Biomaterial Database

Taste preferences and feeding behaviour in children with phenylketonuria on a semisynthetic diet. 2000

M Owada, and K Aoki, and T Kitagawa

Department of Paediatrics, Nihon University Hospital, Tokyo, Japan.

Low-phenylalanine formula for phenylketonuria (PKU) made from free amino acids as a protein source (AAM formula) has a poor taste and smell. We developed a more palatable formula using low-phenylalanine peptide (LPP) as a protein source. Palatability tests performed by 41 healthy adults confirmed that the palatability of LPP formula was significantly better than that of AAM formula. A group of 48 patients with PKU who had been administered AAM formula since the newborn period were assessed for their preference between the AAM and LPP formulae and their feeding behaviour was compared to that in healthy children. Of patients, 90.9% and 66.6% of healthy infants less than 18 months of age took both formulae without apparent preference, suggesting that sensitivity to taste and smell is more immature in infancy than in later life. Of patients with PKU aged between 18 months and 11 years, 29.1% liked AAM formula rather than the LPP formula, while 66.7% took both formulae without apparent preference. Most healthy children in the same age group who had never previously tasted therapeutic formulae disliked it, although they tended to prefer the LPP formula. Of patients aged between 11 and 17 years, 84.6% preferred the LPP formula while 15.4% preferred the AAM formula. In the controls of this age group, 33% disliked therapeutic formulae, but they tended to prefer the LPP formula. CONCLUSIONS In some young children with phenylketonuria the characteristic taste of amino acid mixture formula encountered in early life is considered to be imprinted and remains as a preference for a long time. Since school children with phenylketonuria usually obtain about 50% of their energy intake from natural food containing small amounts of protein, these patients are considered to have come to have similar preferences as healthy people which result from a waning of the imprinted taste of amino acid mixture formula.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D010661	Phenylketonurias	A group of autosomal recessive disorders marked by a deficiency of the hepatic enzyme PHENYLALANINE HYDROXYLASE or less frequently by reduced activity of DIHYDROPTERIDINE REDUCTASE (i.e., atypical phenylketonuria). Classical phenylketonuria is caused by a severe deficiency of phenylalanine hydroxylase and presents in infancy with developmental delay; SEIZURES; skin HYPOPIGMENTATION; ECZEMA; and demyelination in the central nervous system. (From Adams et al., Principles of Neurology, 6th ed, p952).	Biopterin Deficiency,Dihydropteridine Reductase Deficiency Disease,Hyperphenylalaninemia, Non-Phenylketonuric,Phenylalanine Hydroxylase Deficiency Disease,BH4 Deficiency,DHPR Deficiency,Deficiency Disease, Dihydropteridine Reductase,Deficiency Disease, Phenylalanine Hydroxylase,Deficiency Disease, Phenylalanine Hydroxylase, Severe,Dihydropteridine Reductase Deficiency,Folling Disease,Folling's Disease,HPABH4C,Hyperphenylalaninaemia,Hyperphenylalaninemia Caused by a Defect in Biopterin Metabolism,Hyperphenylalaninemia, BH4-Deficient, C,Hyperphenylalaninemia, Tetrahydrobiopterin-Deficient, Due To DHPR Deficiency,Non-Phenylketonuric Hyperphenylalaninemia,Oligophrenia Phenylpyruvica,PAH Deficiency,PKU, Atypical,Phenylalanine Hydroxylase Deficiency,Phenylalanine Hydroxylase Deficiency Disease, Severe,Phenylketonuria,Phenylketonuria I,Phenylketonuria II,Phenylketonuria Type 2,Phenylketonuria, Atypical,Phenylketonuria, Classical,QDPR Deficiency,Quinoid Dihydropteridine Reductase Deficiency,Tetrahydrobiopterin Deficiency,Atypical PKU,Atypical Phenylketonuria,Biopterin Deficiencies,Classical Phenylketonuria,Deficiency, BH4,Deficiency, Biopterin,Deficiency, DHPR,Deficiency, Dihydropteridine Reductase,Deficiency, PAH,Deficiency, Phenylalanine Hydroxylase,Deficiency, QDPR,Deficiency, Tetrahydrobiopterin,Disease, Folling,Disease, Folling's,Hyperphenylalaninemia, Non Phenylketonuric,Non Phenylketonuric Hyperphenylalaninemia,Non-Phenylketonuric Hyperphenylalaninemias
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D005247	Feeding Behavior	Behavioral responses or sequences associated with eating including modes of feeding, rhythmic patterns of eating, and time intervals.	Dietary Habits,Eating Behavior,Faith-based Dietary Restrictions,Feeding Patterns,Feeding-Related Behavior,Food Habits,Diet Habits,Eating Habits,Behavior, Eating,Behavior, Feeding,Behavior, Feeding-Related,Behaviors, Eating,Behaviors, Feeding,Behaviors, Feeding-Related,Diet Habit,Dietary Habit,Dietary Restriction, Faith-based,Dietary Restrictions, Faith-based,Eating Behaviors,Eating Habit,Faith based Dietary Restrictions,Faith-based Dietary Restriction,Feeding Behaviors,Feeding Pattern,Feeding Related Behavior,Feeding-Related Behaviors,Food Habit,Habit, Diet,Habit, Dietary,Habit, Eating,Habit, Food,Habits, Diet,Pattern, Feeding,Patterns, Feeding,Restrictions, Faith-based Dietary
D005518	Food Preferences	The selection of one food over another.	Food Selection,Food Preference,Food Selections,Preference, Food,Preferences, Food,Selection, Food,Selections, Food
D005526	Food, Formulated	Food and dietary formulations including elemental (chemically defined formula) diets, synthetic and semisynthetic diets, space diets, weight-reduction formulas, tube-feeding diets, complete liquid diets, and supplemental liquid and solid diets.	Diet, Chemically Defined,Diet, Elemental,Diet, Formula,Diet, Synthetic,Dietary Formulations,Chemically Defined Diet,Synthetic Diet,Chemically Defined Diets,Dietary Formulation,Diets, Chemically Defined,Diets, Elemental,Diets, Formula,Diets, Synthetic,Elemental Diet,Elemental Diets,Foods, Formulated,Formulated Food,Formulated Foods,Formulation, Dietary,Formulations, Dietary
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D013649	Taste	The ability to detect chemicals through gustatory receptors in the mouth, including those on the TONGUE; the PALATE; the PHARYNX; and the EPIGLOTTIS.	Gustation,Taste Sense,Gustations,Sense, Taste,Senses, Taste,Taste Senses,Tastes