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

• Үндсэн зохиолчид: Eric Tibesar (Зохиогч), Christine Karwowski (Зохиогч), Paula Hertel (Зохиогч), Ann Scheimann (Зохиогч), Wikrom Karnsakul (Зохиогч)
• Формат: Ном
• Хэвлэсэн: Hindawi Limited, 2014-01-01T00:00:00Z.
• Нөхцлүүд: article
• Онлайн хандалт: 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...