J Korean Med Sci.  2017 Apr;32(4):656-660. 10.3346/jkms.2017.32.4.656.

Epidemiology of Biliary Atresia in Korea

• Lee KJ
• Kim JW
• Moon JS
• Ko JS

• KMID: 2371452
• DOI: http://doi.org/10.3346/jkms.2017.32.4.656

Abstract

Biliary atresia (BA) is the major cause of cholestasis and the leading indication for liver transplantation (LT). However, the incidence of BA in Korea has not been reported. The aim of this study was to investigate the incidence and clinical outcomes of BA in Korea. We used the Korean universal health insurance database and extracted data regarding BA patients younger than 18 years of age admitted between 2011 and 2015. The incidence of BA was calculated by dividing the number of BA patients by the number of live births. Two hundred forty infants were newly diagnosed with BA. A total of 963 BA patients younger than 18 years of age were followed up for 5 years. The overall incidence of BA was 1.06 cases per 10,000 live births. The incidence of BA was 1.4 times higher for female patients than for male patients. Additionally, significant seasonal variation was observed; in particular, the incidence of BA was 2 times higher from June through August than from December through February. Congenital anomalies were found in 38 of 240 patients (15.8%). Congenital heart diseases were major associated congenital anomalies (6.3%). Several complications developed during the study period, including cholangitis (24.0%), varix (6.2%), and gastrointestinal bleeding (4.4%). Three hundred and one of the 963 BA patients under 18 years of age (31.3%) received LT for BA. The incidence of BA is higher in Korea than that in Western countries. We also report significant gender-associated differences and seasonal variation with respect to the incidence of BA.

Keyword

Biliary Atresia; Incidence; Liver Transplantation

Figure

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  Fig. 1 Seasonal variation in the incidence of BA. BA = biliary atresia, RR = relative risk. *P < 0.05.

Reference

1. Yoon PW, Bresee JS, Olney RS, James LM, Khoury MJ. Epidemiology of biliary atresia: a population-based study. Pediatrics. 1997; 99:376–382.

2. Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Auvert B. Epidemiology of biliary atresia in France: a national study 1986-96. J Hepatol. 1999; 31:1006–1013.

3. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet. 2000; 355:25–29.

4. Perlmutter DH, Shepherd RW. Extrahepatic biliary atresia: a disease or a phenotype? Hepatology. 2002; 35:1297–1304.

5. 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.

6. Wada H, Muraji T, Yokoi A, Okamoto T, Sato S, Takamizawa S, Tsugawa J, Nishijima E. Insignificant seasonal and geographical variation in incidence of biliary atresia in Japan: a regional survey of over 20 years. J Pediatr Surg. 2007; 42:2090–2092.

7. Tiao MM, Tsai SS, Kuo HW, Chen CL, Yang CY. Epidemiological features of biliary atresia in Taiwan, a national study 1996-2003. J Gastroenterol Hepatol. 2008; 23:62–66.

8. Grizelj R, Vuković J, Novak M, Batinica S. Biliary atresia: the Croatian experience 1992-2006. Eur J Pediatr. 2010; 169:1529–1534.

9. Lin YC, Chang MH, Liao SF, Wu JF, Ni YH, Tiao MM, Lai MW, Lee HC, Lin CC, Wu TC, et al. Decreasing rate of biliary atresia in Taiwan: a survey, 2004-2009. Pediatrics. 2011; 128:e530–6.

10. Utterson EC, Shepherd RW, Sokol RJ, Bucuvalas J, Magee JC, McDiarmid SV, Anand R; SPLIT Research Group. Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J Pediatr. 2005; 147:180–185.

11. McDiarmid SV, Anand R, Lindblad AS; SPLIT Research Group. Studies of Pediatric Liver Transplantation: 2002 update. An overview of demographics, indications, timing, and immunosuppressive practices in pediatric liver transplantation in the United States and Canada. Pediatr Transplant. 2004; 8:284–294.

12. Byun J, Yi NJ, Lee JM, Suh SW, Yoo T, Choi Y, Ko JS, Seo JK, Kim H, Lee HW, et al. Long term outcomes of pediatric liver transplantation according to age. J Korean Med Sci. 2014; 29:320–327.

13. Balistreri WF, Grand R, Hoofnagle JH, Suchy FJ, Ryckman FC, Perlmutter DH, Sokol RJ. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology. 1996; 23:1682–1692.

14. Davenport M. Biliary atresia: outcome and management. Indian J Pediatr. 2006; 73:825–828.

15. Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K. Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg. 2003; 38:997–1000.

16. Houwen RH, Kerremans II, van Steensel-Moll HA, van Romunde LK, Bijleveld CM, Schweizer P. Time-space distribution of extrahepatic biliary atresia in The Netherlands and West Germany. Z Kinderchir. 1988; 43:68–71.

17. Nakamizo M, Toyabe S, Kubota M, Komata O, Suzuki H, Akazawa K. Seasonality in the incidence of biliary atresia in Japan. Acta Paediatr. 2006; 95:509–510.

18. Wildhaber BE, Majno P, Mayr J, Zachariou Z, Hohlfeld J, Schwoebel M, Kistler W, Meuli M, Le Coultre C, Mentha G, et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr. 2008; 46:299–307.

19. Livesey E, Cortina Borja M, Sharif K, Alizai N, McClean P, Kelly D, Hadzic N, Davenport M. Epidemiology of biliary atresia in England and Wales (1999-2006). Arch Dis Child Fetal Neonatal Ed. 2009; 94:F451–5.

20. Guttman OR, Roberts EA, Schreiber RA, Barker CC, Ng VL; Canadian Pediatric Hepatology Research Group. Biliary atresia with associated structural malformations in Canadian infants. Liver Int. 2011; 31:1485–1493.

21. Jimenez-Rivera C, Jolin-Dahel KS, Fortinsky KJ, Gozdyra P, Benchimol EI. International incidence and outcomes of biliary atresia. J Pediatr Gastroenterol Nutr. 2013; 56:344–354.

22. Tayler R, Barclay AR, Rogers P, McIntyre K, Russell RK, Devadason D, Bisset WM, Ling SC, McGrogan P. Scottish outcomes for extra hepatic biliary atresia post-rationalisation of services. Arch Dis Child. 2013; 98:381–383.

23. Smith BM, Laberge JM, Schreiber R, Weber AM, Blanchard H. Familial biliary atresia in three siblings including twins. J Pediatr Surg. 1991; 26:1331–1333.

24. Shneider BL, Brown MB, Haber B, Whitington PF, Schwarz K, Squires R, Bezerra J, Shepherd R, Rosenthal P, Hoofnagle JH, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr. 2006; 148:467–474.

25. Hol L, van den Bos IC, Hussain SM, Zondervan PE, de Man RA. Hepatocellular carcinoma complicating biliary atresia after Kasai portoenterostomy. Eur J Gastroenterol Hepatol. 2008; 20:227–231.

26. Hadžić N, Quaglia A, Portmann B, Paramalingam S, Heaton ND, Rela M, Mieli-Vergani G, Davenport M. Hepatocellular carcinoma in biliary atresia: King’s College Hospital experience. J Pediatr. 2011; 159:617–622.e1.

27. Brunati A, Feruzi Z, Sokal E, Smets F, Fervaille C, Gosseye S, Clapuyt P, de Ville de Goyet J, Reding R. Early occurrence of hepatocellular carcinoma in biliary atresia treated by liver transplantation. Pediatr Transplant. 2007; 11:117–119.

28. Shirouzu Y, Kasahara M, Takada Y, Taira K, Sakamoto S, Uryuhara K, Ogawa K, Doi H, Egawa H, Tanaka K. Development of pulmonary hypertension in 5 patients after pediatric living-donor liver transplantation: de novo or secondary? Liver Transpl. 2006; 12:870–875.

29. Soh H, Hasegawa T, Sasaki T, Azuma T, Okada A, Mushiake S, Kogaki S, Matsushita T, Harada T. Pulmonary hypertension associated with postoperative biliary atresia: report of two cases. J Pediatr Surg. 1999; 34:1779–1781.

30. Kim JM, Kim KM, Yi NJ, Choe YH, Kim MS, Suh KS, Kim SI, Lee SK, Lee SG. Pediatric liver transplantation outcomes in Korea. J Korean Med Sci. 2013; 28:42–47.