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  • Book
  • © 2010

Fibrocystic Diseases of the Liver

  • Unique focus on fibrocystic diseases
  • The gold-standard reference for these conditions
  • Includes supplementary material: sn.pub/extras

Part of the book series: Clinical Gastroenterology (CG)

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Table of contents (22 chapters)

  1. Front Matter

    Pages i-xiv
  2. Pathophysiology and Development

    1. Front Matter

      Pages 2-2
    2. Embryology and Development of the Ductal Plate

      • Clifford W. Bogue
      Pages 3-21
    3. Cholangiocyte Biology as Relevant to Cystic Liver Diseases

      • Silvia Lecchi, Luca Fabris, Carlo Spirli, Massimiliano Cadamuro, Romina Fiorotto, Mario Strazzabosco
      Pages 23-43
    4. Cholangiocyte Cilia and Basal Bodies

      • Tatyana V. Masyuk, Anatoliy I. Masyuk, Nicholas F. LaRusso
      Pages 45-70
    5. Genetics of Fibrocystic Diseases of the Liver and Molecular Approaches to Therapy

      • Maya Tuchman, William A. Gahl, Meral Gunay-Aygun
      Pages 71-102
  3. Diagnosis

    1. Front Matter

      Pages 104-104
    2. Radiologic Findings in the Fibrocystic Diseases

      • Orpheus Kolokythas, Grace Phillips
      Pages 105-132
    3. Pathology of Fibrocystic Diseases of the Liver

      • Camtu D. Nguyen Truong, Matthew M. Yeh
      Pages 133-155
  4. Disease States

    1. Front Matter

      Pages 158-158
    2. Alagille Syndrome and JAGGED1/NOTCH Sequence

      • Binita M. Kamath, Kathleen M. Loomes, David A. Piccoli
      Pages 159-178
    3. Biliary Atresia and the Ductal Plate

      • Ronald J. Sokol, Cara L. Mack
      Pages 179-199
    4. Meckel and Joubert Syndromes

      • Melissa A. Parisi, Laura S. Finn, Ian A. Glass
      Pages 221-256
    5. Bardet–Biedl and Jeune Syndromes

      • Aoife M. Waters, Philip L. Beales
      Pages 257-285
    6. Congenital Disorders of Glycosylation and Their Effects on the Liver

      • Erik A. Eklund, Hudson H. Freeze
      Pages 287-317
    7. Autosomal Recessive Polycystic Kidney Disease

      • Jodi M. Smith, Ruth A. McDonald
      Pages 319-330
    8. Caroli Disease, Caroli Syndrome, and Congenital Hepatic Fibrosis

      • Ronen Arnon, Henrietta Kotlus Rosenberg, Frederick J. Suchy
      Pages 331-358
    9. Simple Hepatic Cysts/Choledochal Cysts

      • André A. S. Dick, Patrick J. Healey
      Pages 359-369
    10. Autosomal Dominant Polycystic Liver Disease

      • Qi Qian, Vicente E. Torres, Stefan Somlo
      Pages 371-386

About this book

In recent years there have been huge advances in the understanding of the genetic and molecular basis of the fibrocystic diseases. This volume provides a thorough review of fibrocyctic diseases that affect the liver. It contains in-depth discussions of the genetics, molecular biology, pathogenesis, histology, clinical presentations, complications of, treatment, and prognosis of the conditions affecting children and adults, and hence will be the gold-standard reference for these conditions. In addition, the histological features that distinguish these conditions from other potentially fibrosing hepatopathies are illustrated. Conditions with syndromic features involving the kidney or other organ systems are also reviewed. Thorough review of the clinical phenotypes, their presentations, treatment, potential complications of, and prognosis is discussed.

Fibrocystic Diseases of the Liver will be an invaluable resource for hepatologists, gastroenterologists, nephrologists, and hepatic surgeons who care for children and adults with liver disease, as well as basic scientists in molecular genetics, hepatobiliary pathophysiology, hepatology and nephrology.

Editors and Affiliations

  • School of Medicine, Children's Hospital & Regional, University of Washington, Seattle, USA

    Karen F. Murray

  • St. Paul University Hospital, Dallas, USA

    Anne M. Larson

Bibliographic Information

  • Book Title: Fibrocystic Diseases of the Liver

  • Editors: Karen F. Murray, Anne M. Larson

  • Series Title: Clinical Gastroenterology

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

  • Publisher: Humana Totowa, NJ

  • eBook Packages: Medicine, Medicine (R0)

  • Copyright Information: Springer Science+Business Media, LLC 2010

  • Hardcover ISBN: 978-1-60327-523-1Published: 22 March 2010

  • Softcover ISBN: 978-1-61779-685-2Published: 06 April 2012

  • eBook ISBN: 978-1-60327-524-8Published: 23 July 2010

  • Series ISSN: 2197-7399

  • Series E-ISSN: 2197-7704

  • Edition Number: 1

  • Number of Pages: XIV, 512

  • Number of Illustrations: 91 b/w illustrations, 70 illustrations in colour

  • Topics: Gastroenterology, Hepatology, Nephrology, Pediatrics, Abdominal Surgery

Buy it now

Buying options

eBook USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book USD 299.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Other ways to access