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

dc.contributor.authorSun, Gavin R.
dc.contributor.authorBurns, Michele
dc.date.accessioned2018-09-27T11:29:28Z
dc.date.available2018-09-27T11:29:28Z
dc.date.issued2015-06-02
dc.date.updated2018-09-27T11:29:27Z
dc.description.abstractHepatic 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.
dc.description.versionPeer Reviewed
dc.identifier.citationGavin R. Sun and Michele Burns, “Progressive Familial Intrahepatic Cholestasis: A Rare Cause of Cirrhosis in Young Adult Patients,” Case Reports in Medicine, vol. 2015, Article ID 428638, 5 pages, 2015. doi:10.1155/2015/428638
dc.identifier.doihttps://doi.org/10.1155/2015/428638
dc.identifier.urihttp://hdl.handle.net/1880/108165
dc.identifier.urihttps://doi.org/10.11575/PRISM/44002
dc.language.rfc3066en
dc.rights.holderCopyright © 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.
dc.titleProgressive Familial Intrahepatic Cholestasis: A Rare Cause of Cirrhosis in Young Adult Patients
dc.typeJournal Article
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