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Wilson Disease - 1st Edition - ISBN: 9780128110775, 9780128110782

Wilson Disease

1st Edition

Pathogenesis, Molecular Mechanisms, Diagnosis, Treatment and Monitoring

Editors: Karl Heinz Weiss Michael Schilsky
Paperback ISBN: 9780128110775
eBook ISBN: 9780128110782
Imprint: Academic Press
Published Date: 22nd May 2019
Page Count: 264
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Wilson Disease: Pathogenesis, Molecular Mechanisms, Diagnosis, Treatment and Monitoring translates both clinical and experimental findings into a comprehensive approach for anyone involved in research and patient care. While the clinical variability of Wilson Disease poses a challenge from a diagnostic approach, the book uses the translational impact of new research findings to relate to new treatment concepts. Comprehensive chapters include common knowledge, guideline consensus statements, and discussions of clinical evidence. This is a must-have reference for researchers and clinicians in translational research.

Key Features

  • Delivers a substantial overview of evidence driven diagnostic pathways and treatment concepts for Wilson disease
  • Provides an understanding of the translational impact of new research findings in Wilson Disease
  • Reveals the current controversies in treatment decisions for Wilson disease, providing the best decisions for optimal patient care


Clinical and basic scientists with a research interest in Wilson Disease; Hepatology and Gastroenterology Clinicians

Table of Contents

  1. History of WD
  2. Pathogenesis
    2.1.) Normal copper metabolism
    2.2.) ATP7B function
    2.3.) Copper endpoints and cellular copper toxicity
  3. Epidemiology and genetics
    3.1.) Epidemiology and regional differences
    3.2.) Genotype – Phenotype correlation and Phenotype definitions
  4. Diagnosis
    4.1.) Diagnostic work up
    4.2.) Biochemical marker
    3.3.) Hepatic Wilson Disease
    4.4.) Neurologic Wilson Disease
    4.5.) Liver pathology in WD and genetic testing
  5. Treatment Decisions
    5.1.) General considerations and the need for Liver transplantation
    5.2.) Chelation therapy: D-penicilamine
    5.3.) Chelation therapy: Trientine
    5.4.) Zinc
    5.5.) Other treatment regimens and emerging therapies
    5.6.) Symptomatic treatment of residual neurologic or psychiatric disease



No. of pages:
© Academic Press 2019
22nd May 2019
Academic Press
Paperback ISBN:
eBook ISBN:

About the Editors

Karl Heinz Weiss

Dr. Weiss is an Associate Professor at the University Hospital of Heidelberg, Department of Gastroenterology in Heidelberg, Germany. He is the author numerous articles dedicated to Wilson Disease which feature international collaboration. He has been the recipient of The German National Merit Foundation Award as well as the Young Investigator Award. His main areas of research include Wilson Disease, Hepatobiliary Tumors, and Metabolic Liver Diseases.

Affiliations and Expertise

Department of Gastroenterology, Associate Professor, University Hospital of Heidelberg, Heidelberg, Germany

Michael Schilsky

Michael Schilsky

Michael Schilsky, MD, became medical director of liver transplantation at Yale-New Haven Hospital in 2007, with appointments in medicine and surgery at the Yale School of Medicine. Dr. Schilsky received his medical degree from the University of Chicago, Pritzker School of Medicine. He completed his residency and fellowship in gastroenterology and research training in liver diseases at Albert Einstein College of Medicine and Montefiore Medical Center. His clinical and research interests include transplant hepatology, inherited metabolic disorders of the liver, Wilson disease and hemochromatosis. Dr. Schilsky previously served as director of the liver medicine clinic at the Recanati/Miller Transplantation Institute at Mount Sinai Medical Center and medical director for liver transplantation at the New York Weill Cornell Medical Center, where he developed a comprehensive program for the care and evaluation of liver transplant patients. Dr. Schilsky coauthored the AASLD practice guidelines for Wilson disease and is author of numerous original manuscripts and reviews.

Affiliations and Expertise

Medical Director, Adult Liver Transplant, Yale-New Haven Transplantation Center, New Haven, CT, USA

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