Abstract: Objective Dietary copper restriction has long been considered an important treatment for patients with hepatolenticular degeneration (HLD). However, evidence supporting this approach is limited. There are no published randomised controlled trials for the recommendation due to rarity of the disease and variable presentation. This review summarized current knowledge on the absorption and regulation of copper in humans and its relevance to patients with HLD. Studies have demonstrated that as the level of dietary copper increases, the proportion absorbed decreases. This observation implies that ‘high copper' foods that HLD patients are generally advised to avoid would need to be consumed in large amounts to impact markedly on the quantity absorbed. Dietary copper restriction is unlikely to reduce the amount absorbed significantly and is not only difficult to manage but restricts food supply unnecessarily, detracting from the provision of substrates essential for improving nutritional status in a nutritionally compromised group. Medical management for HLD is effective in compliant patients, allowing stabilization of the liver disease. Based on current evidence, dietary copper restrictions in stable HLD patients who are adherent to medical therapy are unnecessary with two food exceptions (shellfish and liver organ).

Key words: Hepatolenticular degeneration, Copper restricted diet, Therapy