In babies with biliary atresia, the ducts that are to drain bile from the liver to the intestinal tract do not work, causing the bile to back up in the liver. Inevitably, the liver will scar and begin to malfunction causing a serious need for a transplant. Biliary atresia is the most prevalent cause of liver transplantation in children in the United States; however, it is a very rare disease that will present itself at birth or shortly thereafter. It is important to note that biliary atresia is not an inherited disease and its specific cause is not known. The disorder is more likely to affect girls, premature babies, as well as Asian and African-American babies.
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Honor Society of Nursing (STTI) answered
Johns Hopkins Medicine answered
Biliary atresia, often detected in infancy, is a condition in which the bile ducts that drain bile from the liver to the intestine are blocked or absent. As a result of the blockage, the liver cannot perform its normal functions. The cause of the disease is unknown, but it is believed not to be hereditary. Symptoms of the disease may include poor weight gain and jaundice. Testing used to diagnose biliary atresia include a liver biopsy, a Tc-HIDA scan of the liver and an intra-operative cholangiogram. If preliminary tests indicate biliary atresia, the child will need exploratory surgery. A Kasai procedure may be performed in which the non-functioning portion of the bile duct is replaced with a piece of small intestine (jejunum), providing a route for bile drainage. The Kasai procedure may often be a temporary measure, although it can postpone a transplant for several years. Approximately one third of patients have long-term, good results from the Kasai procedure; one third of the patients develop cirrhosis in early or late childhood and require transplantation; and one third of the patients develop rapid early cirrhosis and require transplant in early infancy.