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Biliary atresia

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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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Abbreviations

BA:

biliary atresia

BASM:

biliary atresia splenic malformation

PCR:

polymerase chain reaction

RT:

reverse transcriptase

HLA:

human leukocyte antigen

A1AT:

alpha-1-antitrypsin

CMV:

cytomegalovirus

HHV:

human herpes virus

HPV:

human papillomavirus

TNF:

tumour necrosis factor

IFN:

interferon

APC:

antigen-presenting cell

TH :

T helper

SCID:

severe combined immune deficiency

ICAM:

intercellular adhesion molecule

sICAM:

soluble ICAM

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Authors and Affiliations

  1. Paediatric Liver Centre, Institute of Liver Studies, King’s College London School of Medicine, King’s College Hospital, Denmark Hill, London, SE5 9RS, UK

    Giorgina Mieli-Vergani

  2. Institute of Liver Studies, King’s College London School of Medicine, King’s College Hospital, Denmark Hill, London, SE5 9RS, UK

    Diego Vergani

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  1. Giorgina Mieli-Vergani
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  2. Diego Vergani
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Correspondence to Giorgina Mieli-Vergani.

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Mieli-Vergani, G., Vergani, D. Biliary atresia. Semin Immunopathol 31, 371–381 (2009). https://doi.org/10.1007/s00281-009-0171-6

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  • DOI: https://doi.org/10.1007/s00281-009-0171-6

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