Journal of Pediatric Psychiatry, Vol 24, 281-299, Copyright © 1999 by Society of Pediatric Psychology
JE Sullivan and P Chang
OBJECTIVE: To examine 17 studies of the psychological sequelae of early-
treated phenylketonuria (PKU) with emphasis on the impact of dietary
control on functioning. Two questions are addressed: (1) What is the
typical psychological profile associated with PKU? (2) Is emotional and
behavioral disturbance more prevalent in PKU-affected individuals compared
to appropriate controls? METHOD: Computerized searches of PsycINFO
identified studies using behavioral, personality, and diagnostic measures.
RESULTS: Findings converge upon a profile including attentional
difficulties, depression, anxiety, and low self- esteem. Methodological
constraints limit conclusions regarding the nature and severity of observed
difficulties. A single study has used comparison groups appropriate for the
simultaneous examination of the questions posed (Waisbren and Levy, 1991).
CONCLUSIONS: We discuss results using a biopsychosocial framework,
addressing the factors and processes that may influence emotional and
behavioral functioning in this neurodevelopmental disorder. We outline
potential lines of new investigation that address critical methodological
factors.
ARTICLE
Review: emotional and behavioral functioning in phenylketonuria
University of Minnesota, USA. jsulivan@enteract.com
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