目的 研究低苯丙氨酸饮食治疗的苯丙酮尿症儿童饮食治疗过程中蛋白质、脂类的营养状况,为寻求最佳治疗方案提供依据.方法 选取1997年12月-2005年6月经青岛市新生儿疾病筛查中心确诊为苯丙酮尿症的患儿46例作为病例组,选取性别、年龄与病例组匹配、无任何躯体及精神疾病的正常儿童61例作为对照组,对所有研究对象进行血生物化学指标检测和血氨基酸分析.结果 ①两组儿童血总蛋白、白蛋白含量的差异有统计学意义(t值分别为0.989、0.093,均P<0.05),血清球蛋白含量的差异有高度统计学意义(t=1.041,均P<0.01);②两组儿童血清天冬氨酸、甲硫氨酸、苯丙氨酸、精氨酸、瓜氨酸、甘氨酸、鸟氨酸含量的差异均有高度统计学意义(t值分别为3.314、2.006、12.725、9.892、6.669、5.867、2.713,均P<0.01).结论 苯丙酮尿症儿童治疗过程中蛋白质、脂类、氨基酸虽在正常范围之内,但仍低于正常儿童.定期对低苯丙氨酸饮食治疗的苯丙酮尿症儿童进行蛋白质、脂类的监测,及时发现并纠正苯丙酮尿症儿童存在的蛋白质、脂类的营养偏差,以寻求苯丙酮尿症儿童最佳的饮食治疗方案.%Objective To analyze the nutritional condition of protein and lipids in dietotherapy for children with phenylketonuria ( PKU ), so as to provide basis for best treatment. Methods 46 children ( case group ) with PKU diagnosed with neonatal screening in Qingdao from December 1997 to June 2005 were studied and compared to 61 matched healthy children ( control group ) bom at the same time. Nutritional state of the children was evaluated on blood biochemical parameters and analysis of amino acids in serum respectively. Results There were differences in serum total protein, serum albumin ( t＝ 0. 989,0. 093 respectively, P ＜ 0.05 ) and significant differences in serum globulin (t＝ 1. 041, P ＜ 0. 01 ) between two groups. There were significant differences in blood aspartic acid, methionine, phenylalanine, arginine, citrulline, glycine, omithine between children with PKU and the controls ( t＝ 3. 314, 2. 006, 12. 725, 9. 892, 6. 669, 5. 867, 2. 713 respectively, P ＜ 0.01 ). Conclusion Protein, amino acids and lipids nutrient are deficient in children with PKU treated with dietotherapy although there are in normal range. Timely identification and correction of the nutrient deficiencies in children with PKU are important. The best dietotherapy program is needed for children with PKU.
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