NDLI: Biliary atresia

Content Provider	Springer Nature Link
Author	Mieli Vergani, Giorgina Vergani, Diego
Copyright Year	2009
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.
Starting Page	371
Ending Page	381
Page Count	11
File Format	PDF
ISSN	18632297
Journal	Springer Seminars in Immunopathology
Volume Number	31
Issue Number	3
e-ISSN	18632300
Language	English
Publisher	Springer-Verlag
Publisher Date	2009-06-17
Publisher Place	Berlin, Heidelberg
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Conjugated hyperbilirubinaemia Neonatal liver disease Viral infection Immune pathogenesis Internal Medicine Immunology
Content Type	Text
Resource Type	Article
Subject	Immunology and Allergy Immunology

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