Biliary atresia is a rare condition in newborn children in which the biliary tract between the liver and the intestine is blocked or absent. If unrecognised, the condition leads to liver failure but not (as one might think) to kernicterus. It has no known cause, and the only effective treatment is by surgery. more...
Symptoms and diagnosis
Initially, the symptoms are indistinguishable from neonatal jaundice, a common phenomenon. Prolonged jaundice that is resistant to phototherapy and/or exchange transfusions should prompt a search for secondary causes. By this time, liver enzymes are generally measured, and these tend to be grossly deranged, hyperbilirubinaemia is conjugated and therefore does not lead to kernicterus. Ultrasound investigation or other forms of imaging can confirm the diagnosis.
There is no known cause of biliary atresia, although it may be associated with a number of rare syndromes, such as malrotation of the intestine.
As the biliary tract cannot transport bile to the intestine, bile is retained in the liver and results in damage and the ultimate destruction of that organ.
If the intrahepatic biliary tree is unaffected, surgical reconstruction of the extrahepatic biliary tract is possible.
If the atresia is complete, only liver transplantation is a therapeutic option.
Intro. to pediatric blood tests for liver function
Choledochal cyst associated with extrahepatic bile duct atresia
Biliary Atresia Network
Children's Liver Association for Support Services
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