Nutrient intake of treated infants with phenylketonuria. 1977

P B Acosta, and E Wenz, and M Williamson

Growth, energy, and nutrient intake of 88 treated infants in the Collaborative Study of Children Treated for Phenylketonuria were evaluated longitudinally and compared to normative data and by treatment group. Growth parameters (height and weight) did not differ according to treatment group assignment, nor did they differ from normative data. Subjects in treatment group 2 had a significantly higher intake of phenylalanine than did subjects in treatment group 1. Differences in intake of other nutrients disappeared when intake was compared on an energy or body weight basis. Differences in intake by males and females also disappeared when compared on an energy and a body weight basis. From none to 10% of the subjects had energy intakes below two-thirds of the 1968 Recommended Dietary Allowances (RDA). Low individual energy intakes were more commonly found in the first and fourth quarters, and only during the first quarter of infancy did mean energy intake meet the RDA. From 48 to 80% of subjects had intakes of preformed niacin below two-thirds of the 1968 RDA. Few subjects had low intakes of protein, iron, or vitamin A, and intakes of all subjects were greater than 67% of the RDA for calcium, phosphorus, thiamin, riboflavin, and ascorbic acid. It is suggested from the data presented that a supplemental casein hydrolysate supplies adequate essential amino acids and nitrogen to support normal growth in infants when protein and energy intakes are fed at the levels described.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007501 Iron A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN. Iron-56,Iron 56
D008297 Male Males
D009751 Nutritional Requirements The amounts of various substances in food needed by an organism to sustain healthy life. Dietary Requirements,Nutrition Requirements,Dietary Requirement,Nutrition Requirement,Nutritional Requirement,Requirement, Dietary,Requirement, Nutrition,Requirement, Nutritional,Requirements, Dietary,Requirements, Nutrition,Requirements, Nutritional
D010649 Phenylalanine An essential aromatic amino acid that is a precursor of MELANIN; DOPAMINE; noradrenalin (NOREPINEPHRINE), and THYROXINE. Endorphenyl,L-Phenylalanine,Phenylalanine, L-Isomer,L-Isomer Phenylalanine,Phenylalanine, L Isomer
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
D010758 Phosphorus A non-metal element that has the atomic symbol P, atomic number 15, and atomic weight 31. It is an essential element that takes part in a broad variety of biochemical reactions. Black Phosphorus,Phosphorus-31,Red Phosphorus,White Phosphorus,Yellow Phosphorus,Phosphorus 31,Phosphorus, Black,Phosphorus, Red,Phosphorus, White,Phosphorus, Yellow
D001827 Body Height The distance from the sole to the crown of the head with body standing on a flat surface and fully extended. Body Heights,Height, Body,Heights, Body
D001835 Body Weight The mass or quantity of heaviness of an individual. It is expressed by units of pounds or kilograms. Body Weights,Weight, Body,Weights, Body

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