Epidemiology

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 in the US.[10][11]​​ Reported incidence rates vary worldwide with Asian countries having a higher incidence (1:8000).[12] The highest incidence has been reported in French Polynesia (1:3500).[13]​ It is the most common cause of neonatal jaundice requiring surgical intervention and the most common indication for liver transplantation in children.[14] There is a slight female predominance. In the US, there is increased incidence among African-Americans.[15] Seasonal clustering has been reported that varies between regions and states.[13][16][17]​​​​​ 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.[18]

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