Wu Wei, Sheng Diying, Shao Jie, Zhao Zhengyan
Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou, Zhejiang, China.
J Paediatr Child Health. 2011 Jul;47(7):441-7. doi: 10.1111/j.1440-1754.2010.01996.x. Epub 2011 Feb 18.
This study was designed to evaluate mental and motor development, psychosocial adjustment (temperament and behavioural problems) in children with phenylketonuria (PKU).
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.
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.
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.
本研究旨在评估苯丙酮尿症(PKU)患儿的智力和运动发育、心理社会适应情况(气质和行为问题)。
采用贝利婴幼儿发育量表(BSID)、皮博迪发育运动量表(PDMS-II)、幼儿气质问卷(TTQ)和艾森克儿童行为量表(CBCL)对28名3至36个月大的早期治疗的PKU中国患儿进行评估。同时还探讨了相关因素,包括血苯丙氨酸(Phe)水平以及患儿家庭的社会经济地位(SES)。
与健康对照组相比,早期治疗的PKU患儿在智力和运动发育方面没有延迟。然而,他们在九个气质维度中的四个维度上表现出更高的水平(活动水平更高、反应更强烈、负面情绪更多、注意力更分散)。他们在抑郁、攻击和破坏行为方面也有更多的行为问题。较低的Phe水平和较高的患儿家庭SES对发育指标、气质特征和行为问题有积极影响。
早期治疗的PKU患儿在智力和运动发育方面正常。然而,他们在九个气质维度中的四个维度上表现出更高的水平,并且有更多的行为问题。建议对年幼的PKU患儿进行儿科管理并结合心理辅导。