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Seminars in Immunopathology
Published in: Seminars in Immunopathology 3/2009

01-09-2009 | Review

Biliary atresia

Authors: Giorgina Mieli-Vergani, Diego Vergani

Published in: Seminars in Immunopathology | Issue 3/2009

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Abstract

Biliary atresia (BA) is a condition unique to infancy. It results from inflammatory destruction of the intrahepatic and extrahepatic bile ducts. It is the most frequent surgically correctable liver disorder in infancy and the most frequent indication for liver transplantation in paediatric age. Clinical presentation is in the first few weeks of life with conjugated hyperbilirubinaemia (dark urine and pale stools); other manifestations of liver disease, such as failure to thrive, splenomegaly and ascites, appear only later, when surgery is unlikely to be successful. Hence, all infants with conjugated hyperbilirubinaemia must be urgently referred to specialised centres for appropriate treatment. Success of surgery depends on the age at which it is performed. With corrective surgery, followed, when necessary, by liver transplantation, the overall survival rate is approximately 90%. The cause of BA is unknown, but there is evidence for the involvement of infectious, genetic and immunologic mechanisms, which will be discussed in this review.
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Metadata
Title
Biliary atresia
Authors
Giorgina Mieli-Vergani
Diego Vergani
Publication date
01-09-2009
Publisher
Springer-Verlag
Published in
Seminars in Immunopathology / Issue 3/2009
Print ISSN: 1863-2297
Electronic ISSN: 1863-2300
DOI
https://doi.org/10.1007/s00281-009-0171-6

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