Advertisement

Journal of Inherited Metabolic Disease

, Volume 28, Issue 6, pp 845–854 | Cite as

The intake of total protein, natural protein and protein substitute and growth of height and head circumference in Dutch infants with phenylketonuria

  • M. Hoeksma
  • M. Van Rijn
  • P. H. Verkerk
  • A. M. Bosch
  • M. F. Mulder
  • J. B. C. de Klerk
  • T. J. De Koning
  • E. Rubio-Gozalbo
  • M. De Vries
  • P. J. J. Sauer
  • F. J. van Spronsen
Article

Summary

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.

Keywords

Phenylalanine Energy Intake Protein Intake Natural Protein Head Circumference 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Acosta PB (1996) Recommendations for protein and energy intakes by patients with phenylketonuria. Eur J Pediatr 155(Supplement 1): S121–S124.PubMedGoogle Scholar
  2. Acosta PB, Yannicelli S (1994) Protein intake affects phenylalanine requirements and growth of infants with phenylketonuria. Acta Paediatr 407(Supplement): 66–67.Google Scholar
  3. Acosta PB, Wenz RD, Williamson M (1977) Nutrient intake of treated infants with phenylketonuria. Am J Clin Nutr 30: 198–208.PubMedGoogle Scholar
  4. Acosta PB, Yannicelli S, Marriage B (1998) Nutrient intake and growth of infants with phenylketonuria undergoing therapy. J Pediatr Gastroenterol Nutr 27: 287–291.CrossRefPubMedGoogle Scholar
  5. Anonymous (1971) Malnutrition with early treatment of phenylketonuria. Nutr Rev 29: 11–13.Google Scholar
  6. Carver JD, Wu PY, Hall RT, et al (2001) Growth of preterm infants fed nutrient-enriched or term formula after hospital discharge. Pediatrics 107: 683–689.CrossRefPubMedGoogle Scholar
  7. Chang P, Weisberg S, Fisch RO (1984) Growth development and its relationship to intellectual functioning of children with phenylketonuria. J Dev Behav Pediatr 5: 127–131.PubMedGoogle Scholar
  8. 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
  9. Dewey GK, Beaton G, Fjeld C, Lönnerdal B, Reeds P (1996) Protein requirements of infants and children. Eur J Clin Nutr 50(Supplement 1): S119–150.PubMedGoogle Scholar
  10. Dobbelaere D, Michaud L, Debrabander A, et al (2003) Evaluation of nutritional status and pathophysiology of growth retardation in patients with phenylketonuria. J Inherit Metab Dis 26: 1–11.CrossRefPubMedGoogle Scholar
  11. Dolk H (1991) The predictive value of microcephaly during the first year of life for mental retardation at seven years. Dev Med Child Neurol 33: 974–983.PubMedGoogle Scholar
  12. Fomon SJ (1991) Requirements and recommended dietary intakes of protein during infancy. Pedriatr Res 30: 391–395.Google Scholar
  13. Fredriks AM, van Buuren S, Burgmeijer RJ, et al (2000) Continuing positive secular growth change in The Netherlands 1955–1997. Pediatr Res 47: 316–323.PubMedGoogle Scholar
  14. Giovannini M, Biasucci G, Agostoni C, Luotti D, Riva E (1995) Lipid status and fatty acid metabolism in phenylketonuria. J Inherit Metab Dis 18: 265–272.CrossRefPubMedGoogle Scholar
  15. 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
  16. 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
  17. 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
  18. Kindt E, Lunde HA, Gjessing LR, et al (1988) Fasting plasma amino acid concentrations in PKU children on two different levels of protein intake. Acta Paediatr Scand 77: 60–66.PubMedGoogle Scholar
  19. Lindley AA, Benson JE, Grimes C, Cole IM, Herman AA (1999) The relationship in neonates between clinically measured head circumference and brain volume estimated from head CT-scans. Early Hum Dev 56: 17–29.CrossRefPubMedGoogle Scholar
  20. McCabe L, Ernest AE, Neifert MR, et al (1989) The management of breast feeding among infants with phenylketonuria. J Inherit Metab Dis 12: 467–474.CrossRefPubMedGoogle Scholar
  21. Przyrembel H (1996) Recommendations for protein and amino acid intake in phenylketonuria patients. Eur J Pediatr 155(Supplement 1): S130–S131.PubMedGoogle Scholar
  22. Räihä N, Minoli I, Moro G (1986) Milk protein intake in the term infant. I. Metabolic responses and effects on growth. Acta Paediatr Scand 75: 881–886.PubMedGoogle Scholar
  23. Schaeffer F, Burgard P, Bazler U, et al (1994) Growth and skeletal maturation in children with phenylketonuria. Act Paediatr 83: 534–541.Google Scholar
  24. 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
  25. van Spronsen FJ, Verkerk PH, van Houten M, et al (1997) Does impaired growth of PKU patients correlate with the strictness of dietary treatment? Acta Paediatr 86: 816–818.PubMedGoogle Scholar
  26. Verkerk PH, van Spronsen FJ, Smit GPA, Sengers RCA (1994) Impaired prenatal and postnatal growth in Dutch patients with phenylketonuria. Arch Dis Child 71: 114–118.PubMedGoogle Scholar
  27. Watemberg N, Silver S, Harel S, Lernam-Sagie T (2002) Significance of microcephaly among children with developmental disabilities. J Child Neurol 17: 117–122.PubMedGoogle Scholar
  28. Xiang M, Alfven G, Blennow M, Trygg M, Zetterstrom R (2000) Long-chain polyunsaturated fatty acids in human milk and brain growth during early infancy. Acta Paediatr 89: 142–147.PubMedGoogle Scholar

Copyright information

© SSIEM and Springer 2005

Authors and Affiliations

  • M. Hoeksma
    • 1
  • M. Van Rijn
    • 1
    • 2
  • P. H. Verkerk
    • 3
  • A. M. Bosch
    • 4
  • M. F. Mulder
    • 5
  • J. B. C. de Klerk
    • 6
  • T. J. De Koning
    • 7
  • E. Rubio-Gozalbo
    • 8
  • M. De Vries
    • 9
  • P. J. J. Sauer
    • 1
  • F. J. van Spronsen
    • 1
    • 10
  1. 1.Section of Metabolic Diseases, Beatrix Children's HospitalUniversity Medical Centre of Groningen, University of GroningenGroningenThe Netherlands
  2. 2.Department of DieteticsUniversity Medical Centre of Groningen, University of GroningenGroningenThe Netherlands
  3. 3.TNO Prevention and HealthLeidenThe Netherlands
  4. 4.Department of Paediatrics, Academic Medical CentreUniversity of AmsterdamAmsterdamThe Netherlands
  5. 5.Department of PaediatricsUniversity Hospital VUAmsterdamThe Netherlands
  6. 6.Department of Paediatrics, Division of Metabolic Diseases and GeneticsErasmus Medical Centre/Sophia Children's HospitalRotterdamThe Netherlands
  7. 7.Department of Metabolic DiseasesUniversity Medical Centre UtrechtUtrechtThe Netherlands
  8. 8.Department of PaediatricsUniversity Hospital MaastrichtMaastrichtThe Netherlands
  9. 9.Department of Metabolic DiseasesUniversity Medical Centre NijmegenNijmegenThe Netherlands
  10. 10.Beatrix Children's HospitalUniversity Medical Centre of GroningenGroningenThe Netherlands

Personalised recommendations