Main description:

Wilson Disease provides a comprehensive guide on this inherited genetic disorder that has devastating consequences for both the liver and neurologic/psychiatric health. This disease is of increasing interest to neurologists, hepatologists, and geneticists, but when the disease is diagnosed early, it is treatable, with patients living normal lives.

This volume describes the molecular pathophysiology of WD and the clinical and pathological effects of copper. Separate sections address both diagnosis and medical and surgical approaches to treatment. Both adult and pediatric perspectives on diagnosis and treatment are addressed, and a section on genetics highlights advances in molecular diagnostics. Patient support groups that can aid in coping with this disease are also discussed, as are animal models for those interested in basic research on cell biology, pathophysiology, and treatment.

Contents:

1. History of Wilson disease: a personal account
J M. Walshe
2. Epidemiology and introduction to the clinical presentation of Wilson disease
Christine Lo and Oliver Bandmann
3. The Genetics of Wilson disease
Sihoun Hahn
4. Genetic and environmental modifiers of Wilson disease
Valentina Medici and Karl Heinz Weiss
5. Pathogenesis of Wilson disease
Petr Dusek, Radan Bruha and Ivo Florin Scheiber
6. Animal models of Wilson disease
Dominik Huster and Valentina Medici
7. Wilson disease - liver pathology
Maciej Pronicki
8. Wilson disease - brain pathology
France Woimant, Aurelia Poujois and Jacqueline Mikol
9. Hepatic features of Wilson disease
Michael L. Schilsky, Aftab Ala and Salih Boga
10. Wilson disease: neurologic features
Anna Czlonkowska, Tomasz Litwin and Grzegorz Chabik
11. Cognitive and psychiatric symptoms in Wilson disease
Paula C. Zimbrean and Joanna Seniow
12. Wilson's disease in children
Eve A. Roberts and Piotr Socha
13. Other organ involvement and clinical aspects of Wilson disease
Karolina Dziezyc, Tomasz Litwin and Anna Czlonkowska
14. Diagnosis of Wilson disease
Peter Ferenci
15. Wilson disease - currently used anti-copper therapy
Anna Czlonkowska and Tomasz Litwin
16. Liver transplantation for Wilson disease
Michael L. Schilsky
17. Wilson disease - symptomatic liver therapy
Wolfgang Stremmel and Karl Heinz Weiss
18. Symptomatic treatment of neurologic symptoms in Wilson disease.
Tomasz Litwin, Petr Dusek and Anna Czlonkowska
19. Novel perspectives on Wilson disease treatment
Christian Rupp, Wolfgang Stremmel and Karl Heinz Weiss
20. Patient support groups in the management of Wilson disease
Michael L. Schilsky and Mary L. Graper