Biliary Atresia

Biliary atresia, also known as "extrahepatic ductopenia" and "progressive obliterative cholangiopathy" is a congenital or acquired disease of the liver and one of the principal forms of chronic rejection of a transplanted liver allograft. As a birth defect in newborn infants, it has an occurrence of 1/10,000 to 1/15,000 cases in live births in the United States. In the congenital form, the common bile duct between the liver and the small intestine is blocked or absent. The acquired type most often occurs in the setting of autoimmune disease, and is one of the principal forms of chronic rejection of a transplanted liver allograft.

Infants and children with biliary atresia have progressive cholestasis with all the usual concomitant features: pruritus, malabsorption with growth retardation, fat-soluble vitamin deficiencies, hyperlipidemia, and eventually cirrhosis with portal hypertension. If unrecognized, the condition leads to liver failure -- but not kernicterus, as the liver is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood-brain barrier. The cause of the condition is unknown. The only effective treatments are certain surgeries such as the kasai procedure, or liver transplantation.

This text uses material from Wikipedia licensed under CC BY-SA

Latest Spotlight News

Promising progress for new treatment of type 1 diabetes

New research from Uppsala University shows promising progress in the use of anti-inflammatory cytokine for treatment of type 1 diabetes. The study, published in the open access journal Scientific Reports, reveals that administration ...

Blocking a gene reduces fat

By blocking the expression of a certain gene in patients, University of Montreal researchers have contributed to the demonstration of great decreases in the concentration of triglycerides in their blood, even in various severe ...

Surprising similarity in fly and mouse motion vision

At first glance, the eyes of mammals and those of insects do not seem to have much in common. However, a comparison of the neural circuits for detecting motion shows surprising parallels between flies and mice. Scientists ...