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Monitoring Treatment of Wilson Disease

  • Tamir Miloh
  • Michael L. Schilsky
Chapter
Part of the Clinical Gastroenterology book series (CG)

Abstract

Treatment of Wilson disease (WD) with medical therapy and degrees of dietary restriction of copper intake is lifelong, but adjustments of treatments and determining their effectiveness require appropriate monitoring. There are two categories of treatment monitoring, monitoring for effectiveness and monitoring for safety. Monitoring requires assessing for changes in health status, including physical exam, blood and urine testing, and imaging. The frequency of monitoring may change according to the clinical status, type of therapy, and phase of treatment. Special attention is given to symptoms related to liver disease, neurologic findings, the appearance of KF rings, psychiatric findings, and quality of life. In addition, monitoring for adherence to treatment links to monitoring for effectiveness as adherence is critical to achieving best patient outcomes. Copper status should be monitored regularly, as some patients may be overtreated, resulting to overlapping symptomatology due to copper excess.

Keywords

Wilson disease Chelation Copper Zinc Adherence Monitoring 

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatric Hepatology and Liver Transplant MedicineTexas Children’s HospitalHoustonUSA
  2. 2.Baylor College of MedicineHoustonUSA
  3. 3.Division of Digestive Diseases and Transplant and Immunology, Departments of Medicine and SurgeryYale UniversityNew HavenUSA

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