| Abstract: | 威爾森氏症(Wilson’s disease; WD)是由於染色體基因ATP7B發生異常，導致銅代謝功能障礙而蓄積於肝、腎、腦、眼睛等部位的罕見遺傳疾病。WD的治療以藥物為主，但有動物和人體實驗結果發現，輔以低銅飲食能避免肝臟症狀惡化及緩解神經症狀。然而對WD患者來說，居家製備低銅飲食並不容易，故本研究在於選用台灣在地生鮮食材來設計七天低銅菜單，並將食物中銅含量之數值轉化為「銅點數表」，希望能增加WD病友在採行低銅飲食的便利性。以19歲以上成年人為對象之七天低銅飲食菜單的設計，主要是依據臨床營養工作手冊之飲食原則，並參照每日飲食指南及國人膳食營養素參考攝取量(dietary reference intakes; DRIs)第七版的要點，然後進行實作。食物之銅含量數值是以台灣食品營養成分資料庫為主，而以美國、中國與日本的資料庫為輔。銅點數的制定規則為將25 μg<Cu≦75 μg訂為0.5點(平均50 μg)，75 μg<Cu≦125 μg為1點(平均100 μg)，依此類推，一日攝取不得超過9點(900 μg)，另增加0 μg為0點、0 μg<Cu≦25 μg為0.125點，以避免銅點數的低估。結果顯示，七天低銅飲食菜單的銅含量皆不超過900 μg；點數表雖然有較為高估的情形，但仍能反映菜單中銅含量的多寡。故本研究所設計的七天低銅飲食菜單可做為WD病友在製備低銅飲食的參考，然而銅點數表在實際應用上仍有諸多限制及考量，尚需進一步的調整及修正，因此WD患者於執行低銅飲食時，仍建議先以銅含量食物分類表為參考，盡量選用中、低銅含量的食物，並注意避開全穀類、豆類、動物內臟、蝦蟹貝類等高銅含量食物，必要時搭配適當補充劑，以預防潛在營養素缺乏的問題。
Wilson’s disease (WD) is a genetic disorder due to pathogenic mutation in ATP7B gene, which affects normal copper metabolism and makes copper to accumulate in the body, especially in the liver, kidneys, brain and eyes. Taking medications is the main treatment for WD, however, the results from both animal and human studies indicated that the low-copper diet could alleviate liver and neurological complications of WD. However, it is not easy for the WD patients to prepare a low-copper diet at home. Therefore, the purposes of this study were to design a seven-day low-copper diet meal plan with Taiwanese fresh and local food items as well as to develop a copper content counting system of food to help the WD patients to implement the low-copper diet. The contents of copper in food were primarily obtained from the Taiwan Food Composition Database and were supplemented with Food Composition Databases of the United States, China and Japan. The seven-day low-copper diet meal plan, based on the dietary principles of the low-copper diet, Taiwanese daily food guide and the seventh revision of Taiwan Dietary Reference Intakes, was designed and prepared for the adult WD patients. The copper points were set as follows: 0.5 point means 25 μg < Cu ? 75 μg (average 50 μg), 1 point means 75 μg < Cu ? 125 μg (average 100 μg), and so on. The daily copper intake should not exceed 9 points (900 μg). In addition, 0 point means 0 μg and 0.125 point means 0 μg < Cu ? 25 μg to avoid underestimating copper contents. The results showed that the copper contents of all seven-day designed low-copper diets were less than 900 μg. Although the counting system slightly overestimated the copper contents, but it still could reflect the amounts of copper in the designed diets. The designed low-copper diets could be used as a reference for the WD patients to implement their low-copper diet. However, the preliminary copper content counting system still has many limitations in practical application and needs to be improved and corrected for future application. Therefore, it is recommended for the WD patients to make food choices based on the copper contents, that is, to select medium- and low-copper foods, and particularly to restrict the consumption of high-copper foods such as whole grains, beans, organ meats and shellfish, and take appropriate dietary supplements to prevent nutritional deficiency. |
