Biliary atresia (BA) or extrahepatic biliary atresia (EHBA)
is a disorder of infants in which there is an obliteration
or discontinuity of the extrahepatic (outside of the liver)
biliary system, resulting in obstruction of bile flow.
The incidence of EHBA is 1 in 10,000 to 13,000 newborns.
The disorder occurs more often in girls than boys, and
20-30% of cases area ssociated with developmental abnormalities
of the heart, digestive tract, or spleen (polysplenia
[more than one spleen] or asplenia [no spleen]) or other
abnormalities. Between 400-600 new cases of BA are encountered
in the United States every year.
Because early intervention is so important, any infant
older than 14 days with jaundice needs to be evaluated.
Surgery is usually required to repair damage or malformations
to promote normal flow of bile. Liver transplant may be
required in severe cases.
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