https://www.ncbi.nlm.nih.gov/pubmed/31437071 Cholestasis
Fetal Pediatr Pathol. 2019 Aug 22:1-17. doi: 10.1080/15513815.2019.1652376. [Epub ahead of print]
Non Syndromic Paucity of Interlobular Bile Ducts in Children - A Clinicopathological Study.
Mohanty S1, Das K2, Anne Correa MM1.

Abstract
Background: Non syndromic paucity of interlobular bile ducts (NS-PILBD) constitutes a miniscule of infantile cholestasis. Method: Clinical details, investigations, surgical findings, management and outcome of cases of NS-PILBD at liver biopsy were analyzed. Specific histopathological features including bile duct to portal tract ratio were studied. Results: Eighteen cases (1993-2013) are detailed. Clinical presentation and investigations were similar to biliary atresia. Hepatic scintigraphy showed no gut excretion in 13/18 and operative cholangiogram was normal in all. Liver biopsy showed a median Scheuer fibrosis stage of 2, the mean bile duct/portal tract ratio was 0.29. The average age at last follow up of twelve cases was 54.9 months . Ten were asymptomatic and anicteric, the liver function tests had normalized over 3-15 months. Conclusion: Histopathology differentiated NS-PILBD from other causes of infantile cholestasis .The idiopathic form generally had a favorable long term outcome with medical management.