primary sclerosing cholangitis

Peribiliary glands and biliary tree stem cells are involved in the pathogenesis of cholangiocarcinoma arising in patients affected by primary sclerosing cholangitis

Background and Aims: Primary sclerosing cholangitis (PSC) is a chronic cholangiopathy characterized by inflammation and bile duct fibrosis. Massive proliferation of biliary tree stem/progenitor cell (BTSC), expansion of peribiliary glands (PBG) and dysplasia were observed in PSC. Cholangiocarcinoma (CCA) frequently complicates the course of PSC. The aims of the present study were to evaluate the involvement of PBGs and BTSCs in CCA emerging in PSC patients.

Biliary tree stem cells and peribiliary glands are involved in primary sclerosing cholangitis and cholangiocarcinoma.

Peribiliary glands (PBGs) represent the niche of biliary tree stem/progenitor cells (BTSCs) [1]. BTSCs are multipotent stem cells able to differentiate into hepatocytes, cholangiocytes, and pancreatic islets. Primary sclerosing cholangitis (PSC) is a chronic inflammation involving extra-hepatic biliary tree, and is complicated by the risk of cholangiocarcinoma (CCA) development [2]. We aimed to evaluate the involvement of PBGs and BTSCs in PSC and their role in CCA insurgence [2].

Peribiliary Gland Niche Participates in Biliary Tree Regeneration in Mouse and in Human Primary Sclerosing Cholangitis

Background and Aims: Mechanisms underlying the repair of extrahepatic biliary tree (EHBT) after injury have been scarcely explored. The aims of this study were to evaluate, by using a lineage tracing approach, the contribution of peribiliary gland (PBG) niche in the regeneration of EHBT after damage and to evaluate, in vivo and in vitro, the signaling pathways involved. Approach and Results: Bile duct injury was induced by the administration of 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC) diet for 14 days to Krt19CreTdTomatoLSL mice.

Functional Role of the Secretin/Secretin Receptor Signaling During Cholestatic Liver Injury

Liver diseases are a major health concern and affect a large proportion of people worldwide. There are over 100 types of liver disorders, including cirrhosis, cholangiocarcinoma (CCA), hepatocellular carcinoma, and hepatitis. Despite the relevant number of people who are affected by liver diseases, and the increased awareness with regard to these disorders, the number of deaths corresponding to liver injury is expected to increase in the foreseeable future.

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