Epidemiology

Biliary atresia occurs in approximately 1 in 18,000 live births in Western Europe.[10] In the world, the reported incidence varies from 5 to 32 cases per 100,000 live births, and is highest in Asia and the Pacific region. Females are affected slightly more often than males.[10] Estimates of the incidence of biliary atresia in Taiwan and Japan range from 1.1 to 3.7 cases per 10,000 live births.[11][12][13]

In the US, biliary atresia occurs with an estimated frequency of 1 in 8000 to 15,000 live births, resulting in 250 to 400 new cases per year.[14][15] The highest incidence has been reported in French Polynesia (1:3500).[16]​​​ It is the most common cause of neonatal jaundice requiring surgical intervention and the most common indication for liver transplantation in children.[17] There is increased incidence among African-Americans.[18] Seasonal clustering has been reported that varies between regions and states.[16][19][20]​​ ​​​A possible genetic component remains elusive; identical twins are not similarly affected, yet there have been several isolated case reports of multiple first-degree relatives, including twins, being affected.[21]

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