| CASE REPORT |
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| Year : 2010 | Volume
: 53
| Issue : 2 | Page : 334-336 |
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PFIC type III in infant presenting as acute liver cell failure
Syed Ahmed Zaki1, Preeti Shanbag1, Anjali Amarapurkar2
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
 Source of Support: None, Conflict of Interest: None  | Check |
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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