What's New in Biliary Atresia?

Abstract

The main problem with biliary atresia (BA) is three-fold. Early diagnosis and timely therapy is mandatory in this rare and life-threatening disease. In as much as the aetiology of BA is unknown, all therapeutic attempts are still symptomatically orientated and finally, at the end-stage of the disease, the majority of the patients need organ replacement. Due to promising interdisciplinary cooperation and improved outcomes after liver transplantation, the overall survival rate of patients with BA increased remarkably during the last decades. Additionally, every effort was made in clinical and basic research to obtain a better understanding of the disease and its clinical course. Nevertheless, the nature of biliary atresia still remains unclear and therapeutic options are unsatisfactory. Numerous papers about BA have appeared, reflecting clinical and scientific activity. Considering recent publications and prospective activities, in the following we will summarise what is new in biliary atresia.

Relationship between prognosis of biliary atresia and infection of cytomegalovirus

“…Though about 30% to 40% of patients will survive for more than 5 years with their native liver after Kasai procedure, [1,2] their long-term outcome remains controversial. BA is a progressive disease and hepatic fi brosis will continue even though a successful HPE was performed.…”

Analysis of the Pathomorphology of the Intra- and Extrahepatic Biliary System in Biliary Atresia

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Introduction

!The pathogenesis of biliary atresia (BA) is still unclear [7,17]. The most likely pathogenesis currently under consideration [5,19,21] is that certain viruses [4] induce an injury to the biliary epithelial cell, triggering a disorder of the immune system [8,10,15] which leads to several pathological changes, such as liver fibrosis, apoptosis of the biliary epithelial cell and intracellular cholestasis.

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High-dose steroids do not improve early outcome in biliary atresia

“…In addition, to make an impact on the outcome of infants with BA, several studies investigating its pathophysiology have been widely conducted. The results lead to the refinement of postoperative treatment [1,4]. Even so, the fundamental problem of BA is that we do not fully understand the underlying disease process.…”