Mental and motor development and psychosocial adjustment of Chinese children with phenylketonuria.

摘要

AIM: This study was designed to evaluate mental and motor development, psychosocial adjustment (temperament and behavioural problems) in children with phenylketonuria (PKU). METHODS: Twenty-eight Chinese children aged from three to 36 months with early-treated PKU were evaluated by Bayley Scales of Infant Development (BSID), Peabody Developmental Motor Scale (PDMS-II), Toddler Temperament Questionnaire (TTQ) and Achenbach's Child Behavior Checklist (CBCL). The related factors, including blood phenylalanine (Phe) levels, and the socio-economic status (SES) of the children's family were also explored. RESULTS: In comparison with healthy control, early-treated PKU children showed no delay in the mental and motor development. However, they presented higher levels on four of the nine temperamental dimensions (higher level in activity, more intense in reaction, more negative mood and more distraction). They also had more behavioural problems in depression, aggression and destructibility. A lower Phe level and a higher SES of children's family showed a positive effect on development index, temperamental characteristics and behavioural problems. CONCLUSION: Early-treated PKU children were normal in the mental and motor development. However, they presented higher level on four of the nine temperamental dimensions and had more behavioural problems. Paediatric control combined with psycho-counselling was suggested for young PKU children.