Biliary Atresia
Definition
Biliary atresia is the failure of a fetus to develop an adequate pathway for bile to drain from the liver to the intestine.
Description
Biliary atresia is the most common lethal liver disease in children, occurring once every 10,000–15,000 live births. Half of all liver transplants are done for this reason.
The normal anatomy of the bile system begins within the liver, where thousands of tiny bile ducts collect bile from liver cells. These ducts merge into larger and larger channels, like streams flowing into rivers, until they all pour into a single duct that empties into the duodenum (first part of the small intestine). Between the liver and the duodenum this duct has a side channel connected to the gall bladder. The gall bladder stores bile and concentrates it, removing much of its water content. Then, when a meal hits the stomach, the gall bladder contracts and empties its contents.
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