Abstract
Even early-treated patients with phenylketonuria (PKU) have a higher risk of psychosocial maladjustment. This study was performed to determine whether emotional and behavioural problems are specific in phenylketonurics and whether they depend on the quality of biochemical control. This comparative study covered 42 PKU patients aged 10–18 years (mean 14.7 years) and 42 diabetic patients matched for sex, age and socioeconomic status. Patients' groups were compared with a control sample of healthy controls (n=2900) from an epidemiological study. We used the Child Behavior Check List (CBCL) according to Achenbach, intelligence quotient (IQ) test according to Weiss, and monitoring of blood phenylalanine concentrations and HBA1 concentrations. Internalizing problems such as depressive mood, anxiety, physical complaints or social isolation were significantly elevated in both phenylketonuric and diabetic patients, whereas externalizing problems were not. The two patient groups did not differ significantly either in the degree or in the pattern of their psychological profile. In both groups no significant correlations could be computed between the psychological characteristics and the biochemical control, the IQ, and the socioeconomic status. No patient was undergoing psychiatric treatment or psychotherapy. Our results strongly support a psychological perspective for the development of behavioural and emotional problems in both phenylketonuric and diabetic patients. Thus, medical treatment should be accompanied by psychological support for the families.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
REFERENCES
Achenbach TM (1991) Manual for the Child Behavior Check-List 4–18 and 1991 Profile (1991). Burlington, VT: University of Vermont, Department of Psychiatry.
Awiszus D, Unger I (1990) Co** with PKU: results of narrative interviews with parents. Eur J Pediatr 149 (supplement 1): 45–51.
Brown JS, Achenbach TM (1994) Bibliography of published studies using the Child Behavior Check List and related materials: 1994 Edition. Burlington, VT: University of Vermont, Department of Psychiatry.
Burgard P, Armbruster M, Schmidt E, Rupp A (1994) Psychopathology of patients treated early for phenylketonuria: results of the German collaborative study of phenylketonuria. Acta Paediatr 407 (supplement): 108–110.
Burgard P, Bremer HJ, Bührdel P, et al (1999) Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997. Eur J Pediatr 158: 46–54.
Döpfner M, Schmeck K, Bur W (1984) Handbuch: Elternfragebogen das V erhalten von über Kindern und Jugendlichen. Köln: KJFD.
Döpfner M, Schmeck K, Berner W, Lehmkuhl G, Poustka F (1994) Zur Reliabilität und faktoriellen Validität der Child Behavior Checklist-eine Analyse in einer klinischen und einer Feldstichprobe. Zeitschrift Kinder-und Jugendpsychiatrie 22: 189–205.
Eiser C (1990) Psychological Effects of chronic disease J Child Psychol Psychiatry 31: 85–98.
Esser G, Schmidt MH, Woerner W (1990) Epidemiology and course of psychiatric disorders in school-age children-results of a longitudinal study. J Child Psychol Psychiatry 31: 243–263.
Fisch OR, Sines LK, Chang PN (1981) Personality characteristics of nonretarded phenylketonurics and their family members. J Clin Psychiatry 42: 106–113.
Fölling A (1934) Ausscheidung von Phenylbrenztraubensäure in den Harn als Stoffwechselanomalie in Verbindung mit Imbezillität. Hoppe-Seylers Z Physiol Chem 227: 169–176.
Güttler F, Lou H (1986) Dietary problems of phenylketonuria: e.ect on transmitters and their possible role in behavior and neuropsychological function. J Inherit Metab Dis 9(supplement 2): 169–177.
Kazak AE, Reber M, Snitzer L (1988) Childhood chronic disease and family functioning. A study of phenylketonuriä. J Pediatr 81: 224–230.
Marholin D, Pohl RF, Stewart RM, Touchette PE, Townsend NM, Kolodny EH (1978) Effects of diet and behavior therapy on social and motor behavior of retarded phenylketonuric adults: an experimental analysis. Pediatr Res 12: 179–187.
MRC Working Party Phenylketonuria (1993) Recommendations on the dietary management of phenylketonuria.Arch Dis Child 68: 426–427.
Pietz J, Fätkenheuer B, Burgard P, Armbruster M, Esser G, Schmidt H (1997) Psychiatric disorders in adult patients with early-treated phenylketonuria.Pediatrics 99: 345–350.
Realmuto GM, GarFInkel BD, Tuchman M, et al (1986) Psychiatric diagnosis and behavioral characteristics of phenylketonuric children. J Nerv Ment Dis 174: 536–540.
Reber M, Kazak AE, Himmelberg P (1987) Phenylalanine control and family functioning in early treated phenylketonuria.Dev Behav Pediatr 8: 311–317.
St Vincent declaration (1998) Cited according to L. Thomas, ed. Labor und Diagnose. Frankfurt/Main: TH-Books Verlagsgesellschaft.
Smith I, Beasly M, Wol. DH, Ades EA (1988) Behaviour disturbance in 8-year-old children with early treated phenylketonuria.J Pediatr 112: 403–408.
Steinhausen HC, Winkler Metzke C, Meier M, Kannenbaerg R (1997) Behavioral and emotional problems reported by parents for ages 6 to 17 in a Swiss epidemiological study. Eur Child Adolesc Psychiatry 16: 136–141.
Stevenson JE, Hawcraft J, Lobascher M, Smith I, Wol. OH, Graham PJ (1987) Behavioral deviance in children with early treated phenylketonuria.Arch Dis Child 54: 14–18.
Verhulst FC, Achenbach TM (1995) Empirically based assessment and taxonomy of psychopathology: cross-cultural applications. A review. Eur Child Adolesc Psychiatry 4: 61–76.
Waisbren SE, Zaff J (1995) Personality disorders in young women with treated phenylketonuria. J Inherit Metab Dis 17: 584–592.
Waisbren SE, Schnell R, Levy HL (1984) Intelligence and personality characteristics in adults with untreated atypical phenylketonuria or mild hyperphenylalaninemia. J Pediatr 105: 955–958.
Weglage J, Fünders B, Ullrich K, Rupp A, Schmidt E (1996) Psychosocial aspects in phenylketonuria. Eur J Pediatr 155 (supplement 1): 101–104.
Weglage J, Fünders B, Wilken B, et al (1992) Psychological and social FIndings in adolescents with phenylketonuria.Eur J Pediatr 151: 522–525.
Weglage J, Rupp A, Schmidt E (1994) Personality characteristics in early treated phenylketonuria. Pediatr Res 35: 611–613.
Weglage J, Ullrich K, Pietsch M, Fünders B, Güttler F, Harms E (1997) Intellectual, neurologic, and neuropsychologic outcome in untreated subjects with nonphenylketonuria hyperphenylalaninemia. Pediatr Res 42: 378–384.
Weiss RH (1987) Grundintelligenztest Skala 2 CFT 20. Göttingen: Verlag für Psychologie, Dr CJ Hogrefe.
Wood AC, Friedmann CJ, Steisel IM (1967) Psychological factors in phenylketonuria.Am J Orthopsychiatry 37: 671–679.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Weglage, J., Grenzebach, M., Pietsch, M. et al. Behavioural and emotional problems in early-treated adolescents with phenylketonuria in comparison with diabetic patients and healthy controls. J Inherit Metab Dis 23, 487–496 (2000). https://doi.org/10.1023/A:1005664231017
Issue Date:
DOI: https://doi.org/10.1023/A:1005664231017