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

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...

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Detalles Bibliográficos
Autores principales: Tibesar, Eric, Karwowski, Christine, Hertel, Paula, Scheimann, Ann, Karnsakul, Wikrom
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4060322/
https://www.ncbi.nlm.nih.gov/pubmed/24991443
http://dx.doi.org/10.1155/2014/185923