Stroup Bridget M, Murali Sangita G, Nair Nivedita, Sawin Emily A, Rohr Fran, Levy Harvey L, Ney Denise M
Department of Nutritional Sciences, University of Wisconsin-Madison, WI, United States.
Division of Genetics and Genomics, Boston Children׳s Hospital, Harvard Medical School, Boston, MA, United States.
Data Brief. 2017 Jun 7;13:377-384. doi: 10.1016/j.dib.2017.06.004. eCollection 2017 Aug.
This article provides original data on median dietary intake of 18 amino acids from amino acid medical foods, glycomacropeptide medical foods, and natural foods based on 3-day food records obtained from subjects with phenylketonuria who consumed low-phenylalanine diets in combination with amino acid medical foods and glycomacropeptide medical foods for 3 weeks each in a crossover design. The sample size of 30 subjects included 20 subjects with classical phenylketonuria and 10 with a milder or variant form of phenylketonuria. Results are presented for the Delis-Kaplan Executive Function System and the Cambridge Neuropsychological Test Automated Battery; the tests were administered at the end of each 3-week dietary treatment with amino acid medical foods and glycomacropeptide medical foods. The data are supplemental to our clinical trial, entitled "Glycomacropetide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial, 2016 (1) and "Metabolomic changes demonstrate reduced bioavailability of tyrosine and altered metabolism of tryptophan via the kynurenine pathway with ingestion of medical foods in phenylketonuria, 2017 (2). This data has been made public and has utility to clinicians and researchers due to the following: 1) This provides the first comprehensive report of typical intakes of 18 amino acids from natural foods, as well as amino acid and glycomacropeptide medical foods in adolescents and adults with phenylketonuria; and 2) This is the first evidence of similar standardized neuropsychological testing data in adolescents and adults with early-treated phenylketonuria who consumed amino acid and glycomacropeptide medical foods.
本文提供了基于3天食物记录的原始数据，这些记录来自苯丙酮尿症患者，他们采用低苯丙氨酸饮食，并结合氨基酸医用食品和糖巨肽医用食品，以交叉设计分别食用3周。30名受试者的样本包括20名典型苯丙酮尿症患者和10名症状较轻或变异型苯丙酮尿症患者。文中呈现了德利-卡普兰执行功能系统和剑桥神经心理测试自动成套测验的结果；这些测试在每3周的氨基酸医用食品和糖巨肽医用食品饮食治疗结束时进行。这些数据是我们临床试验的补充资料，相关临床试验题为《糖巨肽用于苯丙酮尿症的营养管理：一项随机、对照、交叉试验，2016年》(1)以及《代谢组学变化表明，苯丙酮尿症患者摄入医用食品后，酪氨酸的生物利用度降低，色氨酸通过犬尿氨酸途径的代谢发生改变，2017年》(2)。这些数据已公开，对临床医生和研究人员有用，原因如下：1) 本文首次全面报告了苯丙酮尿症青少年和成人从天然食物以及氨基酸和糖巨肽医用食品中摄入18种氨基酸的情况；2) 这是首次有证据表明，食用氨基酸和糖巨肽医用食品的早期治疗苯丙酮尿症青少年和成人有类似的标准化神经心理测试数据。
