A BETTER ORGANOLEPTIC NUTRITIONAL PRODUCT FOR PKU

目前对年龄较大的儿童和青少年的治疗 高苯丙氨酸血症(HPA)是不令人满意的，因为可用元素 医疗食品(EMF)未按推荐量摄入，原因是 令人不快的感官(味觉)品质。 这项建议的长期目标是改善 HPA患者的治疗和生化控制。 具体目标是开发和测试一种新的电磁场的安全性和有效性 (俄勒冈州的EMF。)俄勒冈州的电动势旨在实现以下目标：1) 降低动势蛋白：卡路里密度；2)改变个体氨基酸水平 酸(AAs)以满足推荐膳食摄入量(RDAs)；3)减少 口感不佳的AAs水平；4)合成和品尝 口感不佳的衍生品，必不可少的AA、蛋氨酸。 俄勒冈州电磁场的临床安全性和代谢有效性将得到确定 在6岁及以上的15-20名HPA患者中。身体上、心理上和 营养评估和实验室数据将根据 膳食用新产品临床评价指南 美国科学院营养委员会新陈代谢疾病管理 儿科医生。 其他措施将用于确定对俄勒冈州电磁场的接受程度， 就餐行为和学校进步。

Current treatment of older children and adolescents with hyperphenylalaninemia (HPA) is unsatisfactory because available elemental medical foods (EMFs) are not consumed in recommended amounts due to unpleasant organoleptic (taste) qualities. The long-term objective of this proposal is to improve the nutritional treatment and biochemical control of patients with HPA. Specific aims are to develop and test for safety and efficacy a new EMF (the Oregon EMF.) The Oregon EMF is designed to achieve the following: 1) reduce EMF protein: calorie density; 2) alter levels of individual amino acids (AAs) to meet Recommended Dietary Allowances (RDAs); 3) diminish levels of unpleasant-tasting AAs; and 4) synthesize and taste-test derivatives of the unpleasant-tasting, indispensable AA, methionine. Clinical safety and metabolic efficacy of the Oregon EMF will be determined in 15-20 HPA patients six years and older. Physical, psychological and nutritional assessments and laboratory data will be collected according to The Guidelines for Clinical Evaluation of New Products Used in the Dietary Management of Metabolic Disorders, Committee on Nutrition, American Academy of Pediatrics. Additional measures will be used to determine acceptance of the Oregon EMF, mealtime behaviors and school progress.