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CASE REPORT
Year : 2010  |  Volume : 53  |  Issue : 2  |  Page : 334-336

PFIC type III in infant presenting as acute liver cell failure


1 Department of Pediatrics, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai-400 022, India
2 Department of Pathology, TN Medical College and BYL Nair Hospital, Mumbai Central, Mumbai 400 008, India

Correspondence Address:
Syed Ahmed Zaki
Room no. 509, new RMO quarters, LTMG hospital, Sion, Mumbai-400 022
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.64326

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An eight-month-old female, delivered to consanguineous parents, presented with acute liver cell failure. Her investigations showed progressive cholestatic jaundice, high liver enzymes and high gamma-glutamyl transferase. Hepatitis and inborn errors of metabolism were excluded. The liver biopsy showed a prominent parenchymal bile stasis without features of bile obstruction or paucity of bile ducts. These findings wee suggestive of Byler disease or progressive familial intra hepatic cholestasis type III (PFIC III) which begins in infancy and usually progresses to cirrhosis and hepatic failure in the first few years of life.


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