Biliary atresia (BA)
is a disastrous liver disease of the newborn with unpredictable outcome. Occurring in about 1 in 14.000 live births, first clinical symptoms of this rare disease are difficult to distinguish from the frequent and mostly benign neonatal jaundice. Therefore, diagnosis and surgical therapy are often decided too late, worsening the chance for a better outcome. However, despite successful surgery, fibrosis of the liver is an ongoing process in the majority and patients with BA are the most frequent indication for liver transplantation (LTx) in childhood.
The long and eventful history of this disease is a story of innovative ideas and promising therapeutic options, as well as curative helplessness and frustrating attempts against an etiologically incomprehensible entity. Nowadays, interdisciplinary and internationally cooperating experts coordinate their competence, taking up the challenge to uncover the etiology and to improve the situation of BA-patients.
In order to put these ideas into practice, the generation of closely aligned, larger databases (multicenter, multinational) will enable high-resolution analyses of broad scope, facilitate regional and even continental collaboration, and foster a “continuously learning health system strategy” as outlined by the Institute of Medicine.
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