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Case Reports in Pediatrics
Volume 2014, Article ID 185923, 4 pages
Case Report

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

1Division of Pediatric Gastroenterology, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Baltimore, MD 21287, USA
2Division of Pediatric Gastroenterology, Texas Children’s Hospital, 6621 Fannin Street, Houston, TX 77030, USA

Received 24 February 2014; Revised 8 May 2014; Accepted 20 May 2014; Published 2 June 2014

Academic Editor: Junji Takaya

Copyright © 2014 Eric Tibesar et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.