The most common functional disorders of the biliary tract and pancreas relate to the activity of the sphincter of Oddi. The sphincter of Oddi is a small smooth muscle sphincter strategically placed at the junction of the bile duct, pancreatic duct and duodenum. Disorder in its motility is called sphincter of Oddi dysfunction. Clinically this presents either with recurrent abdominal pain or epis...

Mitchell-Riley syndrome is a genetic disorder characterized by neonatal diabetes, pancreatic hypoplasia, intestinal atresia and/or malrotation, biliary atresia, and gallbladder aplasia or hypoplasia. It was considered a variant of the Martinez-Frias syndrome with similar phenotypic characteristics, except for neonatal diabetes and tracheoesophageal fistula. However, the genetic mutation in (reg...

Primary biliary cirrhosis (PBC) is an autoimmune disease characterized by the presence of anti-mitochondrial antibodies (AMA). Whilst asymptomatic distal tubular acidosis (DTA) is the commonest renal lesion reported in PBC, tubulointerstitial nephritis (TIN) has also been reported as a rare association. Although PBC could be a familial disorder, there have been no previous reports of familial c...

Forty-two patients with autoimmune liver disease have been investigated. Renal tubular acidosis was detected in 60% of the patients with primary biliary cirrhosis, in 30% with active chronic hepatitis, and in one out of seven cases with cryptogenic cirrhosis. The presence of the defect of renal acidification was not related to the level of plasma potassium, copper, or total globulin, nor to the...

A 45-year-old woman with suspected Functional Biliary Sphincter Disorder (FBSD) developed Clostridium perfringens related emphysematous cholecystitis after ERCP. A low index of suspicion for emphysematous cholecystitis in this young, otherwise healthy woman led to a significant delay in making the correct diagnosis, and air in the gallbladder was wrongly attributed to a possible gallbladder per...

We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This conditio...

A 63-year-old man with extrahepatic portal vein thrombosis presented with biliary obstruction and hemobilia after a liver biopsy. Balloon sweep of the common bile duct removed clotted blood, and cholangiogram showed a common bile duct narrowing, treated with biliary stenting. A percutaneous biliary catheter was later required for recurrent biliary obstruction and hemobilia, and repeat cholangio...

Pancreas cancer has increased morbidity and mortality. It generally result in biliary obstruction which life threatening importance. Main biliary drainage method is endoscopic retrograde cholangiopancreatography. When endoscopic retrograde cholangiopancreatography is not successful, second preferred method is percutaneous biliary drainage. Percutaneous biliary drainage has some complications wh...

Unusual cases of acute cholecystitis and cholangitis include (1) pediatric biliary tract infections, (2) geriatric biliary tract infections, (3) acalculous cholecystitis, (4) acute and intrahepatic cholangitis accompanying hepatolithiasis (5) acute biliary tract infection accompanying malignant pancreatic-biliary tumor, (6) postoperative biliary tract infection, (7) acute biliary tract infectio...

conclusions we outlined its biliary drainage and blood supply, as well as our surgical approach, which may be a guide to surgeons who encounter hepatic choristomas in their clinical practice. introduction hepatic choristomas, defined as completely separated ectopic liver tissue, are rare. they are most commonly found adjacent to the gallbladder. details of their anatomy, such as blood supply an...