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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Online Access: | https://doi.org/10.1177/2324709619896876 |
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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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