The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria
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In a previous study, Dutch children with phenylketonuria (PKU) were found to be slightly shorter than their healthy counterparts. In the literature, it has been hypothesized that a higher protein intake is necessary to optimize growth in PKU patients. The study aimed to investigate whether protein intake (total, natural and protein substitute) in this group might be an explanatory factor for the observed growth. Growth of height and head circumference and dietary data on protein intake (total, natural and protein substitute) from 174 Dutch PKU patients born between 1974 and 1996 were analysed retrospectively for the patients' first 3 years of life. Analyses were corrected for energy intake during the first year of life and for the clinical severity of the deficiency of phenylalanine hydroxylase by means of plasma phenylalanine concentration at birth. Neither protein nor energy intake correlated with height growth. A positive, statistically significant relation between head circumference growth and natural protein and total protein intake was found, but not with the intake of the protein substitute or energy. Therefore, this study suggests that improvement of the protein substitute rather than an increase of total protein intake may be important in optimizing head circumference growth in PKU patients.
KeywordsPhenylalanine Energy Intake Protein Intake Natural Protein Head Circumference
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- Acosta PB, Yannicelli S (1994) Protein intake affects phenylalanine requirements and growth of infants with phenylketonuria. Acta Paediatr 407(Supplement): 66–67.Google Scholar
- Anonymous (1971) Malnutrition with early treatment of phenylketonuria. Nutr Rev 29: 11–13.Google Scholar
- Cockburn F, Clark BJ, Caine EA, et al (1996) Fatty acids in the stability of neuronal membrane: relevance to PKU. Int Pediatr 1: 56–60.Google Scholar
- Fomon SJ (1991) Requirements and recommended dietary intakes of protein during infancy. Pedriatr Res 30: 391–395.Google Scholar
- Gropper SS, Acosta PB (1991) Effect of simultaneous ingestion of L-amino acids and whole protein on plasma amino acid and urea nitrogen concentrations in humans. J Parenter Enteral Nutr 15: 48–53.Google Scholar
- Herrmann ME, Brosicke HG, Keller M, Monch E, Helge H (1994) Dependence of the utilization of a phenylalanine-free amino acid mixture on different amounts of single dose ingested. A case report Eur J Pediatr 153: 501–503.Google Scholar
- Huijbregts SCJ, de Sonneville LMJ, van Spronsen FJ, et al (2003) Motor function under lower and higher controlled processing demands in early and continuously treated phenylketonuria. Neuro- psychology 17: 369–379Google Scholar
- Schaeffer F, Burgard P, Bazler U, et al (1994) Growth and skeletal maturation in children with phenylketonuria. Act Paediatr 83: 534–541.Google Scholar
- Scriver CR, Kaufman S (2001) Hyperphenylalaninemia: phenylalanine hydroxylase deficiency. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds; Childs B, Kinzler KW, Vogelstein B, assoc. eds. The Metabolic and Molecular Bases of Inherited Disease, 8th edn. New York: McGraw-Hill, 1667–1724.Google Scholar