【摘要】 目的:了解低苯丙氨酸(phe)饮食疗法对苯丙酮尿症(PKU)患儿蛋白质营养状况的影响。方法:采用氨基酸分析仪对18例PKU患儿治疗前后的血清必须氨基酸水平进行测定。结果:PKU患儿血phe浓度治疗前为(1580.9±545.4)μmol/L,治疗后为(326.5±178.6)μmol/L,治疗后较治疗前明显降低(P<0.01),除phe外的其他必需氨基酸浓度,治疗后较治疗前明显提高(P<0.05或P<0.01);体格和智能发育满意。结论:低phe饮食疗法可以改善PKU患儿的蛋白质营养状况。更多还原

【Abstract】 Objective:To know the effect of low phenylalanine diet on protein status of patients with phenylalanineia(PKU).Methods:The serum amino acids levels of 18 patients with PKU before and after treatment were determined by using the amino acid analyzer.Results:Serum phenylalanine level after treatment (326.5±178.6) μmol/L was significant lower than that before treatment (1 580.9±545.4)μmol/L,P<0.01,while the other necessary amino acids levels greatly increased(P<0.05 or P<0.01). The physical and intellectual development of patients were nearly normal.Conclusion:Low phenylalanine diet treatment may improve the protein status in nutrition.更多还原