European biliary atresia registries: summary of a symposium

Article Article scientifique Article European biliary atresia registries: summary of a symposium eng 2008 author Petersen C. author Harder D. author Abola Z. author Alberti D. author Becker T. author Chardot Christophe 594877 author Davenport M. author Deutschmann A. author Khelif K. author Kobayashi Hajime 542748 author Kvist N. author Leonhardt J. author Melter M. author Pakarinen M. author Pawlowska Joanna 758359 author Petersons A. author Pfister E-D author Rygl M. author Schreiber R. author Sokol R. author Ure B. author Veiga C. author Verkade H. author Wildhaber Barbara 595596 author Yerushalmi B. author Kelly D. 111-116 10.1055/s-2008-1038479 18437656 0939-7248 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. Biliary Atresia/*epidemiology/surgery Europe/epidemiology *European Continental Ancestry Group Humans Incidence Infant, Newborn International Cooperation *Registries Survival Analysis Treatment Outcome Dewey 590 Dewey 618 44 Département de génétique et évolution 162 58 Département de pédiatrie, gynécologie et obstétrique 223 false 546 Recherche clinique en chirurgie pédiatrique 886 European journal of pediatric surgery 18 2 99da91ee-977d-4b8f-a30a-2d1ee3b250ae Article Restricted file1.pdf 98349 application/pdf restrictedAccess Faculté des sciences / Section de biologie / Département de génétique et évolution Faculté de médecine / Section de médecine clinique / Département de pédiatrie, gynécologie et obstétrique

Title	Published in	Access level	OA Policy	Year	Views	Downloads

European biliary atresia registries: summary of a symposium	European journal of pediatric surgery			2008	626	0

Archive ouverte UNIGE

Pawlowska, Joanna