Chapter 5. Congenital and Inherited Anomalies

  1. Hans G. Beger MD, FACS(Hon) Founding Editor Emeritus Professor of Surgery2,
  2. Andrew L. Warshaw MD Surgeon-in-Chief Chairman3,4,
  3. Markus W. Büchler MD Chairman Head Professor of Surgery5,
  4. Richard A. Kozarek MD Director6,
  5. Markus M. Lerch MD, FRCP Professor Chair7,
  6. John P. Neoptolemos MA, MB, BChir, MD, FRCS, FMedSci Head Professor of Surgery8,9,10,
  7. Keiko Shiratori MD Chair Professor11,
  8. David C. Whitcomb MD, PhD Professor of Medicine Chief12 and
  9. Bettina M. Rau MD Coordinating Editor Associate Professor of Surgery13
  1. Martin Zenker MD1 and
  2. Markus M. Lerch MD, FRCP Professor Chair7

Published Online: 4 FEB 2009

DOI: 10.1002/9781444300123.ch5

The Pancreas: An Integrated Textbook of Basic Science, Medicine, and Surgery, Second Edition

The Pancreas: An Integrated Textbook of Basic Science, Medicine, and Surgery, Second Edition

How to Cite

Zenker, M. and Lerch, M. M. (2009) Congenital and Inherited Anomalies, in The Pancreas: An Integrated Textbook of Basic Science, Medicine, and Surgery, Second Edition (eds H. G. Beger, A. L. Warshaw, M. W. Büchler, R. A. Kozarek, M. M. Lerch, J. P. Neoptolemos, K. Shiratori, D. C. Whitcomb and B. M. Rau), Blackwell Publishing Ltd., Oxford, UK. doi: 10.1002/9781444300123.ch5

Editor Information

  1. 2

    c/o Universitätsklinikum Ulm, University of Ulm, Germany

  2. 3

    Department of Surgery, Massachusetts General Hospital, Boston, MA, USA

  3. 4

    W. Gerald Austen Professor of Surgery, Harvard Medical School, Boston, MA, USA

  4. 5

    Department of General and Visceral Surgery, University of Heidelberg, Germany

  5. 6

    Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA, USA

  6. 7

    Department of Gastroenterology, Endocrinology and Nutrition, Ernst-Moritz-Arndt University, Greifswald, Germany

  7. 8

    The Owen and Ellen Evans Chair of Cancer Studies, University of Liverpool, UK

  8. 9

    Division of Surgery and Oncology, University of Liverpool, UK

  9. 10

    School of Cancer Studies, University of Liverpool, UK

  10. 11

    Department of Gastroenterology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan

  11. 12

    Division of Gastroenterology, Hepatology and Nutrition, University of Pittsburgh, PA, USA

  12. 13

    Department of General, Thoracic, Vascular and Transplantation Surgery, University of Rostock, Germany

Author Information

  1. 1

    Institute of Human Genetics, University of Erlangen-Nuremberg, Erlangen, Germany

  2. 7

    Department of Gastroenterology, Endocrinology and Nutrition, Ernst-Moritz-Arndt University, Greifswald, Germany

Publication History

  1. Published Online: 4 FEB 2009
  2. Published Print: 11 APR 2008

ISBN Information

Print ISBN: 9781405146647

Online ISBN: 9781444300123

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Keywords:

  • pancreatic agenesis and hypoplasia;
  • congenital and inherited anomalies;
  • pancreas divisium - absent or incomplete fusion;
  • congenital pancreatic cysts;
  • congenital secretory insufficiency;
  • Shwachman–Diamond syndrome (SDS);
  • isolated enzyme deficiencies;
  • von Hippel–Lindau syndrome (VHL);
  • Beckwith–Wiedemann syndrome (BWS);
  • hereditary pancreatitis

Summary

This chapter contains sections titled:

  • Introduction

  • Primary malformations

  • Congenital secretory insufficiency (excluding cystic fibrosis)

  • Isolated congenital disorders of pancreatic endocrine function

  • Other hereditary disorders with variable pancreatic involvement and metabolic diseases affecting the pancreas

  • Inherited metabolic disorders affecting the pancreas

  • Hereditary pancreatitis

  • References