The syndromic form of biliary atresia accounts for 10-25% and is associated with a poor prognosis due to associated anomalies. We report a case of extrahepatic biliary atresia and polysplenia syndrome with jaundice since 19th day of life and who had undergone surgical correction of malrotation in the neonatal period. Inspite of successful Kasai's portoenterostomy at 52nd day of life, the child ...

Cu BA, T. Serum and Urine Amino Acid Analysis in Children with Biliary Atresia. Tohoku J. exp. Med., 1983, 139 (1), 61-66 Serum and urine amino acid levels were measured preoperatively and at varying time following successful hepatic portoenterostomy. All of the essential amino acids measured returned to normal levels during the late postoperative period over five years, whereas many of the non...

Biliary atresia is a rare idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extrahepatic biliary ducts. As the disease is progressive all cases will develop portal fibrosis, cirrhosis, and portal hypertension with the sequelae of varices, jaundice, and eventually liver failure requiring a transplant. Survival rates have improved considerably with ma...

Gallbladder duplication is an extremely rare anomaly. Association of gall bladder agenesis with duodenal atresia and biliary atresia has been described. However, association of gall bladder duplication with duodenal atresia hasn't been described so far; we report a case in view of its rarity.

In the development of the human biliary system, the extrahepatic bile ducts (EHBD) develop from the embryonic hepatic diverticulum, while the intrahepatic bile ducts (IHBD) originate within the liver from the ductal plate. The ductal plate is a flat muralium of primitive biliary epithelium that develops in the mesenchyme along the branches of the portal vein, by a process which requires a delic...

Biliary atresia in infants occasionally presents as intracranial, nasal or gastrointestinal bleeding, instead of the classical triad of jaundice, acholia and choluria. We present two female infants aged four and two months, who were hospitalized with convulsive episode, cephalohematoma and drowsiness. Computed tomography findings were subdural hemorrhage in one patient and intraventricular and ...

Biliary atresia is a severe progressive cholangiopathy which leads to early liver cirrhosis and is uniformly fatal. Early surgical intervention (the Kasai procedure) is needed for an improved outcome. However, early recognition and diagnosis is not easy during the neonatal period because of the high incidence of neonatal jaundice, ill-informed and less than urgent appraisal of the clinical mani...

Inherited syndromes of intrahepatic cholestasis and biliary atresia are the most common causes of chronic liver disease and the prime indication for liver transplantation in children. Our understanding of the pathogenesis of these diseases has increased substantially by the discovery of genetic mutations in children with intrahepatic cholestasis and the findings that inflammatory circuits are o...

The aetiology of biliary atresia, the leading cause of neonatal extrahepatic jaundice and the main indication for liver transplantation in children, is unknown. Recent research has focused on an infectious aetiology and the development of viral models in animals. The few published epidemiological studies report conflicting results for seasonal, geographical, and racial variations in incidence. ...

The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicin...