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Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review

Background: Treatment of Wilson’s disease (WD), an inherited disease characterized by copper overload, is lifelong and there is the possibility that copper deficiency (CD) may occur. We systematically reviewed the literature to describe treatment patterns, symptoms and outcomes associated with CD. M...

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Autores principales: Litwin, Tomasz, Antos, Agnieszka, Bembenek, Jan, Przybyłkowski, Adam, Kurkowska-Jastrzębska, Iwona, Skowrońska, Marta, Członkowska, Anna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10377829/
https://www.ncbi.nlm.nih.gov/pubmed/37510170
http://dx.doi.org/10.3390/diagnostics13142424
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author Litwin, Tomasz
Antos, Agnieszka
Bembenek, Jan
Przybyłkowski, Adam
Kurkowska-Jastrzębska, Iwona
Skowrońska, Marta
Członkowska, Anna
author_facet Litwin, Tomasz
Antos, Agnieszka
Bembenek, Jan
Przybyłkowski, Adam
Kurkowska-Jastrzębska, Iwona
Skowrońska, Marta
Członkowska, Anna
author_sort Litwin, Tomasz
collection PubMed
description Background: Treatment of Wilson’s disease (WD), an inherited disease characterized by copper overload, is lifelong and there is the possibility that copper deficiency (CD) may occur. We systematically reviewed the literature to describe treatment patterns, symptoms and outcomes associated with CD. Methods: Using preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines, the PubMed database was searched up to 6 April 2023. Results: Across 17 articles, 20 cases of CD were described, most commonly (15 cases) in WD patients treated with zinc salts (ZS), less often on combined chelator and ZS therapy (3 cases), molybdate salts plus ZS (1), or molybdate alone (1). CD symptoms occurred insidiously, including sideroblastic anemia, neutropenia, axonal sensory neuropathy, posterior cord myelopathy and increased ratio of epileptic seizures (or epilepsy). CD diagnosis was based on symptoms and severely reduced urinary copper excretion (<20 µg/24 h [<0.3 µmol/24 h] on ZS, or <100 µg/24 h [<1.6 µmol/24 h] on chelators) with low total serum copper and ceruloplasmin. Conclusions: Awareness of CD and regular monitoring of copper metabolism is needed during WD treatment. Temporary cessation of anti-copper treatment usually reverses serum copper reductions as well as pancytopenia; however, some symptoms, especially neuropathy and myelopathy, may persist.
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spelling pubmed-103778292023-07-29 Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review Litwin, Tomasz Antos, Agnieszka Bembenek, Jan Przybyłkowski, Adam Kurkowska-Jastrzębska, Iwona Skowrońska, Marta Członkowska, Anna Diagnostics (Basel) Systematic Review Background: Treatment of Wilson’s disease (WD), an inherited disease characterized by copper overload, is lifelong and there is the possibility that copper deficiency (CD) may occur. We systematically reviewed the literature to describe treatment patterns, symptoms and outcomes associated with CD. Methods: Using preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines, the PubMed database was searched up to 6 April 2023. Results: Across 17 articles, 20 cases of CD were described, most commonly (15 cases) in WD patients treated with zinc salts (ZS), less often on combined chelator and ZS therapy (3 cases), molybdate salts plus ZS (1), or molybdate alone (1). CD symptoms occurred insidiously, including sideroblastic anemia, neutropenia, axonal sensory neuropathy, posterior cord myelopathy and increased ratio of epileptic seizures (or epilepsy). CD diagnosis was based on symptoms and severely reduced urinary copper excretion (<20 µg/24 h [<0.3 µmol/24 h] on ZS, or <100 µg/24 h [<1.6 µmol/24 h] on chelators) with low total serum copper and ceruloplasmin. Conclusions: Awareness of CD and regular monitoring of copper metabolism is needed during WD treatment. Temporary cessation of anti-copper treatment usually reverses serum copper reductions as well as pancytopenia; however, some symptoms, especially neuropathy and myelopathy, may persist. MDPI 2023-07-20 /pmc/articles/PMC10377829/ /pubmed/37510170 http://dx.doi.org/10.3390/diagnostics13142424 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Systematic Review
Litwin, Tomasz
Antos, Agnieszka
Bembenek, Jan
Przybyłkowski, Adam
Kurkowska-Jastrzębska, Iwona
Skowrońska, Marta
Członkowska, Anna
Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review
title Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review
title_full Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review
title_fullStr Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review
title_full_unstemmed Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review
title_short Copper Deficiency as Wilson’s Disease Overtreatment: A Systematic Review
title_sort copper deficiency as wilson’s disease overtreatment: a systematic review
topic Systematic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10377829/
https://www.ncbi.nlm.nih.gov/pubmed/37510170
http://dx.doi.org/10.3390/diagnostics13142424
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