Registries for Biliary Atresia and Related Disorders

Abstract

Most neonatal cholestatic disorders are rare diseases (RD), but they represent the leading indication for pediatric liver transplantation. From the pediatric surgical point of view, biliary atresia (BA) and choledochal cyst are from particular interest because diagnostic pathways and therapeutic algorithms are diverging as well as outcome parameters of follow-up studies. In addition, screening programs for BA are still at an early stage while transition of those patients with and without liver transplantation is not established yet. The newly created Web site www.bard-online.com focuses on BA and related diseases and includes decentralized online registries, information for patients and health workers, as well as a platform for interdisciplinary communication and service offerings. This initiative was started to compensate for disadvantages for patients with RD and to help unifying the community of clinicians and researchers in this field.

Keywords

biliary atresia - neonatal cholestasis - registries - choledochal cyst

• References

• 1 Regulation (EC) No 1786/2002 of the European Parliament and of the Council of 23 Sep 2002 adopting a programme of Community action in the field of public health (2003–2008). Official Journal of the European Communities. 2002; L271: 1-11
• 2 Petersen C, Davenport M. Aetiology of biliary atresia: what is actually known?. Orphanet J Rare Dis 2013; 8: 128-141
• 3 Reuben A. The sensei of Sendai: correcting the uncorrectable. Hepatology 2003; 37 (4) 952-955
• 4 Petersen C. Pathogenesis and treatment opportunities for biliary atresia. Clin Liver Dis 2006; 10 (1) 73-88 , vi
• 5 Hsiao CH, Chang MH, Chen HL , et al; Taiwan Infant Stool Color Card Study Group. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 2008; 47 (4) 1233-1240
• 6 Wildhaber BE. Screening for biliary atresia: Swiss stool color card. Hepatology 2011; 54 (1) 367-368 , author reply 369
• 7 Schreiber RA, Masucci L, Kaczorowski J , et al. Home-based screening for biliary atresia using infant stool colour cards: a large-scale prospective cohort study and cost-effectiveness analysis. J Med Screen 2014; 21 (3) 126-132
• 8 Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Auvert B. Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996. Hepatology 1999; 30 (3) 606-611
• 9 Petersen C, Harder D, Abola Z , et al. European biliary atresia registries: summary of a symposium. Eur J Pediatr Surg 2008; 18 (2) 111-116
• 10 Davenport M, De Ville de Goyet J, Stringer MD , et al. Seamless management of biliary atresia in England and Wales (1999-2002). Lancet 2004; 363 (9418) 1354-1357
• 11 Chardot C, Buet C, Serinet MO , et al. Improving outcomes of biliary atresia: French national series 1986-2009. J Hepatol 2013; 58 (6) 1209-1217
• 12 Leonhardt J, Kuebler JF, Leute PJ , et al. Biliary atresia: lessons learned from the voluntary German registry. Eur J Pediatr Surg 2011; 21 (2) 82-87
• 13 Shneider BL, Brown MB, Haber B , et al; Biliary Atresia Research Consortium. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 2006; 148 (4) 467-474
• 14 Davenport M, Ong E, Sharif K , et al. Biliary atresia in England and Wales: results of centralization and new benchmark. J Pediatr Surg 2011; 46 (9) 1689-1694
• 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 (7) 997-1000
• 16 Schreiber RA, Barker CC, Roberts EA, Martin SR ; Canadian Pediatric Hepatology Research Group. Biliary atresia in Canada: the effect of centre caseload experience on outcome. J Pediatr Gastroenterol Nutr 2010; 51 (1) 61-65
• 17 Wildhaber BE, Majno P, Mayr J , et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr 2008; 46 (3) 299-307
• 18 de Vries W, de Langen ZJ, Groen H , et al; Netherlands Study Group of Biliary Atresia and Registry (NeSBAR). Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008. J Pediatr 2012; 160 (4) 638-644.e2
• 19 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 (1) 62-66