Schulz B, Bremer H J
Department of General Paediatrics, University Children's Hospital, Heidelberg, Germany.
Acta Paediatr. 1995 Jul;84(7):743-8. doi: 10.1111/j.1651-2227.1995.tb13748.x.
Food and nutrient intake was assessed in 99 PKU patients (12-29 years old) by two food protocols (7 days and 4 days, respectively). Ninety-three patients completed at least one 7-day food record and 83 both records. Nineteen of 93 patients had already stopped taking the phenylalanine-free amino acid mixture (AAM), which is enriched with vitamins, minerals and trace elements. Plasma phenylalanine levels in this group were significantly higher than in patients who were still taking the AAM. Even without the AAM, protein intake still met the recommendations, but thiamin, riboflavin, folate, calcium and iron levels were below 80% of the US RDA in most patients. For those still taking the AAM, calorie, protein, vitamin and mineral intakes were above the recommendations. The diet was characterized by a low intake of fiber (median 14 (range 8-35) g/day), fat (27 (10-47) cal%) and cholesterol (75 (13-417) mg/day) as well as a high ratio of polyunsaturated/saturated fatty acids (0.7 (0.2-2.4)). Problems with dietary compliance in adolescents and young adults may lead to a combination of marginal nutrient intake and high phenylalanine levels.
通过两种食物记录方案（分别为7天和4天）对99名苯丙酮尿症患者（12 - 29岁）的食物和营养摄入情况进行了评估。93名患者完成了至少一份7天的食物记录，83名患者完成了两份记录。93名患者中有19名已经停止服用不含苯丙氨酸的氨基酸混合物（AAM），该混合物富含维生素、矿物质和微量元素。该组患者的血浆苯丙氨酸水平显著高于仍在服用AAM的患者。即使不服用AAM，蛋白质摄入量仍符合推荐标准，但大多数患者的硫胺素、核黄素、叶酸、钙和铁水平低于美国推荐膳食摄入量（RDA）的80%。对于仍在服用AAM的患者，热量、蛋白质、维生素和矿物质摄入量高于推荐标准。该饮食的特点是纤维（中位数14（范围8 - 35）克/天）、脂肪（27（10 - 47）千卡%）和胆固醇（75（13 - 417）毫克/天）摄入量低，以及多不饱和/饱和脂肪酸比例高（0.7（0.2 - 2.4））。青少年和青年成人的饮食依从性问题可能导致营养素摄入量边缘性不足和苯丙氨酸水平升高的综合情况。
