Skip to main content
Log in

Personality disorder in young women with treated phenylketonuria

  • Published:
Journal of Inherited Metabolic Disease

Summary

Twenty-eight young women with phenylketonuria (PKU) attending a Maternal PKU Summer Camp were interviewed and administered a personality inventory, the Minnesota Multiphasic Personality Inventory (MMPI). The 12 young women who were either late-treated (treatment initiated after 90 days) or who had terminated the diet for a period of at least 5 years (the extended exposure group) were compared to the 16 women who were early-treated and had remained continuously on diet (the continuously treated group). Although the mean blood phenylalanine and tyrosine concentrations at the camp for the two groups were comparable (973±344 and 1033±284 µmol/L for phenylalanine and 43±16 and 40±25 µmol/L for tyrosine), the women in the extended exposure group evidenced significantly greater psychopathology as measured by the MMPI and self-report; thought disorder and mood disturbances were associated with diet termination in PKU.

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

  • Berry HK, O'Grady DJ, Perlmutter LJ, Bofinger MK (1979) Intellectual development and academic achievement of children treated early for phenylketonuria.Dev Med Child Neurol 21: 311–320.

    Google Scholar 

  • Bickel H, Grubel-Kaiser S (1982) Inborn errors of metabolism — consequences of long-term treatment for the individual, as derived from observations in phenylketonuria. In Cockburn F, Gitzelmann R, eds.Inborn Errors of Metabolism in Humans. Lancaster: MTP Press.

    Google Scholar 

  • Dobson JC, Kushida E, Williamson M (1976) Intellectual performance of 36 phenylketonuric patients and their nonaffected siblings.Pediatrics 58: 53–58.

    Google Scholar 

  • Exner JE (1974)The Rorschach. A Comprehensive System, vol. 1. New York: Wiley.

    Google Scholar 

  • Fisch RO, Sines LK, Chang P (1981) Personality characteristics of nonretarded phenylketonurics and their family members.J Clin Psychiatry 42: 106–113.

    Google Scholar 

  • Fishler K, Azen CG, Henderson R, Friedman EG, Koch R (1987) Psychoeducational findings among children treated for phenylketonuria.Am J Ment Defic 92: 65–73.

    Google Scholar 

  • Greene RL (1991)Clinical scales. MMPI-2/MMPI: An Interpretive Manual. Needham, MA: Allyn and Bacon.

    Google Scholar 

  • Hathaway SR, McKinley JC (1940) A multiphasic personality schedule (Minnesota): III.J Psych 10: 249–254.

    Google Scholar 

  • Holtzman NA, Kronmal RA, Van Doorninck W, Azen C, Koch R (1986) Effect of age at loss of dietary control on intellectual performance and behavior of children with phenylketonuria.N Engl J Med 314: 593–598.

    Google Scholar 

  • Jervis GA (1937) Phenylpyruvic oligophrenia.Arch Neurol Psychiatr 38: 944.

    Google Scholar 

  • Koch R, Azen C, Friedman EG, Williamson ML (1984) Paired comparisons between early treated PKU children and their matched sibling controls on intelligence and school achievement test results at eight years of age.J Inher Metab Dis 7: 86–90.

    Google Scholar 

  • Koff E, Boyle P, Pueschel S (1977) Perceptual-motor functioning in children with phenylketonuria.Am J Dis Child 131: 1084–1087.

    Google Scholar 

  • Levy HL, Shih VE, Kraolkewicz V et al (1971) Persistent mild hyperphenylalaninemia in the untreated state. A prospective study.N Engl J Med 285: 424–429.

    Google Scholar 

  • MacCready RA (1974) Admissions of phenylketonuric patients to residential institutions before and after screening programs of the newborn infant.J Pediatr 85: 383–385.

    Google Scholar 

  • Murawski BJ, Chazan BI, Balodimos MC, Ryan JR (1970) Personality patterns in patients with diabetes mellitus of long duration.Diabetes 19: 259–263.

    Google Scholar 

  • Pennington BF, Van Doorninck WJ, McCabe LL, McCabe ER (1985) Neuropsychological deficits in early treated phenylketonuric children.Am J Ment Defic 89: 467–474.

    Google Scholar 

  • Realmuto GM, Garfinkel BD, Tuchman M et al (1986) Psychiatric diagnosis and behavioral characteristics of phenylketonuric children.J Nerv Ment Dis 174: 536–540.

    Google Scholar 

  • Rorschach H (1984)Psychodiagnostics. New York: Grune and Stratton.

    Google Scholar 

  • Schuett VE, Brown ES, Michals K (1985) Reinstitution of diet therapy in PKU patients from twenty-two US cities.Am J Pub Health 75: 39–42.

    Google Scholar 

  • Scriver C (1989) Genetic counseling for phenylketonuria.J Pediatr 114: 683–684.

    Google Scholar 

  • Smith I, Beasley MG, Wolff OH, Ades AE (1988) Behavior disturbances in 8-year-old children with early treated phenylketonuria.J Pediatr 112: 403–408.

    Google Scholar 

  • Sonneville LMJ de, Schmidt E, Michel U, Batzler U (1990) Preliminary neuropsychological test results.Eur J Pediatr 149: S39–S44.

    Google Scholar 

  • Stevenson JE, Hawcroft J, Lobascher M, Smith I, Wolff OH, Graham PJ (1979) Behavioral deviance in children with early treated phenylketonuria.Arch Dis Child 54: 14–18.

    Google Scholar 

  • Waisbren SE, Levy HL (1991) Agoraphobia in phenylketonuria.J Inher Metab Dis 14: 755–764.

    Google Scholar 

  • Waisbren SE, Schnell RR, Levy HL (1980) Diet termination in children with phenylketonuria: A review of psychological assessments to determine outcome.J Inher Metab Dis 3: 149–153.

    Google Scholar 

  • Waisbren SE, Mahon B, Schnell RR, Levy HL (1987) Predictors of intelligence quotient and intelligence quotient change in persons treated for phenylketonuria early in life.Pediatrics 79: 351–355.

    Google Scholar 

  • Walsh MC, Pennington BC, Ozonoff S, Rouse B, McCabe ERB (1990) Neuropsychology of early-treated phenylketonuria: Specific executive function deficits.Child Dev 61: 1697–1713.

    Google Scholar 

  • Williamson ML, Koch R, Azen C, Chang C (1981) Correlates of intelligence test results in treated phenylketonuric children.Pediatrics 68: 161–167.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Waisbren, S.E., Zaff, J. Personality disorder in young women with treated phenylketonuria. J Inherit Metab Dis 17, 584–592 (1994). https://doi.org/10.1007/BF00711596

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00711596

Keywords

Navigation