肝豆状核变性的饮食和药物治疗  

Diet and drug therapy for Wilson's disease

作　　者：方微园 王建设 FANG Wei-yuan;WANG Jian-she(The Center for Pediatric Liver Diseases,Children's Hospital of Fudan University,Shanghai 201102,China)

机构地区：[1]复旦大学附属儿科医院儿童肝病中心,上海201102

出　　处：《中国实用儿科杂志》2023年第4期297-300,306,共5页Chinese Journal of Practical Pediatrics

摘　　要：肝豆状核变性是由于ATP7B基因变异所致的一种原发性铜代谢障碍性疾病,是可治疗的遗传代谢病之一。治疗包括低铜饮食、药物及肝移植等。虽然肝豆状核变性是因为体内铜过多引起,然而,单纯限铜饮食并不能阻止疾病的进展,必须使用驱铜药物或锌剂治疗。一般建议在治疗的第一年限制富含铜的食物,病情平稳后可适当放宽对铜摄入的限制,无需严格的终身限铜饮食。过度限铜饮食有可能会引发铜缺乏,影响患者的生活质量和营养状态。药物治疗仍是肝豆状核变性治疗的基本措施,饮食治疗是重要的辅助手段。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.

关 键 词：肝豆状核变性 限铜饮食 治疗 铜缺乏 

分 类 号：R72[医药卫生—儿科]