system in a patient with intrahepatic lithiasis, liver cirrhosis, and surgically altered anatomy A 35-year-old woman underwent a Rouxen-Y hepaticojejunostomy in 2001 for a bileduct injury thatoccurredduringacholecystectomy for cholelithiasis. After the surgery, she began to experience episodes of relapsing cholangitis, approximately once a year, which were treated with antibiotics. However, fro...

Congenital intrahepatic arterioportal fistula is a rare but treatable cause of portal hypertension for which early recognition may lead to successful radiological management. We report an infant presenting with severe failure to thrive, melena and splenomegaly due to a congenital intrahepatic arterioportal fistula, successfully ablated after multiple trials of superselective transarterial embol...

BACKGROUND Progressive familial intrahepatic cholestasis has been only infrequently reported from India. CASE CHARACTERISTICS An Indian girl with progressive cholestatic liver disease beginning during infancy, normal gamma-glutamyl transpeptidase levels, parental consanguinity, positive family history and a fatal outcome. OBSERVATION A novel, homozygous mutation (c.[589_592inv;592_593insA])...

Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rapidly developing hepatic disease that leads to early childhood cirrhosis and liver failure. We present a diagnostically challenging case of a 20-year-old male with 8 years history of recurrent icteric episodes and constantly normal serum levels of GGT. Genetic study disclosed two novel mutations in gene ABCB11 and liver histopa...

This article gives an overview of the molecular and cellular mechanisms of cholestasis. Topics reviewed include the pathomechanisms of hereditary cholestasis syndromes, such as progressive familial intrahepatic cholestasis, and hepatocellular transporter defects encountered in various acquired cholestatic disorders, such as intrahepatic cholestasis of pregnancy, drug-induced cholestasis, inflam...

Bile duct cancer is subdivided according to the anatomic location of origin into intrahepatic and extrahepatic cholangiocarcinoma, gallbladder cancer, or ampulla of Vater cancer. While biliary tract cancer is uncommon in Western countries, the incidence is relatively high in Asia, including Japan, and Latin America. However, in biliary tract cancers, the incidence of intrahepatic cholangiocarci...

The ramifications of the portal vein in the liver of horses were studied by using corrosion casting and gross dissection. The portal vein in the horse supplies the hepatic lobes by six major branches. The right lobe is supplied by three main branches, namely the dorsal diaphragmatic, the right caudodorsal and the right intermediate branches. The left branch supplies the left lateral and medial ...

Caroli's disease, which is a rare condition with congenital dilatation of the intrahepatic bile ducts, is usually diagnosed postoperatively. The clinical suspicion in a patient with gallstones and choledocholithiasis presenting with dilated intrahepatic biliary radicles and jaundice is usually an obstructive etiology. However, scintigraphic evaluation of this entity, as in this case, gives addi...

Congenital (spontaneous) intrahepatic portosystemic shunt is rare in the English literature. Most cases of portosystemic shunt occur after trauma, surgery, liver biopsy or as a result of chronic portal hypertension. Chronic shunting may result in encephalopathy, bleeding or hyperinsulinism. We report a case of an asymptomatic adult female with a presumed congenital intrahepatic portosystemic sh...

We report a case of secondary sclerosing cholangitis that manifested itself during pregnancy. A tentative diagnosis of intrahepatic cholestasis of pregnancy was considered, but after her third delivery, a liver biopsy and imaging, as well as review of past records, confirmed the diagnosis of secondary sclerosing cholangitis. Maternal and fetal outcomes of primary sclerosis cholangitis have been...