Review: Emotional and behavioral functioning in phenylketonuria

Objective: To examine 17 studies of the psychological sequelae of early-tre ated phenylketonuria (PKU) with emphasis on the impact of dietary control o n functioning. Two questions are addressed: (1) What is the typical psychol ogical profile associated with PKU? (2) Is emotional and behavioral disturb ance more prevalent in PKU-affected individuals compared to appropriate con trols? Method: Computerized searches of PsycINFO(R) identified studies using behav ioral, personality, and diagnostic measures. Results: Findings converge upon a profile including attentional difficultie s, depression, anxiety, and low self-esteem. Methodological constraints lim it 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, addressi ng the factors and processes that may influence emotional and behavioral fu nctioning in this neurodevelopmental disorder. We outline potential lines o f new investigation that address critical methodological factors.