Intrahepatic bile duct primary cilia in biliary atresia

2018 ◽  
Vol 48 (8) ◽  
pp. 664-674 ◽  
Author(s):  
Roberta Frassetto ◽  
Filippo Parolini ◽  
Salvatore Marceddu ◽  
Giulia Satta ◽  
Valeria Papacciuoli ◽  
...  
Keyword(s):  
Bile Duct ◽  
Biliary Atresia ◽  
Primary Cilia ◽  
Intrahepatic Bile Duct

Intrahepatic bile duct loss in biliary atresia despite portoenterostomy: A consequence of ongoing obstruction?

1992 ◽  
Vol 102 (6) ◽  
pp. 2126-2133 ◽  
Author(s):  
Gregor W. Nietgen ◽  
Joseph P. Vacanti ◽  
Antonio R. Perez-Atayde
Keyword(s):  
Bile Duct ◽  
Biliary Atresia ◽  
Intrahepatic Bile Duct

Distortion in TGFβ1 peptide immunolocalization in biliary atresia: Comparison with the normal pattern in the developing human intrahepatic bile duct system

1995 ◽  
Vol 45 (11) ◽  
pp. 815-824 ◽  
Author(s):  
Carolyn E. L. Tan ◽  
Vivian S. W. Chan ◽  
Rita Y. Y. Yong ◽  
Vijayalaxmy Vijayan ◽  
Wan L. Tan ◽  
...  
Keyword(s):  
Bile Duct ◽  
Biliary Atresia ◽  
Intrahepatic Bile Duct ◽  
Duct System ◽  
Normal Pattern ◽  
Bile Duct System

Biliary Atresia and Cytomegalovirus Infection: A DNA Study

1999 ◽  
Vol 2 (1) ◽  
pp. 11-14 ◽  
Author(s):  
G.P. Jevon ◽  
J.E. Dimmick
Keyword(s):  
In Situ Hybridization ◽  
Bile Duct ◽  
Biliary Atresia ◽  
Intrahepatic Bile Duct ◽  
Infectious Agent ◽  
Formalin Fixed Paraffin ◽  
Formalin Fixed Paraffin Embedded ◽  
Polymerase Chain ◽  
Formalin Fixed

The cause of extrahepatic biliary atresia (EHBA) is undetermined in most instances, but an infectious agent is widely suspected. Cytomegalovirus (CMV) infection has been associated with intrahepatic bile duct destruction and paucity raising the question of its role in EHBA. We identified 12 children in the past 5 years with biliary atresia and examined the bile duct biopsy. These showed acute/chronic inflammation and epithelial degeneration. CMV inclusions were not identified. We used in situ hybridization and the polymerase chain reaction (PCR) for CMV-DNA on formalin-fixed, paraffin-embedded tissue. All samples showed the presence of amplifiable DNA using β-globin primers. No biopsy tissue showed CMV DNA using specific probes and primers. The absence of demonstrable CMV DNA by in situ hybridization and PCR in EHBA biopsies implies that it is unlikely that this virus has any major role in the pathogenesis of this condition.

Proliferation of intrahepatic bile-duct epithelium in biliary atresia

1996 ◽  
Vol 11 (2-3) ◽  
pp. 126-129
Author(s):  
Moazzem Hossain ◽  
Osamu Murahashi ◽  
Hisami Ando ◽  
Kentiro Kaneko ◽  
Takahiro Ito
Keyword(s):  
Bile Duct ◽  
Biliary Atresia ◽  
Intrahepatic Bile Duct ◽  
Duct Epithelium ◽  
Bile Duct Epithelium

scholarly journals Caroli's Disease: Current Knowledge of Its Biliary Pathogenesis Obtained from an Orthologous Rat Model

2012 ◽  
Vol 2012 ◽  
pp. 1-10 ◽  
Author(s):  
Yasunori Sato ◽  
Xiang Shan Ren ◽  
Yasuni Nakanuma
Keyword(s):  
Bile Duct ◽  
Primary Cilia ◽  
Current Knowledge ◽  
Intrahepatic Bile Duct ◽  
Fluid Secretion ◽  
Polycystic Kidney ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Cell Matrix ◽  
Cell Matrix Interactions

Caroli's disease belongs to a group of hepatic fibropolycystic diseases and is a hepatic manifestation of autosomal recessive polycystic kidney disease (ARPKD). It is a congenital disorder characterized by segmental saccular dilatations of the large intrahepatic bile duct and is frequently associated with congenital hepatic fibrosis (CHF). The most viable theory explaining its pathogenesis suggests that it is related to ductal plate malformation. The development of the polycystic kidney (PCK) rat, an orthologous rodent model of Caroli's disease with CHF as well as ARPKD, has allowed the molecular pathogenesis of the disease and the therapeutic options for its treatment to be examined. The relevance of the findings of studies using PCK rats and/or the cholangiocyte cell line derived from them to the pathogenesis of human Caroli's disease is currently being analyzed. Fibrocystin/polyductin, the gene product responsible for ARPKD, is normally localized to primary cilia, and defects in the fibrocystin from primary cilia are observed in PCK cholangiocytes. Ciliopathies involving PCK cholangiocytes (cholangiociliopathies) appear to be associated with decreased intracellular calcium levels and increased cAMP concentrations, causing cholangiocyte hyperproliferation, abnormal cell matrix interactions, and altered fluid secretion, which ultimately result in bile duct dilatation. This article reviews the current knowledge about the pathogenesis of Caroli's disease with CHF, particularly focusing on studies of the mechanism responsible for the biliary dysgenesis observed in PCK rats.

Localized Intrahepatic Bile Duct Dilatation without a Visible Mass or Stone as depicted on CT Images: Findings of Malignancy Prediction

2008 ◽  
Vol 59 (3) ◽  
pp. 163
Author(s):  
Ju Wan Choi ◽  
Gab Chul Kim ◽  
Han Young Jeong ◽  
Hui Joong Lee ◽  
Jae Hyuck Lee ◽  
...  
Keyword(s):  
Bile Duct ◽  
Intrahepatic Bile Duct ◽  
Ct Images ◽  
Bile Duct Dilatation ◽  
Duct Dilatation
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