Volume : 2, Issue : 4, April - 2013

Non Syndromic Paucity of Interlobular Bile Ducts: A Rare Cause of Neonatal Cholestasis

Sanjay A Natu, Ujjwala S Keskar, M K Behera

Abstract :

A one month male baby presented with cholestatic jaundice of recent onset. Ultrasound was normal and TC99 HIDA scan showed no excretion. Liver biopsy revealed paucity of bile ducts. In absence of other features of Alagille syndrome, a diagnosis of non syndromic paucity of interlobular bile ducts was made. Patient was started on supportive management but developed severe pruritis and eventually succumbed to liver cell failure.