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Ultra-Structural Analysis of the Intrahepatic Bile Duct System
Published in Gianfranco Alpini, Domenico Alvaro, Marco Marzioni, Gene LeSage, Nicholas LaRusso, The Pathophysiology of Biliary Epithelia, 2020
Luca Marucci, Anne Marie Jezequel, Antonio Benedetti
Interlobular bile ducts are larger, 20–100 µm in cross-section, and are located at a distance from the edge of portal spaces (Fig. 2). The lining cells are taller than in bile ductules, the nucleocytoplasmic ratio is lower and the cellular organelles are distributed in a somewhat similar fashion. However, the nucleus tends to migrate towards the basal pole of the cells, accompanied by the components of the Golgi apparatus.2
Structural Organization of the Liver
Published in Robert G. Meeks, Steadman D. Harrison, Richard J. Bull, Hepatotoxicology, 2020
Bile in the bile ductules flows into the interlobular bile ducts in the portal tract. These ducts measure 30–40 μm in diameter and form a continuous network for biliary passage. They are lined by a single layer of cuboidal or columnar epithelial cells with short microvilli on their lumenal surface and are encircled by a basal lamina.
Microdeletion Syndromes
Published in Merlin G. Butler, F. John Meaney, Genetics of Developmental Disabilities, 2019
Gopalrao V. N. Velagaleti, Nancy J. Carpenter
The clinical diagnosis of AGS is based on the criteria established by Alagille et al. (1,3). These criteria are: (i) the histologic finding of paucity of the interlobular bile ducts on liver biopsy, and (ii) at least three of these five major clinical features: chronic cholestasis, cardiac disease, skeletal abnormalities, ocular abnormalities, and characteristic facial features (Fig. 1).The clinical features associated with the syndrome can be classified into five major groups.
Central Hepatic Regenerative Nodules in Alagille Syndrome: A Clinicopathological Review
Published in Fetal and Pediatric Pathology, 2021
The characteristic liver pathology in Alagille syndrome is interlobular bile duct paucity. Alagille [2] describes a ratio of the number of interlobular bile ducts to the number of portal tracts of less than 0.4 and recommends examining at least 10 portal tracts to derive this ratio. This paucity of bile ducts increases after infancy and has been reported in 60% of infants versus 95% of older patients [4]. Additional features often seen in Alagille syndrome patients include cholestasis, portal inflammation and fibrosis [2, 4]. Emerick et al. [4] report progression to bridging fibrosis in 48% of patients with fibrosis on their initial biopsy and cirrhosis in an additional 26% of patients in this subgroup. In cirrhotic patients, the paucity of interlobular bile ducts is well established and usually without a significant ductular reaction (Fig. 1A,B). Lobular cholestatic changes are variably seen. Cytokeratin 7 immunohistochemical stain can be helpful to highlight bile duct loss and aberrant cytokeratin 7 expression in the lobular hepatocytes (Fig. 1C). Similar to other cholestatic liver disorders, copper deposition is frequently identified in the periportal hepatocytes (Fig. 1D).
Non Syndromic Paucity of Interlobular Bile Ducts in Children – A Clinicopathological Study
Published in Fetal and Pediatric Pathology, 2020
Suravi Mohanty, Kanishka Das, Marjorie Maria Anne Correa
The portal tract has a single, usually round interlobular bile duct lined by cuboidal epithelium located near the hepatic artery and portal venule. In most cases, the bile duct lies within three diameters of the accompanying arteriole and has a similar size. In contrast, ductules have a smaller lumen and are located at the periphery of the portal tract [17, 18]. The identification of bile ducts is feasible by Hematoxylin & Eosin stain; however in certain situations (dense inflammation, fibrosis, inconspicuous bile ducts formed by a few epithelial cells without a patent lumen), Masson’s trichrome stain and immunohistochemistry with Cytokeratin-7 help to delineate them for calculation of the BD/PT (bile duct to portal tract ratio) [10, 13, 17]. We have used cytokeratin-7 wherever necessary.
Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy
Published in Fetal and Pediatric Pathology, 2019
Santosh Kumar Mahalik, Suvradeep Mitra, Susama Patra, Kanishka Das
The liver biopsy showed marked portal fibrosis with portal-portal bridging forming biliary nodules. These portal tracts were expanded by edema and fibrosis (Fig. 3A and B). The portal inflammation was minimal to mild. There was a bile ductular reaction and the interlobular bile ducts contained bile plugs at places (Fig. 3C). Some of the centrally located bile ducts showed abnormally angulated, irregular, non-canalized and malformed outlines. Concentric tubular structures around prominent hepatic arterioles embedded in a myxoid mesenchyme suggested a ductal plate malformation. These abnormal ductal structures were seen in addition to the peripheral rim of ductules and were associated with hypoplastic portal veins (Fig. 3D and E). The periportal hepatocytes showed cytoplasmic rarefaction indicating cholestasis (Fig. 3D). The hepatic lobules showed architectural disarray along with feathery changes, canalicular bile plugs, psuedoacinar transformation and occasional giant cell formation. There was no extramedullary hematopoiesis. CK7 highlighted the bile ductules and there was strong diffuse membranous CD56 expression in the ducts and the ductules (Fig. 3D inset). The morphology of the cyst and the liver biopsy suggested an intermediate entity in the spectrum of choledochal cyst and cystic biliary atresia with attendant hepatic changes.