors Pathogenesis Primary Sclerosing Cholangitis Young Adult Fatores imunológicos Primary Sclerosing Cholangitis Adulto Jovem |
title_short |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood |
title_full |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood |
title_fullStr |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood |
title_full_unstemmed |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood |
title_sort |
The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood |
author |
Priscila Menezes Ferri Liu |
author_facet |
Priscila Menezes Ferri Liu Ana Cristina Simões e Silva Soraya Luiza Campos Silva Diego Junior Queiroga de Aquino Eleonora Druve Tavares Fagundes Débora Marques de Miranda Alexandre Rodrigues Ferreira |
author_role |
author |
author2 |
Ana Cristina Simões e Silva Soraya Luiza Campos Silva Diego Junior Queiroga de Aquino Eleonora Druve Tavares Fagundes Débora Marques de Miranda Alexandre Rodrigues Ferreira |
author2_role |
author author author author author author |
dc.contributor.author.fl_str_mv |
Priscila Menezes Ferri Liu Ana Cristina Simões e Silva Soraya Luiza Campos Silva Diego Junior Queiroga de Aquino Eleonora Druve Tavares Fagundes Débora Marques de Miranda Alexandre Rodrigues Ferreira |
dc.subject.por.fl_str_mv |
Immune Factors Pathogenesis Primary Sclerosing Cholangitis Young Adult |
topic |
Immune Factors Pathogenesis Primary Sclerosing Cholangitis Young Adult Fatores imunológicos Primary Sclerosing Cholangitis Adulto Jovem |
dc.subject.other.pt_BR.fl_str_mv |
Fatores imunológicos Primary Sclerosing Cholangitis Adulto Jovem |
description |
Primary 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. |
publishDate |
2016 |
dc.date.issued.fl_str_mv |
2016-12-16 |
dc.date.accessioned.fl_str_mv |
2023-07-28T22:00:29Z |
dc.date.available.fl_str_mv |
2023-07-28T22:00:29Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/1843/57182 |
dc.identifier.doi.pt_BR.fl_str_mv |
https://doi.org/10.1155/2016/3905240 |
dc.identifier.issn.pt_BR.fl_str_mv |
16876121 |
url |
https://doi.org/10.1155/2016/3905240 http://hdl.handle.net/1843/57182 |
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16876121 |
dc.language.iso.fl_str_mv |
eng |
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eng |
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Gastroenterology Research and Practice |
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openAccess |
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Universidade Federal de Minas Gerais |
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UFMG |
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Brasil |
dc.publisher.department.fl_str_mv |
MED - DEPARTAMENTO DE PEDIATRIA |
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Universidade Federal de Minas Gerais |
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UFMG |
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UFMG |
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