关键词: 肝豆状核变性, 限铜饮食, 治疗, 铜缺乏

Abstract: Wilson’s disease is a primary copper metabolic disorder caused by ATP7B gene variation. It’s one of the treatable genetic metabolic diseases.The management included dietary restriction of copper，medical therapy and liver transplantation etc，However，dietary copper restriction can’t be the only treatment for Wilson‘s disease since low copper diet alone cannot prevent the progression，and copper-removal rugs or zinc agents must be used for treatment. Dietary restriction of copper-rich foods is advised during the first year after diagnosis，but whole life strict low copper diet is not recommended for Wilson’s disease.Overly copper restrictive diets are also associated with secondary copper deficiency，which affects life quality and nutritional status of Wilson’s disease patients.Medical therapy is still the primary treatment for Wilson’s disease；and dietary treatment is an important auxillary means.

Key words: Wilson’s disease, dietary restriction of copper, treatment, copper deficiency