The role of genetic and immune factors for the pathogenesis of primary sclerosing cholangitis in childhood
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Universidade Federal de Minas Gerais
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Artigo de periódico
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Membros da banca
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.
Abstract
Assunto
Fatores imunológicos, Primary Sclerosing Cholangitis, Adulto Jovem
Palavras-chave
Immune Factors, Pathogenesis, Primary Sclerosing Cholangitis, Young Adult
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https://www.hindawi.com/journals/grp/2016/3905240/