Clinical practice: management of biliary atresia

Basem A Khalil, M Thamara P R Perera, Darius F Mirza

Research output: Contribution to journalArticlepeer-review

23 Citations (Scopus)

Abstract

Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown. Optimal management at specialist centres with resultant better overall outcomes is achieved through a multidisciplinary team approach. The Kasai portoenterostomy performed early in life remains the only surgical repair procedure. Two thirds of patients will clear their jaundice after a Kasai procedure, but only about one third will retain their livers after the first decade of life. Failure of this procedure leaves liver transplantation as the only chance for survival, and this disease is the commonest indication for liver transplantation in children. With modern medical care and refinements in surgical techniques, survival after either or both of these procedures is about 90%. Early referral to specialist centres and long-term specialist care remains the key to successful treatment of this condition.

Original languageEnglish
Pages (from-to)395-402
Number of pages8
JournalEuropean Journal of Pediatrics
Volume169
Issue number4
DOIs
Publication statusPublished - Apr 2010

Keywords

  • Biliary Atresia
  • Child
  • Cholangitis
  • Humans
  • Liver Function Tests
  • Portoenterostomy, Hepatic
  • Preoperative Care

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