RCT of Steroids Following Kasai Portoenterostomy for Biliary Atresia.

• clearance of jaundice (<20 umol/L) [ Time Frame: 1 year ]
• Proportion transplanted or died [ Time Frame: 1 year ]

Biliary atresia is a congenital disorder of bile duct development or destruction of established but immature bile ducts. The study tests the hypothesis that post-operative steroids improve outcome following the Kasai procedure - the commonest surgical treatment.

Biliary atresia is a potentially fatal condition of infants presenting as persisting jaundice in the first few weeks of life. The disease is characterised by obstruction and damage to the intra and extrahepatic parts of the biliary tree. Within the liver there is also a pronounced inflammatory response. The initial treatment is an attempt, by surgery, to restore bile flow by excising the obliterated extrahepatic bile ducts and joining part of the intestine to the bile "root" of the liver (the porta hepatis). This is known as the Kasai procedure. This is successful in ~50% of cases in reducing the level of jaundice to near-normal values. The use of steroids post-operatively has been suggested as improving outcome by diminishing the inflammatory response.

• Drug: prednisolone

  2 mg/kg /day from post-op day 7 - day 21

  1 mg/kg /day from post-op day 22 - day 30

• Drug: placebo

• Active Comparator: A
  oral corticosteroids
  Intervention: Drug: prednisolone
• Placebo Comparator: B
  as for active regimen
  Intervention: Drug: placebo

Inclusion Criteria:

• biliary atresia

Exclusion Criteria:

• <100 days at portoenterostomy
• no other anomalies (e.g. Biliary Atresia Splenic Malformation syndrome)
• anu contra-indications to corticosteroids