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35 - Biliary atresia

from Part IV - Abdomen

Published online by Cambridge University Press:  08 January 2010

By
Edward R. Howard
Edited by
Mark D. Stringer ,
Keith T. Oldham  and
Pierre D. E. Mouriquand
Edward R. Howard
Affiliation:
King's College Hospital, London, UK
Mark D. Stringer
Affiliation:
University of Otago, New Zealand
Keith T. Oldham
Affiliation:
Children's Hospital of Wisconsin
Pierre D. E. Mouriquand
Affiliation:
Debrousse Hospital, Lyon
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Summary

Introduction

Biliary atresia presents in the neonatal period, occurs with a frequency of between 1 in 8000 and 1 in 16000 live births and accounts for more than 50% of pediatric liver transplantation. The cause of the disease remains obscure but current evidence suggests that there is more than one etiologic factor. Both the intra- and extrahepatic bile ducts are affected and affected infants present with jaundice and pale stools within the first few weeks of life. The intrahepatic pathology, which has been likened to sclerosing cholangitis, is accompanied by an inflammatory sclerosing lesion of the extrahepatic bile ducts, which results in obstruction of the lumen and, in some cases, complete disappearance of segments of the biliary tract. Death from cirrhotic liver failure occurs within 2 years in untreated cases and biliary atresia represents the most frequent reason for liver transplantation in childhood.

Historical issues

Thomson3 published the first major review in 1892. He collected 49 cases from the literature and added a further case of his own. He recorded that “the children themselves are either jaundiced at birth, or they become so within the first week or so of life; otherwise they are healthy and well nourished.” He concluded that, whatever the etiology of the condition, it was characterized by a progressive destructive inflammatory lesion of the biliary tract.

Type
Chapter
Information
Pediatric Surgery and Urology
Long-Term Outcomes
 , pp. 446 - 464
Publisher: Cambridge University Press
Print publication year: 2006

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