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]Schreiber RA, Kleinman RE. Genetics, immunology, and biliary atresia: an opening or a diversion? J Pediatr Gastroenterol Nutr. 1993;16:111-113.
http://www.ncbi.nlm.nih.gov/pubmed/8450373?tool=bestpractice.com
[11]Cavallo L, Kovar EM, Aqul A, et al. The epidemiology of biliary atresia: exploring the role of developmental factors on birth prevalence. J Pediatr. 2022 Jul;246:89-94.e2.
https://www.jpeds.com/article/S0022-3476(22)00288-8/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/35364097?tool=bestpractice.com
Reported incidence rates vary worldwide with Asian countries having a higher incidence (1:8000).[12]Tiao MM, Tsai SS, Kuo HW, et al. Epidemiological features of biliary atresia in Taiwan, a national study 1996-2003. J Gastroenterol Hepatol. 2008;23:62-66.
http://www.ncbi.nlm.nih.gov/pubmed/17725591?tool=bestpractice.com
The highest incidence has been reported in French Polynesia (1:3500).[13]Schreiber RA, Harpavat S, Hulscher JBF, et al. Biliary atresia in 2021: epidemiology, screening and public policy. J Clin Med. 2022 Feb 14;11(4):999.
https://www.mdpi.com/2077-0383/11/4/999
http://www.ncbi.nlm.nih.gov/pubmed/35207269?tool=bestpractice.com
It is the most common cause of neonatal jaundice requiring surgical intervention and the most common indication for liver transplantation in children.[14]Yoon PW, Bresee JS, Olney RS, et al. Epidemiology of biliary atresia: a population-based study. Pediatrics. 1997;99:376-382.
http://www.ncbi.nlm.nih.gov/pubmed/9041292?tool=bestpractice.com
There is a slight female predominance. In the US, there is increased incidence among African-Americans.[15]Sokol RJ, Mack C, Narkewicz MR, et al. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr. 2003;37:4-21.
http://www.ncbi.nlm.nih.gov/pubmed/12827000?tool=bestpractice.com
Seasonal clustering has been reported that varies between regions and states.[13]Schreiber RA, Harpavat S, Hulscher JBF, et al. Biliary atresia in 2021: epidemiology, screening and public policy. J Clin Med. 2022 Feb 14;11(4):999.
https://www.mdpi.com/2077-0383/11/4/999
http://www.ncbi.nlm.nih.gov/pubmed/35207269?tool=bestpractice.com
[16]Ayas MF, Hillemeier AC, Olson AD. Lack of evidence for seasonal variation in extrahepatic biliary atresia during infancy. J Clin Gastroenterol. 1996;22:292-294.
http://www.ncbi.nlm.nih.gov/pubmed/8771425?tool=bestpractice.com
[17]Caton AR, Druschel CM, McNutt LA. The epidemiology of extrahepatic biliary atresia in New York State, 1983-98. Paediatr Perinat Epidemiol. 2004;18:97-105.
http://www.ncbi.nlm.nih.gov/pubmed/14996248?tool=bestpractice.com
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]Smith BM, Laberge JM, Schreiber R, et al. Familial biliary atresia in three siblings including twins. J Pediatr Surg. 1991;26:1331-1333.
http://www.ncbi.nlm.nih.gov/pubmed/1812269?tool=bestpractice.com