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...

OBJECTIVES To define the sensitivity, specificity and accuracy of the ultrasound triangular cord sign and hepatic histopathology, in isolation or in combination, for diagnostic differentiation between biliary atresia and intrahepatic cholestasis. METHODS This was a retrospective study carried out between January 1990 and December 2004. Fifty-one cases of biliary atresia and 45 of intrahepatic...

OBJECTIVE To assess the feasibility of screening for cholestatic hepatobiliary disease and extrahepatic biliary atresia by using tandem mass spectrometry to measure conjugated bile acids in dried blood spots obtained from newborn infants at 7-10 days of age for the Guthrie test. SETTING Three tertiary referral clinics and regional neonatal screening laboratories. DESIGN Unused blood spots f...

Biliary atresia is a neonatal obstructive cholangiopathy characterized by a fibrosclerosing obliteration of the extrahepatic bile duct that uniquely presents in the first months of life (1). The condition occurs in approximately 1 in 8,000 to 1 in 15,000 live births and accounts for 30% of all cases of cholestasis in young infants. Biliary atresia is the most frequent cause of chronic end-stage...

Three infants who presented with clinical features of neonatal cholestasis and clinically suspected to have biliary atresia were found on imaging studies and surgery to have extrahepatic bile duct atresia in association with choledochal cyst. All patients were treated by bilio-enteric bypass procedure. Post operatively jaundice cleared in only two patients. The prognosis of this association dep...

During a period of three years from 1996 to 1998, 124 infants (64 male and 60 female) with an age range of 1-6 months (mean age 1.5 months) with cholestasis were studied. Idiopathic neonatal hepatitis was the most common cause of cholestasis, accounting for 48 cases with a rate of 3'8.70% in a total of 124 patients, followed by galactosemia in 29 patients (23.38%) and extrahepatic biliary ...

Cholestasis in early infancy represents a diagnostic dilemma and most of these infants suffer either from extrahepatic biliary atresia or idiopathic neonatal hepatitis. Differentiation between the two conditions may be extremely difficult both clinically and biochemically, and a diagnostic liver biopsy is usually required. We report on a Sudanese infant who presented at the age of 4 weeks with ...