Biliary atresia overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Biliary atresia, is a disease of the liver in which the bile ducts, inside and outside, are blocked due to damage and scarring. In the congenital form, the common bile duct between the liver and the small intestine is blocked or absent. The acquired type most often occurs in the setting of autoimmune disease, and is one of the principal forms of chronic rejection of a transplanted liver allograft.
Babies born with biliary atresia can undergo surgery called the Kasai procedure before 8 weeks of age, however, a liver transplant may still be necessary.
Classification
Biliary atresia can be classified into several subtypes, since it does not arise from a specific cause. As such, it is broadly classified according to syndromic and non-syndromic expression. Davenport et al. grouped biliary atresia based on the similarities between the different presentations.
Pathophysiology
Causes
There is no known cause of biliary atresia.
Epidemiology and Demograhpics
Biliary atresia is a very rare disorder. About one in 10,000 to 20,000 babies in the U.S are affected every year. Biliary atresia seems to affect girls slightly more often than boys. Within the same family, it is common for only one child in a pair of twins or only one child within the same family to have it. Asians and African-Americans are affected more frequently than Caucasians. There does not appear to be any link to medications or immunizations given immediately before or during pregnancy.