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Orphanet J Rare Dis. 2013 Aug 29;8:128. doi: 10.1186/1750-1172-8-128.

Aetiology of biliary atresia: what is actually known?

Author information

1
Department of Pediatric Surgery, Hannover Medical School, Carl-Neuberg-Str, 1, 30625 Hannover, Germany. petersen.claus@mh-hannover.de.

Abstract

Biliary atresia (BA) is a rare disease of unknown etiology and unpredictable outcome, even when there has been timely diagnosis and exemplary surgery. It has been the commonest indication for liver transplantation during childhood for the past 20 years. Hence much clinical and basic research has been directed at elucidating the origin and pathology of BA. This review summarizes the current clinical variations of BA in humans, its occasional appearance in animals and its various manifestations in the laboratory as an experimental model.

PMID:
23987231
PMCID:
PMC3766137
DOI:
10.1186/1750-1172-8-128
[Indexed for MEDLINE]
Free PMC Article

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