Biliary atresia is a serious but rare disease of the liver that affects newborn infants.
It occurs in about one in 10,000 children and is more common in girls than in boys and in Asian and African-American newborns than in Caucasian newborns. The cause of biliary atresia is not known, and treatments are only partially successful. Biliary atresia is the most common reason for liver transplantation in children in the United States and most of the Western world.
The liver damage incurred from biliary atresia is caused by injury and loss (atresia) of the bile ducts that are responsible for draining bile from the liver. Bile is made by the liver and passes through the bile ducts and into the intestines where it helps digest food, fats, and cholesterol. The loss of bile ducts causes bile to remain in the liver. When bile builds up it can damage the liver, causing scarring and loss of liver tissue. Eventually the liver will not be able to work properly and cirrhosis will occur. Once the liver fails, a liver transplant becomes necessary. Biliary atresia can lead to liver failure and the need for liver transplant within the first 1 to 2 years of life.