Primary biliary cirrhosis (PBC) is an autoimmune cholestatic disorder of the liver. A diagnostic serum marker for PBC is an anti-mitochondrial antibody. Most prominent histologic findings of PBC are portal inflammation and destruction of interlobular bile ducts. The PBC occurs only in 40 to 400 individuals per million in the general population. About 1.8 - 5.6% of individuals with this rare dis...

An 82-year-old woman with primary biliary cholangitis was diagnosed with an irreversible neurological disorder, caused by metronidazole (MNZ)-induced encephalopathy. Although the disorder is a reversible pathological condition, in rare cases, it can cause serious sequelae or could even be fatal. Therefore, medications should be administered carefully, particularly in patients who require long-t...

Rendu-Osler-Weber disease is a rare autosomal dominant disorder. Hepatic involvement manifests itself as vascular, parenchymal, and biliary lesions with characteristic telangiectasias and vascular shunts. In a 37-year-old female patient, dynamic contrast-enhanced upper abdominal CT and MRI were performed. CT and MRI revealed dilated celiac trunk and hepatic artery. On early arterial phase, dila...

Agenesis of the gallbladder is a rare anomaly that is usually asymptomatic, but sometimes the patients may have symptoms compatible with a biliary disorder like cholelithiasis. Its preoperative diagnosis is often difficult, especially if dysplastic cyst is associated and simulates the gallbladder. When the diagnosis is doubtful its confirmation and treatment of dysplastic cyst require open surg...

BILIARY atresia is the most common indication for liver transplants in children, and this disorder is associated with some congenital vascular anomalies. Complex vascular anomalies increase the technical difficulties of the operation and previously resulted in high mortality in these patients. The role of the radiologist is to define the conditions in which transplantation is high risk or contr...

The Alagille Syndrome (AGS) is a heritable disorder affecting the liver and other organs. Causative dominant mutations in human Jagged 1 have been identified in most AGS patients. Related organ defects occur in mice that carry jagged 1 and notch 2 mutations. Multiple jagged and notch genes are expressed in the developing zebrafish liver. Compound jagged and notch gene knockdowns alter zebrafish...

We report a case of posttransplantation lymphoproliferative disorder manifesting as an isolated, unilateral iris tumor. A 2-year-old boy who had undergone liver transplantation for biliary atresia at age 4 months was seen with a 2-month history of an enlarging iris nodule. Histopathologic examination of the iris lesion demonstrated a mixed population of lymphoid cells. To our knowledge, this is...

Several Helicobacter species have recently been isolated from the bile and hepatobiliary systems of murine species, and are well recognized as a pathogen of the hepatobiliary disorder. This study was planned to investigate whether Helicobacter species possess a causative potential for human hepatobiliary disease, especially for hepatobiliary carcinogenesis. Bile and hepatobiliary tissue samples...