Centre de référence des maladies héréditaires du métabolisme de Nancy, INSERM U 954, Hôpital d'Enfants, CHU Brabois, Allée du Morvan, Vandoeuvre les Nancy 54500, France.
J Inherit Metab Dis. 2010 Dec;33(6):659-64. doi: 10.1007/s10545-010-9043-4. Epub 2010 Feb 12.
A phenylalanine (Phe)-restricted diet is the mainstay of phenylketonuria (PKU) treatment, and, in recent years, the nutritional management of PKU has become more complex in order to optimize patients' growth, development and diet compliance. Dietary restriction of Phe creates a diet similar to a vegan diet, and many of the nutritional concerns and questions applicable to vegans who wish to avoid animal products are also relevant to patients with PKU. Owing to their nutritional characteristics, breast milk and breastfeeding should be given greater consideration as a useful food in patients with PKU and in those with other inborn errors of metabolism. Further key issues for consideration include the quality of the available amino acid substitutes, the neurotrophic and neuroprotective effects of added long-chain polyunsaturated fatty acids (e.g. docosahexaenoic acid), micronutrient deficiencies, bone disease and antioxidant status. Long-term dietary guidance and monitoring of the nutritional status of patients with PKU should be part of a follow-up programme that continues for life.
苯丙氨酸（Phe）限制饮食是苯丙酮尿症（PKU）治疗的主要方法，近年来，为了优化患者的生长、发育和饮食依从性，PKU 的营养管理变得更加复杂。Phe 的饮食限制会创建一种类似于纯素饮食的饮食，许多适用于希望避免动物产品的素食者的营养问题和疑问，也与 PKU 患者相关。由于其营养特性，母乳和母乳喂养应该在 PKU 患者和其他先天性代谢缺陷患者中被更多地视为有用的食物。进一步需要考虑的关键问题包括可用氨基酸替代品的质量、添加长链多不饱和脂肪酸（如二十二碳六烯酸）的神经营养和神经保护作用、微量营养素缺乏、骨骼疾病和抗氧化状态。长期的饮食指导和 PKU 患者营养状况的监测应该是终身随访计划的一部分。
