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Dietary copper restriction in Wilson's disease
Kylie Russell1, Lyn K Gillanders2, David W Orr3
1Nutrition and Dietetics, Auckland City Hospital, Auckland, New Zealand. krussell@adhb.govt.nz.
Dietary copper restriction is likely unnecessary for most Wilson's disease (WD) patients on medication. Current evidence suggests it offers minimal benefit and can negatively impact nutrition.
Area of Science:
- Biochemistry
- Human Nutrition
- Gastroenterology
Background:
- Dietary copper restriction is a traditional recommendation for Wilson's disease (WD).
- Evidence supporting this dietary approach is limited, with no randomized controlled trials available due to disease rarity.
Purpose of the Study:
- To review current knowledge on copper absorption and regulation in humans.
- To assess the relevance and efficacy of dietary copper restriction in managing Wilson's disease.
Main Methods:
- Literature review summarizing studies on human copper absorption and WD management.
- Analysis of the relationship between dietary copper intake and absorption proportions.
Main Results:
- Copper absorption proportion decreases as dietary intake increases.
- Large quantities of high-copper foods are needed to significantly impact copper absorption.
- Dietary copper restriction is difficult to manage and can impair nutritional status.
Conclusions:
- Dietary copper restriction is generally unnecessary for stable Wilson's disease patients adhering to medical therapy.
- Exceptions include avoiding large amounts of shellfish and liver.
- Focus should remain on effective medical management and nutritional support.
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