Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2 Presenting with Severe Coagulopathy without Jaundice

Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2 Presenting with Severe Coagulopathy without Jaundice

Progressive familial intrahepatic cholestasis (PFIC) type 2 results from a mutation in the bile salt exporter pump, impeding bile acid transport. Patients usually present with jaundice, pruritus, growth failure, and fat soluble vitamin deficiencies. We present two patients diagnosed with PFIC type 2...

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Main Authors: Eric Tibesar, Christine Karwowski, Paula Hertel, Ann Scheimann, Wikrom Karnsakul
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Pediatrics
Online Access:http://dx.doi.org/10.1155/2014/185923
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