Biliary Atresia Registry

12/01/2022 to 01/01/2025

Biliary Atresia (BA) is a rare liver disease with an estimated prevalence of 1-9/ 100.000 in
Europe. It´s etiology is still unknown but increasing evidence points to an immunological dysregulation
on the basis of a perinatal infection leading to bile duct obliteration and fibrosis. Organizing care for BA
cases is challenging as only approximately 50% of patients are treated in expert (ERN RARE LIVER
certified) centers in Europe and many patients are identified in a later stage of the disease which leads
to impaired long-term outcome following the Kasai-intervention (porto-enterostomy). In consequence,
a decrease of survival with the native liver ensues with need for early liver transplantation in the first 2
years of life. Novel approaches to therapy are currently being studied in trials, their impact on outcome
is unclear.
Aim of the registry to
- determine the incidence of BA in Europe
- document quality of care indicators (e.g. , “age at time of Kasai”, an ESPGHAN quality of care indicator))
- document co-morbidities (e.g., “failure to thrive”)
- document outcome (endpoints: “survival” and “survival with native liver”)
- document the impact of currently available and novel treatments on outcome