Skip to main content
PMC home page
American Journal of Public Health logoLink to American Journal of Public Health
. 1985 Jan;75(1):39–42. doi: 10.2105/ajph.75.1.39

Reinstitution of diet therapy in PKU patients from twenty-two US clinics.

V E Schuett, E S Brown, K Michals
PMCID: PMC1646129  PMID: 3966596

Abstract

In a nationwide survey we found 72 PKU (phenylketonuria) patients who had terminated diet but later returned to diet. Sixty-one patients resumed diet due to clinical problems. Age at initial diet discontinuation ranged from three to 20 years. The most prevalent problems reported were poor school performance, and mood and/or behavior changes. Following diet reinstitution, only positive changes were noted for 42 patients, no changes for 19 patients, and 11 had one or more negative changes. Improvements and blood phenylalanine levels were not significantly correlated, but only 11 patients maintained levels less than 10 mg/dl. The number of improvements was significantly correlated with length of time on diet (p less than 0.001). After a median of 10 months on diet, 22 patients had again discontinued due to poor diet control, lack of motivation, poor formula tolerance, lack of apparent benefits and/or changes for the worse. Median time on diet for the 50 second-time continuers was two years nine months.

Full text

PDF
39

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bickel H., Kaiser-Grubel S. Uber die Phenylketonurie. Psychometrische Erfolgsbeuteilung der phenylalaninarmen Diät bei phenylketonurischen Kindern. Dtsch Med Wochenschr. 1971 Sep 3;96(36):1415–1423. doi: 10.1055/s-0028-1110153. [DOI] [PubMed] [Google Scholar]
  2. Brown E. S., Warner R. Mental development of phenylketonuric children on or off diet after the age of six. Psychol Med. 1976 May;6(2):287–296. doi: 10.1017/s0033291700013842. [DOI] [PubMed] [Google Scholar]
  3. Cabalska B., Duczyńska N., Borzymowska J., Zorska K., Koślacz-Folga A., Bozkowa K. Termination of dietary treatment in phenylketonuria. Eur J Pediatr. 1977 Nov 4;126(4):253–262. doi: 10.1007/BF00477051. [DOI] [PubMed] [Google Scholar]
  4. Koch R., Azen C. G., Friedman E. G., Williamson M. L. Preliminary report on the effects of diet discontinuation in PKU. J Pediatr. 1982 Jun;100(6):870–875. doi: 10.1016/s0022-3476(82)80503-9. [DOI] [PubMed] [Google Scholar]
  5. Lenke R. R., Levy H. L. Maternal phenylketonuria and hyperphenylalaninemia. An international survey of the outcome of untreated and treated pregnancies. N Engl J Med. 1980 Nov 20;303(21):1202–1208. doi: 10.1056/NEJM198011203032104. [DOI] [PubMed] [Google Scholar]
  6. Murphy D. Termination of dietary treatment of phenylketonuria. Ir J Med Sci. 1969 Apr;8(4):177–183. doi: 10.1007/BF02954680. [DOI] [PubMed] [Google Scholar]
  7. Schuett V. E., Brown E. S. Diet policies of PKU clinics in the United States. Am J Public Health. 1984 May;74(5):501–503. doi: 10.2105/ajph.74.5.501. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Schuett V. E., Gurda R. F., Brown E. S. Diet discontinuation policies and practices of PKU clinics in the United States. Am J Public Health. 1980 May;70(5):498–503. doi: 10.2105/ajph.70.5.498. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Smith I., Lobascher M. E., Stevenson J. E., Wolff O. H., Schmidt H., Grubel-Kaiser S., Bickel H. Effect of stopping low-phenylalanine diet on intellectual progress of children with phenylketonuria. Br Med J. 1978 Sep 9;2(6139):723–726. doi: 10.1136/bmj.2.6139.723. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Williamson M., Koch R., Berlow S. Diet discontinuation in phenylketonuria. Pediatrics. 1979 May;63(5):823–824. [PubMed] [Google Scholar]
  11. Woolf L. I. Late onset phenylalanine intoxication. J Inherit Metab Dis. 1980;2(1):19–20. doi: 10.1007/BF01805558. [DOI] [PubMed] [Google Scholar]

Articles from American Journal of Public Health are provided here courtesy of American Public Health Association

RESOURCES