Biliary atresia (BA) is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly). We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inv...

Biliary atresia (BA) is an inflammatory cholangiopathy of unknown etiology. Maternal microchimerism has been identified in the livers of patients with BA. We analyzed the human leukocyte antigen (HLA) compatibility between 57 BA patient-mother pairs and 50 control-mother pairs. The HLA class I matching was significantly more frequent in BA pairs (odds ratio [OR]=2.46) than controls. Similar res...

To study the impairment of cholangiocyte primary cilia caused by prolonged cold preservation and its correlation with graft cholangiopathy after orthotopic liver transplantation (OLT). Subjects were 60 male Wistar rats that were divided into 2 groups: a control group (n = 30) receiving a donor liver preserved for 1 h and a study group (n = 30) receiving a donor liver preserved for 12 h. A two-c...

Secondary sclerosing cholangitis is a rare condition caused by disorders directly damaging the biliary tree. We present a case of a 34-year-old man with no pre-existing hepatobiliary disease who developed significant cholestasis and subsequent cholangitis while in the intensive care unit for multiorgan failure secondary to H1N1 influenza A (swine flu). After discharge from the intensive care un...

Biliary innate immunity is involved in the pathogenesis of cholangiopathies in patients with various biliary diseases. Biliary epithelial cells possess an innate immune system consisting of the Toll-like receptor (TLR) family and recognize pathogen-associated molecular patterns (PAMPs). Recently, regulatory mechanisms by intracellular negative regulators including peroxisome proliferator-activa...

Autoimmune pancreatitis (AIP) is a benign disorder and a unique form of chronic pancreatitis with several characteristic features. A cystic formation that mimics a pseudocyst is a rare finding. There have been a few reports of AIP complicated by pancreatic cysts. We present a case of AIP with multiple pseudocysts and obstructive jaundice caused by IgG4-associated cholangitis. We initially misse...

The clinicopathologic features of liver allograft dysfunction occurring in 51 symptomatic recipients after more than 5 years' survival (mean 7.1 years) with the same hepatic allograft were compared with those of a similar group of 14 asymptomatic patients (mean survival, 9.9 years) who underwent a nonclinically indicated protocol liver biopsy evaluation. Predictably, patients who had clinically...