Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations

Background . Available treatments for Wilson disease (WD) prevent longterm complications of copper accumulation. Current anti-copper agents include zinc salts, penicillamine, and trientine. Patients with WD may switch between the agents for a number of reasons. Due to the different mechanisms of act...

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Main Authors: Marcia Leung MD, Jaimie Wu Lanzafame MD, Valentina Medici MD
Format: Article
Language:English
Published: SAGE Publishing 2020-01-01
Series:Journal of Investigative Medicine High Impact Case Reports
Online Access:https://doi.org/10.1177/2324709619896876
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spelling doaj-64f59fc21a2649fe83ddbe26e85fefcd2020-11-25T03:46:39ZengSAGE PublishingJournal of Investigative Medicine High Impact Case Reports2324-70962020-01-01810.1177/2324709619896876Switching Pharmacological Treatment in Wilson Disease: Case Report and RecommendationsMarcia Leung MD0Jaimie Wu Lanzafame MD1Valentina Medici MD2University of California Davis, Sacramento, CA, USAUniversity of California Davis, Sacramento, CA, USAUniversity of California Davis, Sacramento, CA, USABackground . Available treatments for Wilson disease (WD) prevent longterm complications of copper accumulation. Current anti-copper agents include zinc salts, penicillamine, and trientine. Patients with WD may switch between the agents for a number of reasons. Due to the different mechanisms of action between the copper chelators and zinc salts, transitioning could require a period of overlap and increased monitoring. There are no large studies that investigate the best transition strategies between agents. In this article, we review the treatments for WD and how to monitor for treatment efficacy. Case Summary . The patient had been diagnosed with WD for over 20 years prior to establishing care in our Hepatology Clinic. During his initial course, he was transitioned from penicillamine to zinc due to evidence suggesting penicillamine had greater adverse effects in the long term. Later, he was switched to trientine. His liver enzymes and 24-hour urine copper were monitored. During these years, he intermittently had some financial hardship, requiring him to be on penicillamine rather than trientine. He also had developed acute kidney injury. Overall, his liver disease remained under control and he never had signs of decompensated cirrhosis, but had fluctuations of liver enzymes over the years. Conclusion . Anti-copper treatment for WD has to be tailored to medication side effects profile, patient’s chronic and emerging comorbidities, as well as costs. Transitioning regimens is often challenging, and it requires closer monitoring, with no predictors of response.https://doi.org/10.1177/2324709619896876
collection DOAJ
language English
format Article
sources DOAJ
author Marcia Leung MD
Jaimie Wu Lanzafame MD
Valentina Medici MD
spellingShingle Marcia Leung MD
Jaimie Wu Lanzafame MD
Valentina Medici MD
Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations
Journal of Investigative Medicine High Impact Case Reports
author_facet Marcia Leung MD
Jaimie Wu Lanzafame MD
Valentina Medici MD
author_sort Marcia Leung MD
title Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations
title_short Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations
title_full Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations
title_fullStr Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations
title_full_unstemmed Switching Pharmacological Treatment in Wilson Disease: Case Report and Recommendations
title_sort switching pharmacological treatment in wilson disease: case report and recommendations
publisher SAGE Publishing
series Journal of Investigative Medicine High Impact Case Reports
issn 2324-7096
publishDate 2020-01-01
description Background . Available treatments for Wilson disease (WD) prevent longterm complications of copper accumulation. Current anti-copper agents include zinc salts, penicillamine, and trientine. Patients with WD may switch between the agents for a number of reasons. Due to the different mechanisms of action between the copper chelators and zinc salts, transitioning could require a period of overlap and increased monitoring. There are no large studies that investigate the best transition strategies between agents. In this article, we review the treatments for WD and how to monitor for treatment efficacy. Case Summary . The patient had been diagnosed with WD for over 20 years prior to establishing care in our Hepatology Clinic. During his initial course, he was transitioned from penicillamine to zinc due to evidence suggesting penicillamine had greater adverse effects in the long term. Later, he was switched to trientine. His liver enzymes and 24-hour urine copper were monitored. During these years, he intermittently had some financial hardship, requiring him to be on penicillamine rather than trientine. He also had developed acute kidney injury. Overall, his liver disease remained under control and he never had signs of decompensated cirrhosis, but had fluctuations of liver enzymes over the years. Conclusion . Anti-copper treatment for WD has to be tailored to medication side effects profile, patient’s chronic and emerging comorbidities, as well as costs. Transitioning regimens is often challenging, and it requires closer monitoring, with no predictors of response.
url https://doi.org/10.1177/2324709619896876
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