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

• প্রধান লেখক: Eric Tibesar (লেখক), Christine Karwowski (লেখক), Paula Hertel (লেখক), Ann Scheimann (লেখক), Wikrom Karnsakul (লেখক)
• বিন্যাস: গ্রন্থ
• প্রকাশিত: Hindawi Limited, 2014-01-01T00:00:00Z.
• বিষয়গুলি: article
• অনলাইন ব্যবহার করুন: Connect to this object online.

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...