A 4 week old new born, in the neonatal unit presents with jaundice and clay white stools. On liver biopsy giant cells with ballooning degeneration of the cytoplasm are seen. Most likely diagnosis is:
Correct Answer: Neonatal hepatitis with extra-hepatic biliary atresia
Description: In this scenario the neonates condition cannot be explained by one single condition. Giant cells with ballooning degeneration of cytoplasm is a finding in neonatal hepatitis and the clinical symptoms points towards extra-hepatic biliary atresia. In neonatal hepatitis liver biopsy shows altered lobular architecture, focal hepatocellular necrosis, giant cells with ballooning degeneration of cytoplasm. These changes are seen by 4 weeks of age. Neonates with biliary atresia are usually full term, look healthy presents with jaundice, pass dark urine and clay coloured stool. The other signs include hepatomegaly and splenomegaly (late). Ref: Kliegman, Behrman, Jenson, Stanton (2008), Chapter 353, "Cholestasis", In the book, "NELSON TEXTBOOK OF PEDIATRICS", Volume 2, 18th Edition, New Delhi, Pages 1671-1673 ; O P Ghai Essential Pediatrics, 6th Edition, Pages 295-96
Category:
Pediatrics
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