Abstract：Objective To study the efficacy of a low-copper diet guidance based on food exchange portions in children with hepatolenticular degeneration. Methods A self-controlled study was conducted from July 2021 to June 2022, including 30 children under the age of 18 who were diagnosed with hepatolenticular degeneration and poorly controlled with a low-copper diet. During the medical visit, personalized low-copper diet guidance was provided to the children and their parents using a copper-containing food exchange table and a copper food exchange chart. During home care, compliance with the low-copper diet of the children was improved by recording dietary diaries and conducting regular follow-ups. The changes in 24-hour urine copper level, liver function indicators, and the low-copper diet knowledge of the children's parents were observed before and after the intervention, with no change in the original drug treatment. Results After 8, 16, and 24 weeks of intervention, the 24-hour urine copper level decreased significantly compared to before intervention (P<0.05). When compared to 8-week intervention, the urine copper level decreased significantly after 16 and 24 weeks of intervention. The 24-hour urine copper level after 24 weeks of intervention decreased significantly compared to 16 weeks of intervention (P<0.05).After 24 weeks of intervention, the alanine aminotransferase and aspartate aminotransferase levels decreased significantly compared to before intervention (P<0.05). Additionally, in 16 of the cases (53%), alanine aminotransferase and aspartate aminotransferase returned to normal levels. Following 8 weeks of intervention, the low-copper diet knowledge of the children's parents increased significantly (P<0.05). Conclusions A low-copper diet guidance based on food exchange portions can effectively decrease the urine copper level and improve liver function in children with hepatolenticular degeneration. Furthermore, it can increase the low-copper diet knowledge of the children's parents.
CHEN Ying-Xiang,QIU Zheng-Qing,SUN Jing et al. Application of a low copper diet guidance based on food exchange portions in children with hepatolenticular degeneration[J]. CJCP, 2023, 25(6): 612-618.
7 Rivard AM. Dietary Copper and Diet Issues for Patients with Wilson Disease[M]. Cham: Springer International Publishing, 2018: 63-85.
8 Adachi Y, Ogimoto T, Moriyama K. An examination of mineral content ratios of food for copper-reduced diets for Wilson's disease: scatter plots estimation based on standards tables of food composition in Japan[EB/OL]. (2020-03-06)[2022-11-10]. https://jglobal.jst.go.jp/en/detail?JGLOBAL_ID=202002268199272245.
Baranovsky AY, Belodedova AS, Fedorova TF, et al. The new aspects of clinical nutrition at Wilson disease: actuality and perspectives[J]. Vopr Pitan, 2019, 88(4): 12-17. PMID: 31722136. DOI: 10.24411/0042-8833-2019-10036.
Cleary A, Dowling M. Knowledge and attitudes of mental health professionals in Ireland to the concept of recovery in mental health: a questionnaire survey[J]. J Psychiatr Ment Health Nurs, 2009, 16(6): 539-545. PMID: 19594676. DOI: 10.1111/j.1365-2850.2009.01411.x.
