Hospital for Children and Adolescents, Centre of Paediatric Research (CPL), Department of Women and Child Health, University Hospitals, University of Leipzig, Leipzig, Germany.
Department of Pediatrics, University Hospital, University of Münster, Münster, Germany.
Eur J Clin Nutr. 2014 Jan;68(1):119-24. doi: 10.1038/ejcn.2013.218. Epub 2013 Nov 20.
To investigate micronutrient supply in phenylketonuria (PKU) patients on a relaxed diet.
SUBJECTS/METHODS: Sixty-seven patients (6-45 years) with a phenylalanine tolerance ≥ 600 mg/day were included in the study. From a 3-day diet record, protein supply as well as consumption of essential amino acids and several micronutrients were assessed and compared with the current recommendations and data for the healthy population.
Protein supply and consumption of all essential amino acids were sufficient in all patients. Supply of micronutrients depended on dietary regime. Patients with a total protein supply of 120% or more of the recommended amount and at least 0.5 g protein per kg body weight from amino-acid mixture (AAM) were sufficiently supplied with all investigated micronutrients. All patients without AAM supplement showed severe micronutrient deficiencies in their diet records.
PKU patients under a relaxed diet are at risk of an insufficient nutrient supply, if they have first no substitution with AAM, second a protein supply less than 0.5 g per kg body weight from AAM or third a total protein supply less than 120% of the recommendations. Therefore, close monitoring, specific dietary counseling and potential supplementation is mandatory to prevent micronutrient deficiencies in PKU patients.
研究宽松饮食条件下苯丙酮尿症（PKU）患者的微量营养素供应情况。
对象/方法：本研究纳入了 67 名（6-45 岁）苯丙氨酸耐受量≥600mg/天的患者。通过 3 天的饮食记录，评估了蛋白质供应以及必需氨基酸和几种微量营养素的摄入量，并与当前推荐的摄入量和健康人群的数据进行了比较。
所有患者的蛋白质供应和所有必需氨基酸的摄入量均充足。微量营养素的供应取决于饮食方案。总蛋白质供应量达到推荐量的 120%或以上，且从氨基酸混合物（AAM）中摄入至少 0.5g/kg 体重蛋白质的患者，所有调查的微量营养素均供应充足。所有未补充 AAM 的患者的饮食记录中均显示严重的微量营养素缺乏。
在宽松饮食条件下，PKU 患者如果没有首先用 AAM 替代，其次 AAM 提供的蛋白质摄入量低于 0.5g/kg 体重，或者总蛋白质供应量低于推荐量的 120%，则存在营养物质供应不足的风险。因此，为了预防 PKU 患者的微量营养素缺乏，必须进行密切监测、特殊的饮食咨询和潜在的补充。
