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

نویسندگان

  • Eric Tibesar
  • Christine Karwowski
  • Paula Hertel
  • Ann Scheimann
  • Wikrom Karnsakul
چکیده

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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عنوان ژورنال:

دوره 2014  شماره 

صفحات  -

تاریخ انتشار 2014