Skip to main content
Log in

Iatrogenic and transient hyperglycinemia in patients with phenylketonuria

  • Original Investigations
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Two patients with phenylketonuria detected by newborn screening for inborn errors of metabolism were treated with low phenylanine formulae.

Serum phenylalanine levels were well controlled, but serum glycine levels were elevated until 4 or 5 months of age. This was probable due to the high content of glycine in the formulae. Glycine level returned to be normal in these patients, even though they were kept on the same formula, suggesting, immature metabolism of glycine during this period.

No clinical problems were encountered in either patient.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Bickel, H., Gerrard, J., Hickman, E. M.: Influence of phenylalanine intake on phenylketonuria. Lancet 1953 II, 812–813

    Google Scholar 

  • De Groot, G. J., Boelieverts, V., Touwen, B. C. L., Hommes, F. A.: Nonketotic hyperglycinemia (NKH): An inborn error of metabolism affecting brain function exclusively. In Maturation of the nervous system. Progress in Brain Research, Vol. 48 (ed. by M. A. Corner et al.), pp. 199–205. North Holland Biomedical Press: Elsevier 1978

    Google Scholar 

  • Gerritsen, T., Kaveggia, E., Waisman, H. A.: A new type of idiopathic hyperglycinemia with hypo-oxaluria. Pediatrics 36, 882–891 (1964)

    Google Scholar 

  • Harper, A. E., Benevenga, N. J., Wohlhuetter, R. M.: Effect of ingestion of disproportionate amounts of amino acids. Physiol. Rev. 50, 428–558 (1970)

    Google Scholar 

  • Mamus, P., Prince, P. E., Thornton, N. H., Hunt, P., Hitchock, E. S.: Intellectual deficits after transient tyrosinemia in the term neonate. Pediatrics 57, 675–680 (1976)

    Google Scholar 

  • Menkes, J. H., Welcher, D. W., Levis, H. S., Dallas, J., Gretsky, N. E.: Relationship of elevated blood tyrosine to the ultimate intellectual performance of premature infants. Pediatrics 49, 218–224 (1972)

    Google Scholar 

  • Nyhan, W. L.: Nonketotic hyperglycinemia. In: Hereditable disorders of amino acid metabolism (ed. by Nyhan, W. L.), pp. 309–323. New York: John Wiley & Sons 1974

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

This investigation was supported by a Research grant by the Ministry of Health and Welfare of Japan 1978

Rights and permissions

Reprints and permissions

About this article

Cite this article

Nagata, N., Shinozuka, S., Matsuda, I. et al. Iatrogenic and transient hyperglycinemia in patients with phenylketonuria. Eur J Pediatr 132, 17–20 (1979). https://doi.org/10.1007/BF00443200

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00443200

Key words

Navigation