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

Eric Tibesar | Zendy; Christine Karwowski | Zendy; Paula M. Hertel | Zendy; Ann Scheimann | Zendy; Wikrom Karnsakul | Zendy

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

Author(s) -

Eric Tibesar,

Christine Karwowski,

Paula M. Hertel,

Ann Scheimann,

Wikrom Karnsakul

Publication year - 2014

Publication title -

case reports in pediatrics

Language(s) - English

Resource type - Journals

eISSN - 2090-6803

pISSN - 2090-6811

DOI - 10.1155/2014/185923

Subject(s) - medicine , progressive familial intrahepatic cholestasis , jaundice , coagulopathy , cholestasis , gastroenterology , consumptive coagulopathy , liver transplantation , transplantation

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 due to severe coagulopathy and bleeding without jaundice.

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