title: Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2 Presenting with Severe Coagulopathy without Jaundice.
abstract: 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.
authors.affiliation: Division of Pediatric Gastroenterology, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Baltimore, MD 21287, USA.
authors.forename: Eric
authors.identifier: 0000-0002-6975-2134
authors.initials: E
authors.lastname: Tibesar
authors.affiliation: Division of Pediatric Gastroenterology, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Baltimore, MD 21287, USA.
authors.forename: Christine
authors.initials: C
authors.lastname: Karwowski
authors.affiliation: Division of Pediatric Gastroenterology, Texas Children's Hospital, 6621 Fannin Street, Houston, TX 77030, USA.
authors.forename: Paula
authors.identifier: 0000-0002-2314-7297
authors.initials: P
authors.lastname: Hertel
authors.affiliation: Division of Pediatric Gastroenterology, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Baltimore, MD 21287, USA.
authors.forename: Ann
authors.identifier: 0000-0001-5615-7662
authors.initials: A
authors.lastname: Scheimann
authors.affiliation: Division of Pediatric Gastroenterology, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Baltimore, MD 21287, USA.
authors.forename: Wikrom
authors.initials: W
authors.lastname: Karnsakul