New Video on how to use EBAR - The European Biliary Atresia Registry of the ERN RARE-LIVER

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Biliary Atresia (BA) is a rare paediatric liver disease with an estimated prevalence of 1 / 14.000-21.000 births in Europe (~ 270 cases / year). Its aetiology is still unknown but recent studies favour an aetiology through microbe- or toxin-triggered immune dysregulation in liver and bile ducts leading to obliteration and fibrosis.

Management of BA is challenging as only 50% of patients in Europe are treated in centres with multidisciplinary expertise and many patients are identified in a later stage of the disease which leads to impaired long-term outcome following the Kasai procedure (portoenterostomy). Novel approaches to therapy are currently being studied in trials, however, their impact on outcome is yet unclear.
The European Biliary Atresia Registry (EBAR) is implemented in the European Reference Network RARE LIVER to analyse and improve care for BA. EBAR can facilitate standardization of care, implementation of novel therapies and connect centers to drive research on BA.

Key Aims
▪ Improve data on the incidence, diagnostic features and treatment of BA in Europe
▪ Document quality of care indicators (e.g., “age at time of Kasai”)
▪ Document co-morbidities (e.g., “failure to thrive”)
▪ Document outcome (e.g., “survival”, “pre-transplant status”, “survival with native liver”)
▪ Document the impact of currently available and novel treatments on outcome

Data Collection
▪ Prospective registry design, focus on all incident cases
▪ eCRF (electronic case report form) on Castor- as simple as possible and as detailed as necessary
▪ Core and expanded data displayed by a “traffic light system”)
▪ Sections:
− Patient population (e.g., consent, demographics)
− Initial diagnosis (e.g., clinical findings, clinical chemistry, ultrasound, liver biopsy, treatment, complications)
− Surgical interventions (Kasai procedure, liver transplantation)
− Additional reports (further diagnostics, treatment, follow-ups)
− End of study (discontinuation)

Administration
▪ Funded by unrestricted grants
▪ Cooperative administration by University Medical Centres Tübingen and Hamburg
▪ Steering group: Patient representative, C. Schramm (Hamburg), C. Slavetinsky & E. Sturm (Tübingen), O. Madadi-Sanjani & C. Petersen (Hannover),
B. Fischler (Stockholm), B. Hansen (Rotterdam)
▪ Accessibility: ERN website, email, periodic mailings
▪ Inclusivity: Step-wise opening, ERN members and non-members
▪ Obligatory contribution to ERN RARE-LIVER centres
▪ Data sharing agreement with the Hamburg centre required (one R-LIVER agreement; contributors to e.g., AIH register need no further agreement)
▪ Contributors will obtain the right to authorship and will be compensated financially

Introducing EBAR  - the European Biliary Atresia Registry of the ERN RARE-LIVER

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If you have any questions contact Biliary Atresia Registry: biliaryatresia.registry@med.uni-tuebingen.de
If you want to join the EBAR (European Biliary Atresia Registry) contact the ERN RARE-LIVER Office.