目的 :探讨河南地区苯丙酮尿症 (PKU)的发病率及治疗效果 ;方法 :非选择的新生儿 46778例 ,出生后 72小时充分哺乳后足跟采血 ,应用细菌抑制法测血标本中苯丙氨酸 (Phe)含量 ,凡确诊病人立即服用低Phe奶粉及食物并定期进行体格发育、智力发育及Phe含量监测。结果 :46778例新生儿中PKU病人 4例 ,发病率 1/ 11695与我国其它地区的发病率相比偏高。结论 :河南地区PKU发病率为 1/ 11695 ,略高于我国总的发病水平 ,早期治疗效果满意 Objective: To investigate the incidence and therapeutic effect of phenylketonuria (PKU) in Henan Province.Methods: 46778 non-selected newborns were collected from the heel after full lactation 72 hours after birth. Blood samples were tested for bacterial propofol (Phe) content, where the diagnosis of patients taking low Phe milk powder and food and regular physical development, mental development and Phe content monitoring. Results: There were 4 cases of PKU in 46778 newborns, the incidence was 1/11695, which was higher than other regions in China. Conclusion: The incidence of PKU in Henan Province is 1/11695, which is slightly higher than the total incidence of PKU in our country. The results of early treatment are satisfactory