Seamless management of biliary atresia in England and Wales (1999--2002).

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Bibliographic Details
Title:	Seamless management of biliary atresia in England and Wales (1999--2002).
Authors:	Davenport, Mark, De Goyet, J. De Ville, Stringer, M. D., Mieli-Vergani, G., Kelly, D. A., McClean, P., Spitz, L.
Source:	Lancet. 4/24/2004, Vol. 363 Issue 9418, p1354-1357. 4p.
Subjects:	Biliary atresia, Infant disease treatment, Liver transplantation, Neonatal jaundice, Medical care research, Medical research, Therapeutics
Geographic Terms:	United Kingdom
Abstract:	Background Before 1999, infants born in the UK with suspected biliary atresia were investigated in regional centers, and, if confirmed, a Kasai operation was done there. Since 1999, all infants with suspected biliary atresia in England and Wales, UK, have been referred to one of three designated centers where both the Kasai operation and liver transplantation (if necessary) could be done. Methods We assessed clearance of jaundice (bilirubin <20 mol/L) as an early outcome in all cases of biliary atresia referred from one of the three centers. We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation or death. Findings 148 infants with biliary atresia were treated between January, 1999, and June, 2002. A primary portoenterostomy was done in 142 (96%) infants and a primary liver transplant in five (3%). One child died before any intervention. Early clearance of jaundice after portoenterostomy was achieved in 81 of 142 (57%) infants. Liver transplantation was done in 52 (37%) of those undergoing portoenterostomy. 13 (9%) infants died. Of the 135 children who survived, 84 (62%) still have their native liver and 51 (38%) had transplantation. The median follow-up of survivors was 2.13 (range 0.5--4.1) years. The overall 4-year estimated actuarial survival was 89% (95% CI 82--94). The 4-year estimated actuarial survival with native liver was 51% (42--59%). Interpretation Our early results suggest that surgical outcome can be improved by centralization of care to supraregional centers. [ABSTRACT FROM AUTHOR]
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Database:	Psychology and Behavioral Sciences Collection

Description
Abstract:	Background Before 1999, infants born in the UK with suspected biliary atresia were investigated in regional centers, and, if confirmed, a Kasai operation was done there. Since 1999, all infants with suspected biliary atresia in England and Wales, UK, have been referred to one of three designated centers where both the Kasai operation and liver transplantation (if necessary) could be done. Methods We assessed clearance of jaundice (bilirubin <20 mol/L) as an early outcome in all cases of biliary atresia referred from one of the three centers. We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation or death. Findings 148 infants with biliary atresia were treated between January, 1999, and June, 2002. A primary portoenterostomy was done in 142 (96%) infants and a primary liver transplant in five (3%). One child died before any intervention. Early clearance of jaundice after portoenterostomy was achieved in 81 of 142 (57%) infants. Liver transplantation was done in 52 (37%) of those undergoing portoenterostomy. 13 (9%) infants died. Of the 135 children who survived, 84 (62%) still have their native liver and 51 (38%) had transplantation. The median follow-up of survivors was 2.13 (range 0.5--4.1) years. The overall 4-year estimated actuarial survival was 89% (95% CI 82--94). The 4-year estimated actuarial survival with native liver was 51% (42--59%). Interpretation Our early results suggest that surgical outcome can be improved by centralization of care to supraregional centers. [ABSTRACT FROM AUTHOR]
ISSN:	01406736
DOI:	10.1016/S0140-6736(04)16045-5