Teufel-Schäfer Ulrike, Forster Christine, Schaefer Nikolaus
Department of General Pediatrics, Adolescent Medicine and Neonatology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Mathildenstr. 1, 79106 Freiburg, Germany.
Children (Basel). 2022 Jul 28;9(8):1132. doi: 10.3390/children9081132.
Wilson's disease (WD) is an autosomal recessive inherited disease in which a pathological storage of copper in various organs is the mean pathophysiological mechanism. The therapy consists of drug therapy with chelating agents or zinc. For patients, nutrition is always an important issue. The aim of this review was to determine whether there are clear recommendations for a low copper diet for WD patients, or whether the essential trace element zinc plays a role? We were able to show that some of the foods with high copper content would have to be consumed in such large quantities that this is regularly not the case. Furthermore, there are also different absorption rates depending on the copper content. A lower copper intake only prevents the re-accumulation of copper. In summary, consistent adherence to drug therapy is more important than a strict diet. Only two foods should be consistently avoided: Liver and Shellfish.
威尔逊病（WD）是一种常染色体隐性遗传病，其病理生理机制主要是铜在各个器官中的病理性蓄积。治疗方法包括使用螯合剂或锌进行药物治疗。对患者来说，营养始终是一个重要问题。本综述的目的是确定对于WD患者是否有明确的低铜饮食建议，或者必需微量元素锌是否发挥作用？我们能够证明，一些铜含量高的食物必须大量食用，但实际情况并非如此。此外，根据铜含量的不同，吸收率也有所不同。较低的铜摄入量只能防止铜的重新蓄积。总之，持续坚持药物治疗比严格的饮食更重要。只有两种食物应始终避免：肝脏和贝类。
