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Autosomal Recessive Polycystic Kidney Disease

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Fibrocystic Diseases of the Liver

Part of the book series: Clinical Gastroenterology ((CG))

Summary

Autosomal recessive polycystic kidney disease (ARPKD) is an inherited disorder involving cystic dilatation of the renal collecting ducts as well as varying degrees of hepatic abnormalities consisting of cysts, fibrosis, and portal hypertension. The ARPKD locus has been mapped to chromosome 6p21 and encodes a novel protein product named fibrocystin or polyductin. There are several modes of presentation depending on the age and the predominance of hepatic or renal involvement. With improvements in medical management and continued progress in end-stage renal disease therapy in young infants, further improvements in survival and rehabilitation can be anticipated.

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Authors and Affiliations

  1. Seattle Children’s Hospital, Seattle, WA, USA

    Jodi M. Smith MD, MPH & Ruth A. McDonald MD

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  1. Jodi M. Smith MD, MPH
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  2. Ruth A. McDonald MD
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Editors and Affiliations

  1. School of Medicine, Children's Hospital & Regional, University of Washington, Sand Point Way NE. 4800, Seattle, 98105, USA

    Karen F. Murray

  2. St. Paul University Hospital, Harry Hines Blvd. 5939, Dallas, 75390-9258, USA

    Anne M. Larson

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Smith, J.M., McDonald, R.A. (2010). Autosomal Recessive Polycystic Kidney Disease. In: Murray, K., Larson, A. (eds) Fibrocystic Diseases of the Liver. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-524-8_13

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  • DOI: https://doi.org/10.1007/978-1-60327-524-8_13

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  • Print ISBN: 978-1-60327-523-1

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