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.

The European Biliary Atresia Registry (EBAR) was founded in 2001 in order to raise awareness of the rare disease biliary atresia and to reinforce international and interdisciplinary cooperation. The number of participating centres and registered patients increases continuously. EBAR activities can be found at the website www.biliary-atresia.com. A first evaluation of the EBAR data is scheduled for 2006, when the registry will alter its infrastructure. This meeting will be organized together with the European Federation for Biliary Atresia Research (EFBAR), a scientific committee, which was founded in 2003. The objectives of this association are to create clinical and basic research studies, which are based on existing registries in Europe.

No abstract

The European Biliary Atresia Registry (EBAR) was founded in 2001 in order to raise awareness of the rare disease biliary atresia and to reinforce international and interdisciplinary cooperation. The number of participating centres and registered patients increases continuously. EBAR activities can be found at the website http://www.biliary-atresia.com. A first evaluation of the EBAR data is scheduled for 2006, when the registry will alter its infrastructure. This meeting will be organized together with the European Federation for Biliary Atresia Research (EFBAR), a scientific committee, which was founded in 2003. The objectives of this association are to create clinical and basic research studies, which are based on existing registries in Europe.

No abstract