Primary biliary cirrhosis (PBC) is histologically characterized by chronic nonsuppurative destructive cholangitis (CNSDC) associated with destruction of small bile ducts. The pathogenesis of PBC, predominance of female, or the reason why biliary duct is selectively involved, however, remains unknown. Infectious or non-infectious noxious insults such as xenobiotic chemicals may precipitate in th...

Biliary complications cause significant morbidity and mortality in liver transplantation. Warm ischemia can induce biliary duct injury. This study aimed to investigate the effects of warm ischemia on the peribiliary vascular plexus in rat liver transplantation. A total of 102 Sprague-Dawley rats were divided into three groups: sham-operation group, non-ischemic group, and ischemic group. Liver ...

Inflammatory bowel diseases are associated with various hepatobiliary disorders, reported both in Crohn's disease and ulcerative colitis. They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions used in different studies. Fatty liver is considered as the most common hepatobiliary complication in inflam...

The molecular pathogenesis of cystic fibrosis (CF) liver disease is unknown. This study investigates its earliest pathophysiological manifestations employing a mouse model carrying DeltaF508, the commonest human CF mutation. We hypothesized that, if increased bile salt spillage into the colon occurs as in the human disease, then this should lead to a hydrophobic bile salt profile and to "hyperb...

OBJECTIVES Biliary atresia (BA) is a progressive fibroinflammatory cholangiopathy affecting the bile ducts of neonates. Although BA is the leading indication for pediatric liver transplantation, the etiology remains elusive. Adducin 3 (ADD3) and X-prolyl aminopeptidase 1 (XPNPEP1) are 2 genes previously identified in genome-wide association studies as potential BA susceptibility genes. Using ze...

Biliary innate immunity is involved in the pathogenesis of cholangiopathies in patients with primary biliary cirrhosis (PBC) and biliary atresia. Biliary epithelial cells possess an innate immune system consisting of the Toll-like receptor (TLR) family and recognize pathogen-associated molecular patterns (PAMPs). Tolerance to bacterial PAMPs such as lipopolysaccharides is also important to main...

The diagnosis of perihilar and distal cholangiocarcinoma (CCA) remains an important clinical challenge. Current detection methods are mostly based on biliary brushing samples with suboptimal sensitivity and/or specificity, leading to late higher mortality rates. We aimed identify a diagnostic biomarker using targeted DNA methylation sequencing in classical training validation study-design. Bili...

BACKGROUND Immunoglobulin G4 (IgG4)-associated diseases mostly involve the pancreaticobiliary tree and pancreatic parenchyma. This disease complex is characterized by marked response to corticosteroid therapy and response to steroids is incorporated in the diagnostic algorithm of IgG4 associated diseases. However, there is much unknown about the sequences and duration of healing during the cort...