Intrahepatic bile ducts (IHBDs) develop from bipotential liver progenitor cells in contact with the mesenchyme of the portal vein and thus form the "ductal plates." The ductal plates are remodeled into mature tubular ducts. Lack of remodeling results in the persistence of periportal epithelial sleeves or " @entity513 " (DPM). A proposal is that virtually all @entity66 of IHBDs represent examples of DPM. Some early, severe types of extrahepatic bile duct @entity1146 are characterized by DPM, a suggestion of a prenatal beginning of the disease. Several @entity66 are characterized by dilatation of segments of IHBDs and variable degrees of @entity82 . Such " @entity3923 " represent DPM at different levels of the biliary tree. @entity8 represents DPM of interlobular bile ducts, associated with tubular dilatation of collecting renal tubules. @entity7872 may derive from the same type of @entity409 , through a superimposed destructive type of cholangiopathy associated with scarring @entity82 . @entity13550 represents DPM of the larger IHBDs, whereas @entity13550 combines the lesions of @entity13550 and @entity7872 represent DPM of smaller interlobular ducts; their dilatation gives rise to the @entity96 in @entity8 . Finally, DPM is a component of the tissue abnormalities in so-called @entity4607 .

Ludwig symposium on biliary disorders--part I. Pathogenesis of XXXX .

@entity8 :: ('MESH:D007674', 'Disease') :: ['Autosomal recessive polycystic kidney disease', 'autosomal dominant polycystic kidney disease'] @entity82 :: ('MESH:D005355', 'Disease') :: ['fibrosis'] @entity66 :: ('MESH:D004194', 'Disease') :: ['congenital diseases'] @entity96 :: ('MESH:D008113', 'Disease') :: ['liver cysts'] @entity409 :: ('MESH:D008107', 'Disease') :: ['liver lesion'] @entity513 :: ('MESH:D044584', 'Disease') :: ['ductal plate malformation'] @entity3923 :: ('MESH:D005348', 'Disease') :: ['fibrocystic diseases'] @entity13550 :: ('MESH:D016767', 'Disease') :: ["Caroli's syndrome", "Caroli's disease"] @entity7872 :: ('MESH:C562378', 'Disease') :: ['congenital hepatic fibrosis. von Meyenburg complexes', 'Congenital hepatic fibrosis'] @entity1146 :: ('MESH:D018633', 'Disease') :: ['atresia'] @entity4607 :: ('MESH:D006222', 'Disease') :: ['mesenchymal hamartoma']
@entity513 :: (MESH:D044584,Disease) :: ['ductal plate abnormalities']
