Objectives: 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.
目的: 探讨基于食物交换份法的低铜饮食指导在肝豆状核变性患儿中的应用效果。方法: 采用自身前后对照研究设计，于2021年7月—2022年6月纳入年龄18周岁以下、诊断为肝豆状核变性、低铜饮食控制情况差的患儿共30例。就诊期间采用含铜食物交换表及铜食物交换图谱对患儿及其家长进行个体化低铜饮食指导。居家期间通过记录饮食日记和定期随访提高患儿低铜饮食的依从性。在原有药物治疗不变的情况下，观察患儿干预前后的24 h尿铜水平、肝功能指标及患儿家长低铜饮食知识水平的变化。结果: 干预8周、16周、24周后的24 h尿铜水平均较干预前下降（P<0.05）；干预16周和24周后的24 h尿铜水平较干预8周后下降（P<0.05）；干预24周后的24 h尿铜水平较干预16周后下降（P<0.05）。干预24周后，谷丙转氨酶和谷草转氨酶较干预前下降（P<0.05）；16例患儿（53%）谷丙转氨酶和谷草转氨酶降至正常水平。干预8周后，患儿家长的低铜饮食知识得分明显高于干预前（P<0.05）。结论: 基于食物交换份法的低铜饮食指导可使肝豆状核变性患儿的尿铜水平下降，肝功能得到改善，患儿家长低铜饮食知识水平得以提高。.
Keywords: Child; Food exchange; Hepatolenticular degeneration; Low copper diet.