Biliary Atresia Clinical Care Program

About Biliary Atresia

Biliary atresia is a serious liver disorder that occurs shortly after birth when a newborn's bile ducts (the tubes that carry bile from the liver) become blocked. The body needs bile to aid digestion and carry wastes from the liver out of the body. When blocked ducts prevent bile from being excreted, the liver becomes damaged and other body functions are affected.

Causes and forms

The cause of biliary atresia is a mystery. Two forms of biliary atresia are generally recognized though more may exist.

The most common is the perinatal form which accounts for 65-90% of all cases. These children are typically healthy appearing at birth. As a newborn, these infants pass normally colored stool and are of average weight. Between 4-8 weeks of age, jaundice develops.

The less common, embryonic or fetal form occurs in 10-35% of cases. These children are jaundiced as newborns and frequently there are associated birth anomalies such as abnormalities of the heart, major blood vessels, spleen and intestines. Neither form is thought to be inherited. Many theories about the causes of biliary atresia have been proposed, but none have been proven.

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