苯丙酮尿症儿童膳食营养补充现状分析

doi:10.12377/1671-4393.22.10.19

摘要/Abstract

摘要： 苯丙酮尿症（PKU）是一种代谢异常的常染色体隐性遗传病，可引起患儿神经认知及神经运动障碍、自闭症行为、湿疹和癫痫等并发症。在治疗过程中，通过低苯丙氨酸（Phe）限制饮食的同时，还需关注患儿其他营养素缺乏的营养问题，因此开发适用于1~10岁PKU儿童的特殊医学用途氨基酸代谢障碍配方粉，对保障患儿正常生长发育具有重要意义。本文在PKU分型、饮食疗法、PKU患儿营养素缺乏等方面进行了综述，重点分析低蛋白饮食、基于游离氨基酸的特殊医学用途氨基酸代谢障碍配方粉以及详述了PKU患儿体内某些营养素的缺乏与补充，以期为PKU饮食治疗中的营养问题以及特殊医学用途氨基酸代谢障碍配方粉的开发提供理论指导与参考。

关键词: 苯丙酮尿症, 饮食疗法, 特殊医学用途氨基酸代谢障碍配方粉, 营养素

Abstract: Phenylketuria（PKU）is an autosomal recessive disorder with abnormal metabolism，which can cause neurocognitive and neuromotor disorders，autistic behavior，eczema，epilepsy and other complications in children. In the course of treatment，while limiting diet through low Phe，it is also necessary to pay attention to the nutritional problems of other nutrients in children. Therefore，the development of formula powder with amino acid metabolic disorders for special medical purpose suitable for PKU children aged 1~10 years is of great significance to ensure the normal growth and development of children. In this paper，the classification of PKU，diet therapy and nutrient deficiency of PKU children were reviewed，focusing on the analysis of low protein diet，formula powder with amino acid metabolic disorders for special medical purpose based on free amino acids and the lack and supplement of some nutrients in PKU children，in order to provide theoretical guidance and reference for the nutrition problems in PKU diet therapy and the development of formula powder with amino acid metabolic disorders for special medical purpose.

Key words: phenylketonuria, dietotherapy, formula powder with amino acid metabolic disorders for special medical purpose, nutrients

王艳聪. 苯丙酮尿症儿童膳食营养补充现状分析[J]. 中国乳业, 2022, 0(10): 104-110.

WANG Yancong. Analysis of Dietary Nutrition Supplement in Children with Phenylketonuria[J]. China Dairy, 2022, 0(10): 104-110.

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