目的：探究肠内营养粉剂在1 岁以下苯丙酮尿症患儿中的应用效果。方法：120 例年龄＜1 岁的苯丙酮尿症患儿持续32

周喂养肠内营养粉剂，20 g/kg，qd。比较治疗前、治疗8 周、治疗32 周时患儿的苯丙氨酸水平、身高、体质量、头围、智商水平、各项

营养指标变化和不良反应情况。结果：治疗8、32 周时，患儿的苯丙氨酸水平较治疗前逐渐降低，身高、体质量、头围较治疗前逐渐

增长，差异有统计学意义（P＜0.05）。患儿的智商水平治疗8 周时优于治疗前，治疗32 周时优于治疗8 周时，差异均具有统计学意

义（P＜0.05）。治疗8、32 周时，患儿各项营养指标均较治疗前显著改善，且治疗32 周时患儿的丙氨酸转氨酶（ALT）、白蛋白（Alb）

和血清总蛋白（TP）水平显著优于治疗8 周时，差异有统计学意义（P＜0.05）。治疗32 周时，患儿的尿素氮（BUN）、总胆红素

（TBil）、前白蛋白（Pa）水平与治疗8 周时比较差异均无统计学意义（P＞0.05）。有66 例患儿在治疗中发生181 次不良反应，主要为

10 次腹泻和126 次上呼吸道感染，其中1 例患儿出现中度胃肠炎，经对症处理后均恢复正常。结论：给予1 岁以下苯丙酮尿症患儿

肠内营养粉剂，能够有效控制患儿的苯丙氨酸水平，避免其智商水平的下降，改善其营养状况，进而满足患儿正常发育生长的营养

需求，且具有较好的安全性和耐受性。

OBJECTIVE：To explore the effects of enteral nutrition powder in children with phenylketonuria（PKU）under one

year. METHODS：120 PKU children under one year were selected and given enteral nutrition powder（20 g/kg，qd）for consecutive

32 weeks. The level of benzene，height，weight，head circumference，IQ level，nutrition indicators and ADR were compared before

treatment，8 weeks after treatment，32 weeks after treatment. RESULTS：8 and 32 weeks after treatment，the level of benzene

decreased gradually，and height，weight and head circumference increased gradually，with statistical significance（P＜0.05）. IQ level

of children after 8 weeks treatment were better than before，and those of children after 32 weeks treatment were better than after

8 weeks treatment，with statistical significance（P＜0.05）. 8 and 32 weeks after treatment，nutrition indicators were improved significantly，

compared with before treatment；ALT，Alb and TP levels 32 weeks after treatment were significantly better than 8

weeks after treatment，with statistical significance（P＜0.05）.There was no statistically significant difference in BUN，TBil and Pa

levels between 32 weeks after treatment and 8 weeks after treatment（P＞0.05）. 66 cases suffered from 181 times of ADR，mainly

including 10 times diarrhea and 126 times upper respiratory tract infection，among which one children developed moderate gastroenteritis.

They were all recovered after symptomatic treatment. CONCLUSIONS：For PKU children under one year，enteral nutrition

powder can effectively control the phenylalanine levels，prevent the decrease of IQ and improve the nutritional status so as to meet

the normal growth and development of children nutritional requirements，with better safety and tolerance.