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Choledochal Cysts and Fibrocystic Diseases of the Liver

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Diseases of the Liver in Children

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Abstract

Cholangiocyte differentiation and biliary development rely on complex interactions between cellular signaling, cholangiocyte biology, genetics, and extra-biliary influences. Alteration of or disruptions to any of the normal developmental processes may result in one of a number of liver and biliary diseases characterized by fibrosis. Many of the primary biliary fibrosing diseases also present with variable cystic dilation of the biliary system, commonly contributing to the symptoms that bring these conditions to clinical attention. This chapter will review the normal biliary development and most basic of cholangiocyte biology as a background to understand the pathogenesis of the most common biliary cystic conditions and fibrocystic diseases of the liver. As separate chapters are devoted to biliary atresia, conditions associated with intrahepatic paucity of the bile ducts, and chronic inflammatory hepatopathies that can lead to fibrosis, these conditions will not be reviewed here.

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Authors and Affiliations

  1. Division of Pediatric Gastroenterology and Hepatology, Seattle Children’s Hospital, University of Washington School of Medicine, 4800 Sand Point Way NE, 5371/OB 9.620.1, Seattle, WA, 98105, USA

    Karen F. Murray MD

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  1. Karen F. Murray MD
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Correspondence to Karen F. Murray MD .

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Editors and Affiliations

  1. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Karen F. Murray

  2. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Simon Horslen

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Murray, K.F. (2014). Choledochal Cysts and Fibrocystic Diseases of the Liver. In: Murray, K., Horslen, S. (eds) Diseases of the Liver in Children. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9005-0_14

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  • DOI: https://doi.org/10.1007/978-1-4614-9005-0_14

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-9004-3

  • Online ISBN: 978-1-4614-9005-0

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