background liver cirrhosis is one of the major causes of hospitalization and mortality in children. a wide spectrum of disorders including developmental abnormali­ties, infections, metabolic and genetic disorders can lead to liver cirrhosis in pediatric patients. determination of its etiology is important for treatment mo­dality, prevention of progressive liver damage, family counseling and pri...

results hla-drb1*07 (rr 5.3, p = 0.0008) and hla-drb1*08 (rr n.c. p = 0.0005) were significantly associated with the risk of pbc development. patients younger than 45 years had significantly higher alanine aminotransferase (p = 0.038) and alkaline phosphatase levels (p = 0.047) than older cases. in comparison to non-cc rs12979860, patients with cc rs12979860 genotype showed an early histologica...

conclusions co-occurrence of pbc with wd is rare, which can cause diffusely intrahepatic copper deposition. early liver biopsy and genetic testing are necessary for the diagnosis. the combination of ursodeoxycholic acid with zinc and sodium dimercaptopropane sulfonate is effective. introduction both primary biliary cirrhosis (pbc) and wilson’s disease (wd) can cause copper retention in the live...

Peroral cholangioscopy is useful in differentiating benign from malignant biliary strictures. However, when conventional biliary access via endoscopic retrograde cholangiopancreatography (ERCP) fails, percutaneous transhepatic cholangioscopy (PTCS) via the SpyGlass cholangioscopy system can be used to achieve a diagnosis. Four patients with biliary strictures in whom conventional ERCP was not p...

Cholecystenteric fistula is one of the rarest complications of biliary lithiasis, with a frequency of less than 1%. Bouveret syndrome is a gastric outlet obstruction produced by gallstone(s) located in the distal stomach or proximal duodenum. The route of gallstone migration to the bowel is most commonly via a cholecystoduodenal fistula; however, fistulization of the stomach is a rarer variatio...

chronic liver diseases in children is the result of many different diseases including: metabolic, genetic, infectious, toxic and idiopathic causes. this was a case series study on 133 infants and children with age range 6 month to 12 years old, who presented clinically with manifestation of chronic liver disease and were admitted to children hospital medical center from year 1999 to 2000. in th...

a 63-year-old woman suffering from progressive systemic sclerosis for about 20 years disclosed symptoms of liver disease within the last three years. diagnosis of biliary cirrhosis was established on the basis of clinical picture, pathological examination of the hepatic tissue sample, immunological tests, and x-ray studies. association of systemic sclerosis with primary biliary cirrhosis is bri...

background: biliary atresia is a 100% fatal disorder without any treatment in infants as a leading cause of cirrhosis and kasai operation, as the only operative choice, which plays a crucial role in increasing their rate of survival. nonetheless, many patients end up with liver transplantation in the future owing to various inevitable hepatic and biliary problems which do remain after the kasai...

Biliary atresia is the most common cause of pathologic jaundice in young infants and results from the obstruction of the extrahepatic bile ducts by an inflammatory and fibro-obliterative process. Although the pathogenesis of the disease is multifactorial, recent patient- and animal-based studies began deciphering the molecular pathways involved in biliary injury and duct obstruction. Using larg...

Schwannomas are derived from Schwann cells, the most common sites being upper extremiteis, trunk, retroperitoneum, head and neck, mediastinum. However they can arise from the gastrointestinal tract too, including the biliary system. We present a case of a 40 year old gentleman, who presented with obstructive jaundice whose computed tomography of the abdomen was suggestive of mass lesion at the ...