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BILIARY ATRESIA: POLITICS AND PROGRESS – A UK PERSPECTIVE
Author(s) -
Stringer M. D.
Publication year - 2007
Publication title -
anz journal of surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.426
H-Index - 70
eISSN - 1445-2197
pISSN - 1445-1433
DOI - 10.1111/j.1445-2197.2007.04125_8.x
Subject(s) - medicine , biliary atresia , liver transplantation , jaundice , incidence (geometry) , liver disease , pediatrics , etiology , transplantation , intensive care medicine , general surgery , surgery , physics , optics
Biliary atresia (BA) is a congenital obliterative cholangiopathy of unknown aetiology. It is a rare condition with an incidence of ∼1 in 17,000 live births in the UK. BA must be diagnosed promptly in any infant with conjugated hyperbilirubinaemia since prognosis is improved by early detection and timely surgery (Kasai portoenterostomy). In the UK, a survey of all infants with BA conducted between 1993 and 1995 demonstrated that outcome was related to centre experience. Consequently, in 1999 the management of BA in England and Wales was centralised to three supra‐regional paediatric liver units. The results of this national change in practice were carefully audited. This showed that approximately 60% of all infants undergoing Kasai portoenterostomy for BA in these centres now achieve clearance of jaundice (plasma bilirubin <20 umol/l). Infants with BA that require a liver transplant remain under the care of the same team and can be listed for transplant at the optimum time. An additional benefit of centralised management has been to facilitate clinical research as a result of concentrating a relatively rare disease into specialist centres with multidisciplinary teams. Recent advances include improved diagnostic accuracy of BA using ultrasound scanning and a better understanding of the role of adjunctive postoperative corticosteroids in improving outcome. Despite these advances, BA remains the commonest indication for liver transplantation in children. Five‐year survival after liver transplantation for BA is currently 80–90%. Current medical and surgical management has transformed a disease that was almost invariably fatal in the 1960s into one with an overall 5‐year survival of about 90%.