Division of Endocrinology, Diabetes, and Clinical Nutrition, University Hospital Zurich, Zurich, Switzerland.
Ann Nutr Metab. 2017;71(3-4):129-135. doi: 10.1159/000479746. Epub 2017 Aug 26.
Adult phenylketonuria (PKU) patients often reduce their intake of amino acid mixture (AAM) to less than the prescribed amounts. Effects of reduced AAM intake on nutrient supply were evaluated.
Nutrient intake was calculated in 20 adult PKU patients based on a structured food record and complemented by laboratory assessment of nutritional status. Patients were classified into 2 groups, (A) regular AAM intake, or (B) AAM intake below calculated requirements.
Group B consumed a higher proportion of natural protein (60 ± 23 vs. 33 ± 12%, p = 0.002); however, the total protein intake was below the recommended amounts in 60% of patients in group B versus 7% in group A (p = 0.03). Fat intake was higher in group B (39 ± 9% of energy vs. 31 ± 6%, p = 0.03), mainly from saturated fats. Selenium, folate, and vitamin B12 intake was below the recommended intake in group B. However, serum concentrations of these analytes remained within the normal range in both groups, although vitamin B12 levels were lower in group B. Plasma tyrosine correlated with AAM intake, and hydroxyproline correlated with the amount of natural protein consumed.
Relaxed AAM intake resulted in insufficient nutrient supply, despite a compensatory increase in consumption of natural protein. Care needs to be taken to ensure adequate nutrition in adults with PKU.
成人苯丙酮尿症（PKU）患者常减少氨基酸混合物（AAM）的摄入量，低于规定量。评估减少 AAM 摄入对营养供应的影响。
根据结构化食物记录计算 20 名成年 PKU 患者的营养摄入量，并通过实验室评估营养状况进行补充。患者分为 2 组，（A）常规 AAM 摄入，或（B）AAM 摄入量低于计算需求。
B 组摄入了更高比例的天然蛋白质（60 ± 23 对 33 ± 12%，p = 0.002）；然而，B 组 60%的患者总蛋白质摄入量低于推荐量，而 A 组仅为 7%（p = 0.03）。B 组脂肪摄入量较高（占能量的 39 ± 9%对 31 ± 6%，p = 0.03），主要来自饱和脂肪。B 组硒、叶酸和维生素 B12 摄入量低于推荐量。然而，两组血清分析物浓度均在正常范围内，尽管 B 组维生素 B12 水平较低。血浆酪氨酸与 AAM 摄入量相关，羟脯氨酸与天然蛋白质的摄入量相关。
尽管天然蛋白质的摄入量增加，但放松 AAM 摄入会导致营养供应不足。需要注意确保 PKU 成人的充足营养。
