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

Abstract

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

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

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Analysis of Dietary Nutrition Supplement in Children with Phenylketonuria

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