Reber M, Kazak A E, Himmelberg P
Department of Psychiatry, Medical College of Pennsylvania, Philadelphia 19129.
J Dev Behav Pediatr. 1987 Dec;8(6):311-7.
The association between effective metabolic control and patients' intelligence test performance and behavior in phenylketonuria (PKU) has been demonstrated frequently. The present study reexamined this relationship in a population of 41 young children with early-treated PKU, and added a dimension of family investigation to determine relationships between dietary phenylalanine control and patient functioning, family functioning and phenylalanine control, and family functioning and patient functioning. Significant correlations were found between concurrent phenylalanine control and patients' intelligence test scores, and lifetime phenylalanine control and patients' social competence. Parent-report measures of family psychological adjustment, stress, interaction, and socioeconomic status showed no significant association with children's dietary phenylalanine control. Family cohesion and adaptability correlated positively with patients' cognitive performance. Results support a policy of diet continuation in PKU, and suggest that family interaction patterns influence patient functioning. Longitudinal study of family factors in PKU is indicated.
有效代谢控制与苯丙酮尿症（PKU）患者智力测试表现及行为之间的关联已被多次证实。本研究在41名早期接受治疗的PKU幼儿群体中重新审视了这种关系，并增加了家庭调查维度，以确定饮食苯丙氨酸控制与患者功能、家庭功能与苯丙氨酸控制以及家庭功能与患者功能之间的关系。研究发现，同时期的苯丙氨酸控制与患者智力测试分数之间存在显著相关性，终生苯丙氨酸控制与患者社会能力之间也存在显著相关性。家长报告的家庭心理调适、压力、互动及社会经济地位指标与儿童饮食苯丙氨酸控制无显著关联。家庭凝聚力和适应性与患者认知表现呈正相关。研究结果支持PKU患者持续饮食控制的政策，并表明家庭互动模式会影响患者功能。有必要对PKU患者家庭因素进行纵向研究。
