Skip to main content

Biliary atresia: the Croatian experience 1992–2006

Abstract

The objective of this study was to determine the outcomes of Croatian children with biliary atresia. Health records of infants born in Croatia between January 1, 1992 and December 31, 2006 who were diagnosed with biliary atresia and treated at a single university center were reviewed. Survival rates were calculated with the Kaplan–Meier method. Twenty-nine patients with biliary atresia were identified. Incidence was one in 23,600 live births (95% confidence interval 1/17,400–1/27,200). The median age at Kasai operation was 66 days (range 22–192). Median follow-up was 2.65 years (range 0.2–14.3). Overall survival rates for the patients who underwent Kasai portoenterostomy, including those six (20%) who subsequently underwent liver transplantation, were 75.6%. Five and 10-year native liver survival rates were 51.7% and 38.8%, respectively (median survival time was 7.88 years). Survival rate curves in two groups of patients according to the biliary atresia phenotype (fetal or perinatal form) were divergent. Survival was 87.7% in perinatal form and 43.8% in fetal form (Breslow chi-square 8.082, p < 0.01). Overall survival rates of patients with biliary atresia in Croatia compared unfavorably with current international standards; this could be improved with earlier referral for liver transplantation. Results of Kasai operation (native liver survival rates) compared favorably with results reported elsewhere.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. 1.

    Altman P, Lilly JR, Greenfeld J et al (1997) A multivariate risk factor analysis of the portoenterostomy procedure for biliary atresia: twenty-five years of experience from two centers. Ann Surg 226:348–355

    CAS  Article  PubMed  Google Scholar 

  2. 2.

    Balistreri WF, Grand R, Hoofnagle JH et al (1996) Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology 23:1682–1692

    CAS  Article  PubMed  Google Scholar 

  3. 3.

    Caton AR, Druschel CM, McNutt LA (2004) The epidemiology of extrahepatic biliary atresia in New York State, 1983–98. Paediatr Perinat Epidemiol 18:97–105

    Article  PubMed  Google Scholar 

  4. 4.

    Chardot C, Carton M, Spire-Bendelac N et al (1999) Epidemiology of biliary atresia in France: a national study 1986–96. J Hepatol 31:1006–1013

    CAS  Article  PubMed  Google Scholar 

  5. 5.

    Davenport M (2005) A challenge on the use of the words embryonic and perinatal in the context of biliary atresia. Hepatology 41:403–404

    Article  PubMed  Google Scholar 

  6. 6.

    Davenport M, Tizzard SA, Underhill J et al (2006) The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study. J Pediatr 149:393–400

    Article  PubMed  Google Scholar 

  7. 7.

    Fischler B, Haglund B, Hjern A (2002) A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia. J Pediatr 141:217–222

    Article  PubMed  Google Scholar 

  8. 8.

    Henriksen NT, Drablos PA, Aagenaes O (1981) Cholestatic jaundice in infancy. The importance of familial and genetic factors in aetiology and prognosis. Arch Dis Child 56:622–627

    CAS  Article  PubMed  Google Scholar 

  9. 9.

    Houwen RH, Kerremans II, van Steensel-Moll HA et al (1988) Time-space distribution of extrahepatic biliary atresia in The Netherlands and West Germany. Z Kinderchir 43:68–71

    CAS  PubMed  Google Scholar 

  10. 10.

    Mack C, Sokol RJ (2005) Unraveling the pathogenesis and etiology of biliary atresia. Pediatr Res 57(No 5, Pt 2):87R–94R

    Article  PubMed  Google Scholar 

  11. 11.

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

    CAS  Article  PubMed  Google Scholar 

  12. 12.

    Nio M, Ohi R (2000) Biliary atresia. Semin Pediatr Surg 9:177–186

    CAS  Article  PubMed  Google Scholar 

  13. 13.

    Nio M, Ohi R, Miyano T et al (2003) Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg 38:997–1000

    Article  PubMed  Google Scholar 

  14. 14.

    Petersen C, Harder D, Abola Z et al (2008) European biliary atresia registries: summary of a symposium. Eur J Ped Surg 18:111–116

    CAS  Article  Google Scholar 

  15. 15.

    Schreiber BA, Barker CC, Roberts EA et al (2007) Biliary atresia: the Canadian experience. J Pediatr 151:659–665

    PubMed  Google Scholar 

  16. 16.

    Serinet MO, Broue P, Jacquemin E et al (2006) Management of patients with biliary atresia in France: results of a decentralized policy 1986–2002. Hepatology 44:75–84

    Article  PubMed  Google Scholar 

  17. 17.

    Serinet M-O, Wildhaber BE, Broue P et al (2009) Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 123:1280–1286

    Article  PubMed  Google Scholar 

  18. 18.

    Shneider BL, Brown MB, Haber B et al (2006) A multicenter study of the outcome of biliary atresia in United States, 1997 to 2000. J Pediatr 148:467–474

    Article  PubMed  Google Scholar 

  19. 19.

    Sokol RJ, Mack C, Narkewicz MR et al (2003) Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 37:4–21

    Article  PubMed  Google Scholar 

  20. 20.

    Sokol RJ, Shepherd RW, Superina R et al (2007) Screening and outcomes in biliary atresia: summary of National Institutes of Health workshop. Hepatology 46(2):566–581

    CAS  Article  PubMed  Google Scholar 

  21. 21.

    Strickland AD, Shannon K (1982) Studies in the etiology of extrahepatic biliary atresia: time-space clustering. J Pediatr 100:749–753

    CAS  Article  PubMed  Google Scholar 

  22. 22.

    Tiao MM, Tsai SS, Kuo HW et al (2008) Epidemiological features of biliary atresia in Taiwan, a national study 1996–2003. J Gastroenterol Hepatol 23:62–66

    PubMed  Google Scholar 

  23. 23.

    Vazquez J, Lopez Gutierrez JC, Gamez M et al (1995) Biliary atresia and the polysplenia syndrome: its impact on final outcome. J Pediatr Surg 30:485–7

    CAS  Article  PubMed  Google Scholar 

  24. 24.

    Wildhaber BE, Majno P, Mayr J et al (2008) Biliary atresia: swiss national study 1994–2004. J Pediatr Gastroenterol Nutr 46:299–307

    Article  PubMed  Google Scholar 

  25. 25.

    Yoon PW, Bresee JS, Olney RS et al (1997) Epidemiology of biliary atresia: a population-based study. Pediatrics 99:376–382

    CAS  Article  PubMed  Google Scholar 

Download references

Author information

Affiliations

Authors

Corresponding author

Correspondence to Jurica Vuković.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Grizelj, R., Vuković, J., Novak, M. et al. Biliary atresia: the Croatian experience 1992–2006. Eur J Pediatr 169, 1529–1534 (2010). https://doi.org/10.1007/s00431-010-1266-8

Download citation

Keywords

  • Biliary atresia
  • Children
  • Incidence
  • Kasai portoenterostomy