Biliary atresia epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Saud Khan M.D.

Overview

Biliary atresia is a very rare disorder. About one in 10,000 to 20,000 babies in the U.S are affected every year. About 400-600 new cases are described in the United States each year (according to rarediseases.org). 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.

Epidemiology and Demographics

It is common for only one child in a pair of twins or within the same family to have the condition. There seems to be no link to medications or immunizations given immediately before or during pregnancy. Diabetes during pregnancy particularly during the first trimester seems to predispose to a number of distinct congenital abnormalities in the infant such as sacral agenesis, transposition of the great vessels and the syndromic form of biliary atresia.

Incidence

• The incidence of biliary atresia is 1 in 10,000-15,000 newborn infants.

Race

• Biliary atresia seems to affect Asians and African Americans more often than Caucasians.

Gender

• Gender bias remains controversial. Some studies suggest that biliary atresia affects females slightly more often than males, others report a clear male predilection.