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

A challenge to expedite Genervon's new ASL drug

(Medical Xpress)—The Ice Bucket Challenge to raise awareness for Amyotrophic lateral sclerosis (ALS) went viral on social media last summer. Over 1.2 million videos were posted on Facebook alone. The difficulty ...

Using stem cells to grow new hair

In a new study from Sanford-Burnham Medical Research Institute (Sanford-Burnham), researchers have used human pluripotent stem cells to generate new hair. The study represents the first step toward the development ...

Lung cancer clues found in downstream pathway

Despite the promise of the gene KRAS as a target for treating lung cancer, finding effective therapies has been challenging. Now researchers are traveling down the pathway to find what makes KRAS cancerous.