Author | Brewer, George J. author |
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Title | Wilson's Disease [electronic resource] : A Clinician's Guide to Recognition, Diagnosis, and Management / by George J. Brewer |
Imprint | Boston, MA : Springer US : Imprint: Springer, 2001 |
Connect to | http://dx.doi.org/10.1007/978-1-4615-1645-3 |
Descript | XXVIII, 190 p. online resource |
1. Overview of the Disease for the Clinician -- Cause of Wilson's disease -- Inheritance -- Clinical Presentations -- Recognition Problem -- Some Screening Tests are Accurate -- Diagnosis is Usually Straightforward -- Treatment and Management Concepts -- Monitoring and Compliance -- Prognosis -- 2. The Clinician's Challenge: Recognizing Wilson's Disease -- The Neurological Presentation -- The Hepatic Presentation -- The Behavioral Change Presentation -- The Presymptomatic Patient -- Other Situations in Which Wilson's Disease May be Present -- Key Points with Respect to Recognition of Possible Wilson's Disease -- 3. Simple Approaches to Screening and Definitive Diagnosis -- Screening Tests and Procedures -- How to Screen Patients for Wilson's disease -- Making a Definitive Diagnosis of Wilson's Disease -- Disasters and Near Disasters Related to Screening and Diagnostic Tests -- 4. Description of the Anticopper Drugs Which are Used in Wilson's Disease Therapy -- Zinc -- Trientine -- Tetrathiomolybdate -- Penicillamine -- Other Anticopper Drugs -- 5. Overview of Management for the Clinician: What's Important and What's Not -- Anticopper Drug Therapy OverviewโImportant -- DietโGenerally Not Important -- Drinking WaterโOccasionally Important -- Patients Signs and SymptomsโAlways Important -- 6. Initial Treatment of Patients Who Present With Liver Disease -- Initial Therapy of Patients With Hepatic Failure -- Initial Therapy of Patients with Hepatitis -- 7. Initial Treatment of Patients Who Present With Neurological and/or Psychiatric Disease -- Neurological Worsening of Patients with Initial Penicillamine Therapy -- Recommended TherapyโTetrathiomolybdate -- Alternative Therapies -- 8. Maintenance Therapy -- Recommended Maintenance TherapyโZinc -- Alternative Maintenance Therapy -- 9. Therapy of the Presymptomatic, the Pediatric, and the Pregnant Patient -- Therapy for the Presymptomatic Patient -- Therapy for the Pediatric Patient -- Therapy for the Pregnant Patient -- 10. Risk Factors during Maintenance Therapy and Prognosis -- A General Risk FactorโPoor Compliance -- Hepatic Risk Factors -- Neurological Risk Factors -- Psychiatric Risk Factors -- Prognosis -- 11. Disease Pathogenesis and Genetics -- Does Copper Toxicity Cause Wilson's Disease? -- Normal Copper Handling -- Defective Biliary Excretion of Copper in Wilson's Disease -- The Genetics of Wilson's Disease -- From Gene Defect to Copper Accumulation, Organ Damage, and Symptoms -- Animal Models -- Summary of Our Knowledge of Pathogenesis -- 12. Disease Pathology -- The Nature of Copper Toxicity -- Liver Pathology -- Brain Pathology -- Pathology in Other Organs -- Summary -- 13. History, Important Milestones, and the Future -- History of Wilson's Disease -- Future Challenges -- Summary