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  • Book
    Michael L. Schilsky, editor.
    Digital : Springer2018
    Intro; Preface; Contents; Contributors; Chapter 1: Introduction to Copper Metabolism and Wilson Disease; Introduction; Cellular Mechanisms of Copper Transport; Wilson Disease; Abnormal Copper Metabolism and Injury in Wilson Disease; Ceruloplasmin: A Marker of Wilson Disease and Copper Status; When Copper Is in Excess; Summary and Conclusion; References; Chapter 2: Diagnosis Confirmation and Screening of Wilson Disease; Introduction; Patients to Test for Wilson Disease; Liver Disease and Extrahepatic Involvement in Wilson Disease The Neuropsychiatric Manifestations of Wilson Disease: More than Meets the EyeTremor; The Cranial Nerves; The Cerebellum; Psychiatric Symptoms; Other Organs Involved in Wilson Disease; Confirming the Diagnosis; Slit-Lamp Examination for Kayser-Fleischer (KF) Rings; Serum Ceruloplasmin; Free (Unbound) Serum Copper; Twenty-Four-Hour Urinary Copper; Genetic Analysis: Testing for ATP7B Mutations; Liver Biopsy: Histology and Hepatic Copper Concentration; Hemolysis (Breakdown of Red Blood Cells); Imaging Tests; Acute Liver Failure Secondary to Wilson Disease; Family Screening; Conclusion Dietary Modifications in Wilson DiseaseFood; Water; Cooking Methods; Nutritional Supplements; Special Considerations; Liver Disease; Neurological Involvement; Dysphagia; Nutrition Support; Resources; One-on-One RD Assistance; USDA Food/Nutrient Database; Conclusion; References; Chapter 5: Treating Other Symptoms of Wilson Disease: The Liver; Introduction; Asymptomatic or Mild Symptoms; Portal Hypertension; Varices (Esophageal and Gastric); Management of Variceal Bleed; Ascites; Treatment of Resistant Ascites; Spontaneous Bacterial Peritonitis; Sepsis; Hepatic Encephalopathy; Malignancy Acute Liver FailureBibliography; Chapter 6: Treatment of Neurological Symptoms in Wilson Disease; Introduction; Neurological Manifestations of WD; Tremor; Medications for Action Tremor; Dystonia; Treatment Options for Dystonia; Botulinum Toxin Injections; Oral Medications; Parkinsonism; Cerebellar Ataxia; Dysphagia; Neurological Manifestations in Children; Clinical Scales Used for Neurological Evaluation of WD; Neuroimaging in WD; Conclusion; Bibliography; Chapter 7: Psychiatric Aspects of Wilson Disease; Introduction; Evaluation of Psychiatric Symptoms in WD