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.- Addresses the molecular pathophysiology of WD and the clinical and pathological effects of copper- Offers coverage of both diagnosis and medical and surgical approaches to treatment- Includes perspectives on both adult and pediatric diagnosis and treatment- Edited work with chapters authored by leaders in the field from around the globe—the broadest, most expert coverage available
Table of Contents
1. History of Wilson disease: a personal accountJ M. Walshe2. Epidemiology and introduction to the clinical presentation of Wilson diseaseChristine Lo and Oliver Bandmann 3. The Genetics of Wilson diseaseSihoun Hahn4. Genetic and environmental modifiers of Wilson diseaseValentina Medici and Karl Heinz Weiss 5. Pathogenesis of Wilson diseasePetr Dusek, Radan Bruha and Ivo Florin Scheiber 6. Animal models of Wilson diseaseDominik Huster and Valentina Medici 7. Wilson disease – liver pathologyMaciej Pronicki8. Wilson disease – brain pathologyFrance Woimant, Aurelia Poujois and Jacqueline Mikol 9. Hepatic features of Wilson disease Michael L. Schilsky, Aftab Ala and Salih Boga 10. Wilson disease: neurologic featuresAnna Czlonkowska, Tomasz Litwin and Grzegorz Chabik 11. Cognitive and psychiatric symptoms in Wilson diseasePaula C. Zimbrean and Joanna Seniow 12. Wilson's disease in childrenEve A. Roberts and Piotr Socha 13. Other organ involvement and clinical aspects of Wilson diseaseKarolina Dziezyc, Tomasz Litwin and Anna Czlonkowska 14. Diagnosis of Wilson diseasePeter Ferenci15. Wilson disease - currently used anti-copper therapyAnna Czlonkowska and Tomasz Litwin 16. Liver transplantation for Wilson disease Michael L. Schilsky17. 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 treatmentChristian Rupp, Wolfgang Stremmel and Karl Heinz Weiss 20. Patient support groups in the management of Wilson diseaseMichael L. Schilsky and Mary L. Graper
