Biliary atresia

Biliary atresia is a rare conditionin newborn childrenin which the biliary tractbetween the liverand the intestineis blocked or absent. If unrecognised, the condition leads to liver failurebut not (as one might think) to kernicterus. It has no known cause, and the only effective treatment is by surgery.

Symptoms and diagnosis

Initially, the symptoms are indistinguishable from neonatal jaundice, a common phenomenon. Prolonged jaundice that is resistant to phototherapy and/or exchange transfusionsshould prompt a search for secondary causes. By this time, liver enzymesare generally measured, and these tend to be grossly deranged, hyperbilirubinaemia is conjugated and therefore does not lead to kernicterus. Ultrasoundinvestigation or other forms of imaging can confirm the diagnosis.

Pathophysiology

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, bileis retained in the liver and results in damage and the ultimate destruction of that organ.

Treatment

If the intrahepatic biliary tree is unaffected, surgical reconstruction of the extrahepatic biliary tract is possible.

If the atresia is complete, only liver transplantationis a therapeutic option.

External links

E-medicine overview

Intro. to pediatric blood tests for liver function

Research Links

Choledochal cyst associated with extrahepatic bile duct atresia

Support groups

Biliary Atresia Network

Children's Liver Association for Support Services

Liver Families

This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Biliary+atresia Wikipedia article Biliary atresia.