Review: emotional and behavioral functioning in phenylketonuria.
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?


METHODS
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.
Sullivan
J E
JE
University of Minnesota, USA. jsulivan@enteract.com
Chang
P
P
D002648:Child; D002653:Child Behavior Disorders / Q000209:etiology*; D002908:Chronic Disease; D017281:Cost of Illness; D006801:Humans; D010553:Personality Development; D010661:Phenylketonurias / Q000150:complications / Q000178:diet therapy / Q000523:psychology*