Abstract: Objective To investigate the influence of feeding behavior of parents on the blood phenylalanine (Phe) concentration and deterioration quotient (DQ) of children with phenylketonuria(PKU). Methods Totally 49 cases of classic PKU children and their long-term following parents were chosen,who were diagnosed by newborn screening center of Taian between January 2000 to May 2010 and continued insurance treatment for six years(28 boys,21 girls;parents:father 9 cases and mother 40 cases).The dietary feeding behavior of parents was investigated by a self-made questionnaire and analyzed by single factor analysis of variance and multiple stepwise regression analysis. Results The multiple stepwise regression analysis of 26 items were carried out,high protein diet,tend to high protein diet behavior,food preparation(food pattern,whether parence cook dinner for the children specially),had significant influence on the blood Phe concentration and DQ (P＜0.05). Conclusion The feeding behaviors of parents of classic PKU children have significant influence on the curative effect of children.

Key words: classic phenylketonuria children, feeding behavior, deterioration quotient, phenylalanine concentration

摘要： 目的 研究经典苯丙酮尿症(PKU)患儿家长饮食喂养行为对患儿血苯丙氨酸(Phe)浓度和智能发育(DQ)的影响。方法 选择2000年1月-2010年5月在泰安市新生儿疾病筛查中心确诊并持续保偿治疗6年的经典PKU患儿及其长期随访家长49例,其中患儿:男28例,女21例;家长,父亲9例,母亲40例。家长饮食喂养行为采用自制问卷进行调查,统计分析采用单因素方差分析和多元逐步回归分析。结果 26项分布各异的患儿家长饮食喂养行为,经多元逐步回归结果显示:高蛋白饮食喂养行为、趋向高蛋白饮食喂养行为、单独做饭、饭菜种类对患儿血Phe浓度、DQ的影响有统计学意义(P值均＜0.05)。结论 家长饮食喂养行为对经典PKU患儿的治疗效果影响显著。

关键词: 经典苯丙酮尿症患儿, 喂养行为, 智能发育, 苯丙氨酸浓度