European biliary atresia registries: Summary of a symposium

Claus Petersen, D. Harder, Zane Ābola, D. Alberti, T. Becker, C. Chardot, M. Davenport, A. Deutschmann, K. Khelif, H. Kobayashi, N. Kvist, J. Leonhardt, M. Melter, M. Pakarinen, J. Pawlowska, Aigars Pētersons, E. D. Pfister, M. Rygl, R. Schreiber, R. SokolB. Ure, C. Veiga, H. Verkade, B. Wildhaber, B. Yerushalmi, D. Kelly

Research output: Contribution to journalArticlepeer-review

32 Citations (Scopus)

Abstract

Biliary atresia (BA) is a rare but potentially devastating disease. The European Biliary Atresia Registry (EBAR) was set up to improve data collection and to develop a pan-national and interdisciplinary strategy to improve clinical outcomes. From 2001 to 2005, 100 centers from 22 countries registered with EBAR via its website (www.biliary-atresia.com). In June 2006, the first meeting was held to evaluate results and launch further initiatives. During a 5-year period, 60 centers from 19 European countries and Israel sent completed registration forms for a total of 514 BA patients. Assuming the estimated incidence of BA in Europe is 1:18 000 live births, 35% of the expected 1488 patients from all EBAR participating countries were captured, suggesting that reporting arrangements need improvement. At the meeting, the cumulative evaluation of 928 BA patients including patients from other registries with variable follow-up revealed an overall survival of 78% (range from 41% to 92%), of whom 342 patients (37%) have had liver transplants. Survival with native liver ranged from 14% to 75%. There was a marked variance in reported management and outcome by country (e.g., referral patterns, timing of surgery, centralization of surgery). In conclusion, EBAR represents the first attempt at an overall evaluation of the outcome of BA from a pan-European perspective. The natural history and outcome of biliary atresia is of considerable relevance to a European population. It is essential that there is further support for a pan-European registry with coordination of clinical standards, further participation of parent support groups, and implementation of online data entry and multidisciplinary clinical and basic research projects.

Original languageEnglish
Pages (from-to)111-116
Number of pages6
JournalEuropean Journal of Pediatric Surgery
Volume18
Issue number2
DOIs
Publication statusPublished - Apr 2008
Externally publishedYes

Keywords*

  • Biliary atresia
  • Clinical research
  • Registry

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database

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