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Management of Wilson disease : a pocket guide

Author: Michael L Schilsky
Publisher: Cham, Switzerland : Humana Press, [2018]
Series: Clinical gastroenterology (Series)
Edition/Format:   eBook : Document : EnglishView all editions and formats
Summary:
This pocket guide provides a concise yet comprehensive overview of the pathogenesis, treatment, and management of Wilson disease. Drawing upon expertise from the Center of Excellence at Yale University in liver disease, neurology, and psychiatry for adult and pediatric patients, as well as dieticians, clinical trial coordinators, representatives from the Wilson Disease Association and outside experts from the US and  Read more...
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Genre/Form: Electronic books
Additional Physical Format: (OCoLC)1031460957
Material Type: Document, Internet resource
Document Type: Internet Resource, Computer File
All Authors / Contributors: Michael L Schilsky
ISBN: 9783319915272 3319915274
OCLC Number: 1050110389
Notes: Includes index.
Description: 1 online resource.
Contents: 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
Series Title: Clinical gastroenterology (Series)
Responsibility: Michael L. Schilsky, editor.

Abstract:

This pocket guide provides a concise yet comprehensive overview of the pathogenesis, treatment, and management of Wilson disease. Drawing upon expertise from the Center of Excellence at Yale University in liver disease, neurology, and psychiatry for adult and pediatric patients, as well as dieticians, clinical trial coordinators, representatives from the Wilson Disease Association and outside experts from the US and UK as well, this guide provides a balanced and easy to digest approach to understanding treatment and care for Wilson disease patients. The text reviews different treatment options and other care needed to address clinical symptoms, as well as the testing needed for monitoring efficacy and adherence. Furthermore, the volume discusses these issues in the context of a best care model, which is an integrative care model where patients and their caregivers partner with Centers of Excellence with expertise in this rare disorder, to achieve best long-term outcomes. Written by experts in the field, Management of Wilson Disease: A Pocket Guide is a valuable resource for clinicians and primary care physicians who treat patients with Wilson disease.

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