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苯丙酮尿症（ＰＫＵ）是最常见的先天性代谢病之一，从１９８４年１１月至１９９３年４月收治ＰＫＵ患儿１２０例，其中６１例用无苯丙氨酸（ＰＡ）奶粉和低ＰＡ奶粉加低ＰＡ饮食疗法为奶粉治疗组，５９例用单纯饮食疗法为非奶粉组，年龄分为０－１月、１－３月、３－６月、６－１岁，１－２岁、＞２岁。两组同时做智商和脑电图（ＥＥＧ），奶粉治疗组０－３组治疗者智商及ＥＥＧ均正常，３－６月智商在正常低限，＞６月者低于正常，但有少数坚持合理治疗者，智商有的达正常水平。说明对于ＰＫＵ患儿的早期诊断和早期治疗是十分必要的，两组疗效比较有显著差异（Ｐ＜０．０５），两组ＥＥＧ异常发生率分别为５２．４６％，６０．１％差异显著（ｔ＝３．１８７６６，Ｐ＜０．０１），对于６月以下患儿用特殊奶粉加低ＰＡ饮食大大低于单纯饮食疗法，强调用无或低ＰＡ奶粉治疗是防止智力低下，改善预后的重要手段。
Phenylketonuria (PKU) is one of the most common forms of congenital metabolic disease, and 120 patients with PKU were admitted from November 1984 to April 1993, of which 61 were treated with phenylalanine (PA) milk powder and low PA milk powder plus low PA diet therapy for the milk powder treatment group, 59 patients with simple diet therapy for non-milk powder group, age is 0-1, 1-3 months, 3-6 months, 6-1 years old, 1-2 years> 2 years old. IQ and EEG were done in both groups at the same time. The IQ and EEG of 0-3 group were normal in the milk powder treatment group. IQ was lower than normal in 3-6 months, but lower than normal in> 6 months, but a few persisted Reasonable treatment, some IQ up to normal levels. It is very necessary for the early diagnosis and early treatment of children with PKU. There are significant differences between the two groups (P <0.05). The incidence of EEG abnormalities in both groups were 52.46% and 60.1%, respectively (T = 3.18766, P <0.01), for children with less than 6 months with special milk with low PA diet significantly lower than simple diet therapy, emphasizing the use of low or no PA milk powder treatment is to prevent mental retardation and improve prognosis means.