肝豆状核变性患儿低铜饮食管理现状的研究

Current status of low copper diet management in children with hepatolenticular degeneration

目的分析肝豆状核变性患儿低铜饮食管理现状,为个体化低铜饮食指导提供参考和帮助。方法采用问卷调查法与半结构式访谈法。问卷调查时采用便利抽样法,选取2021年8月—2022年10月于中国医学科学院北京协和医院儿科门诊及病房治疗的113名肝豆状核变性患儿的家长为研究对象,并采用自行设计的《肝豆状核变性患儿低铜饮食现状调查问卷》对其进行调查。半结构式访谈部分采用目的抽样法,募集符合入选标准的研究对象,信息达到饱和时不再纳入,最终纳入16名访谈对象。结果问卷调查结果显示,肝豆状核变性患儿家长低铜饮食知识问卷部分,正确率最低的条目分别是"根据食物中含铜量决定该食物是否可以食用,如100 g食物中若含铜量超0.5 mg则禁止食用"和"以下豆类食物中(豆腐、腐竹、豆腐干、油豆腐),哪个含铜量最高"。113例患儿中,由家长完全管理低铜饮食的有55例,53例患儿与家长共同管理低铜饮食,自我管理低铜饮食的患儿4例。64例患儿目前遵循低铜饮食原则,只吃含铜量低的几大类食物;32例患儿在确保每日食物中总含铜量不超标的情况下,保证饮食多元化。半结构式访谈提炼的主题包括:患儿家长对低铜饮食的认知不足、家长对患儿在外就餐的担忧、家长对如何兼顾低铜饮食和营养均衡存在疑惑、希望医护人员在低铜饮食指导方面提供一定帮助。结论肝豆状核变性患儿低铜饮食管理情况不佳;患儿家长缺乏低铜饮食相关知识且存在较多困惑,亟须医务人员提供更具体、丰富、操作性强的低铜饮食指导。

Objective To analyze the current situation of low copper diet management in children with hepatolenticular degeneration,so as to provide reference and help for individualized low copper diet guidance.Methods Questionnaire survey and semi-structured interview were used.In the questionnaire survey,a total of 113 parents of children with hepatolenticular degeneration who were treated in the Pediatric Outpatient Department and ward of Chinese Academy of Medical Sciences,Peking Union Medical College Hospital from August 2021 to October 2022 were selected as the research objects.And a self-designed Low Copper Diet Status Questionnaire for Children with Hepatolenticular Degeneration was used for investigation.In the semi-structured interview,the purposive sampling method was used to recruit research objects that met the inclusion criteria.When the information reached saturation,they were no longer included,and finally 16 interviewees were included.Results The results of the questionnaire showed that the items with the lowest accuracy in the part of the low-copper diet knowledge questionnaire for parents of children with hepatolenticular degeneration were"whether the food was edible according to the amount of copper in the food,for example,if the copper content in 100 g food exceeded 0.5 mg,it was forbidden to eat"and"in the following bean food(tofu,yuba,dried bean curd,oily bean curd),which one had the highest copper content".Among the 113 children,55 were completely managed by their parents on a low copper diet,53 children were jointly managed with their parents on a low copper diet,and 4 children were self-managed on a low copper diet.A total of 64 children currently followed the principle of a low copper diet,only consuming a few major categories of foods with low copper content.A total of 32 children ensured a diversified diet while ensuring that the total copper content in their daily food did not exceed the standard.The themes extracted from semi-structured interviews included insufficient awareness of low copper diet among parents of affected children,concerns about their children dining outside,doubts about how to balance low copper diet and nutritional balance among parents,and hopes that medical staff could provide some assistance in low copper diet guidance.Conclusions The management of low-copper diet in children with hepatolenticular degeneration is poor.The parents of the affected children lack knowledge about low copper diet and have many confusions,and there is an urgent need for medical personnel to provide more specific,rich and practical guidance on low copper diet.