Tutorial Review for Understanding of Cholangiopathy

Abstract

The biliary tree consists of intrahepatic and extrahepatic bile ducts and is lined by biliary epithelial cells (or cholangiocytes). There are also peribiliary glands around the intrahepatic large bile ducts and extrahepatic bile ducts. The biliary tree is a conduit of bile secreted by hepatocytes and biliary epithelial cells and also of the peribiliary glands and has several physiological roles. A number of diseases affect mainly the intrahepatic and extrahepatic biliary tree, and, in this special issue, these cholangiopathies are reviewed in detail with respect to genetics, pathogenesis, and pathology. In this paper, the anatomy and physiology of the biliary tree, basic injuries to biliary epithelial cells from stress and bile duct damage, and representative cholangiopathies are briefly reviewed.

Physiology of Cholangiocytes

“…In addition, large intrahepatic ducts are lined peripherally (within their fibromuscular walls) by peribiliary glands, as are the extrahepatic ducts (208). …”

CCN1 induces hepatic ductular reaction through integrin αvβ5–mediated activation of NF-κB

“…The peribiliary vascular plexus (PBP) that supplies blood to the bile ducts is an anastomotic network derived from the hepatic arterial branches and flows into the hepatic sinusoids. Capillaries of the PBP are highly fenestrated, facilitating the exchange of molecules between the bile duct and the PBP (38). Thus, when injected into WT mice via retro-orbital delivery, purified CCN1 protein is rapidly and extensively localized in the liver (Supplemental Figure 3).…”

Modifying effects of 1,2-dichloropropane on N-nitrosobis(2-oxopropyl)amine-induced cholangiocarcinogenesis in male Syrian hamsters

“…To the best of our knowledge, this is the first study to determine the carcinogenic effects of 1,2-DCP in hamsters. Results of our thorough histopathological examination of livers (17 sections of liver per animal) show that 1,2-DCP did not enhance the development of BOP-induced atypical biliary hyperplasia that is associated with development of bile duct cancer (Kurashina et al, 1988;Nakanuma, 2012;Hailey et al, 2014) and did not induce any lesions in liver bile ducts when administered alone to hamsters. Notably, 1,2-DCP had no effect on the proliferative activity of bile duct epithelial cells regardless of BOP-initiation.…”

“…Preneoplastic biliary lesions in the livers were diagnosed as atypical biliary hyperplasia that were characterized by an irregular proliferation of bile duct with irregular lumina, multi-layers of atypical epithelial cells, with or without intestinal metaplasia (Kurashina et al, 1988;Nakanuma, 2012;Hailey et al, 2014).…”