Application Of Low Copper Diet Guidance Based On Food Exchange Method In Children With Hepatolenticular Degeneration

Background:Hepatolenticular degeneration is a common autosomal recessive disorder of copper metabolism in childhood.At present,there is no specific therapeutic method,and the core therapeutic principle is to reduce copper intake and increase copper excretion.Under normal circumstances,copper intake mainly comes from food.Low-copper diet can delay the onset of symptoms and control the progression of hepatolenticular degeneration,which is an important part of the integrated treatment of the disease.The survey found that children with hepatolenticular degeneration and their parents did not know how to implement a low-copper diet in their daily diet,and the existing research at home and abroad did not make a detailed introduction on how to specifically quantify the implementation of a low-copper diet,which was low operational.In this study,0.05mg copper was used as one food exchange portion to verify the intervention effect of low copper diet guidance based on food exchange portion method on children with hepatolenticular degeneration.Objectives:To explore the effects of low-copper diet guidance intervention based on food exchange method on 24-hour urinary copper value,liver function indexes(alanine aminotransferase,aspartate aminotransferase)and parents’ knowledge level of lowcopper diet in children with hepatolenticular degeneration.Methods:A total of 30 children under 18 years old who were diagnosed with hepatolenticular degeneration and poorly controlled with a low-copper diet were enrolled from July 2021 to June 2022.We used the copper food exchange table and copper food exchange map during hospitalization to guide the children and their parents with an individualized low-copper diet.Children’s compliance with a low-copper diet was improved by keeping a food diary and regular follow-up during the home period.After 24 weeks of intervention,the changes in 24-hour urinary copper value,liver function index,and parents’ knowledge level of low-copper diet were observed.Results:At the 8th,16th,and 24th week of intervention,the 24-hour urinary copper value of children was significantly lower than that before intervention(P<0.01),and the difference between the four time periods was statistically significant(P=0.032).Liver function indexes were decreased compared with those before intervention(P<0.01),and 16 cases(53.33%)had normal transaminase after the intervention.After the intervention,the low copper diet knowledge score(16.07±2.24)points was significantly higher than that before the intervention(11.07±2.18)points,and the difference was statistically significant(P<0.01).Conclusions:The dietary guidance of low copper based on the food exchange method can reduce the 24-hour urinary copper of children with Wilson’s disease,improve the liver function index,and improve the knowledge level of parents of children with Wilson’s disease.The copper food exchange table and copper food exchange map,as quantifiable and operable tools for children with Wilson’s disease,provide a new idea for the low copper diet of children with Wilson’s disease.