Neonatal cholestasis is a prolonged elevation of the serum levels of conjugated bilirubin beyond the first 14 days of life. Commonest etiologies include extrahepatic conditions like biliary atresia, intrahepatic like congenital malformations, infections and inborn errors of metabolism. TORCH infections constitute 22% cases of neonatal hepatitis, of which Cytomegalovirus is the commonest agent. ...

Biliary atresia is a rare birth defect that affects 1 in 12,000 to 1 in 19,500 live births. We used data from the National Birth Defects Prevention Study, a multistate case-control study, to identify potential risk factors for isolated biliary atresia (no additional unrelated major birth defects diagnosed). Infants were identified from eight states from 1997 to 2002, with clinical information a...

Cholestasis may result from a failure in bile secretion in hepatocytes or ductular cells, or from a blockade to the free bile flow. Human cholestasis may be induced by many drugs, being antibiotics the more common. Other types of cholestasis seen in humans are a group of familial cholestatic disorders, obstructive cholestasis, primary biliary cirrhosis, extrahepatic biliary atresia, primary scl...

The potential usefulness of orthotopic liver transplantation (OLTx) in the treatment of end-stage liver diseases was recognized in the 1950s, and the first clinical attempt was carried out at the University of Colorado in Denver, on March 1, 1963 [1,2]. It was unsuccessful: the 3 year old patient, affected by extrahepatic biliary atresia, exsanguinated on the operating table. Among the next 8 p...

The intra and extrahepatic bile ducts of the liver are developmentally distinct, and may be differentially affected by certain diseases. However, differences between intra and extrahepatic cholangiocytes, and between neonatal and adult cells, are not well understood. Methods for the isolation of cholangiocytes from intrahepatic bile ducts are well established(1-4). Isolation of extrahepatic duc...

Alagille syndrome may mimic biliary atresia in early infancy. Since mutations in JAG1 typical for Alagille syndrome type 1 have also been found in biliary atresia, we aimed to identify JAG1 mutations in newborns with proven biliary atresia (n = 72). Five biliary atresia patients with cholestasis, one additional characteristic feature of Alagille syndrome and ambiguous liver histology were singl...

The biliary tree is composed of intrahepatic and extrahepatic ducts and is lined by simple columnar/cuboidal epithelium. Particularly in evaluation of extrahepatic bile ducts, cytology is commonly utilized. Herein we review common indications, methodology and microscopic features as relates to biliary tract cytologic diagnoses. Additionally, we briefly discuss newer techniques that can be used ...

Introduction Early diagnosis of biliary atresia is very important for better outcome of treatment. Ultrasonography is one of the diagnostic tools for early differentiation of biliary atresia from other causes of neonatal cholestasis. It has been reported that triangular cord sign (TACS) in sonography is a reliable sign for diagnosis of biliary atresia. The aim of this study was to re-assess the...