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Case Reports in Medicine
Volume 2015 (2015), Article ID 428638, 5 pages
Case Report

Progressive Familial Intrahepatic Cholestasis: A Rare Cause of Cirrhosis in Young Adult Patients

1Department of Internal Medicine, University of Calgary, Calgary, AB, Canada T2N 4N1
2University of Calgary, Rockyview General Hospital, Calgary, AB, Canada T2V 1P9

Received 13 March 2015; Accepted 21 May 2015

Academic Editor: Timothy J. Craig

Copyright © 2015 Gavin R. Sun and Michele Burns. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Hepatic cirrhosis is an important cause of morbidity and mortality. An unusual case of cirrhosis and portal hypertension in an 18-year-old patient secondary to Progressive Intrahepatic Cholestasis is discussed. The clinical and biochemical findings are discussed and a clinical approach to determining the underlying etiology of cirrhosis is outlined. Significant complications of portal hypertension include ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, varices, and hepatic encephalopathy. A clinical approach to these complications of cirrhosis is presented. Progressive Familial Intrahepatic Cholestasis (PFIC) is a rare congenital metabolic abnormality. There are 3 subtypes and Type 3 PFIC commonly presents in late adolescence and early adulthood. Clinical and laboratory findings as well as management for the condition are described.