Biliary atresia
When this surveillance occurred, Biliary Atresia was an uncommon disease of infancy which was fatal if not treated. The aetiology and incidence of the condition in the United Kingdom and the Republic of Ireland were unknown. The BPSU survey sought to answer: What was the national incidence of biliary atresia?, if there were any recognisable aetiological features, the current pattern of referral, and why is referral delayed and the outcome for affected children following modern management (and how many will eventually need liver transplants).
Lead investigator
Deirdre A Kelly
About the study
When this surveillance occurred, Biliary Atresia was an uncommon disease of infancy which was fatal if not treated. It was the commonest cause of liver disease in children of such severity as to require liver transplantation. The aetiology and incidence of the condition in the United Kingdom and the Republic of Ireland were unknown.
The BPSU survey sought to answer the following questions:
- What is the national incidence of biliary atresia?
- Are there any recognisable aetiological features?
- What is the current pattern of referral, and why is referral delayed
- What is the outcome for affected children following modern management, and how many will eventually need liver transplants?
The researchers concluded that as a result of late referral, many children with Biliary Atresia were undergoing surgery too late to obtain maximum benefit. They also found that too many surgical centres were operating on children with Biliary Atresia, resulting in dilution of surgical and medical expertise.
Duration
March 1993 – February 1995
Published papers
McKiernan, Patrick J et al.
The Lancet, Volume 355, Issue 9197, 25 – 29
Seamless management of biliary atresia in England and Wales (1999–2002)
Davenport, Mark et al.
The Lancet, Volume 363, Issue 9418, 1354 – 1357
BPSU Tenth Annual Report 1995 – 1996
Support Group
Children’s Liver Disease Foundation
Web: https://childliverdisease.org Email: info@childliverdisease.org