Can Patients with Wilson's Disease Develop Copper Deficiency?

source extracted confidence: high 2026-04-09
#adult #female #humans #male #copper

Can Patients with Wilson's Disease Develop Copper Deficiency?

Chevalier et al., 2023 | Mov Disord Clin Pract | Systematic Review

Citation

Chevalier Kevin, Obadia Mickaël Alexandre, ... Poujois Aurélia. Can Patients with Wilson's Disease Develop Copper Deficiency?. Mov Disord Clin Pract. 2023-Sep;10(9):1306-1316. doi:10.1002/mdc3.13813

Abstract

BACKGROUND: Wilson's disease (WD) is a rare genetic condition characterized by a copper overload in organs secondary to mutation in ATP7B gene. Lifelong decoppering treatments are the keystone of the treatment but must be regularly adapted to obtain a correct copper balance and could lead to copper deficiency (CD). OBJECTIVES: Study the characteristics of CD in WD patients. METHODS: CD cases from our cohort of 338 WD patients have been investigated. CD was defined by the association of serum copper, exchangeable copper and urinary copper excretion assays less than two standard deviations from the mean with cytopenia and/or neurological damage of spinal cord origin. A systematic review of literature about cases of CD in WD patient was performed in PubMed database according to PRISMA guidelines. RESULTS: Three WD patients were diagnosed with CD in our cohort. Review of the literature found 17 other patients. Most of the patients had anemia and neutropenia associated with neurological symptoms (especially progressive posterior cord syndrome). All the patients were treated with Zinc salts and the symptoms occurred more than a decade after the initiation of treatment. The adaptation of the treatment allowed a correction of the cytopenia but only a partial improvement of the neurological symptoms. CONCLUSIONS: WD patients can develop CD after many years of zinc therapy. Anemia and neutropenia are red flags that should evoke CD.

Key Findings

Three WD patients were diagnosed with CD in our cohort. Review of the literature found 17 other patients. Most of the patients had anemia and neutropenia associated with neurological symptoms (especially progressive posterior cord syndrome). All the patients were treated with Zinc salts and the symptoms occurred more than a decade after the initiation of treatment. The adaptation of the treatment allowed a correction of the cytopenia but only a partial improvement of the neurological symptoms.

Outcomes Measured

  • Requires manual extraction

Population

Field Value
Population See abstract
Sample Size See abstract
Age Range See abstract
Condition deficiency

MeSH Terms

  • Adult
  • Female
  • Humans
  • Male
  • Copper
  • Hepatolenticular Degeneration
  • Young Adult
  • Middle Aged
  • Aged

Evidence Classification

  • Level: Systematic Review
  • Publication Types: Journal Article, Systematic Review, Case Reports
  • Vertical: copper

Provenance

Source extracted via PubMed E-utilities API on 2026-04-10