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

Sperm can carry Ebola for 82 days: WHO

Sperm can carry the Ebola virus for at least 82 days, the World Health Organization said Friday, urging men recovering from the disease to use condoms for three months after the onset of symptoms.

'Chatty' cells help build the brain

The cerebral cortex, which controls higher processes such as perception, thought and cognition, is the most complex structure in the mammalian central nervous system. Although much is known about the intricate ...

Duality in the human genome

Humans don't like being alone, and their genes are no different. Together we are stronger, and the two versions of a gene – one from each parent – need each other. Scientists at the Max Planck Institute ...

"Body recognition" compares with fingerprint ID

(Medical Xpress)—University of Adelaide forensic anatomy researchers are making advances in the use of "body recognition" for criminal and missing persons cases, to help with identification when a face ...